American Journal of Physical Medicine and Rehabilitation

December 2007, Volume 86, Issue 12,PP 957-1038

Research Article Musculoskeletal Disorders in Referrals for Suspected 957 Lumbosacral Radiculopathy. Daniel E. Cannon, BS; Timothy R. Dillingham, MD; Haiyan Miao, MS; Michael T. Andary, MD; Liliana E. Pezzin, PhD Research Article Understanding Pain After Traumatic Brain Injury: Impact on 962 Community Participation. Jeanne M. Hoffman, PhD; Kathleen F. Pagulayan, PhD; Nadya Zawaideh, BA; Sureyya Dikmen, PhD; Nancy Temkin, PhD; Kathleen R. Bell, MD Research Article Myopathic Dropped Head Syndrome: An Expanding 970 Clinicopathological Spectrum. Jenny P. Liao, MD; Andrew J. Waclawik, MD; Barend P. Lotz, MD; Sharhriar M. Salamat, MD, PhD; Brad R. Beinlich, MD; Benjamin R. Brooks, MD Research Article Upper-Limb Prosthetics: Critical Factors in Device Abandonment. 977 Elaine Biddiss, PhD; Tom Chau, PhD Research Article A Dynamic Seating Intervention for Wheelchair Seating 988 Discomfort. Barbara A. Crane, PhD, PT, ATP; Margo B. Holm, PhD, OTR/L; Douglas Hobson, PhD; Rory A. Cooper, PhD; Matthew P. Reed, PhD CME Article * 2007 Series * Number 5 CME Cyproheptadine for Pediatric Intrathecal Baclofen 994 Withdrawal: A Case Report. Joseph A. Saveika, MD; Jean E. Shelton, MD Media Review Physical Medicine and Rehabilitation Board Review: Palm Edition. 997 Lam Nguyen, DO Research Survey Perceived Preparedness for Physiatric Specialization and Future 1001 Career Goals of Graduating Postgraduate Year IV Residents during the 2004-2005 Academic Year. Vishwa S. Raj, MD; Diana H. Rintala, PhD

Review & Analysis Outcome Assessment in Randomized Controlled Trials of Stroke 1007 Rehabilitation. Katherine L. Salter, BA; Robert W. Teasell, MD; Norine C. Foley, MSc; Jeffrey W. Jutai, PhD Review & Case Reports Persistent Hiccups During Rehabilitation Hospitalization: Three 1013 Case Reports and Review of the Literature. John A. Schuchmann, MD; Barry A. Browne, PharmD Case Report Persistent Hiccup Associated with Intrathecal Morphine Infusion 1019 Pump Therapy. Xiulu Ruan, MD; John Patrick Couch, MD; Rinoo Shah, MD; Frank Wang, MD; Hai Nan Liu, MS Commentary Ashley X. 1023 Kristi L. Kirschner, MD; Rebecca Brashler, LCSW; Teresa A. Savage, PhD, RN Visual Vignette Exacerbation of Habitual Dislocation of Ulnar Nerve by Concurrent 1030 Dislocation of Triceps Muscle: Complementary Role of Dynamic Ultrasonography to Electrodiagnosis. Myung Jae Yoo, MD; Dennis DJ Kim, MD; Mooyeon Oh-Park, MD Letters to the Editor Re: Introduction to Nanotechnology: Potential Applications in 1031 Physical Medicine and Rehabilitation. Govindasamy Agoramoorthy; Chiranjib Chakraborty Re: Introduction to Nanotechnology: Potential Applications in 1032 Physical Medicine and Rehabilitation: Response Letter. Assaf T. Gordon, MD Re: Sacroiliac Joint Pain: Anatomy, Biomechanics, Diagnosis, and 1032 Treatment. Myron M. LaBan, MD, MMSc, FACP, FAAPMR Re: Sacroiliac Joint Pain: Anatomy, Biomechanics, Diagnosis, and 1033 Treatment: Response Letter. Brian S. Foley, MD; Ralph M. Buschbacher, MD Re: The Myofascial Trigger Point Region: Correlation Between the 1033 Degree of Irritability and the Prevalence of Endplate Noise. Jay P. Shah, MD; Sagar Parikh, MS IV; Jerome Danoff, PT, PhD; Lynn H. Gerber, MD Re: The Myofascial Trigger Point Region: Correlation Between the 1034 Degree of Irritability and the Prevalence of Endplate Noise: Response Letter. Chang-Zern Hong, MD; Ta-Shen Kuan, MD Re: Characteristics and Treatment of Headache After Traumatic 1034 Brain Injury. Myron M. LaBan, MD, MMSc Re: Characteristics and Treatment of Headache After Traumatic 1035 Brain Injury: Response Letter. Henry L. Lew, MD, PhD; David J Clark, MD, PhD; William C. Walker, MD

1035 Re: Shoulder Impingement Syndrome: Relationships Between Clinical, Functional, and Radiologic Findings. Ilknur Aktas, MD; Kenan Akgun, MD Re: Shoulder Impingement Syndrome: Relationships Between 1036 Clinical, Functional, and Radiologic Findings: Response Letter. F Figen Ayhan, MD; Z Rezan Yorgancioglu, MD Erratum Erratum. 1038

Authors: Daniel E. Cannon, BS Timothy R. Dillingham, MD Musculoskeletal Haiyan Miao, MS Michael T. Andary, MD Liliana E. Pezzin, PhD

Afﬁliations: RESEARCH ARTICLE From the Medical College of Wisconsin, Milwaukee, Wisconsin (DEC); Departments of Physical Medicine and Rehabilitation (TRD) and Medicine (HM, LEP), Medical Musculoskeletal Disorders in College of Wisconsin, Milwaukee, Wisconsin; and Department of Referrals for Suspected Physical Medicine and Rehabilitation, Michigan State University College of Lumbosacral Radiculopathy Osteopathic Medicine, East Lansing, Michigan (MTA).

Correspondence: ABSTRACT All correspondence and requests for Cannon DE, Dillingham TR, Miao H, Andary MT, Pezzin LE: Musculoskeletal reprints should be addressed to disorders in referrals for suspected lumbosacral radiculopathy. Am J Phys Med Timothy R. Dillingham, Department Rehabil 2007;86:957–961. of Physical Medicine and Rehabilitation, 9200 W Wisconsin Objective: An electrodiagnostic evaluation is often requested for pa- Avenue, Milwaukee, WI 53226-3596. tients with suspected lumbosacral radiculopathy. Although musculoskele- 0894-9115/07/8612-0957/0 tal disorders can produce lower-limb symptoms, their prevalence in this American Journal of Physical referral population is unknown. The purpose of this study was to determine Medicine & Rehabilitation the prevalence of common lower-limb musculoskeletal disorders in pa- Copyright © 2007 by Lippincott Williams & Wilkins tients referred for lower-limb electrodiagnosis and determine whether these findings predict study outcome. DOI: 10.1097/PHM.0b013e31815b614a Design: Subjects undergoing an electrodiagnostic study for lower-limb symptoms and suspected lumbosacral radiculopathy constituted the sample. A standardized clinical and electrodiagnostic evaluation was performed for all patients. Results: There were 170 subjects included. The mean age was 52 (SD ϭ 17), and 45% were males. The total prevalence of musculoskeletal disorders in the sample was 32%. The prevalence in those with a normal study was 55% as compared with 21% in those with lumbosacral radiculopathy (P Ͻ 0.0001). Conclusions: Musculoskeletal disorders are common in patients suspected of having lumbosacral radiculopathy. The high prevalence among both patients with normal studies and those with radiculopathy and other disorders limits the usefulness of this information in predicting study outcome. In particular, it is common for patients to have two or more problems and the presence of a musculoskeletal disorder should not preclude such testing.

Key Words: Radiculopathy, Electrodiagnosis, Musculoskeletal Diseases, Myofascial Pain Syndromes

December 2007 Musculoskeletal Disorders in Radiculopathy 957 ter, Tacoma, WA; Womack Army Medical Center, A large number of conditions are included in Fort Bragg, NC; and Walter Reed Army Medical the differential diagnosis of patients referred for Center, Washington, DC. Patients were asked to electrodiagnostic testing. Suspected radiculopathy participate if they were referred for lower-limb is a common reason patients are referred to an symptoms or suspected lumbosacral radiculopathy electrodiagnostic laboratory.1,2 Lumbosacral radic- to the electrodiagnostic laboratory in the depart- ulopathy is a pathologic condition involving the ment of physical medicine and rehabilitation at one lumbosacral nerve roots. The most common symp- of the participating institutions. Subjects partici- tom is pain that is often increased or precipitated pated on a voluntary basis after giving written by standing or sitting. Other symptoms that can informed consent. The study protocol was ap- occur are parasthesias and weakness in a part of the proved by the appropriate institutional review com- limb. The distribution of the symptoms depends on mittee at each of the participating institutions. the nerve roots involved.3 Likewise, musculoskeletal conditions are common causes of lower-limb Data Collection symptoms and may be mistaken for lumbosacral Standardized procedures and data collection radiculopathy.4–7 sheets were implemented at all participating Myofascial pain is characterized by pain in centers. Patients completed a questionnaire that skeletal muscles that originates from trigger collected detailed information on symptoms and points, areas within the muscle that are highly medical history. For all patients, a standardized sensitive and reproduce the patient’s symptoms physical examination was completed that consisted when palpated.8 Trochanteric bursitis is a condi- of the following: (1) a neurologic examination, tion of inflammation or irritation of either the including manual muscle testing, sensation (vibra- gluteus maximus bursa or the bursa separating the tion and pinprick), and reflex assessments; (2) a greater trochanter from the gluteus medius and musculoskeletal examination; and (3) special tests, gluteus minimus. It presents with pain over the such as the straight-leg raise, and the Patrick test. lateral aspect of the hip.9 Iliotibial band syndrome After the questionnaire and physical examination, occurs when the iliotibial band, a dense fascia on electrodiagnostic evaluations, conducted by at- the lateral aspect of the knee and hip, impinges tending physicians and their supervised resident over the lateral femoral epicondyle. It most com- physicians, were performed for all patients. All par- monly presents with pain over the lateral knee that ticipating attending physicians were certified by can radiate up into the lateral thigh or down to the American Board of Electrodiagnostic Medicine Gerdy tubercle.10 In the present analysis, trochan- and were skilled musculoskeletal physicians. Such teric bursitis and iliotibial band syndrome are expertise was sought to ensure electrodiagnostic grouped together. Plantar fasciitis is an inflamma- and physical exam findings across participating tory condition of the plantar aponeurosis of the centers were consistently interpreted. foot. It typically presents with tenderness on pal- Musculoskeletal disorders were diagnosed if pation of the sole anterior to the calcaneus.11 certain criteria were met in the course of the phys- The purpose of this study was to determine the ical exam. Myofascial pain was diagnosed if palpi- prevalence of these common musculoskeletal disor- tation of the back or buttock region while avoiding ders of the lower limb in patients referred for elect- the area of the sciatic notch reproduced symp- rodiagnostic testing. In addition, whereas previous toms.8 Trochanteric bursitis/iliotibial band syn- studies have examined the influence of physical exam drome was diagnosed if palpation in the lateral hip findings on predicting electrodiagnostic study out- region reproduced symptoms.14,15 Plantar fasciitis comes,12,13 in this study the influences of these spe- was diagnosed if palpation of the sole at the origin cific musculoskeletal disorders on electrodiagnostic of the plantar fascia at the calcaneus reproduced study outcome prediction were examined. symptoms.16 The standardized electrodiagnostic study con- METHODS sisted of at least (1) one lower-limb motor nerve conduction study, (2) one lower-limb sensory Subjects nerve conduction study, and (3) needle EMG with A prospective study was conducted between either monopolar or concentric needles of a stan- July 1996 and April 1998 at the following medical dard set of 11 muscles. The electrodiagnostic centers that treat diverse patient populations and conclusions were classified into three mutually have high-volume Physical Medicine and Rehabil- exclusive categories: normal study, lumbosacral ra- itation electrodiagnostic services: Johns Hopkins diculopathy, or other condition. A stratified data- University, Baltimore, MD; Ingham Regional Med- collection strategy was used to recruit sufficient ical Center, associated with Michigan State Univer- persons in each category because these proportions sity, East Lansing, MI; Madigan Army Medical Cen- in the referral population were different.

958 Cannon et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 Lumbosacral radiculopathy was diagnosed us- cally confirmed conditions also had these disor- ing methods established earlier.17 Specifically, ders, but at lower prevalence than those with nor- lumbosacral radiculopathy was diagnosed if EMG mal studies. findings (spontaneous activity, increased polypha- The only significant finding from the multi- sics, complex repetitive discharges, or reduced re- variate analysis showed that the presence of myo- cruitment) were found in two or more muscles fascial pain meant about a fivefold lower likelihood innervated by the same nerve root but different of having lumbosacral radiculopathy compared peripheral nerves, or if the paraspinal muscles with in a normal study (P ϭ 0.001), given that showed spontaneous activity. Overall, 96% of this other variables in the model were held constant. group had spontaneous activity in at least one muscle. Other diagnoses included sciatic neuropa- DISCUSSION thy, femoral neuropathy, tibial neuropathy, pero- Musculoskeletal disorders are common in pa- neal neuropathy, polyneuropathy, myopathy, and tients suspected of having lumbosacral radiculop- Myasthenia Gravis. The electrodiagnostic out- athy. However, within the study sample there were comes were then analyzed as they related to the significant differences in the prevalence of muscu- presence of musculoskeletal disorders. loskeletal disorders when subjects were grouped according to the outcome of their electrodiagnostic Data Analysis studies. More than half (55%) of those demonstrat- SAS Windows version 9.1 was used to compare ing a normal electrodiagnostic study had a muscu- the prevalence of musculoskeletal disorders be- loskeletal disorder diagnosed on the basis of phys- tween study groups using ␹2 analysis or forward ical exam findings. In comparison, there was a 21% two-sided exact test. To further examine the influ- prevalence of musculoskeletal disorders in those ence of musculoskeletal conditions on study out- with lumbosacral radiculopathies (P Ͻ 0.0001) and come, a multivariate analysis was undertaken us- a 29% prevalence in those with other electrodiag- ing STATA 9.0. Age, gender, and duration of nostic diagnoses. symptoms were controlled for to prevent any po- Whereas the presence of a musculoskeletal dis- tential confounding effects these variables may order suggests that a patient will have a normal have had on the outcome of this analysis. Study study, the fairly high prevalence of musculoskeletal outcomes were defined as normal study (reference disorders in those with a confirmed lumbosacral category), lumbosacral radiculopathy, or other di- radiculopathy or other electrodiagnostic conclu- agnosis. sions limits the usefulness of this information in predicting the results of an electrodiagnostic study. RESULTS A study should not be curtailed solely on the basis There were 170 subjects included in the study. of finding a musculoskeletal disorder on physical The mean age of the subjects was 52 (SD ϭ 17) and exam. 45% were men. The mean duration of symptoms Myofascial pain in particular was common in reported by the subjects was 26 mos (SD ϭ 54). subjects that had a normal study (39%), signifi- The prevalence of musculoskeletal disorders is cantly more so than in those with a lumbosacral shown in Table 1. Those with a normal study radiculopathy (12%, P Ͻ 0.0001) and those with tended to have higher prevalence of the conditions another electrodiagnostic diagnosis (12%, P ϭ studied. However, persons with electrodiagnosti- 0.0362). This result could be an indication of the

TABLE 1 Prevalence of musculoskeletal disorders in patients with suspected lumbosacral radiculopathy Lumbosacral Other EDX Normal Study, % Radiculopathy, % Diagnosis,‡ % Total Sample, % (170 ؍ n) (17 ؍ n) (102 ؍ n) (51 ؍ Musculoskeletal Disorder (n

Myofascial pain syndrome 39 12* 12* 20 Trochanteric bursitis/iliotibial 29 12* 18 18 band syndrome Plantar fasciitis 4 3 18† 5 One or more of the above 55 21* 29 32 * Signiﬁcant at P Ͻ 0.05 compared with normal study. † Signiﬁcant at P Ͻ 0.05 compared with lumbosacral radiculopathy. ‡ Other diagnoses included sciatic neuropathy, femoral neuropathy, tibial neuropathy, peroneal neuropathy, polyneuropathy, myopathy, and Myasthenia Gravis.

December 2007 Musculoskeletal Disorders in Radiculopathy 959 difficulty in distinguishing between myofascial pain. Each of these factors may have contributed to pain and radiculopathy and the degree to which the some of the data being misclassified with respect to two disorders mimic each other. The referred pain musculoskeletal conditions present, electrodiag- patterns seen in myofascial pain can be very similar nostic conclusions, or both. to the dermatomal patterns of the spinal nerve The multivariate analysis showed that the roots and are poorly recognized by most practitio- presence of myofascial pain meant that a patient ners.4 Therefore, it is possible that many patients was about five times less likely to have lumbosacral being referred for electrodiagnostic evaluations ac- radiculopathy than a normal study. This provides a tually have myofascial pain, either alone or along plausible indication of what the results of electro- with radiculopathy. If this is the case, an electrodiagnostic testing would be in a patient diagnosed diagnostic evaluation can help clarify the nature of with myofascial pain. the problem. These findings are also in line with what was Trochanteric bursitis/iliotibial band syndrome concluded in other studies regarding the predictive showed a significantly lower prevalence in subjects ability of physical exam on electrodiagnostic out- with lumbosacral radiculopathies (12%, P ϭ 0.007) come. Physical exam findings are important and than in those with normal studies (29%). It is helpful in guiding the course of an electrodiagnos- possible that this finding reflects difficulty in dis- tic study, but are not sufficient to predict the out- tinguishing between these conditions and lumbo- come of the study.12,13 Similarly, the presence of sacral radiculopathy based solely on physical exam musculoskeletal disorders gives some indication findings, as was proposed with myofascial pain about what the outcome of an electrodiagnostic above. Both cases emphasize the importance of study will be but does not provide enough infor- electrodiagnostic testing in helping distinguish mation to definitively predict the results of the musculoskeletal disorders from lumbosacral radic- study. ulopathy when evidence is present for both and treatment strategies for the musculoskeletal con- CONCLUSIONS ditions are not effective. However, electrodiagnos- Musculoskeletal disorders are common in pa- tic studies are not advocated for all patients with tients referred for electrodiagnostic assessment. musculoskeletal disorders. Rather, the clinician However, the presence of a musculoskeletal prob- should consider the possibility of radiculopathy or lem does not accurately predict who will have a other disorders even in the presence of such con- normal electrodiagnostic study or an electrodiag- ditions. nostically confirmed lumbosacral radiculopathy There are some potential limitations to this with sufficient discriminative ability to curtail the study. It is possible that there was heightened sus- electrodiagnostic evaluation. Whereas there is a picion for certain musculoskeletal conditions given significantly higher prevalence in those with a nor- the nature of the prospective study and the careful mal study compared with those with lumbosacral and systematic examination for these conditions. radiculopathy, the fairly high prevalence in all In addition, there are interrater differences in groups makes it difficult to predict the outcome of physical examination even for skilled practitioners electrodiagnostic testing based on the presence of that could influence our findings. For example, the musculoskeletal disorders. exact finger pressure used to elicit findings during palpation was not standardized. The underlying REFERENCES pain system and peripheral nervous system physi- 1. Levin KH: Electrodiagnostic approach to the patient with ology is quite complex and interrelated. These sys- suspected radiculopathy. Neurol Clin N Am 2002;20:397– tems may augment the pain responses and percep- 421 tions in persons with radiculopathies such that 2. Dumitru D, Zwarts MJ: Radiculopathies, in Dumitru D, physical exam maneuvers are perceived as more Amato AA, Zwarts MJ (eds): Electrodiagnostic Medicine,ed painful than they otherwise might be in persons 2. Philadelphia, Hanley and Belfus, 2002, pp 713–76 with radiculopathy. 3. Ellenburg M, Honet JC: Lumbar radiculopathy, in Frontera W, Silver J (eds): Essentials of Physical Medicine and Re- Finally, not all radiculopathies that produce habilitation, ed 1. Philadelphia, Hanley and Belfus, 2002, pp symptoms can be detected by an electrodiagnostic 243–8 evaluation. For example, radiculopathies that in- 4. Lauder TD: Musculoskeletal disorders that frequently volve only sensory nerve roots will not produce mimic radiculopathy. Phys Med Rehabil Clin N Am 2002; 13:469–85 abnormal EMG findings.18 Also, some radiculopa- 5. Facco E, Ceccherelli F: Myofascial pain mimicking radicular thies may have associated myofascial pain as a syndromes. Acta Neurochir Suppl 2005;92:147–50 component of their disease process. Electrodiag- 6. Flax HJ: Myofascial pain syndromes—the great mimicker. nostically proven radiculopathies with substantial Bol Asoc Med P R 1995;87:167–70 axonal loss may have myotomal pain in the affected 7. Keplinger FS, Gupta N: Trochanteric bursitis, in Frontera muscles that may mimic more typical myofascial W, Silver J (eds): Essentials of Physical Medicine and Re-

960 Cannon et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 habilitation, ed 1. Philadelphia, Hanley and Belfus, 2002, pp 13. Lauder TD: Physical examination signs, clinical symptoms, 297–300 and their relationship to electrodiagnostic ﬁndings and the 8. Borg-Stein J, Simons DG: Myofascial pain. Arch Phys Med presence of radiculopathy. Phys Med Rehabil Clin N Am Rehabil 2002;83:S40–7 2002;13:451–67 9. Anderson M: Trochanteric bursitis, in Ferri FF (ed): Ferri’s 14. Shbeeb MI, Matteson EL: Trochanteric bursitis (greater Clinical Advisor: Instant Diagnosis and Treatment,ed8.St. trochanter pain syndrome). Mayo Clin Proc 1996;71:565–9 Louis, Mosby, 2006, p 867 15. Khaund R, Flynn SH: Iliotibial band syndrome: a common 10. Akuthota V, Stilp SK, Lento P: Iliotibial band syndrome, in source of knee pain. Am Fam Physician 2005;71:1545–50 Frontera W, Silver J (eds): Essentials of Physical Medicine and Rehabilitation, ed 1. Philadelphia, Hanley and Belfus, 16. Aldridge T: Diagnosing heel pain in adults. Am Fam Phy- 2002, pp 328–33 sician 2004;70:332–8 11. Glazer JL, Hosey RG: Soft-tissue injuries of the lower ex- 17. Dillingham TR, Lauder TD, Andary M, et al: Identifying tremity. Prim Care Clin Ofﬁce Pract 2004;31:1005–24 lumbosacral radiculopathies: an optimal electromyographic screen. Am J Phys Med Rehabil 2000;79:496–503 12. Lauder TD, Dillingham TR, Andary M, et al: Effect of history and exam in predicting electrodiagnostic outcome among 18. Wilbourn AJ, Aminoff MJ: AAEM minimonograph 32: elec- patients with suspected lumbosacral radiculopathy. Am J trodiagnosis of radiculopathies. Muscle Nerve 1998;21: Phys Med Rehabil 2000;79:60–8 1612–31

December 2007 Musculoskeletal Disorders in Radiculopathy 961 Authors: Jeanne M. Hoffman, PhD Kathleen F. Pagulayan, PhD Brain Injury Nadya Zawaideh, BA Sureyya Dikmen, PhD Nancy Temkin, PhD Kathleen R. Bell, MD RESEARCH ARTICLE Afﬁliations: From the Departments of Rehabilitation Medicine (JMH, KFP, NZ, SD, NT, KRB) and Department of Neurological Surgery (SD, NT), Understanding Pain After Traumatic University of Washington, Seattle, Washington. Brain Injury

Correspondence: Impact on Community Participation All correspondence and requests for reprints should be addressed to Jeanne Hoffman, PhD, University of ABSTRACT Washington School of Medicine, Department of Rehabilitation Hoffman JM, Pagulayan KF, Zawaideh N, Dikmen S, Temkin N, Bell KR: Medicine, 1959 NE Paciﬁc Street, Box Understanding pain after traumatic brain injury: impact on community participa- 356490, Seattle, WA 98195. tion. Am J Phys Med Rehabil 2007;86:962–969. Objective: Disclosures: To examine the prevalence of pain 1 yr after moderate to severe traumatic brain injury (TBI) and identify predictors from the time of Supported by the National Institute on Disability and Rehabilitation injury. Additionally, factors related to pain at 1 yr after injury were exam- Research, U.S. Department of ined along with the impact of pain on community participation. Education (Grant No. H133A020508). Design: Prospective cohort study of 146 individuals enrolled during Part of the material presented in this manuscript was presented at the acute inpatient rehabilitation for TBI and community follow-up at 1 yr after American Congress of Rehabilitation injury. Medicine Conference, Chicago, October, 2005. Results: Higher reports of depressive symptoms during inpatient rehabilitation and at 1 yr after injury were significantly related to reports of 0894-9115/07/8612-0962/0 American Journal of Physical pain at 1 yr when controlling for demographic and injury characteristics. Medicine & Rehabilitation Being female and nonwhite were also factors related to increased reports Copyright © 2007 by Lippincott of pain. Pain and community participation were significantly related until Williams & Wilkins depression was entered into the model. Depression is a significant factor

DOI: 10.1097/PHM.0b013e31815b5ee5 in the relationship between pain and community participation. Conclusion: Whereas pain was frequently reported 1 yr after injury, injury-related factors were surprisingly unrelated. Further evaluation of the role that depression plays in the relationship between pain and community participation will be important to determine appropriate management of pain and depression and to optimize participation in individuals with TBI.

Key Words: Pain, Traumatic Brain Injury, Rehabilitation

962 Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 using a secondary analysis of a prospectively The experience and explication of pain among collected sample and to examine possible factors individuals with disability is developing into a focus related to overall pain reports in individuals with of rehabilitation research. Studies have been con- TBI. Possible predictors of pain at 1 yr were ducted to understand the prevalence and impact of examined, as well as factors associated with pain pain on individuals with a variety of disabling con- at 1 yr. In addition, the relationship of pain, ditions such as spinal cord injury, amputation, and depression, and community participation was ex- multiple sclerosis.1,2 Pain after traumatic brain in- plored. jury (TBI) has only been partly examined, but has been noted frequently as an area in need of further METHODS research.3–8 Much of the existing research has pri- Study Population marily focused on posttraumatic headache, and One hundred forty-six individuals who com- typically for those with mild TBI.9–11 In addition, pleted the SF-36 at their 1 yr after injury evalua- most studies on pain after TBI have enrolled indi- tion were included in the current study from a total viduals presenting to outpatient brain injury clin- of 202 individuals who were eligible and consecu- ics, rather than prospectively enrolling subjects, tively enrolled in the University of Washington’s which limit the ability to generalize results.12,13 (UW) Traumatic Brain Injury Model System Pain has also been documented as a significant (TBIMS). Twenty-three of the 202 subjects were concern after more severe injury. For instance, lost to follow-up, and 33 did not complete the Walker and colleagues14 observed 109 consecutive SF-36 because of limited testing time. No signifi- Veterans Administration patients with moderate to cant differences between those who completed the severe TBI for 1 yr. Of those patients observed, 38% SF-36 and those who did not were found on gen- reported headache during their inpatient rehabili- der, age, and lowest Glasgow coma scale (GCS) tation, and approximately 50% of those who ini- score within 24 hrs of injury. However, those that tially reported headache continued to endorse did not complete the SF-36 were more likely to be headache at 1 yr after injury. nonwhite individuals. Individuals were enrolled be- Whereas headache has been more widely exam- tween 1998 and 2001 during their inpatient reha- ined, other painful conditions that can develop after bilitation stay. The TBIMS is a longitudinal data- brain injury have not been as frequently evaluated. base and research program sponsored by the Such conditions include spasticity, peripheral nerve National Institute on Disability and Rehabilitation injury, reflex sympathetic dystrophy, contracture, Research. Criteria for enrollment include (1) pri- and heterotopic ossification.15 A case series con- mary diagnosis of TBI on admission to the inpa- ducted on patients presenting to a brain injury clinic tient rehabilitation unit, (2) age greater than 16 found that 58% of individuals with mild TBI and 52% yrs, (3) arrival at the emergency department within of those with moderate to severe TBI reported 24 hrs of injury, and (4) receipt of both acute care chronic pain.12 Headaches were the most common and inpatient rehabilitation within the Model Sys- source of pain for both groups (47 and 34%, respec- tem of care. Individuals were assessed during their tively), followed by neck/shoulder, upper-limb, and inpatient rehabilitation stay (baseline) and at 1 yr lower-limb pain. Bryant and colleagues16 observed 96 after injury. During inpatient rehabilitation, indi- patients with severe TBI for 6 mo after injury and viduals were not consented or evaluated until they found that 31% reported chronic pain associated with had two consecutive scores of 75 or above on the head, neck, back, and limb pain. Other reports of pain Galveston Orientation and Amnesia Test (GOAT),20 vary in the literature but typically exceed 50% of all which places them out of posttraumatic amnesia. patients assessed.13 In addition, study examiners discontinued testing The problems associated with the presence of if there was any question on validity of responses. pain are particularly worrisome for individuals The majority of 1 yr after injury evaluations were with TBI. In noninjured individuals, pain has been conducted in person. Alternatively, when an indi- found to interfere with cognition and exacerbate vidual was unable to come in person due to dis- problems with sleep and fatigue and is strongly tance from the center they were conducted over associated with anxiety and depression, as well as the telephone. The study was approved by the in- other psychological disorders such as posttraumatic stitutional review board at the University of Wash- stress disorder.16–18 Mooney and colleagues19 found ington. that poor recovery after mild TBI was explained in large part by depression and pain. Depression was Measures also found to be highly correlated with headache pain Demographics at 1 yr after injury.14 Age at the time of injury, years of education, The aim of the current study was to deter- gender (female ϭ 1, male ϭ 2), and race (white ϭ mine the prevalence of pain at 1 yr after injury 1, nonwhite ϭ 0) were included in analyses.

December 2007 Pain and Community Participation After TBI 963 Injury Characteristics tion at 1 yr after TBI. The measure includes ques- Severity of injury was measured using the low- tions about home and social integration and est GCS within 24 hrs of injury. GCS measures productive activity level. Scores range from 0 to 29, level of consciousness as indicated by eye opening, with higher scores representing more community participation. Test–retest reliability coefﬁcients verbal response, and motor response to stimuli. 24 Scores can range from 3 to 15, with scores of 3–8 range from 0.83 to 0.97. indicating severe injury, scores of 9–12 indicating a moderate injury, and scores of 13–15 indicating a Pain mild injury.21 Scores were prorated if an individual The two-item Bodily Pain scale of the SF-36 had a score for the best motor component but was Health Survey (SF-36)25 was used to assess pain.26 missing the eye opening and/or verbal response This subscale contains two items that assess pain component of the GCS.22 In the current sample, intensity (ranging from none to very severe) and verbal response was missing in 78% of cases be- pain interference (ranging from not at all to ex- cause subjects were intubated at the time of assess- tremely), which are combined into a composite ment. Eye opening was missing in only one case. score of pain severity and transformed into a 0–100 Cause of injury was categorized into four groups: (no pain) scale, with lower scores indicating more (1) motor vehicle related, 2) fall, 3) violence, and 4) pain. The Bodily Pain scale has demonstrated good all other causes, including pedestrian, bicycle, and test–retest reliability (0.78) and validity.26 In addi- sports-related injuries. In addition, the number of tion, it has been found to have good internal con- associated injuries that occurred at the time of sistency among individuals with TBI.27 The com- injury was included to examine the impact of non– posite score was calculated, using the scoring TBI-related injuries on reports of pain. The total criteria outlined in the SF-36 manual. Norms are number of injuries, including fracture, amputa- also given in the manual from a United States tion, peripheral nerve injury, brachial plexus in- population sample (mean, 75.15, SD, 23.69).25 jury, intraabdominal injury, spinal cord injury, and/or traumatic pneumothorax/hemothorax was Statistical Analysis calculated. Pearson correlation coefﬁcients were calcu- Functional Impairment lated among independent and dependent variables to assess relationships among variables. Two linear The Functional Impairment Measure (FIM) in- regressions were conducted to evaluate (1) baseline strument is a widely used measure of functional predictors of pain level reported on the Bodily Pain status after TBI. This measure includes 18 items Index of the SF-36 at 1 yr and (2) potential asso- that assess level of independence or dependence in ciated variables, measured at the same time as the six domains of functioning (self-care, sphincter con- level of pain. Baseline predictors included demo- trol, mobility, locomotion, communication, and so- graphic variables (age at time of injury, education, cial cognition). Ratings range from 1 (completely race, and sex), injury characteristics (GCS, number dependent) to 7 (independent). This measure was of associated injuries, cause of injury, FIM score at completed for each patient at time of discharge and time of hospital discharge), and baseline BSI de- at 1 yr after injury. pression score. Dummy coded indices of each of Depression Symptom Severity the cause of injury factors (motor vehicle accident, violence, fall, or other cause, which included pe- The depression subscale, one of the nine pri- destrian, bicycle, or sports injuries) were used in mary dimensions from the Brief Symptom Inven- this regression analysis. Potential associated vari- tory, was used to determine the level of distress or ables included baseline demographics as well as the 23 depression. This measure was administered dur- FIM scores, BSI depression scores, and CIQ scores ing inpatient rehabilitation (baseline) and at 1 yr that were completed at 1 yr after injury. after injury. The depression subscale has high in- When the above analyses were completed, ex- ␣ ϭ 23 ternal reliability (Cronbach 0.85). Raw ploratory analyses were conducted to further elu- scores from the subscale were transformed into cidate the relationship between pain, depression at 16 T-scores using nonpatient adult norms. Higher 1 yr after injury, and community participation. A scores indicate more depression. series of analyses were conducted to evaluate the impact of depression on the relationship between Community Participation pain and community participation and involved The Community Integration Questionnaire computation of three regression equations: (1) (CIQ),24 a widely used measure for assessing home pain predicting community participation (␶), (2) and social integration as well as community in- pain (␶=) and depression (␤) predicting community volvement after TBI, was used to assess participa- participation, and (3) depression predicting pain

964 Hoffman et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 36. The average amount of education was 12.4 yrs TABLE 1 Sample characteristics (range 3–20 yr). Only 27% of individuals reported n Percentage of Sample having no pain (a score of 100 on the bodily pain subscale), and 45% reported no interference with Gender Male 111 76.0 activities from pain. The average pain rating on the Female 35 24.0 Bodily Pain Index was 66.7. Approximately 50% of Race the sample sustained their injury in a motor vehi- White 114 78.1 cle accident. Descriptive statistics for FIM scores Nonwhite 32 21.9 and BSI depression scores at baseline and 1 yr after Cause of injury Motor vehicle 71 48.6 injury, and community participation measured related with the CIQ, are also displayed in Table 1. FIM Fall 33 22.6 scores and BSI depression scores at 1 yr were Violence 17 11.6 significantly higher than at baseline, indicating Other 22 15.1 that functional status improved and depression Missing 3 2.1 worsened (P Յ 0.001 and 0.05, respectively). Mean (SD) Range Pearson correlations are presented in Table 2. Age 146 36.1 (14.9) 17–88 Results suggest that higher reports of pain at 1 yr Education 145 12.4 (2.7) 3–20 after injury were significantly related to being fe- Glasgow coma scale 143 9.2 (3.1) 3–15 Number of associated 146 2.0 (1.7) 0–7 male, nonwhite, and having lower function as mea- injuries sured by the FIM instrument at 1 yr after injury. FIM scores Additionally, higher reports of pain were signifi- Baseline 144 112.5 (14.4) 32–126 cantly related to higher rates of depressive symp- 1 yr after injury 140 122.7 (5.5) 86–126 toms both at baseline and 1 yr after injury, and BSI depression Baseline 132 52.9 (9.6) 36–78 lower community participation as measured by the 1 yr after injury 136 55.8 (12.2) 36–80 CIQ. Interestingly, neither severity of TBI nor CIQ functional score 142 17.7 (6.0) 2.5–29 number of associated injuries was associated with SF-36 bodily pain 146 66.7 (28.4) 0–100 pain at 1 yr after injury. Higher rates of depressive symptoms at baseline and 1 yr after injury were related to being female, having lower function at 1 yr and lower community participation. Baseline (␣). All equations controlled for the demographic depressive symptoms were also related to lower variables.28 function at baseline. Reports of higher levels of community participation at 1 yr were related to RESULTS younger age, being white, having more education, One hundred forty-six individuals who com- having more injuries, and highly related to better pleted the Bodily Pain subscale of the SF-36 at 1 yr level of function measured by the FIM instrument. after injury were included in the study. Prevalence Evaluation of cause of injury suggests that of pain as well as demographic and injury charac- individuals who were violently injured had signifi- teristics of this sample are shown in Table 1. The cantly higher levels of pain than those in the other majority were white males with an average age of injury category (mean 50.94 compared with 78.32,

TABLE 2 Correlations among variables Bodily Baseline 1-yr Pain Index Depression Postinjury Depression CIQ

Age Ϫ0.07 0.10 0.15 Ϫ0.21* Gender 0.19* Ϫ0.21* Ϫ0.36† Ϫ0.08 Race 0.19* 0.04 0.01 0.17* Education 0.11 Ϫ0.13 Ϫ0.11 0.28† Number of injuries 0.07 0.01 Ϫ0.07 0.17* Glasgow coma scale Ϫ0.07 Ϫ0.08 0.00 0.14 Discharge FIM score 0.10 Ϫ0.17* Ϫ0.02 0.36† 1-yr FIM score 0.23* Ϫ0.30† Ϫ0.33† 0.50† Baseline depression Ϫ0.30† — 1-yr postinjury depression Ϫ0.54† 0.50† — CIQ community participation 0.30† Ϫ0.37† Ϫ0.33† * P Յ 0.05; † P Յ 0.001.

December 2007 Pain and Community Participation After TBI 965 tion measured at 1 yr after injury. Overall, these TABLE 3 Summary of regression analyses for variables accounted for 36% of the variance in the variables predicting pain at 1 yr Ͻ after injury Bodily Pain measure (P 0.001). In this analysis, higher reports of depression at 1 yr after injury and B SE bt being nonwhite were significantly associated with Age Ϫ0.09 0.20 Ϫ0.45 higher levels of pain reported at 1 yr after injury, Education 1.14 1.07 1.08 whereas other demographics, function, and com- Race 15.87 5.79 2.74** munity participation were not significantly related Sex 13.22 5.85 2.26* to reports of pain when the other factors are taken Ϫ Ϫ Glasgow coma scale 0.73 0.86 0.84 into account. Associated injuries Ϫ1.65 1.65 Ϫ1.00 Fall Ϫ1.80 6.54 Ϫ0.27 Violence Ϫ9.90 8.28 Ϫ1.20 Relationship Among Pain, Depression, Other Injury 7.01 7.22 0.97 and Community Participation Discharge FIM score 0.32 0.23 1.37 Depression at baseline Ϫ0.68 0.27 Ϫ2.54* Given the finding that community participation was related to reported pain on a bivariate R2 ϭ 0.25; adjusted R2 ϭ 0.18. * P Ͻ 0.05; ** P Ͻ 0.01. level, but not when included in the regression model, further analyses were conducted to determine how pain, depression, and community participation interrelate. The three regression equations respectively). No other significant differences were were conducted as described in the Statistical Analy- found. sis section, and results are shown in Figure 1. After controlling for demographic variables, pain was asso- Predictors and Correlates of Pain at 1 yr ciated with community participation. However, when After Injury depression was added into the model, the relationship Table 3 displays the results of the regression between pain and community participation becomes model with the outcome of pain at 1 yr after injury nonsignificant. This set of relationships suggest that and predictors from baseline, including demo- depression is an important variable in the relation- graphic factors, injury characteristics, function at ship between pain and community participation. discharge, and depressive symptoms. Overall, these variables accounted for 25% of the variance in the Bodily Pain measure (P Ͻ 0.001). Higher levels of depression during inpatient rehabilitation, and being female and nonwhite were associated with higher levels of reported pain at 1 yr after injury. Consistent with the bivariate correlational analyses, age and injury characteristics were not significant predictors of pain at 1 yr. Similarly, function at baseline was not found to be a significant pre- dictor of pain reports at 1 yr. Table 4 displays the results of the regression model with the outcome of pain at 1 yr after injury including demographic variables, level of function, depressive symptoms, and community participa-

TABLE 4 Association with pain at 1 yr after injury B SE bt FIGURE 1 Exploration of the relationship between Age Ϫ0.01 0.15 Ϫ0.05 pain, depression, and community partic- Ϫ Ϫ Education 0.52 0.87 0.59 ipation. Standardized values are reported Race 16.70 5.38 3.10** in the ﬁgure above. ␶ and ␤ indicate val- Sex 3.68 5.28 0.70 ues when only pain or depression predicts 1-yr FIM score Ϫ0.02 0.45 Ϫ0.05 Depression at 1 yr Ϫ1.10 0.20 Ϫ5.46** CIQ including only demographic vari- CIQ 0.66 0.46 1.44 ables. ␶= and ␤= indicate the value when both pain and depression are included in 2 ϭ 2 ϭ Ͻ R 0.36; adjusted R 0.32. ** P 0.01. the model along with demographic variables.

966 Hoffman et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 DISCUSSION pain, depression, and community participation suggests that depression influences the relation- This study shows that pain is a significant ship between pain and community participation at problem at 1 yr after injury for individuals with 1 yr after injury. In other words, the relationship TBI, with 74% of participants reporting some level between pain and community participation be- of pain and 55% reporting interference with daily comes nonsignificant when depression is taken activities from pain. Whereas these findings sug- into account. This is a complex relationship that gest higher rates of reported pain than previously will require further exploration to determine po- published in the literature,12,14,16 the average pain tential causal relationships and the direction of rating of 66.7 is similar to that reported by Findler that causality. Although direction of causality can- and colleagues,27 who found average pain ratings not be explored with the data available in this on the Bodily Pain Index of 67 for individuals with study, these results do offer some suggestions for moderate to severe TBI. The higher rates of re- future causal hypotheses. One such hypothesis may ported pain may be attributable to a variety of be that depression acts as a mediator in the rela- factors, including the population included, how tionship whereby pain leads to increased depres- pain was assessed, and the development of unmea- sion which leads to decreased participation.30 Al- sured complications (e.g., contractures, hetero- ternatively, depression may lead to increased pain topic ossification, and spasticity), which may de- reports and decreased participation which would velop in the year after injury. suggest that depression is the influential variable Initial evaluation of relationships among vari- which should be the focus of interest. Whereas ables suggested that pain at 1 yr was correlated with reports of depression assessed at baseline and 1 yr being female, nonwhite, lower functional status, and were significantly associated with pain at 1 yr after with higher reports of depressive symptoms at base- injury, both pain and community participation line and 1 yr after injury and with more limited were only assessed at 1 yr making any determina- community participation at 1 yr after injury. Simi- tion of causal relationships impossible in the cur- larly, in the regression models with baseline charac- rent study. Also, it is possible that variables not teristics predicting pain at 1 yr, gender (being fe- measured in this study are important to under- male), race (being nonwhite), and higher reports of standing the relationship among depression, pain, depressive symptomatology were related to higher and community participation. Other potential vari- pain ratings at 1 yr. Injury characteristics including ables of interest may include psychological diag- GCS and number of associated injuries were not noses, such as posttraumatic stress disorder, which found to be related in correlational or regression was found to potentially mediate the relationship analyses. This finding is consistent with Walker and between pain and depression and pain and commu- 14 colleagues, who report that headache pain was un- nity participation in a sample of patients with se- related to injury severity. vere TBI.16 Further examination of the possible At 1 yr after injury, race (being nonwhite) and relationship of depression with pain and function is increased depression were the only variables inde- needed to evaluate the impact of other unmeasured pendently related to higher rates of pain. The sig- variables. nificant impact of depression on individuals after Results of the current study do suggest that TBI has been suggested in the literature and has depression and pain are significant problems for been found to be related to pain after injury. For individuals with TBI. Further evaluation of the 19 example, Mooney and colleagues suggest that poor relationship may assist clinicians in determining outcomes after mild brain injury were related to both best treatment approaches. If depression is the key depression and pain. In addition, Chiu and col- variable leading to increased reports of pain and leagues29 found that psychological distress, depres- decreased participation, then treatment for depression, and poor sleep can lead to lower pain thresholds, sion should lead to improvements in both. How- which may impact reports of pain. The relationship ever, if depression is a mediator in the relationship between being nonwhite and higher reports of pain between pain and participation then treatment is difficult to interpret in the current sample, given would need to focus on decreasing pain first, then the small sample size and the heterogeneous pop- on treating depression to increase participation. ulation included in the nonwhite category. Future Clinicians would benefit from conducting more examination of the impact of race on pain reports detailed evaluations of pain, depression, and func- after TBI is warranted. tional participation when considering treatment Another interesting finding in this study was approaches for individuals with TBI such as those that community participation was associated with described by Martelli and colleagues.31,32 Consid- pain on a bivariate level but was not significantly eration of alternative treatment interventions will associated with pain in the regression analysis. be needed given the potential sedative effects of Further exploration of the relationship among pain medications in individuals with TBI and the

December 2007 Pain and Community Participation After TBI 967 reduced ability to participate in cognitive behav- mine causal relationships. Such research may in- ioral treatments.33 In general, there has been a form clinicians on appropriate assessment and reluctance to treat pain in cognitively impaired treatment recommendations for this population. individuals. However, lack of treatment can negatively impact not only individuals with TBI, but REFERENCES their families and caregivers as well.34 Therefore, 1. Dudgeon BJ, Ehde DM, Cardenas DD, Engel JM, Hoffman AJ, Jensen MP: Describing pain with physical disability: further study is warranted to identify where to narrative interviews and the McGill Pain Questionnaire. focus treatment and on the best treatment meth- Arch Phys Med Rehabil 2005;86:109–15 ods for this population. 2. 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December 2007 Pain and Community Participation After TBI 969 Authors: Jenny P. Liao, MD Andrew J. Waclawik, MD Myopathy Barend P. Lotz, MD Sharhriar M. Salamat, MD, PhD Brad R. Beinlich, MD Benjamin R. Brooks, MD RESEARCH ARTICLE Afﬁliations: From the Department of Neurology, University of Wisconsin Medical School, Madison, Wisconsin. Myopathic Dropped Head Syndrome Correspondence: An Expanding Clinicopathological Spectrum All correspondence and requests for reprints should be addressed to Jenny P. Liao, MD, Clinical Assistant Professor of Neurology, Department ABSTRACT of Neurology, CSC H6/574, University Liao JP, Waclawik AJ, Lotz BP, Salamat SM, Beinlich BR, Brooks BR: Myo- of Wisconsin Medical School, 600 Highland Avenue, Madison, WI pathic dropped head syndrome: an expanding clinicopathological spectrum. Am J 53792. Phys Med Rehabil 2007;86:970–976. Objective: A number of neuromuscular conditions may lead to a Disclosures: dropped head syndrome (DHS), with some patients developing a late A portion of this manuscript was presented in abstract form in onset noninflammatory myopathy affecting only, or predominantly, neck Neurology 1997;48:A445. extensor muscles (NEM). The cause, pathogenesis, and nosological classification of this condition are unclear. To further investigate this condition, 0894-9115/07/8612-0970/0 American Journal of Physical the authors evaluated the clinical, electrodiagnostic and pathologic find- Medicine & Rehabilitation ings in seven patients with a myopathic DHS. Copyright © 2007 by Lippincott Williams & Wilkins Design: Analysis of clinical data, electrodiagnostic studies, and muscle biopsies of seven patients, including one set of identical twins, who DOI: 10.1097/PHM.0b013e3181588331 developed a very late onset myopathy with severe NEM weakness. Results: Age of onset was 61–79 yrs, with the pair of identical twins developing NEM weakness within 1 yr of each other (ages 63 and 64, respectively). Seven patients developed weakness (six slight weakness and one more severe) in muscles other than NEM. The group was characterized by the electromyography (EMG) showing a “myopathic” pattern in cervical paraspinal muscles (7/7), muscle biopsies with nonspecific myopathic changes on histologic stains (7/7), marked abnormalities in NADH dehydrogenase–reacted sections (6/7), desmin-positive sarcoplasmic deposits (1/7), low carnitine levels by biochemical assays (2/7), and mitochondrial changes (3/7). Conclusions: Myopathic DHS encompasses a wide spectrum of conditions that strongly affect NEM; however, as documented in the monozygotic twins, some patients may suffer from a distinct, genetically determined form of late-onset restricted myopathy leading clinically to DHS.

Key Words: Dropped Head, Myopathy, Neck Extensor Muscles, Bent Spine

970 Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 acid Schiff, oil red O, and myophosphorylase, per A number of neuromuscular conditions, includ- standard protocols.15 Where appropriate, succinate ing neurogenic and myopathic entities, as well as dehydrogenase and cytochrome-c oxidase–reacted defective neuromuscular transmission, may present sections were also prepared. Quantitative carnitine with isolated or predominately neck extensor levels in muscle were obtained in three patients weakness.1–10 Motor neuron disease, polymyositis, (patients 1A, 1B, and 2). Immunolabeling for and myasthenia gravis are three conditions most desmin was obtained in one patient (patient 4). commonly associated with severe weakness of neck Electron microscopy (EM) was performed in five extensors. The term dropped head syndrome patients (patients 1A, 1B, 2, 3, 4). (DHS) was introduced by Suarez and Kelly10 in 1992 to designate patients with a late-onset non- RESULTS inflammatory myopathy that affects only, or predominantly, neck extensor muscles (NEM). Several Clinical Presentation case series of DHS have been reported.4,7,10–13 Sub- The clinical and laboratory findings of our sequently the term isolated neck extensor myop- seven patients are summarized in Table 1. The age athy (INEM) was proposed by Katz et al.4,5 to em- of onset was between 58 and 79 yrs old. Patients phasize the distinct clinical presentation of these typically reported insidious onset of NEM weakness patients. for several weeks to months. The time from symp- The cause and pathogenesis of INEM/DHS re- tom onset to diagnosis of DHS was 1–7 yrs. The main unclear. It has been increasingly recognized degree of NEM weakness varied from 1/5 to 5Ϫ/5 by neuromuscular specialists. The nosological clas- on the Medical Research Council (MRC) scale. All sification of INEM/DHS is controversial, and it of our patients experienced at least some degree of likely represents a heterogeneous condition. Some weakness in other muscle groups besides the NEM; authors have included in the DHS spectrum disor- these frequently included the neck flexors, proxi- ders such as parkinsonism,14 which initially may mal arm muscles, or proximal leg muscles. The present with neck extension weakness, adding to creatine kinase (CK) values ranged from normal to the nosological confusion. mildly elevated (up to twice the upper limit of We performed a retrospective review of seven normal value). patients, including a pair of monozygotic twins, Patients 1A and 1B were monozygotic twins who presented with noninflammatory myopathies who developed severe NEM weakness (Fig. 1) at with predominant NEM weakness. Our findings ages 64 and 63, respectively. They have been fol- reveal that in all cases, the weakness and myo- lowed in our neurology clinic for 12 yrs with rela- pathic changes seen on muscle biopsies extended tive stabilization of their NEM weakness. Patient 2 beyond neck extensor muscle group. It seems that had, in addition to NEM weakness, mild involve- myopathic dropped head syndrome is an expanding ment of shoulder and pelvic girdle muscles. Patient clinicopathological spectrum. 3 initially presented with NEM weakness but, within 2 yrs, developed cogwheel rigidity in her PATIENTS AND METHODS limbs, a right upper-limb resting tremor, shuffling Seven patients were reviewed, including a set gait, dysarthria, dysphagia, and limitation in her of monozygotic twins, who were followed in our extraocular eye movements. She was later diag- neuromuscular clinic spanning the years 1993– nosed with multisystem atrophy. Patient 4 initially 2002, and who presented with severe NEM weak- carried the diagnosis of limb girdle muscular dys- ness. All patients underwent serial neurological trophy; he was noted to have accompanying severe examinations, laboratory studies (including creat- weakness of his hip flexors in the range of 1/5 to 2/5 ine kinase and acetylcholine receptor antibody test- on the MRC scale. Patients 5 (Fig. 2) and 6 devel- ing), electrodiagnostic testing (including 2-Hz re- oped NEM weakness during several weeks, which petitive nerve stimulation and needle EMG) and then stabilized. No patients had any electrodiag- muscle biopsy. nostic evidence of a neuromuscular transmission Open muscle biopsy sites included the trape- defect by 2-Hz repetitive nerve stimulation in sev- zius muscle in two patients, deltoid muscle in eral muscle groups, and all patients tested normal four patients, and quadriceps muscle in one pa- for thyroid function and acetylcholine receptor an- tient. The muscle specimens were frozen in liq- tibody studies. There was also absence of ocular uid nitrogen before being cross-sectioned and involvement (except patient 3, who developed lim- stained with hematoxylin and eosin, Gomori itations of extraocular movements late in the trichrome, nicotinamide adenine dinucleotide de- course of her disease as part of her diagnosis of hydrogenase-tetrazolium reductase (NADH-Tr), multisystem atrophy), speech abnormality, or bul- adenosine triphosphatase (at pH 4.3, 4.6, and 9.4), bar signs in our patients on serial neurological acid phosphatase, nonspecific esterase, periodic examinations.

December 2007 Myopathic Dropped Head Syndrome 971 TABLE 1 Summary of clinical and laboratory findings Age at Neck Age at Time of Extension Other Muscle Group CK (Normal Muscle Patient Sex Onset Diagnosis Weaknessa Weakness 0–175 U/liter) Biopsied

1A F 64 65 4 to 5Ϫ/5 4ϩ to 5Ϫ/5 neck 352 Trapezius flexion 1B F 63 65 4 to 4ϩ/5 5Ϫ/5 neck flexion, 181 Trapezius 4ϩ to 5Ϫ/5 hip flexion 2 F 79 84 2 to 3/5 4/5 shoulder girdle, 118 Deltoid 4ϩ/5 neck flexion and pelvic girdle 3 F 61 62 1/5 4ϩ/5 neck flexion 369 Deltoid and arm abduction 4 M 67 74 2/5 4/5 neck flexion and 44 Quadriceps shoulder girdle, 1 to 2/5 hip flexion 5 M 58 59 3/5 4 to 4ϩ/5 neck 306 (0–215) Deltoid flexion, 4 to 4ϩ/5 arm abduction, 5Ϫ/5 hip flexion 6 F 63 65 3 to 4Ϫ/5 4/5 neck flexion, 4ϩ/ 569 (0–250) Deltoid 5 neck lateral rotation, 4ϩ/5 arm abduction, 4ϩ to 5Ϫ/5 elbow flexion/extension a Medical Research Council Scale. CK, creatine kinase.

Magnetic resonance imaging (MRI) of the cervical spine was obtained in all the patients. Aside from what was felt to be age-related degenerative joint disease changes and/or mild disk bulges, there was no signiﬁcant cervical stenosis, cord compression, bony structural abnormalities, or mass lesions.

Electrodiagnostic Data All the patients had nerve conduction study (NCS) and EMG performed on them at least once.

FIGURE 1 Patients 1A and 1B (identical twins). Both patients were barely able to hold their heads up against gravity (neck extension strength 3/5 on the MRC scale) and even- FIGURE 2 Patient 5. Severe neck extension weak- tually required neck collars for support. ness, with dropped head appearance.

972 Liao et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 Two-hertz repetitive nerve stimulations on the NCS did not reveal any decremental responses to suggest a neuromuscular transmission defect. The EMG showed active denervation (as evidenced by fibrillation changes and positive waves) in five of seven patients (patients 1A, 1B, 2, 4, and 6) and myopathic changes (short duration, small ampli- tude, and polyphasic motor units) in the cervical paraspinal muscles in seven of seven patients. Of the proximal upper-limb muscles tested, myopathic motor unit potentials were detected in the triceps and deltoid muscles of five of seven patients (patients 1A, 1B, 4, 5, and 6). Four of seven patients FIGURE 4 Patient 1A. Marked abnormality in the (patients 2, 3, 5, and 6) exhibited myopathic distribution of oxidative enzyme activity. changes in the trapezius and infraspinatus mus- Many fibers seem “mottled” (arrows). cles. Patient 4, who had severe weakness of his hip NADH-Tr stain ϫ380. flexors (1 to 2/5 on the MRC scale), was found to have myopathic motor units in his iliopsoas and anterior tibialis muscles. Of note, not all patients received uniform screening EMG studies; rather, abnormally shaped mitochondria that contained the examiner directed his study according to the dense bodies on EM. The muscle biopsies from the patient’s clinical presentation. monozygotic twins (patients 1A and 1B) revealed some increase in the number of small lipid drop- Muscle Pathology lets. Their muscle tissue tested low for total and The trapezius and deltoid muscles were the free carnitine levels (about half of normal for pa- most commonly biopsied muscles. One patient tient 1A and a fifth of normal for patient 1B). The (patient 4) underwent a quadriceps muscle biopsy. muscle biopsy from patient 4 contained accumula- All muscle biopsies revealed chronic myopathic tions of sarcoplasmic material that tested positive changes (see Figs. 3 and 4) with large variation in for desmin (see Fig. 5). Five of the seven muscle fiber sizes, internalized nuclei, split fibers, and biopsies (patients 1A, 2, 4, 5, and 6) revealed mild reduced oxidative enzyme activity on NADH-Tr neurogenic atrophy in addition to the myopathic staining. There was no evidence for inflammatory changes. changes. In two patients (patients 1A and 1B), the Gomori trichrome–reacted sections revealed some DISCUSSION of the muscle fibers with increased subsarcolem- We agree with previously reported postulates mal accumulations of mitochondria (ragged red that at least a subgroup of patients with a myo- fibers), and one of the patients (patient 3) had pathic DHS most likely represent a unique form of restricted late-onset myopathy affecting predominantly NEM.4,5 However, our experience with patients diagnosed with myopathic DHS indicates that in some patients, in addition to NEM weakness, there may be coexisting mild involvement of other muscle groups, especially in the shoulder girdle region. This may be easily overlooked clinically when the NEM weakness presents in such a dramatic fashion and the mild degree of weakness in other muscle groups frequently becomes difficult to appreciate. Also, muscle biopsies of the NEM are fraught with technical difficulties as the cervical paraspinal muscle group lies deep under the thick layer of the trapezius. Therefore, alternative muscle biopsy sites usually include the trapezius, deltoid, or biceps. Electrodiagnostic studies FIGURE 3 Muscle biopsy from patient 1B. Note increased variation in fiber sizes, hypertro- with EMG and muscle biopsies of these muscles phic and splitting fiber (asterisk), degen- adjacent to or close to the cervical paraspinals erating fibers (arrow), and internalized frequently reveal that the myopathic process (not nuclei (arrowheads). Modified Gomori related to inflammatory, metabolic, or advanced trichrome stain ϫ300. dystrophic process) is not purely limited to the

December 2007 Myopathic Dropped Head Syndrome 973 phy, two patients (a set of twins) had low muscle carnitine levels, and one patient’s muscle biopsy showed sarcoplasmic accumulations of material that stained positive for desmin. The desmin accumulations may be nonspecific but consistent with a myofibrillar myopathy.18,19 The mitochondrial changes seen in our three patients may only reflect the aging process, rather than a primary mitochondrial myopathy.7,14,20,21 Because all of the patients reported so far have been elderly, one cannot exclude mechanical strain as possibly playing a role in the DHS.4,5,14,21 Many of the patients have documented cervical degenerative joint disease, silent radiculopathies, and ky- phosis that may compromise stability, tissue elas- ticity, and musculoskeletal support of the NEM. These underlying age-related changes may further compound and exacerbate the NEM weakness in genetically susceptible individuals. Although the exact association between these conditions and myopathic DHS has not been well delineated, one should consider that DHS is a more heterogeneous disorder than previously realized. Work-up for patients with DHS should be tai- lored according to their clinical presentation, but routine laboratory studies should include CK, FIGURE 5 Patient 4. Foci of abnormal sarcoplasmic erythrocyte-sedimentation rate, C-reactive protein, material depositions (arrows). A, Modified acetylcholine receptor antibodies, and thyroid Gomori trichrome; B, desmin stain. Both studies. Recently introduced to clinical practice, ϫ375. muscle-specific typrosine kinase antibodies (MuSK Ab) may also be useful in detecting atypical seronegative myasthenia gravis patients presenting NEM and represents a more diffuse muscle condi- with DHS, because electrodiagnostic studies may tion. This was not only observed in our case series not be sensitive enough to rule out a neuromus- but also in some patients from previous reports on cular transmission defect. In a publication by DHS.4,10 Sanders et al.,22 MuSK Ab were positive in 12 of 32 During the past decade, scattered reports have patients with generalized seronegative myasthenia surfaced linking several disorders (aside from the gravis. Many of these patients exhibited atypical classic progressive neuromuscular conditions such features; for instance, repetitive nerve stimulation as polymyositis, motor neuron disease, or myasthe- was normal in four patients, jitter was normal in nia gravis) with DHS. These include parkinsonism, six patients, and EMG revealed myopathic motor chronic inflammatory demyelinating polyneurop- units in five patients. Three of the 12 patients athy, inclusion body myositis, and other nonin- presented initially with NEM weakness, and if it flammatory myopathies such as nemaline rod my- were not for the presence of MuSK Ab, they very opathy, desmin storage myopathy, mitochondrial well could have been classified as having DHS myopathy, facioscapulohumeral dystrophy, adult consistent with INEM. Although all our patients acid maltase deficiency, and carnitine deficiency tested negative for acetylcholine receptor antibody myopathy.1–5,14,16 Metabolic conditions, such as (AchR Ab), MuSK Ab was not yet commercially hypothyroidism and hyperparathyroidism, have available to test for any of the patients in our also been described in association with DHS.3–5,7,8 group; thus, atypical myasthenia gravis was not Most recently, Rowin et al.17 have reported three excluded in all of our patients. patients who received remote (12–36 yrs ago) ra- Electrodiagnostic testing may detect myo- diation treatments for Hodgkin’s lymphoma who pathic change, usually limited to the NEM. Cervical subsequently presented with DHS. It is imperative spine MRI should be obtained to evaluate for com- that clinicians be vigilant in their evaluations for pressive or spondylitic lesions that may contribute these conditions as the full clinical picture may be to the DHS. The MRIs of the cervical spine in our masked for some time. Within our case series, one patients were not done specifically to evaluate the patient was later diagnosed with multisystem atro- paraspinal musculature; rather, they were ordered

974 Liao et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 to ensure that there was no significant cervical opathy, particularly given that our monozygotic stenosis, cord compression, bony structural abnor- twins developed this syndrome within 1 yr of each malities, or mass lesions. There have been reports other. They both shared a very similar clinical in the literature of edema,4 atrophy,23 or fatty re- course and muscle pathology findings. They are placement24 of the cervical paraspinal muscles still actively observed in our institution and, de- noted on MRI studies. In selected cases, patients spite progressive NEM weakness requiring the use may undergo muscle biopsies of clinically or EMG- of soft neck collars, the twins have not developed affected muscles to confirm presence of myopathic significant involvement of other muscle groups, changes and to help exclude inflammatory or met- aside from what has been indicated in Table 1. This abolic conditions. Some authors have performed possibility of a genetic factor in the INEM patho- cervical paraspinal muscle biopsies.4,10,14,23,25–27 genesis in some cases may be further supported by However, as already stated, these muscle biopsies some familial cases reported in the bent spine may be technically difficult. Therefore, alternative syndrome,5,24,26 a condition with weakness of the biopsy sites (such as the trapezius, deltoid, or bi- truncal extensor muscles that is felt to be within ceps) may be considered, on the basis of clinical the same spectrum as DHS.27 Identification of and electrodiagnostic findings. more familial cases and further evaluations with There are no established effective therapies for linkage analysis may help elucidate the clinical and patients with myopathic INEM. There are anec- genetic spectrum of this unique condition. dotal reports of treatments with steroids, azathioprine, carnitine, coenzyme Q10, and vitamin ther- REFERENCES apies,5–7,25,28,29 but the overall consensus is that 1. Finsterer J: Dropped head syndrome in mitochondriopathy. these treatments are of dubious utility. The few Eur Spine J 2004;13:652–6 2. Hoffman D, Gutman L: The dropped head syndrome with cases that have reported improvement were pa- chronic inflammatory demyelinating polyneuropathy. Muscle tients with inflammatory changes in their muscle Nerve 1994;17:808–10 5,25,29 biopsies, and they were treated with oral ste- 3. Hund E, Heckl R, Goebel HH, Meinck H: Inclusion body roids, sometimes followed by azathioprine. One can myositis presenting with isolated erector spinae paresis. argue that because DHS is felt to be a noninflam- Neurology 1995;45:993–4 matory myopathic process, the inflammation noted 4. Katz JS, Wolfe GI, Burns DK, Bryan WW, Fleckenstein JL, Barohn RJ: Isolated neck extensor myopathy: a common on the muscle biopsies of these patients may actu- cause of dropped head syndrome. Neurology 1996;46: ally represent focal polymyositis mimicking as 917–21 DHS/INEM. Patient 5 was placed on a short trial of 5. Katz JS, Barohn RJ: Paraspinous myopathies: dropped head oral steroids (unknown dose and duration) by his and bent spine syndromes, in Katirji B, Kaminski HJ, Pres- ton DC, Ruff RL, Shapiro BE (eds): Neuromuscular Disor- local physician, without apparent improvement. ders in Clinical Practice. Boston, Butterworth-Heinemann, Six of our seven patients were placed on at least a trial 2002, pp 1181–6 of carnitine supplementation at different doses (rang- 6. Narayanaswami P, Bertorini TE: The dropped head syn- ing from 990 mg to 3 g total per day) for at least 6 drome. J Clin Neuromusc Dis 2000;2:106–12 mos, and although several of them did report im- 7. Rahim F, Gupta D, Bertorini TE, LeDoux MS: Dropped head presentation of mitochondrial myopathy. J Clin Neuromusc proved muscle strength on carnitine supplementa- Dis 2003;5:108–14 tion, we could not detect any objective improvement. 8. Gourie-Devi M, Nalini A, Sandhya S: Early or late appear- The monozygotic twins in particular were given car- ance of “dropped head syndrome” in amyotrophic lateral nitine because their muscle carnitine levels were low, sclerosis. J Neurol Neurosurg Psychiatry 2003;74:683–6 but we suspect that the carnitine deficiency may be 9. Lomen-Hoerth C, Simmons ML, DeArmond SJ, Layzer RB: Adult-onset nemaline myopathy: another cause of dropped an epiphenomenon of muscle degeneration rather head. Muscle Nerve 1999;22:1146–50 than representing a primary carnitine deficiency my- 10. Suarez GA, Kelly JJ: The dropped head syndrome. 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976 Liao et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 Authors: Elaine Biddiss, PhD Tom Chau, PhD Amputee Afﬁliations: From the Bloorview Research Institute, Toronto, Canada (EB, TC); and Institute of Biomaterials and RESEARCH ARTICLE Biomedical Engineering, University of Toronto, Toronto, Canada (EB, TC). Correspondence: Upper-Limb Prosthetics All correspondence and requests for reprints should be addressed to Tom Critical Factors in Device Abandonment Chau, Bloorview Research Institute, 150 Kilgour Road, Toronto, ON, M4G 1R8. Canada. ABSTRACT Disclosures: Biddiss E, Chau T: Upper-limb prosthetics: critical factors in device abandon- Supported by the Natural Science and ment. Am J Phys Med Rehabil 2007;86:977–987. Engineering Research Council, Canada. Objective: To investigate the roles of predisposing characteristics, established need, and enabling resources in upper-limb prosthesis use 0894-9115/07/8612-0977/0 and abandonment. American Journal of Physical Medicine & Rehabilitation Design: A self-administered, anonymous survey was designed to ex- Copyright © 2007 by Lippincott plore these factors. The questionnaire was available online and in paper Williams & Wilkins format and was distributed through healthcare providers, community DOI: 10.1097/PHM.0b013e3181587f6c support groups, and one prosthesis manufacturer. Two hundred forty-two participants of all ages and levels of upper-limb absence completed the survey. Results: Of participants, 20% had abandoned prosthesis use. Predis- posing factors, namely, origin of limb absence, gender, bilateral limb absence, and, most importantly, level of limb absence, proved influential in the decision not to wear prostheses. Enabling resources such as the availability of health care, cost, and quality of training did not weigh heavily on prosthesis rejection, with the exception of the fitting time frame and the involvement of clients in the prosthesis selection. Conversely, the state of available technology was a highly censured factor in abandonment, specifically in the areas of comfort and function. Perceived need emerged as a predominant factor in prosthesis use. Conclusions: Future research should focus on continued development of more comfortable and functional prostheses, particularly for individuals with high-level or bilateral limb absence. Improved follow-up, repair, and information services, together with active involvement of clients in the selection of prostheses meeting their specific goals and needs, is recommended.

Key Words: Upper Extremity, Limb Prosthesis, Prosthesis Fitting, Prosthesis Design, Rehabilitation

December 2007 Factors in Prosthesis Abandonment 977 with upper-limb absence and a number of promi- From cosmetic hands, to body-powered hooks, nent researchers and clinicians in the field (see to externally powered devices that flatter their nat- Acknowledgments). ural counterpart in attempts of mimicry, prosthe- The first four sections were completed by all

sis design endeavors to address the varying needs participants. Section Vi, exploring reasons for pros- and desires of individuals with upper-limb absence. thesis abandonment, was completed by nonusers

Nevertheless, an estimated 20% of individuals with only, and section Vii, regarding prosthesis use and upper-limb absence reportedly do not use pros- satisfaction, was completed by current or past us- thetic devices.1 Nonuse may be a lifestyle choice ers who wished to comment on previous prosthetic based on personal needs, values, and perspec- experiences. The anonymous survey required tives.2–6 It may result from medical conditions that 20–30 mins to complete and was available online preempt prosthesis fitting.3,7–9 Or, nonuse may be (www.prismlab.org/survey.htm) or in hard copy by a consequence of external factors such as limited contacting the authors. insurance coverage or availability, and inadequate technology.10,10–12 Defining reasons for prosthesis Sample Population and Sampling nonuse is pertinent to health professionals, re- Strategies searchers, and administrators involved in the care All individuals with upper-limb absence were of individuals with upper-limb absence, the design eligible to participate in this study regardless of (1) of improved prosthetics, and the evaluation of clin- level of limb absence, (2) origin of limb absence, (3) ical strategies. bilateral/unilateral limb absence, (4) user status, or In a recent review,13 literature on the motivat- (5) types of prostheses used. Parents/guardians ing factors in prosthesis use and abandonment was were asked to complete the survey for children explored using the Anderson behavioral model for under the age of 12. The survey was available in healthcare use.14 Under this framework, prosthesis English, Spanish, French, and Dutch and was cir- acceptance is modeled as a function of the predis- culated through a number of online support posing characteristics (e.g., gender, level, or origin groups (i.e., Arm-Amp, I-CAN, Stumps R Us, of limb absence), the established need, and the UpperEx) and healthcare providers (i.e., Bloorview enabling resources (e.g., healthcare services). The Kids Rehab, Canada; Shriners Hospital for Children review identifies the following topics as warranting in Los Angeles, CA; Sint Maartenskliniek, the Neth- further exploration13: erlands). In addition, the survey was promoted on ● The potential roles of specific factors (e.g., origin the Otto Bock, Inc., Web site, a prominent manu- of limb absence, incidence of pain, fitting time facturer of upper-limb prostheses. The sample frame) in determining prosthesis use/nonuse. group was self-selected. ● The consequences of prosthesis use/nonuse on overall quality of life and health. Distribution and Data Collection ● The interaction and relative importance of pre- Ten months of data were collected via the disposing characteristics, established need, and online survey. Various measures, as recommended 18 enabling resources in prosthesis rejection. for Web surveys, were implemented to monitor sampling and online data collection: Also of importance in forming a realistic picture of prosthesis use and nonuse is the inclusion a. To mitigate nonresponse attributable to spam of nonwearers who, with one notable exception,6 filtering, incorrect mailing addresses, or mis- are often underrepresented because of the ten- trust, local collaborators were enlisted to recruit dency to sample only those actively involved with participants. Respondents were asked to specify 6,15–17 rehabilitation centers. the organization through which they were re- The specific objective of this study was, there- cruited. fore, to elucidate the motivations for prosthesis use b. Where possible, prenotification (e.g., mailed fly- and/or abandonment by a broad examination of the ers) and reminders (e.g., through e-mail or tele- predisposing characteristics, established need, and enabling resources in a population sampled both phone call) were undertaken to increase re- from hospital- and community-based support net- sponse rates. works. c. A well-established, experienced Web provider (www.vovici.com) was employed to host the sur- METHODS vey and to limit any system incompatibilities, Design such as those introduced by browser settings. The six-part survey, described in Table 1, was d. The survey was made available in paper format, developed in consultation with several individuals to reduce sampling bias.

978 Biddiss and Chau Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 TABLE 1 Breakdown of questionnaire content No. and Type of Section Topic Questions Relevant Factors

I Participant demographics 7, categorical Level of limb loss 1, Likert Length of residua 2, numerical Origin of limb loss Dominant-hand involvement Gender Frequency of associated medical conditions and pain Year of birth Year of amputation II Personal experiences and 1, categorical Occupation status lifestyle 6, Likert Activity level, work/school Activity level, leisure Frequency of exposure to sand, dirt, grease, etc. Quality of life Impact of limb loss on quality of life Acceptance of limb loss III Healthcare experiences 2, Likert Satisfaction with healthcare services provided 1, multiple choice Satisfaction with healthcare information provided 1, categorical Frequency of health visits related to limb loss/prostheses Country of healthcare provision IV Personal views on 1, ordinal Prosthesis design priorities prostheses 1, Likert Perceived need for a prosthesis 1, multiple choice User status

Vi (Nonusers) Past prosthesis use and 1, categorical Past prosthesis use reasons for nonwear 1, Likert Views on future prosthesis use 3, multiple choice Reasons for nonwear 3, numerical Challenges encountered in daily life 2, open ended

Vii (users) Prosthesis use and 3, categorical Time of ﬁtting satisfaction 11, Likert Past prostheses 3, ordinal Current prostheses 14, multiple choice Views on future prosthesis use 4, numerical Primary prosthesis 4, open ended Model/manufacturer of primary prosthesis Technical characteristics of primary prosthesis (e.g., proportional control, control strategy) Cost of prosthesis and repairs/maintenance Frequency of maintenance Personal involvement in prosthesis selection Selection factors Frequency of wear Activities for which worn Functional use Satisfaction (i.e., appearance, function, control, maintenance, comfort, and cost) Detailed design priorities Use of secondary prostheses Reasons for nonwear Challenges encountered in daily life VI Qualitative experiences 1, multiple choice Suggestions for future prosthesis development 3, open ended Suggestions for improvements in healthcare provision Additional comments/feedback

December 2007 Factors in Prosthesis Abandonment 979 e. The Internet protocol (IP) address was used to to the wrist, 54% at the transradial level, 21% at identify and filter repeat respondents. the transhumeral level, and 7% at shoulder level or f. To identify possible false respondents, data were higher. Of participants, 15% had bilateral limb screened for completeness and internal consis- absence. The occurrence of congenital limb ab- tency using information patterns (e.g., series of sence was higher in the pediatric population (91%) than in the adult population (41%) (P Ͻ 0.001). Of dates, detailed descriptions of limb absence, and individuals with acquired limb absence, 54% had prosthesis components). lost their dominant hand. Fifty-one percent of par- g. Participant demographics were meticulously ticipants were male, and 60% were adults (i.e., 19 collected and reported in an effort to contextu- yrs or older), with an average age of 43 Ϯ 15 yrs alize the sample population and identify possi- (19–80 yrs). The average age of the pediatric group ble biases. (n ϭ 97) was 9.5 Ϯ 6 yrs (1–18 yrs) and, conse- quently, 70% of pediatric responses were submit- Data Analysis ted by parents/guardians. Respondents were pri- SPSS 15.0 software was used for statistical marily from Canada (35%), the United States analyses. Nonuser and user groups were compared (43%), and Europe (17%). via the ␹2 test (with Yates correction where appli- Of participants, 14% had never worn a pros- cable) or the Student’s t test for nominal and thesis. Two factors characterized individuals who interval data, respectively. Differences in ordinal had never worn a prosthesis: (a) limb absence distal ratings (i.e., satisfaction, design priorities, quality to wrist was more frequent than in the general of life, etc.) were evaluated using the Mann–Whit- population (P ϭ 0.001), and (b) the mean age of ney U test (for two groups) or the Kruskal–Wallis children was younger than in the general popula- test (for more than two groups). Mean rankings tion (P ϭ 0.01), whereas that of adults was older were determined and compared using the Fried- (P ϭ 0.002). According to detailed comments on man nonparametric ANOVA for repeated measures. patterns of use, 28% of participants who had been Other statistical tools included frequency distribu- fitted with a device were categorized as prosthesis tions and three measures of central tendency (i.e., rejecters (i.e., used a prosthesis once a year or less), mean, median, mode) as appropriate to the distri- whereas 64% were considered frequent wearers bution of data in question. (i.e., either full-time or part-time consistent wear). Of note, 45% of participants recruited from the Ethics community rejected prosthetic devices as com- Ethical approval for this study was obtained pared with 21% of those contacted through reha- from Bloorview Kids Rehab and the University of bilitation centers or hospitals (P ϭ 0.002). Full- Toronto. time users wore the prosthesis for an average of 13 Ϯ 4 hrs on a typical workday and 10 Ϯ 5 hrs on RESULTS a day off. Part-time users reported prosthesis wear Study Population Characteristics for 7 Ϯ 4 hrs per workday and 5 Ϯ 4 hrs per day off. A total of 266 respondents completed the anon- The following will focus on comparisons be- ymous survey of which 242 were included in this tween prosthesis rejecters and frequent wearers study (i.e., responses were excluded on the following with regards to predisposing factors, established basis: repeated IP address and similarity of data [n ϭ need, and enabling resources. 3]; incomplete or inconsistent of data [n ϭ 7]; ineli- gible limb absence [n ϭ 5]; under the age of 12 [n ϭ Predisposing Factors 9]). Survey participants were recruited through rehabilitation centers (52%), online support groups Level of limb loss (39%), the Otto Bock Web site (3%), or through an Level of limb absence emerged as a primary independent Internet search or family/friends (6%). predisposing factor in prosthesis acceptance. Spe- Of submissions, 80% were electronic and 20% were cifically, individuals with limb absence proximal to by hard copy. The completion rate of the online the elbow (high level) or to the wrist (low level) survey (i.e., the number of surveys submitted divided were more likely to reject the device than were by the number of times the survey was accessed) was those with transradial limb absence (P ϭ 0.001). 40%. Previous response rates for surveys addressing upper-limb prosthesis design have ranged from Origin of limb absence 24%19 for large-scale mailing campaigns to 69%15 for The relationship between level of limb absence sampling strategies using more localized and directed and prosthesis rejection persisted for both congen- contact. ital and acquired limb absence. Rejection rates for Levels of limb absence varied, with 16% of the individuals with congenital limb absence were population reporting limb absence at a level distal greater for low-level (63%) or high-level (65%)

980 Biddiss and Chau Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 limb absence and less for transradial (21%) (P ϭ skin irritation (46%), blisters (23%), and upper- 0.02). For acquired limb absence, prostheses were body pain (44%). Residual limb pain, phantom rejected by 0% of individuals with low-level ampu- pain, and upper-body pain were more prevalent in tations, 16% with transradial, and 39% with high- individuals with acquired limb absence (P Ͻ 0.001) level amputations (P Ͻ 0.001). Rejection rates were and in individuals with high-level limb absence comparable for individuals with transradial limb (P Ͻ 0.025). Skin irritation and blisters were more absence, regardless of origin. Individuals with ac- prevalent in frequent wearers than in prosthesis quired amputations at a low or high level seemed rejecters (P Ͻ 0.001). No significant differences in less likely to reject prostheses than those with residual limb, phantom, or upper-body pain were congenital limb absence; however, more data are observed between prosthesis rejecters and frequent required for verification. wearers, whereas the latter were more likely to experience skin irritation and/or blisters (P Ͻ Gender 0.001) as consequences of prosthesis use. In gen- Overall, females (39%) were more likely to eral, reports of discomfort and pain were low and reject a prosthesis than were males (23%), (P ϭ the majority did not experience upper-body pain, 0.022). For males and females with congenital limb skin irritation, blisters, residual limb, or phantom absence at any level, no significant differences in pain at any point during a typical week. prosthesis use were observed. However, females with acquired limb absence were more likely to Age reject prostheses than were males at both the tran- No differences in prosthesis rejection were ob- sradial (P ϭ 0.042) and at high levels (P Ͻ 0.001); served between adults and children with congenital not enough data were available to assess low-level limb absence, irrespective of gender or level of limb limb absence. Females with high-level, acquired absence. The mean age of prosthesis rejecters and limb absence in particular, rejected prostheses in frequent wearers was also not significantly differ- 80% of cases as compared with 15% of males (P Ͻ ent. However, when grouped by different life 0.001). A high rate of prosthesis acceptance was stages, as presented in Figure 1, significant differ- observed in males with acquired limb absence ences in rates of rejection were observed (P ϭ (91%) as compared with males with congenital 0.02), irrespective of origin of limb absence. Rejec- limb absence (60%) (P ϭ 0.001). The opposite tion rates peaked markedly in three age groups, trend was evident in females, for whom 38% with from 4 to 10 yrs, from 24 to 35 yrs, and for those acquired limb absence accepted prostheses, as greater than 65 yrs. These differences are likely compared with 70% with congenital limb absence related to lifestyle and functional needs, as will be (P ϭ 0.011). discussed in the subsequent section.

Bilateral limb absence Established Need In general, rejection rates for bilateral and Participants were asked to rate their perceived unilateral limb absence did not differ significantly, need for a prosthesis on a scale from 1 (not at all irrespective of level of limb absence. Rejection needed for daily life) to 7 (absolutely essential for rates for unilateral and bilateral limb absence were daily life). Perceived need varied significantly for comparable in individuals with acquired amputa- frequent wearers and prosthesis rejecters (P Ͻ tions. However, individuals with congenital, bilat- 0.001), with median ratings of 6 and 1 for wearers eral limb absence had significantly higher rates of and rejecters, respectively. No difference in per- rejection (75%) in comparison with those with ceived need was observed between users of different unilateral limb absence (28%) (P ϭ 0.004). prosthesis types. Participants were asked to comment on any Length of residua activities found to be challenging in everyday life. The length of residua was not a factor in pros- Of the 178 individuals who responded to this ques- thesis rejection at either the transradial or the tion, 16% had not encountered any insurmount- transhumeral level. able challenges in their daily life, whereas 10% of individuals found most activities, particularly bilat- Dominant-hand amputation eral tasks, to be challenging. Almost all individuals Amputation of the dominant hand was not who reported a great number of challenges made associated with prosthesis acceptance or rejection. use of a prosthesis to aid in their daily life, whereas frequent wearers and prosthesis rejecters were Medical factors equally represented in the group not experiencing Discomfort, ranging from predominantly mild challenges. to severe, was reported by participants as follows: Self-reported activity levels (i.e., use of upper residual limb pain (32%), phantom pain (32%), limbs in recreational and job/school activities) and

December 2007 Factors in Prosthesis Abandonment 981 FIGURE 1 Rates of prosthesis rejection observed between adults and children with congenital limb absence, grouped by different life stages.

typical operating environments (i.e., exposure to Enabling Factors water, grease, dirt, etc.) were comparable for fre- Healthcare provision and the state of technol- quent wearers and prosthesis rejecters. Rates of ogy are potential enabling factors in prosthesis unemployment and disability leave were low (num- acceptance and will be explored in this section. bering 3% each) and not statistically different for wearers and rejecters. Figure 2 presents rejection Health care rates for various occupations statuses. Of note, students were more likely to reject prostheses than Satisfaction with health care in all areas, spe- were full-time workers (P ϭ 0.021). ciﬁcally ﬁtting (P Ͻ 0.001), follow-up (P Ͻ 0.001),

FIGURE 2 Prosthesis-rejection rates for various occupation statuses.

982 Biddiss and Chau Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 repair (P Ͻ 0.001), training (P Ͻ 0.007), and in- apparent for individuals with acquired limb ab- formation provision (P ϭ 0.009), were significantly sence. Rejecters were fitted at a median 6 mos after lower for prosthesis rejecters. Prosthesis rejecters amputation, with an IQR of 3 mos to 1 yrs, whereas were less satisfied with the information provided wearers were fitted within a median of 3 mos (IQR: with respect to prosthesis technology (P Ͻ 0.001), 2–5 mos). At the time of follow-up, pediatric wear- sources of funding (P ϭ 0.01), use of multiple ers had worn prostheses for an average of 8 Ϯ 5 yrs, prostheses (P ϭ 0.001), level of expectations set whereas adult wearers had worn them for 21 Ϯ 15 (P Ͻ 0.001), and the overall knowledge and expe- yrs. There was no statistically significant difference rience of healthcare providers (P Ͻ 0.001). Both between the times of follow-up for prosthesis wear- prosthesis rejecters and frequent wearers were in- ers vs. rejecters. terested in receiving better information on non- When asked to rate their involvement in the prosthetic options (i.e., strategies for accomplish- selection of their primary prosthesis on a scale ing activities without use of a prosthesis) and from 1 (no choice) to 7 (entirely my choice), fre- resources for peer support. With these two excep- quent wearers reported a median rating of 5, tions, frequent wearers were generally satisfied whereas rejecters reported a significantly lower de- with all other aspects of health care. Prosthesis gree of involvement of 2 (P ϭ 0.001). Prosthesis rejecters reported neutral feelings (i.e., neither sat- rejection and involvement in prosthesis selection isfied nor dissatisfied) for all other aspects of health were not related to the type of device selected, nor care with the exceptions of training and fitting, was prosthesis rejection. which elicited median ratings of satisfied. Prosthe- sis wearers were in much more frequent contact Prosthesis technology with healthcare providers with regard to upper- Prosthesis rejecters were significantly less sat- limb absence than were rejecters (P Ͻ 0.001), with isfied with all aspects of prosthesis design, includ- appointments occurring at a median frequency of ing appearance (P ϭ 0.014), comfort (P Ͻ 0.001), every 7–12 mos, whereas 51% of prosthesis reject- function (P Ͻ 0.001), ease of control (P Ͻ 0.001), ers had not been in contact with healthcare pro- reliability (P Ͻ 0.001), and cost (P ϭ 0.034). The viders for 6 yrs or more. distribution of prosthesis types used by both Of note, satisfaction with overall health care groups was comparable in terms of the shapes of (P ϭ 0.017) and the knowledge/experience of terminal devices used (i.e., hand or hook) and the healthcare providers (P ϭ 0.022) was rated signif- mode of actuation (i.e., passive, body powered, or icantly lower by bilateral wearers in comparison electric). Frequency of maintenance activities (i.e., with unilateral. Prosthesis wearers with acquired fitting, replacement of components, and repairs) limb absence were less satisfied with all areas of was also not significantly different between fre- health care and information provision (P Ͻ 0.05) quent wearers and prosthesis rejecters. Of prosthe- than were those with congenital limb absence. In- sis rejecters, 74% stated that they might reconsider dividuals with high-level limb absence were less prosthesis use if technological improvements were satisfied than those with transradial limb absence made at a reasonable cost. with information provided on technology (P Ͻ 0.015), nonprosthetic options (P ϭ 0.039), re- Relative Importance of Factors sources for peer support (P ϭ 0.008), and training Respondents were asked to specify the impor- (P ϭ 0.027) and fitting (P ϭ 0.012) services. tance of a variety of factors in their choice not to Satisfaction levels of participants from the wear a prosthesis (a) permanently for the case of three major hospitals involved in this study were nonusers, and (b) on a typical day when the pros- comparable in all aspects with the exception of thesis is not worn for the case of current users. The information on funding (P ϭ 0.003), which was percentages of participants who considered each lower, and information on nonprosthetic options factor to be of some importance in the decision not (P ϭ 0.049) which was higher, for one hospital. to wear a prosthesis and the median ratings are Satisfaction with health care and information pro- presented in Table 2. Evidently, prosthesis rejecters vision was significantly lower for individuals re- discontinue use largely because of a lack of func- cruited from community-based support groups in tional need, discomfort, and impediment to sen- comparison with the hospitals (P Ͻ 0.005). sory feedback. Discomfort is also a primary factor The fitting time frame emerged as an impor- for occasional nonuse in frequent wearers in con- tant factor in prosthesis acceptance for individuals junction with reasons of necessity (i.e., the pros- with congenital limb absence. Prosthesis rejecters thesis must be removed for certain activities like were fitted within a median of 3.9 yrs, with an swimming or sleeping, for medical reasons, or for interquartile range (IQR) of 2–6.6 yrs, whereas repair). Excessive weight was deemed more impor- frequent wearers were fitted more quickly, within tant by prosthesis rejecters (P Ͻ 0.001). Heat was 11 mos (IQR: 5 mos to 1.5 yrs). A similar trend was considered an important factor in prosthesis non-

December 2007 Factors in Prosthesis Abandonment 983 TABLE 2 Factors in the decision not to wear a prosthesis (a) permanently for prosthesis rejecters or (b) on a specific day for prosthesis wearers, based on self-reported ratings of importance from 0 (not at all a factor) to 3 (most important factor) Prosthesis Rejecters Frequent Wearers

Percentage of Median Percentage of Median Factors in Nonwear Respondentsa Rating Respondentsa Rating

Just as or more functional without it* 98 3 60 1 More comfortable without it* 95 3 66 1 Too difﬁcult or tiring to use* 88 2 39 0 Too heavy* 88 2 65 1 Too hot 88 2 77 2 More sensory feedback without it* 85 2 44 0 Inconvenience* 93 2 53 1 Lifestyle 80 2 N/A N/A Dissatisfaction with prosthetic technology 70 1.5 N/A N/A Appearance of the prosthesis* 70 1 33 0 Medical factors (i.e., skin irritation, blisters, etc.) 55 1 64 1 Stopped working and needs repair 49 0 56 1 Cost 48 0 N/A N/A Availability of prostheses 48 0 N/A N/A Availability of healthcare services 51 1 N/A N/A Lack of information about prosthetic options 28 0 N/A N/A Lack of training 28 0 N/A N/A Someone else made the decision 15 0 N/A N/A Moral, cultural, or religious reasons 8 0 N/A N/A Must be removed (i.e., for sleeping, swimming) N/A N/A 71 2 Mood N/A N/A 51 0 Fear of damage N/A N/A 35 0 a The percentage of respondents who considered the factor to be of some importance in the decision not to wear a prosthesis. * Factors that were rated signiﬁcantly more important (P Ͻ 0.001) by prosthesis rejecters than by frequent wearers. N/A: As indicated, some factors (e.g. availability, fear of damage, etc.) were not applicable to both prosthesis rejecters and frequent wearers

wear in both groups, whereas medical factors were the predominant factors in prosthesis use and re- considered to be somewhat important by both fre- jection. Individuals who felt that a prosthesis was quent wearers and prosthesis rejecters in the deci- helpful in their daily activities made use of the sion not to wear, as was need for repairs. With the device, whereas those who felt it to be a hindrance latter three exceptions, prosthesis rejecters rated or unreasonably uncomfortable/difﬁcult to use, did all other factors of nonwear with greater impor- not. It is hypothesized that the decision to wear or tance than frequent wearers (P Ͻ 0.001). Quality of reject a prosthesis is resolved in a manner to best information and training services, along with other meet personal priorities and needs. Although not a enabling factors, such as availability of health care primary factor, more than 50% of those surveyed and prostheses, and cost, were generally not con- considered availability or cost to play at least a sidered important in the decision to reject prosthe- minor role in the decision not to wear a prosthesis. ses, although the latter was noted by approximately 50% of individuals as a minor factor in the deci- Merits and Limitations sion. Technological factors pertaining to prosthesis Probably the most interesting attributes of the design were paramount. study presented above are (a) investigation of predisposing factors through exploration of high-or- DISCUSSION der correlations; (b) the ability to assess the relative Summary of Key Findings importance of predisposing factors, enabling fac- Rejection rates, as determined in this study, tors, and established need on prosthesis use and were comparable with a meta-analysis of previous abandonment; and (c) the opportunity to tap into literature conducted in this area.1 Table 3 provides the opinions and experiences of nonusers recruited a summary of the key ﬁndings with respect to the outside of the rehabilitation center. predisposing factors, enabling resources, and es- A number of Web-based surveys have been tablished need. Established need and satisfaction conducted in the past with regards to health care with available prosthesis technology emerged as on a large scale (i.e., Ͼ100 participants)20–22 and

984 Biddiss and Chau Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 TABLE 3 Summary of key findings Factor Key Findings

Predisposing characteristics Level of limb absence Higher rejection rates associated with low- and high-level limb absence Origin of limb absence Origin of limb absence not a factor in rejection for individuals with transradial limb absence Individuals with congenital amputations at a low or high level more likely to reject prostheses than those with acquired limb absence Gender Females with acquired, high-level limb absence more likely to reject prostheses than males Low rate of prosthesis rejection for males with acquired limb absence No gender differences for individuals with congenital limb absence Bilateral limb absence Individuals with congenital, bilateral limb absence more likely to reject prostheses than those with unilateral limb absence Length of residua Length of residua not associated with prosthesis rejection Dominant-hand amputation Dominant-hand amputation not associated with prosthesis rejection Medical factors Phantom and residual limb pain and upper-body pain more prevalent in individuals with acquired or high-level limb absence, but not associated with prosthesis rejection Skin irritation, blisters, and upper-body pain more prevalent in frequent wearers than in prosthesis rejecters Age 4–10 and 24–35 age groups associated with higher rates of rejection Established need Perceived need for prosthesis Prosthesis rejection associated with lower perceived need Occupation status Prosthesis rejection higher for students than full-time workers Activity levels Self-reported activity levels not associated with prosthesis rejection Enabling resources Health care Satisfaction with overall health care lower for prosthesis rejecters Fitting at an older age for children with congenital limb absence is associated with prosthesis rejection, as is a longer time gap between amputation and ﬁtting for individuals with acquired limb absence Low perceived involvement in the selection of a prosthesis is associated with prosthesis rejection Prosthetic technology Dissatisfaction with prosthesis technology highly associated with prosthesis rejection

prosthetics on a smaller scale (i.e., Ͻ100 partici- use of electric prostheses than the general pop- pants).23,24 Internal consistency and test–retest re- ulation, reflecting the expertise of Bloorview liability between a large variety of questionnaires Kids Rehab in myoelectric prostheses and pedi- administered via the Internet and by other means atric care. Age (i.e., pediatric or adult) and de- (i.e., telephone, mail, etc.) have been demonstrat- vice type were not factors in prosthesis use or ed.22,25 However, it is important to recognize the possible sampling biases inherent to the results abandonment in either Canada or the United presented herein. States. 3. To quantify possible sampling biases introduced 1. The sample population was largely self-selected, as by the use of an online survey, we compared the is often the case in consumer-based surveys, mak- demographic distribution of this study’s elec- ing it difficult to assess the extent to which the ϭ opinions expressed are reflective of the population tronic respondents from the United States (n as a whole. To quantify this potential limitation, 92) with that of a large-scale epidemiologic ϭ rates of prosthesis rejection in samples recruited study (n 2477), also conducted in the United 19 from the three primary pediatric hospitals were States in 1996. No statistically significant dif- compared. Response rates for the three hospitals ferences with respect to age, prevalence of tran- were approximately 40%, 55%, and 100%. No sig- sradial limb absence, or origin of limb absence nificant differences in rates of prosthesis rejection were observed. The prevalence of electric hands or satisfaction with overall health care were ob- and body-powered hooks was not statistically served, irrespective of the rate of self-selection. different. In this study, 51% of participants were 2. It is expected that the study population based in male, as compared with 63% in the Atkins et Canada was, in general, younger, with greater al.19 study (P ϭ 0.02). Previous research has

December 2007 Factors in Prosthesis Abandonment 985 found no association between gender and Inter- REFERENCES net access.26 It is possible, because of the po- 1. Biddiss E, Chau T: Upper extremity prosthesis use and abandonment: a survey of the last 25 years. Prosthet Orthot tential bias of Internet-based surveys to exclude Int 2007;31:236–57 27 low-income individuals, that concerns regard- 2. Scotland TR, Galway HR: A long-term review of children ing high costs of prostheses are actually under- with congenital and acquired upper limb deficiency. J Bone Joint Surg (Br) 1983;65:346–9 estimated in this study. Future research in this 3. Wright TW, Hagen AD, Wood MB: Prosthetic usage in major area is needed. upper extremity amputations. J Hand Surg (Am) 1995;20: 619–22 The results of this study must be considered in 4. 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Gaine WJ, Smart C, Bransby-Zachary M: Upper limb trau- with high-level or bilateral limb absence. Efforts by matic amputees. Review of prosthetic use. J Hand Surg (Br) 1997;22:73–6 healthcare providers to actively involve clients in the 8. Datta D, Selvarajah K, Davey N: Functional outcome of selection of a prosthesis most suited to their personal patients with proximal upper limb deficiency—acquired goals and needs should be escalated. Individuals with and congenital. Clin Rehabil 2004;18:172–7 limb absence are particularly interested in resources 9. Davidson J: A survey of the satisfaction of upper limb for peer support and are desirous to know all of their amputees with their prostheses, their lifestyles, and their abilities. J Hand Ther 2002;15:62–70 options, including nonprosthetic. Better support, 10. 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Biddiss E, Chau T: The roles of predisposing characteristics, established need, and enabling resources on upper extrem- ment: Hanna Heger, Dr. Peter Kyberd, Dr. Dick ity prosthesis use and abandonment. Disabil Rehab Assist Plettenberg, Virginia Wright, Rafael Reyes, John Tech 2007;2:71–84 Krenzel, Julie Shaperman, Sheila Hubbard, Dr. 14. Anderson RM: Revisiting the behavioral model and access to Monique Gignac, Dr. Colin Macarthur, Randall Al- medical care: does it matter? J Health Soc Behav 1995;36:1–10 ley, Karen Tam, and Nora Fayed. For their promo- 15. Kyberd P, Davey J, Morrison J: A survey of upper limb prosthesis users in Oxfordshire. J Prosthet Orthot [serial tion of the survey, we thank: Margriet Poelma, online]. 1998;10. Available at: http://www.oandp.org/jpo/ Joanne Shida, Shawn Swanson, Karen Lundquist, library/1998_04_085.asp. Accessed November 5, 2005 Lisa Artero, Vera van Heijningen, Tim Pauley, Dr. 16. 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December 2007 Factors in Prosthesis Abandonment 987 Authors: Barbara A. Crane, PhD, PT, ATP Margo B. Holm, PhD, OTR/L Wheelchair Douglas Hobson, PhD Rory A. Cooper, PhD Matthew P. Reed, PhD

Afﬁliations: RESEARCH ARTICLE University of Hartford, Physical Therapy Department, College of Education, Nursing and Health Professions, West Hartford, Connecticut (BAC); Departments of A Dynamic Seating Intervention for Occupational Therapy (MBH) and Rehabilitation Science and Wheelchair Seating Discomfort Technology (DH, RAC), School of Health and Rehabilitation Sciences, University of Pittsburgh, Pittsburgh, Pennsylvania; University of Michigan Transportation Research Institute, Ann Arbor, Michigan (MPR). ABSTRACT Crane BA, Holm MB, Hobson D, Cooper RA, Reed MP: A dynamic seating Correspondence: intervention for wheelchair seating discomfort. Am J Phys Med Rehabil 2007; All correspondence and requests for 86:988–993. reprints should be addressed to Barbara Crane, University of Hartford, Objective: The objective of this study was to examine the effectiveness Dana 410C, 200 Bloomﬁeld Ave, West Hartford, CT 06117. of a new user-adjustable wheelchair seating system designed to relieve discomfort for long-duration wheelchair users. Disclosures: Design: This objective was carried out using the newly developed Tool This research was conducted at the School of Health and Rehabilitation for Assessing Wheelchair disComfort (TAWC) as the primary outcome Science, Department of Rehabilitation measure. Two wheelchair users each tested two different designs and Science and Technology, University of Pittsburgh. The study was supported feedback from the wheelchair users regarding the first design was used to by National Institute on Disability guide development of the second design. A single-subject research meth- Rehabilitation and Research, grant #H133E990001. odology was used, allowing long-duration (up to 2 wks per test) evaluation of the wheelchair seating systems and comparison of subject discomfort 0894-9115/07/8612-0988/0 levels with those experienced during a baseline period using their own American Journal of Physical Medicine & Rehabilitation wheelchairs. The experimental wheelchair seating systems employed ex- Copyright © 2007 by Lippincott isting automotive seating with embedded pneumatic bladders that allowed Williams & Wilkins adjustment of the seat and back-support characteristics. The test wheel-

DOI: 10.1097/PHM.0b013e3181583ed9 chair also had tilt, recline, and elevating leg rests. Results: The two subjects completed limited periods of testing with the first design, both finding poor results with either stable or increased levels of discomfort. Subject feedback was used to redesign the wheelchair seat. After redesign, both subjects tested the second design and found it substantially more comfortable. Conclusions: The selected research methodology was a very positive method for a progressive wheelchair seating design and the second design provided improved comfort for both users when compared with that experienced using their own wheelchairs and the first test wheelchair. Future research of this type of user-controlled technology is recommended.

Key Words: Wheelchair Seating, Wheelchair, Discomfort, Disability

988 Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 complishment of the ultimate comfort-related Long-duration wheelchair users (i.e., greater goal, the study design allowed flexibility to remove than 8 hrs of use per day) with intact sensation the initial prototype from further testing and rede- frequently experience problematic levels of discom- sign the seating according to the feedback received fort.1–3 Discomfort has been shown to negatively from the subjects. 4 influence consumer satisfaction, cause decreased The Permobil powered wheelchair used for 5 quality of life, and lead to problems related to testing included powered chair tilt, powered back 6 wheelchair propulsion ergonomics and adoption recline, powered elevating leg rests, and a powered 7 of poor sitting postures —all of which may impair seat lift—all currently available wheelchair seating everyday function and the ability to remain seated features. An automotive bucket-style seat was 8 in a wheelchair. Yet, few researchers have inves- mounted on the Permobil wheelchair base. This tigated the nature and causes of wheelchair seat 1,9 seat consisted of a foam contoured seat and back discomfort, or possible solutions to this problem. with cloth upholstery. Air bladders were embedded However, the automotive seating industry has done in both the seat and back cushions underneath the significant product design research to meet the foam cushions. Four air bladders were added to the comfort needs of long-duration drivers. Therefore seat, and three bladders were added to the back (see we decided to try and incorporate automotive tech- Fig. 1). These air bladders were connected to bat- nologies into wheelchair seating. An additional ad- tery-powered pumps, and the wheelchair user had vantage of this would be the ability to provide lower-cost product options for wheelchair users, controls to inflate or deflate each bladder to adjust because these products are already commercially the support characteristics of the cushion. The available in a much larger market than the wheel- wheelchair controls were adjusted until the partic- chair seating market. ipant was satisfied and able to independently oper- Anecdotal experience by clinicians suggests ate all of the seating and wheelchair controls. that giving users greater control of the seating The Tool for Assessing Wheelchair disComfort 10,11 system increases satisfaction, but no systematic (TAWC), a previously developed and validated studies have supported this observation. Preceding tool, was the main outcome measure, allowing work in this research program developed a vali- quantification of subject discomfort in a real-life dated methodology for assessing the affect of wheelchair technology on sitter comfort. The current paper describes the application of this methodology to the development and refinement of an advanced wheelchair seating system. The system, which is based on off-the-shelf commercial components, was intended to be a test bed for technologies rather than a commercial system. As such, the findings reported here may encourage develop- ers to invest in more configurable seating systems for wheelchair users. The purpose of this study was to examine the effectiveness of a this wheelchair seating system designed to relieve discomfort for long-duration wheelchair users.

METHODS This study involved an iterative process of testing two prototype powered wheelchair seating devices constructed on a Permobil powered wheelchair base. Although the initial seating system design was based on laboratory-based research performed before this study, it was not clear to the researchers that the initial prototype would actually prove to be an effective intervention. The ultimate goal of designing an effective dynamic seating intervention to address the comfort needs of this population of wheelchair users was highly dependent on the outcomes of each design and testing phase. In this study, the initial prototype was FIGURE 1 Arrangement of air bladders in the test tested, but when initial results indicated poor ac- wheelchairs.

December 2007 Wheelchair Seating Discomfort 989 testing process following a single-system, within Likert scale. The GDA score has a possible range of subjects research design. Data from the TAWC 13–91, with higher scores representing increased were used to guide modifications of the wheelchair levels of discomfort. GDA scores were recorded for seating system and to quantify its effectiveness in each 4-hr period of time spent sitting. The Discom- reducing discomfort. After testing of the first pro- fort Intensity Score (DIS) allows subjects to rate totype, the seat of the system was redesigned, and level of discomfort in eight body areas and in the the foam structure was replaced with a custom- body as a whole. The DIS score has a range of 8–99. made, four-quadrant Roho air cushion on the basis A score of 8 indicates no discomfort in any part of of feedback from the participants that the automo- the body, and a score of 99 indicates a maximum tive seat was too firm. In addition to the TAWC amount of discomfort in eight body areas and in discomfort scores, subjects monitored their skin the body as a whole. The reliability and validity of daily for any changes in skin integrity for safety. If the TAWC have been established and reported in an any redness was detected after sitting in the test earlier publication.11 wheelchair, the subject was instructed to stop us- Before data collection, participants signed in- ing the wheelchair and contact the researchers. formed consent documents approved by the Uni- The total amount of time sitting in the wheelchair versity of Pittsburgh’s institutional review board. each day was also monitored via a log completed by the subject each day. For the baseline measures discussed previously, An ABCA single-subject design test protocol participants completed daily logs and TAWC ques- was used. Phase A was the baseline phase, during tionnaires. The researcher contacted each partici- which the subjects used their own wheelchairs. pant several times per week to answer any ques- Phase B involved using the prototype wheelchair tions and clarify procedures as needed. Participants with only the traditional powered wheelchair fea- were also encouraged to call or page the researcher tures operational. The traditional features used if there were any problems or question regarding were power seat tilt, power back recline, power either the documentation or the test wheelchair. elevating leg rests, and power seat lift. Phase C The researcher visited with each participant weekly involved using the prototype wheelchair with the to transition the participant from one intervention traditional wheelchair features as in Phase B, with to the next and to collect data. the new air bladder features operational. Subjects Both traditional graphic visual analyses12 and were randomly assigned to either an ABCA or an specialized semistatistical and statistical proce- ACBA test order to minimize possible interference dures designed for use in single-subject design,12,13 of an order effect. were used to analyze the effectiveness of the seat- Two wheelchair users were recruited for this ing interventions. The comfort effects of three seat- study. The participants reported using powered ing systems were compared. The baseline phases wheelchairs for an average duration of 8 hrs/day. (A1 and A2) (participants’ own chairs) were com- They had severe motor impairment and intact sen- pared with a test chair equipped with traditional sation on their buttocks and lower extremities and powered seating options (B1 and B2) and the same experienced discomfort associated with sitting in test chair equipped with traditional powered seat- their wheelchairs. Neither of the participants had ing options and new user-adjustable seating com- experienced skin breakdown on their seating sur- ponents (adjustable air bladder systems) (C1 and faces (buttocks or posterior thighs) within the year C2). The two intervention systems were also com- before enrollment. Both participants were using pared with each other. Permobil power wheelchairs before the study. The All discomfort-related data were manually en- first participant was using a Permobil standing tered into an SPSS data file, then summary data frame power wheelchair when he was initially re- were transferred into a Microsoft Excel spreadsheet cruited, but he later received a Permobil power wheelchair with power tilt and recline features. and graphs were developed for performing visual Participant 1 used a high-profile Roho seat cush- analyses. Semistatistical and statistical procedures ion, and participant 2 used standard Permobil used included celeration line analyses and the 14 seating including mildly contoured seat and back Tryon C statistic. Before any visual or statistical cushions. analysis, the Bartlett test of the lag-1 autocorrela- 12 The TAWC was used to quantify sitting discom- tion coefficients was performed on each phase of fort experienced by the test subjects under all test data collected for each participant. When serial conditions. This tool is divided into sections that dependency of the data were found, the C statistic result in two discomfort scores.10 The General Dis- alone was relied on for indications of a significant comfort Assessment score (GDA) contains eight intervention effect, because the C statistic remains statements related to discomfort and five state- effective even with data that are serially depen- ments related to comfort, rated on a seven-point dent.14

990 Crane et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 RESULTS TABLE 1 Chair 1 testing: means and standard deviations of Both subjects completed partial tests of chair subject discomfort scores 1. The chair was removed early in both cases be- Phase cause of negative outcomes evidenced by the discomfort scores and development of reddened areas Subject A1 B1 C1 A2 on the buttocks, a preestablished stopping point for safety. Discomfort scores for chair 1 testing are GDA score summarized in Table 1. Both subjects also tested 1 57.1 (6.4) * 52.7 (4.2) 49.6 (4.8) chair 2 after the redesign process. This testing 2 39.6 (9.3) 54.4 (3.9) * 40.1 (10.4) allowed comparison of the two chair designs as well DIS as examining the effectiveness of the new design. score Table 2 contains chair 2 summary results. 1 33.8 (5.1) * 27.4 (2.8) 24.9 (1.9) 2 12.7 (1.0) 14.8 (1.3) * 12.9 (0.9) GDA, General Discomfort Assessment score: 13–91, with higher scores indi- Subject 1 cating greater discomfort; DIS, Discomfort Intensity Score: 8–99, with higher This subject used a Permobil Chairman model scores indicating greater discomfort. * No data collected for these phases. powered wheelchair with a high-profile Roho cushion for the A phases of his first trial. He completed phases A, C, and A when testing the first wheelchair design. He had been randomized to an ACBA de- during phase C (z ϭ 2.78) and his final baseline sign, but he experienced redness under one of his phase (z ϭ 2.37). This indicates lower levels of ischial tuberosities after 5 days of phase C testing discomfort with the test wheelchair with tradi- with this first prototype, and therefore the test was tional power seating, but no further decrease in ended for safety reasons. He did not progress to discomfort with the addition of the new, user- phase B; instead, he returned to his own wheel- adjustable seat features. chair for his return to baseline phase (phase A). His GDA and DIS discomfort scores changed little Subject 2 while he used the prototype wheelchair. The scores The second subject was randomized to an were slightly lower, but they were not particularly ABCA testing order. During his baseline phases, he stable, and none of the semistatistical tests indi- used a Permobil power wheelchair with the stan- cated a significant difference when using the test dard Permobil foam seat and back cushions. His wheelchair. The C statistic analysis indicated sig- own wheelchair had power seat tilt, power back nificantly greater discomfort levels on both the recline, and power elevating leg rests. His first GDA and DIS measures when he was using the first chair trial was interrupted during phase B because test wheelchair. of complaints of increased discomfort and difficulty After the redesign of the test wheelchair, he using chair 1 in his home environment. was contacted and asked whether he would be This participant’s mean discomfort scores in- interested in testing the redesigned wheelchair. He dicated greater discomfort during phase B with the agreed, but because he had just obtained a new first chair than during his baseline phase (phase A). personal wheelchair, he underwent a new trial, including new baseline-phase data collection. His new wheelchair was also a Permobil power wheelchair base, but instead of a standing feature, his TABLE 2 Chair 2 testing: means and standard deviations of new wheelchair had power seating options includ- subject discomfort scores ing power seat tilt and power back recline. He was Phase assigned the same testing schedule: ACBA, for consistency with his first round of testing. Subject A1 B2 C2 A2 Once again, his mean GDA and DIS discomfort GDA scores changed little across phases. Trend lines score illustrated increasing levels of discomfort during 1 50.2 (3.0) 49.5 (2.2) 50.0 (3.2) 51.7 (1.9) all phases of testing. However, the slopes of the 2 39.6 (9.3) 36.0 (8.8) 43.6 (10.2) 40.1 (10.4) DIS trend lines were slightly decreased during phases B score and C. On return to baseline, mean discomfort 1 21.4 (1.6) 19.5 (1.0) 21.3 (2.1) 20.6 (1.2) increased and the trend reversed in slope, indicat- 2 12.7 (1.0) 12.5 (1.4) 12.9 (0.2) 12.9 (0.9) ing increasing discomfort when he returned to GDA, General Discomfort Assessment score: 13–91, with higher scores indi- using his own wheelchair. The DIS data C statistics cating greater discomfort; DIS, Discomfort Intensity Score: 8–99, with higher indicated significantly less discomfort during phase scores indicating greater discomfort. B(z ϭ 2.55) and significantly more discomfort

December 2007 Wheelchair Seating Discomfort 991 FIGURE 2 Participant 2: means (upper panel) and trends (lower panel) for DIS scores.

Trends in discomfort levels indicated stable levels phase B with the second wheelchair was significant of discomfort during both baseline phases and in- (z ϭ 2.69), indicating a significantly lower discom- creasing levels of discomfort during phase B. DIS fort level with chair 2. This was indicative of im- score trends followed a similar pattern as those proved comfort with chair 2 when compared with found with GDA scores with increasing discomfort Chair 1 with the same features. levels during phase B (see Fig. 2). The result of the C statistic testing of DIS scores for phase B indi- DISCUSSION cated a significantly higher discomfort level (z ϭ The newly developed TAWC subjective assess- 2.53) with this intervention. ment tool and associated clinical measures were After redesign of the test wheelchair, this par- used in an iterative testing process to develop a ticipant agreed to complete his trial using chair 2. prototype wheelchair seating system designed to Because his own wheelchair had not changed, the minimize discomfort of wheelchair users. Chair 1, test wheelchair was reintroduced, and he com- the first prototype, underwent initial testing by pleted phases B and C and then returned to using both participants. The TAWC measurements dem- his own wheelchair for his final baseline phase. onstrated that the chair 1 was not providing the Celeration line testing of the GDA scores indicated expected benefits. Because of the results from the greater discomfort during phase C than during initial testing, the wheelchair seating system was phase B. Overall, GDA scores were quite variable, redesigned and then retested as chair 2. The same but with a consistent pattern each day: lower scores participants tested chair 2 after this redesign, and in the morning, and higher scores at the end of the TAWC was again used to assess the effective- each day. DIS scores for this participant were ness of the new intervention. slightly less variable overall, yet they still exhibited One concern with using a within-subjects de- a similar pattern. Celeration line testing of these sign in research is autocorrelated data. Because of scores indicated lower discomfort during phases B this concern, all data were tested for autocorrela- and C, but greater discomfort during phase C when tion. In these two subjects only data from two compared with B. Figure 2 illustrates the DIS score phases resulted in autocorrelated data. For these means and trends. C statistic testing of the DIS results, the C statistic was relied on for testing of scores from phase B with the first wheelchair and significant results. The remainder of the data were

992 Crane et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 not autocorrelated, allowing for both visual and ACKNOWLEDGMENTS semistatistical analysis with all included methods. The authors would like to thank Mark Fried- The results of this testing must be considered man, PhD and Steven Stadelmeier, MSc for their in light of the limitations of this particular re- technical assistance in the design and construction search. The difficulties involved in introducing a of both of the test wheelchairs (test chair 1 and test novel wheelchair design to these participants can- chair 2) used for this study. The following manu- not be overlooked. The wheelchair base itself, facturers supplied equipment used for this re- which was not being studied, may have caused search. The power wheelchair base was donated by enough difficulty for the subjects to interfere with Permobil, USA, and the specialized seat cushion the ability to truly examine the effects of the seat- used in the test chair 2 design was provided by The ing intervention. The fit and function of the wheel- Roho Group. In addition, I would like to thank the chair seat also was suboptimal for the participants. wheelchair users who participated in this study for Because of the close relationship between fit and their great commitment of time and dedication to comfort, this may have caused suboptimal levels of this complex data-collection process. comfort for the subjects unrelated to the dynamic feature effects. Recommendations for future study include (1) REFERENCES studying “standalone” seating products intended 1. Monette M, Weiss-Lambrou R, Dansereau J: In search of a better understanding of wheelchair sitting comfort and for use in the participants’ own wheelchairs; (2) discomfort. Paper presented at: RESNA annual conference, reducing the TAWC scoring to twice per day—once Long Beach, CA, 1999 at 6 hrs and once at bed time—because of the 2. Shaw G, Taylor S: A survey of wheelchair seating problems relatively low levels of discomfort reported with of the institutionalized elderly. Assist Technol 1991;3:5–10 shorter sitting durations; (3) reducing the data- 3. Scherer MJ: Users desires for wheelchairs. Rehab Manag collection labor involved on the part of the partic- 1996:121–3 4. Weiss-Lambrou R, Tremblay C, LeBlanc R, Lacoste M, ipants (perhaps by automating more of the data- Dansereau J: Wheelchair seating aids: how satisfied are collection process) and extending the phase consumers? Assist Technol 1999;11:43–53 lengths to 8–10 days per phase to attain greater 5. Herzberg S: Positioning the nursing home resident: an stability of trends; and (4) increasing the number issue of quality of life. Am J Occup Ther 1993;47:75–7 and pattern of alternating phases—for example, 6. DiGiovine MM, Cooper RA, Boninger ML, Lawrence B, Van- Sickle DP, Renschler A: User assessment of manual wheel- studying one intervention at a time and using an chair ride comfort and ergonomics. Arch Phys Med Rehabil ABABA design. 2000;81:490–4 7. Engstrom B: Ergonomic Seating: A True Challenge—Seat- ing and Mobility for the Physically Challenged, Risks and CONCLUSION Possibilities When Using Wheelchairs, vol 1, ed 2. Stock- holm, Posturalis Books, 2002 The goal of this research was to assess the 8. Crane B, Hobson D: The importance of comfort to wheel- effectiveness of a new, user-adjustable wheelchair chair users—a preliminary study. Paper presented at: 18th seating system in alleviating discomfort. A secondary International Seating Symposium; March 7–9, 2002; Van- goal was to use the subject data in further develop- couver, Canada 9. Shaw G: Wheelchair seat comfort for the institutionalized ment of the wheelchair seating design. These goals elderly. Assist Technol 1992;3:11–23 were accomplished through this within-subjects 10. Crane B, Holm MB, Hobson D, Cooper RA, Reed MP, methodology. The first wheelchair design, developed Stadelmeier S: Development of a consumer-driven Wheel- after short-duration laboratory testing, did not meet chair Seating Discomfort Assessment Tool (WCS-DAT). Int J Rehabil Res 2003;27:85–90 the goal of reducing seating discomfort with long- 11. Crane B, Holm MB, Hobson D, Cooper RA, Reed MP, duration sitting. Therefore, this wheelchair was re- Stadelmeier S: Test-retest reliability, internal item consis- called and redesigned. On construction of the new tency, and concurrent validity of the Wheelchair Seating design, the wheelchair was reintroduced and retested Discomfort Assessment Tool. Assist Technol 2005;17:98– 107 by these two participants. This method of testing 12. Ottenbacher KJ: Evaluating Clinical Change: Strategies for was very helpful in designing a wheelchair that was Occupational and Physical Therapists, vol 1. Baltimore, able to meet the goal of enhancing comfort of users Williams and Wilkins, 1986 from the target population. The within-subjects 13. Franklin RD, Allison DB, Gorman BS (eds): Design and design allowed for in-depth investigation of the Analysis of Single-Case Research. Mahwah, Lawrence Erl- baum Associates, 1996 effectiveness of a user-adjustable seating system for 14. Tryon WW: A simplified time-series analysis for evaluat- long-duration wheelchair users with intact sensa- ing treatment interventions. J Appl Behav Anal 1982;15: tion, using three measures of discomfort. 423–9

December 2007 Wheelchair Seating Discomfort 993 CME Objectives: On completion of this article, the reader should be able to (1) define the biochemical rationale for the use of Spasticity cyproheptadine in the treatment of intrathecal baclofen withdrawal syndrome, (2) identify the signs and symptoms of intrathecal baclofen withdrawal, and (3) formulate an effective treatment plan for the CME ARTICLE ● 2007 SERIES ● NUMBER 5 treatment of intrathecal baclofen withdrawal syndrome. Level: Advanced. Cyproheptadine for Pediatric Accreditation: The Association of Academic Physiatrists is accredited by the Accreditation Council for Intrathecal Baclofen Withdrawal Continuing Medical Education to A Case Report provide continuing medical education for physicians. The Association of Academic Physiatrists designates this continuing medical education activity ABSTRACT for a maximum of 1.5 credits in Category 1 of the Physician’s Saveika JA, Shelton JE: Cyproheptadine for pediatric intrathecal baclofen with- Recognition Award of the American drawal: a case report. Am J Phys Med Rehabil 2007;86:994–997. Medical Association. Each physician should claim only those credits that he Intrathecal baclofen withdrawal syndrome is a known complication of intrathecal or she actually spent in the education baclofen pumps. Its origin is postulated as an independent form of a serotonergic activity. syndrome occurring from loss of ␥-aminobutyric acid B receptor–mediated Disclosures: Disclosure statements inhibition of serotonin. Prodromal symptoms include pruritis, a return of deep have been obtained regarding the tendon reflexes, and increased spastic hypertonia. Previous reports have docu- authors’ relationships with financial supporters of this activity. There are no mented use of cyproheptadine in treatment of this syndrome in adults with apparent conflicts of interest related to positive results. We present the case of a 14-yr-old child with cerebral palsy who the context of participation of the developed pruritis and worsening spastic hypertonia 18 mos after pump implan- authors of this article. tation. She had been previously treated with 520 ␮g/day of intrathecal baclofen. 0894-9115/07/8612-0994/0 Progression of her symptoms was successfully arrested by the administration of American Journal of Physical Medicine both oral and intrathecal baclofen and 6 mg of oral cyproheptadine every 6 hrs & Rehabilitation Copyright © 2007 by Lippincott for 1 day. We postulate that cyproheptadine should be considered a valuable Williams & Wilkins adjuvant therapy for treatment of suspected intrathecal baclofen withdrawal syndrome. DOI: 10.1097/PHM.0b013e31815b5c58 Key Words: Baclofen, Cyproheptadine, Withdrawal Symptoms, Rehabilitation

994 Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 Authors: of a serotonergic antagonist would prove of value. Joseph A. Saveika, MD This concept led to the successful administration of Jean E. Shelton, MD cyproheptadine prophylactically in patients dis- playing early signs and symptoms of ITB with- Affiliations: drawal at the authors’ institution. From the Department of Physical Medicine and Given the increasing use of ITB therapy for Rehabilitation, Eastern Virginia Medical School, spasticity in the pediatric patient, ITB withdrawal Norfolk, Virginia. syndrome is likely to become more common, and a need for treatment of the syndrome has arisen.9 We Correspondence: present the first published use of cyproheptadine in All correspondence and requests for reprints should be ITB withdrawal syndrome in a pediatric patient, addressed to Joseph A. Saveika, MD, Eastern Virginia and we propose that its use in addition to the Medical School, Department of Physical Medicine and administration of baclofen and benzodiazepines Rehabilitation, Hofheimer Hall Suite 646, 825 Fairfax may prove of benefit to the pediatric physiatrist Avenue, Norfolk, VA 23507. who manages children receiving ITB therapy.

CASE REPORT The value of intrathecal baclofen (ITB) therapy A 50-kg, 14-yr-old female with a history of in spastic hypertonia of cerebral or spinal origin cerebral palsy and spastic tetraplegia was previ- has been well established.1,2 Unfortunately, ITB ously treated successfully with ITB for 18 mos. She delivery has been associated with a number of underwent an uneventful pump refill in an outpa- complications ranging in severity from inconve- tient setting. Her reservoir returned less than 1 ml nient to life threatening. The most significant of of baclofen, but no low-volume alarm was noted. these include pump and catheter malfunctions, She returned home without incident. Approxi- withdrawal syndromes, and infection necessitating mately 8 hrs later, she developed severe pruritis removal of hardware.3 and increased spasticity. She was taken to the In ITB administration, one of the most con- emergency room of a pediatric hospital, where she cerning complications is interruption of medica- was evaluated and found to have a low-grade fever tion administration. This interruption is a com- (37.8°C), tachycardia (pulse 123 beats per minute), mon end result of pump malfunction, catheter hyperreflexia, clonus, and increased spasticity. Ag- blockage, or catheter dissociation. Interruption of itation and feelings of fear were noted. Her lower- ITB administration has been shown to lead to a extremity modified Ashworth scale was increased withdrawal syndrome. This syndrome has a wide from 1ϩ to 3, her deep tendon reflexes were in- range of severity, from increased spastic hypertonia creased from 0 to 3ϩ, and her right ankle displayed and pruritis to seizures and audiovisual hallucina- seven to eight beats of clonus, increased from two. tions. In its most severe form, ITB withdrawal Her left ankle displayed no clonus. syndrome presents a constellation of symptoms In the emergency department, her pump was described as mimicking autonomic dysreflexia, interrogated and found to be functioning normally neuroleptic–malignant syndrome, or malignant at her previously programmed dose of 520 ␮g/day, hyperthermia.4 The associated morbidity and mor- simple continuous administration. Radiographic tality of ITB withdrawal syndrome has been well evaluation of pump and catheter were without documented. Given the resemblance of ITB with- change from prior studies, with pump and catheter drawal syndrome to the above syndromes, the stan- in place and catheter tip terminating at the supe- dard of treatment has thus far been the adminis- rior endplate of the sixth thoracic vertebrae. She tration of baclofen and benzodiazepines, with some was given 20 mg of baclofen orally with 6 mg of arguments for the administration of dantrolene.4–7 cyproheptadine (one and one half 4-mg tablets) for The most recent development in the treatment a diagnosis of presumed ITB withdrawal. Symp- of ITB withdrawal syndrome was proposed by toms abated dramatically within 1 hr. Her agitation Meythaler and associates,8 who propose the use of diminished, her tachycardia and fever resolved, and cyproheptadine for treatment of ITB withdrawal. her spasticity, hyperreflexia, and increased right Their logic was based on the similarity of ITB ankle clonus likewise returned to her original base- withdrawal syndrome to serotonergic syndromes, line. After telephone consultation with technical as in overdoses of selective serotonin reuptake in- representatives of the pump manufacturer, a diag- hibitors. They postulated that ITB withdrawal syn- nosis of pump stall was reached, and a 50-␮g in- drome may actually be a form of serotonergic syn- trathecal bolus of baclofen was administered pro- drome caused by downregulation of ␥-amino phylactically via pump. She was admitted for butyric acid (GABA) B receptors, and that addition overnight observation with orders for 20 mg of oral

December 2007 Intrathecal Baclofen Withdrawal Treatment 995 baclofen every 6 hrs and 6 mg of cyproheptadine linked to serotonin syndrome. Meythaler and asso- every 6 hrs for symptoms of ITB withdrawal. ciates8 have advanced the theory that GABA inhib-

On the morning after admission, she was again its the release of serotonin through GABAB recep- evaluated and found to have remained afebrile tors at the level of the brainstem. The serotonergic overnight. Her lower-extremity spasticity was a 1ϩ pathway seems to adjust to this long-term inhibi- on the modified Ashworth scale; she displayed only tion. With abrupt discontinuation of this inhibi- two beats of clonus at the right ankle. Her deep tion, as in our patient, the system seems to swing tendon reflexes could not be elicited. Her pump back toward release of excessive serotonin. This residual volume was changed from 2 to 3 ml to manifests in the patient as a form of serotonin avoid recurrences of ITB withdrawal. After com- syndrome, forming the basis for the use of cypro- pleting 24 hrs of observation, she was discharged heptadine in its treatment. home with a prescription for oral baclofen and Although ITB withdrawal syndrome is perhaps cyproheptadine for use if necessary after telephone the most feared complication of ITB therapy, no consultation with a physician. defined treatment algorithm exists for this entity in the pediatric population. Current pediatric treat- DISCUSSION ment standards are based on experiences in the Baclofen is a selective ligand of the bicuculline adult population and consist of administration of insensitive GABAB receptors present on primary af- baclofen, either orally or intrathecally, and benzo- ferent terminals and highly concentrated in laminae diazepines.4 In our case, the use of oral cyprohep- I to IV of the dorsal horn of the spinal cord. On tadine in conjunction with oral baclofen allowed binding, it inhibits the influx of calcium, which pre- sufficient time for pump interrogation and admin- vents the release of the excitatory neurotransmitters istration of intrathecal baclofen bolus, without pro- glutamate and aspartate. Receptor downregulation 10 gression of the patient’s withdrawal symptoms. Al- was shown in mouse models by Hwang and Wilcox. though it is possible that the symptom abatement In humans, oral baclofen is routinely used as a skel- was attributable entirely to the administration of etal muscle relaxant for spasticity. Central nervous oral baclofen, one would not expect such a pro- system depression limits the maximal allowable oral found response to oral baclofen in a patient receiv- dose. The concept of intrathecal administration is to ing ITB. In addition, the cyproheptadine provided deliver the medication directly to its site of action in excellent subjective symptomatic relief of pruritis. the spinal cord, allowing for much smaller doses and Pruritis is considered a frequent but inconsistent minimizing side effects. Unfortunately, the introduc- symptom of ITB withdrawal syndrome. Although tion of ITB therapy also introduced new complica- not pathognomic, it helps differentiate between tions, related both to the hardware implantation and ITB withdrawal and aggravation of spasticity stem- medication administration. 13 Intrathecal baclofen therapy has been related ming from other causes. In our experience, the to both baclofen overdose and withdrawal. Over- appearance of pruritis and its relief by the admin- dose signs and symptoms include flaccidity, hy- istration of cyproheptadine helped guide clinical poreflexia, respiratory depression, apnea, seizures, decision making to proceed with intrathecal bolus coma, autonomic instability, hallucinations, hypo- of baclofen, despite a normal diagnostic interroga- thermia, and cardiac conduction abnormalities.11 tion of the patient’s intrathecal pump. Management includes supportive care and removal Our success in this case has led to a change in of as much baclofen as possible from the hardware. the standard education of the family of the ITB If not contraindicated, physostigmine may be used pump implantation patient. Previous education did to reduce central side effects such as somnolence not emphasize pruritis, but we now provide infor- and respiratory depression.12 mation regarding the symptoms of ITB withdrawal The use of benzodiazepines and dantrolene syndrome, with particular emphasis on return of sodium in the treatment of ITB withdrawal syn- spasticity and the appearance of pruritis. All pa- drome has been drawn from experience in the tients are now discharged with prescriptions for treatment of neuroleptic malignant syndrome cyproheptadine, in addition to oral baclofen. In the (NMS).8 Both NMS and ITB withdrawal syndrome event of subjective increase of spasticity or pruritic do share some similarities in presentation. The symptoms, patient families are given instructions most overlap has been observed in autonomic to immediately call their physician. If instructed, symptoms, with fever, tachycardia, and hypoten- they administer oral baclofen and cyproheptadine sion documented in cases of each,4 and as observed and proceed to the emergency room of our insti- in our patient. However, NMS is thought to be tution. We continue to provide families with infor- caused by dopamine blockade or reduced transmis- mation to distribute to medical staff of other hos- sion to the thalamus. Intrathecal baclofen with- pitals if they are unable to travel to our institution drawal syndrome, on the other hand, has been or are traveling themselves.

996 Saveika and Shelton Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 As illustrated in the case report, prompt initi- sion of current therapeutic management. J Neurosurg ation of cyproheptadine in conjunction with the 2005;102:1133–6 standard administration of oral and intrathecal ba- 6. Green LB, Nelson VS: Death after acute withdrawal of intrathecal baclofen: case report and literature review. Arch clofen proved successful in averting the progres- Phys Med Rehabil 1999;80:1600–4 sion of ITB withdrawal syndrome in our patient. 7. Khorasani A, Peruzzi WT: Dantrolene treatment for abrupt We recommend that its use in children receive intrathecal baclofen withdrawal. Anesth Analg 1995;80: further study, given the prevalence of implantable 1054–6 ITB pumps in this patient population. 8. Meythaler JM, Roper JF, Brunner RC: Cyproheptadine for intrathecal baclofen withdrawal. Arch Phys Med Rehabil REFERENCES 2003;84:638–42 1. Parke B, Penn RD, Savoy SM, Corcos D: Functional out- 9. Zuckerbraun NS, Ferson SS, Albright AL, Vogeley E: Intra- come after delivery of intrathecal baclofen. Arch Phys Med thecal baclofen withdrawal: emergent recognition and man- Rehabil 1989;70:30–2 agement. Pediatr Emerg Care 2004;20:759–64 ␥ 2. Albright AL, Gilmartin R, Swift D, et al : Long-term intra- 10. Hwang SA, Wilcox GL: Baclofen, -aminobutyric acidB re- thecal baclofen therapy for severe spasticity of cerebral ceptors and substance P in the mouse spinal cord. J Phar- origin. J Neurosurg 2003;98:291–5 macol Exp Ther 1989;248:1026–33 3. Steinbock P, O’Donnel M: Baclofen infusion for spastic 11. Shirley KW, Kothare S, Piatt JH, Adirim TA: Intrathecal cerebral palsy. Clin Neurosurg 2000;47:440–57 baclofen overdose and withdrawal. Pediatr Emerg Care 2006;22:258–61 4. Coffey RJ, Edgar TS, Francisco GE, Graziani V, Meythaler JM, Ridgley PM: Abrupt withdrawal from intrathecal 12. Darbari FP, Melvin JJ, Piatt JJ, Adirim TA, Kothare SV: baclofen: recognition and management of a potentially life- Intrathecal baclofen overdose followed by withdrawal: clin- threatening syndrome. Arch Phys Med Rehabil 2002;83: ical and EEG features. Pediatr Neurol 2005;33:373–7 735–41 13. Ben Smail D, Hugeron C, Denys P, Bussel B: Pruritis after 5. Douglas AF, Weiner HL, Schwartz DR: Prolonged intrathe- intrathecal baclofen withdrawal: a retrospective study. Arch cal baclofen withdrawal syndrome. Case report and discus- Phys Med Rehabil 2005;86:494–7

MEDIA REVIEW Physical Medicine and Rehabilitation Board Review: Palm Edition

by USBMIS. Developed from the book by Sara J. Although the program is largely useful, some sub- Cuccurullo, MD. Published by Demos Medical Pub- jects could use more elaboration. For instance, the or- lishing, New York. $119.00. Available online at: www. thotics and prosthetic section would be better if a quick usbmis.com/store. reference guide were added, including the K1–4 levels and what they mean, how to measure leg-length discrep- This program is a digitized version of the Physical Med- ancy, how to perform the Thomas test, and how to icine and Rehabilitation Board Review book. It allows measure the popliteal angle. Pictures of the different the loading of a condensed version of the book onto a types of sockets, knees, and feet would also be useful. In personal digital assistants (PDA). It is available for Palm the EMG section, pictures of the different waveforms devices as well as Windows-based devices. would be helpful. A chapter about interventional physia- Overall, this is a good program. It was easy to use and try would also be beneﬁcial. Wound care is an important easy to load onto a PDA. Once on the PDA, navigation is topic that does not receive much coverage. fairly easy. The information seems accurate, although the This program was evaluated by several residents. All text is hard to read on a Palm device. Palm devices with agreed that it would be helpful for board review. The older Palm operating system software may have problems book version was felt to be better, but the PDA version reading some of the ﬁles. The text can get distorted, and was considered good as a supplement to be used while on some letters may be changed to symbols. There also seem the go. Their biggest complaint was that the program to be problems running the program off a peripheral stor- was hard to read because of the size of the text. Several age source such as a memory stick or memory card. residents were interested in purchasing the program.

Rating: Lam Nguyen, DO San Antonio, Texas

December 2007 Media Review 997 CME SELF-ASSESSMENT EXAM

INSTRUCTIONS TO OBTAIN CATEGORY 1 CME CREDITS: AMERICAN JOURNAL OF PHYSICAL MEDICINE 1. Read the Designated CME & REHABILITATION Articles in this issue. Vol. 86, No. 12 • December 2007 2. Read the following CME Self- Assessment Exam Questions. 3. Photocopy and complete the his is an adult learning experience and there is no requirement for obtaining CME Self-Assessment Exam T a certain score. The objective is to have each participant learn from the total Answering Sheet and CME experience of studying the article, taking the exam, and being able to immedi- Evaluation. ately receive feedback with the correct answers. For complete information, 4. Send the completed Answering please see “Instructions for Obtaining Continuing Medical Education Credit” at the front of this issue. Sheet and Evaluation to: Every question must be completed on the exam answering sheet to be Bradley R. Johns, Managing eligible for CME credit. Leaving any item unanswered will make void the Editor, CME Department-AAP, participant’s response. This CME activity must be completed and postmarked by American Journal of Physical December 31, 2008. The documentation received will be compiled throughout Medicine & Rehabilitation, the calendar year, and once a year in January, participants will receive a 7240 Fishback Hill Lane, certiﬁcate indicating CME credits earned for the prior year of work. This CME Indianapolis, IN 46278 activity was planned and produced in accordance with the ACCME Essentials.

CME Self Assessment Exam Questions

CME Article Number 5: Saveika, et al. 4. Which of the following is most likely to interrupt delivery of intrathecal baclofen? 1. Baclofen is a ligand of which receptor? A. Pump malfunction A. GABAA B. GABA B. Pump migration B C. Bicuculline C. Systemic infection D. Tetrodotoxin D. Localized trauma ␥ 2. -aminobutyric acid B (GABAB) has been postulated to inhibit which neurotransmitter? 5. Treatment of intrathecal baclofen withdrawal should include A. Serotonin all of the following EXCEPT: B. Dopamine A. Baclofen C. Gutamate B. Cyproheptadine D. Aspartate C. Benzodiazepines D. Clonidine 3. Which of the following is a symptom of intrathecal baclofen withdrawal? A. Pruritis B. Bradycardia C. Hyporeﬂexia D. Psychomotor retardation

998 Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 STANDARDIZED CME SELF-ASSESSMENT EXAM ANSWERING SHEET

The answers to any essay questions AMERICAN JOURNAL OF PHYSICAL MEDICINE must be typed or computer printed & REHABILITATION on a separate piece of paper and at- tached to this page. After finishing this exam: 1. Check your answers with the cor- Please photocopy this form and complete the information rect answers on page 999. required for each CME Activity. 2. Complete the CME Evaluation and Certification on the following page and mail to Bradley R. Johns, Man- Journal Issue Month and Year aging Editor, CME Dept.-AAP, Amer- Volume Number Issue Number ican Journal of Physical Medicine & CME Article Number Rehabilitation, 7240 Fishback Hill CME Article Author’s Name Lane, Indianapolis, IN 46278. 3. This educational activity must be completed and postmarked by De- cember 31, 2008. AAP Members may complete and submit this Circle the appropriate answers. CME Answering Sheet and the fol- 1.ABCD lowing CME Evaluation and Certi- fication page online through the 2.ABCD members-only section of the AAP web page at www.physiatry.org. 3.ABCD

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December 2007 CME Self-Assessment Exam 999 CME EVALUATION & CERTIFICATION

Poor Satisfactory Outstanding Was the article consistent with the stated objectives? 1 2 3

Did reading this article prepare you to achieve 123 its stated objectives?

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I, certify that I have met the criteria for CME credit by studying the designated materials, by responding to the self-assessment questions, by reviewing those parts of the article dealing with any question(s) answered incorrectly, and by referring to the supplemental materials listed in the references.

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1000 CME Self-Assessment Exam Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 Authors: Vishwa S. Raj, MD Diana H. Rintala, PhD Residency Training Afﬁliations: From the Department of Physical Medicine & Rehabilitation, Baylor College of Medicine, Houston, Texas RESEARCH SURVEY (VSR, DHR); The Institute for Rehabilitation and Research, Houston, Texas (VSR); and Michael E. DeBakey Veterans Affairs Medical Center, Houston, Texas (DHR). Perceived Preparedness for Physiatric

Correspondence: Specialization and Future Career Goals All correspondence and requests for of Graduating Postgraduate Year IV reprints should be addressed to Vishwa S. Raj, MD, 1100 Blythe Residents during the 2004–2005 Boulevard, Charlotte, NC 28203. Academic Year 0894-9115/07/8612-01001/0 American Journal of Physical Medicine & Rehabilitation ABSTRACT Copyright © 2007 by Lippincott Raj VS, Rintala DH: Perceived preparedness for physiatric specialization and Williams & Wilkins future career goals of graduating postgraduate year IV residents during the

DOI: 10.1097/PHM.0b013e3181583f44 2004–2005 academic year. Am J Phys Med Rehabil 2007;86:1001–1006. Objective: The purpose of this study was to evaluate trends among postgraduate year (PGY) IV physiatry residents, at the time of graduation from residency, in terms of their perceived experiences in the core clinical areas, confidence with procedural subspecialization, choice in career specialization, and desire to pursue clinical fellowship. Design: Surveys were distributed to 386 PGY IV residents in physiatry at the end of the 2004–2005 academic year. Results: Ninety-three residents (24%) completed responses in a confidential manner. Residents who were generally more confident in core clinical areas, as defined by the Self-Assessment Examination, and specialty prescription writing also believed themselves to be more prepared to practice these topics in their careers. Overall levels of confidence and perceived preparedness correlated positively with months of training and negatively with the belief in the need for postresidency fellowship training to incorporate these areas into clinical practice. Positive correlations also existed among perceived levels of preparedness in performing various physiatric procedures. Statistically significant differences in levels of confidence and preparedness existed among geographic regions when evaluating core physiatric subject matter. Fifty-six percent of residents who responded planned to pursue fellowship training, and a majority of residents intended to perform interventional procedures and musculoskeletal medicine in their practices. Conclusions: These results provide insight into how trainees perceive their current clinical education. With validation of measures for confidence and preparedness, this survey may be useful as an adjunct resource for residency programs to evaluate their trainees. Key Words: PM&R Residency Training, Residency Education, Resident Career Goals, Regional Residency Training, PM&R Procedures, Resident Fellowship Goals

December 2007 Preparedness for Physiatry 1001 TABLE 1 Clinical areas for evaluation as s the field of physical medicine and rehabilita- A defined by the Self Assessment tion evolves, many residency programs have noted Examination (2005) and specialized a trend for recent graduates to pursue fellowship physiatric procedures training and specialization.1 However, data evaluating physiatric residency education and trends in Brain disorders (including stroke and traumatic brain injury) resident career choices is sparse. Though correlations have been shown between quartile ranking on Electrodiagnosis (including electromyography and the Self-Assessment Examination (SAE) and pass- nerve-conduction studies) ing the American Board of Physical Medicine and Industrial rehabilitation (including occupational and performing arts medicine) Rehabilitation (ABPMR) Part 1 Board Certification 2 Joint and connective tissue disorder (including Examination, it is unclear how residents perceive rheumatological disorders) their current education in terms of learning the Medical rehabilitation (including cancer, cardiac, clinical core areas and preparing for future special- general, and pulmonary rehabilitation) ization. During the 1987–1988 academic year, a Musculoskeletal medicine (not including interventional physiatry) survey of chief residents from 70 board-accredited Neuromuscular disorders (including motor neuron physical medicine and rehabilitation residency pro- disease, movement disorders, and multiple grams found great variability in the total months of sclerosis) residency devoted to various clinical specialties in Pediatric rehabilitation physiatry.3 A 1993 survey found that trainees be- Physiatric therapeutics (including modalities and exercise prescription) lieved they needed more clinical and didactic expe- Prosthetics and orthotics rience in industrial medicine, sports medicine, and Spinal cord medicine therapeutic injections.4 During the past few years, Acupuncture however, residents and recent graduates have Botulinum toxin and/or phenol injections shown interest in musculoskeletal medicine, soft- Interventional physiatric procedures (including epidural injections, facet blocks, and medial tissue disorders, therapeutic injections, and nerve 5 branch blocks) blocks. This study was conducted to evaluate Osteopathic manual therapy trends among postgraduate year (PGY) IV physiatry Peripheral joint aspiration and/or injections residents, at the time of graduation from residency, in terms of their perceived experiences in the core clinical areas, confidence with procedural subspecialization, choice in career specialization, and de- prepared. They were asked about the need for fel- sire to pursue clinical fellowship. lowship training to continue a career path after residency (1 ϭ yes, 0 ϭ no), and the number METHODOLOGY months of training devoted to specific disciplines Physiatric residency programs accredited by the during their residency. The second section ad- Accreditation Council for Graduate Medical Educa- dressed perceived levels of preparedness for per- tion (ACGME) were identified using the ACGME Web forming specialized procedures (Table 1). The third page (www.acgme.org) in March 2005. On the basis of section consisted of open-ended questions to deter- information provided, each program coordinator was mine plans for fellowship training and career goals. sent a package in May 2005 and was asked to distrib- Finally, residents rated their confidence levels in ute the contents among graduating PGY IV residents recommending workplace modifications, prescrib- in their respective program. Enclosed for each resi- ing prosthetic or orthotic devices, and prescribing dent were an introductory letter, a questionnaire, and physiatric therapeutics. Though residents did not a postage-paid, preaddressed unmarked envelope to provide identifying markers to protect confidenti- protect confidentiality. Residents were asked to re- ality, they were asked to provide the name of their turn the surveys by mail within 4 wks of receiving them. current residency training program to help catego- The questionnaire was designed to address rize data according to geographic regions (East clinical areas as outlined by the SAE and estab- Coast, Midwest, South, and West Coast). Using the lished by the ABPMR for the 2004–2005 academic Statistical Package for the Social Sciences (SPSS) year (Table 1). In the first section, residents were computer software, the data were analyzed for (a) asked to rate their levels of confidence in evaluat- general descriptive information, (b) relationships ing physiatric diagnoses, and their perceived levels among various factors, and (c) differences among of preparedness to enter a career in specific areas. geographic region. The response options were 1 ϭ very unsure/very unprepared, 2 ϭ somewhat unsure/somewhat un- RESULTS prepared, 3 ϭ neutral, 4 ϭ somewhat confident/ Of 386 surveys distributed, 93 responses were somewhat prepared, and 5 ϭ very confident/very returned, yielding an overall response rate of 24%,

1002 Raj and Rintala Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 TABLE 2 Response rate by geographic region Contribution Number Total Response Region to Study, % of Responses Possible Responses Rate, %

East 41.9 39 170 23 South 28.0 26 76 34 Midwest 14.0 13 95 14 West 16.1 15 45 33 Totals 100 93 386 24

with ranges of 14%–34% per region (Table 2). results for brain disorders, electrodiagnostics, and Ninety-one residents responded that they had spent industrial rehabilitation were not statistically sig- their entire residency within the same program, nificant. Significant relationship also existed be- one resident responded that he or she had not, and tween levels of preparedness and total months of one resident provided no response. Overall, there training (r ϭ 0.246–0.519; P Ͻ 0.01), except that were significant correlations between: prepared- results for electrodiagnostics were not significant. ness and confidence (r ϭ 0.983; P Ͻ 0.001); con- With regard to writing physiatric therapeutic fidence and months of training within specific clin- prescriptions, creating prosthetic and orthotic (P&O) ical areas (r ϭ 0.844; P Ͻ 0.001); preparedness and prescriptions, and recommending workplace modifi- months of training (r ϭ 0.849; P Ͻ 0.001); and cations, there were associations between levels of preparedness and need for fellowship training (r ϭ confidence in the core clinical areas (r ϭ 0.547– Ϫ0.655; P Ͻ 0.03). 0.839; P Ͻ 0.001) and levels of preparedness for Respondents were most confident and believed specialty prescription writing in that clinical area themselves to be most prepared in the areas of (r ϭ 0.514–0.915; P Ͻ 0.001; Table 4). Months of musculoskeletal medicine and electrodiagnosis training correlated positively with confidence in writ- (Table 3). They were least confident and prepared ing P&O prescriptions and prescriptions for physiat- in pediatric rehabilitation. Correlations ranging ric therapeutics (r ϭ 0.428 and 0.463, respectively; from 0.726 to 0.936 (P Ͻ 0.001) existed between P Ͻ 0.001). Confidence in writing P&O prescriptions levels of confidence and preparedness in all core and recommending workplace modifications were rehabilitation specialties. Negative associations negatively related to belief in the need for fellowship with belief in a need for fellowship training were training in the respective core specialty (r ϭϪ0.388 noted for both levels of confidence (r ϭϪ0.216 to and Ϫ0.291, respectively; P Ͻ 0.01). Ϫ0.553; P Ͻ 0.04) and levels of preparedness (r ϭ Residents who believed they were better pre- Ϫ0.209 to Ϫ0.518; P Ͻ 0.05) in specific areas, pared in performing acupuncture also believed except that results for medical rehabilitation were they were better prepared in performing interven- not statistically significant. For most core rehabil- tional physiatric procedures (r ϭ 0.277; P ϭ 0.007) itation specialties, significant relationships existed and osteopathic manual manipulation (r ϭ 0.301; between levels of confidence and total months of P ϭ 0.003). Resident levels of perceived prepared- training (r ϭ 0.268–0.471; P Ͻ 0.02); however, ness in both interventional physiatry and periph-

TABLE 3 Mean responses regarding core clinical areas Belief for Need Months Overall Clinical Area Conﬁdence Preparedness for Fellowship, % of Training

Brain disorders 3.98 3.72 32 4.61 Electrodiagnostics 4.28 4.18 14 6.16 Industrial rehabilitation 3.45 3.35 24 2.39 Joint and connective tissue disorders 3.69 3.52 23 3.40 Medical rehabilitation 3.83 3.79 11 5.42 Musculoskeletal 4.41 4.23 23 7.22 Neuromuscular disorders 3.51 3.35 39 3.96 Pediatric rehabilitation 3.19 3.02 73 2.77 Physiatric therapeutics 4.14 4.11 3 5.16 Prosthetics/orthotics 3.67 3.59 12 3.32 Spinal cord injury 4.16 3.96 38 3.91

December 2007 Preparedness for Physiatry 1003 TABLE 4 Mean perceived preparedness for TABLE 5 Career paths and interests of specialty prescriptions graduates in 2004–2005 and procedures Career Paths and Average Interests Specialty Areas Preparedness Graduates Planned Number of Percentage of to Pursue Responses Respondents Physiatric therapeutic prescriptions 4.04 Peripheral joint injections 4.00 Interventional 43 46 Botox/phenol injections 4.00 procedures Prosthetic/orthotics prescriptions 3.52 practice Workplace modification prescriptions 3.28 Musculoskeletal 32 34 Epidural injections and nerve blocks 2.70 practice OMT 2.19 General practice 17 18 Acupuncture 1.61 Outpatient practice 9 9.7 Sports practice 9 9.7 Inpatient practice 6 6.5 Spinal cord injury 6 6.5 practice Electromyography 4 4.3 eral joint aspiration/injections were significantly practice correlated with preparedness in performing phenol Traumatic brain 2 2.2 and/or botulinum toxin injections (r ϭ 0.256 and injury/stroke 0.359, respectively; P Ͻ 0.02). Finally, a relation- practice ship was noted between levels of preparedness for Private practice 1 1.1 interventional physiatry and peripheral joint aspi- The sum of the results is greater than 100% because ration/injections (r ϭ 0.375; P Ͻ 0.001). respondents had multiple responses. Analyses of variances revealed statistically significant geographic differences in confidence levels and preparedness levels for certain subject matter. with total months of training in core physiatric dis- Specifically, East Coast residents were more confi- ciplines and specialty prescription writing. Trainees dent than West Coast residents in joint and con- who were more confident also believed themselves to nective tissue disorder (means: East Coast: 3.92; ϭ be better prepared to enter careers in those clinical West Coast: 3.13; P 0.004). Southern residents areas, and those individuals who believed they were were more confident than West Coast residents in better prepared generally felt that postresidency fel- prescribing P&O (means: South: 3.92; West Coast: ϭ lowship training was not necessary for professional 3.07; P 0.01), and Southern residents were more clinical work. confident than East Coast residents in spinal cord Surveys of confidence and preparedness have injury medicine (means: South: 4.60; East Coast: ϭ been used by various medical specialties to evaluate 3.76; P 0.001). Residents in both the South and physician competence and clinical experience. A East Coast believed they were more prepared in longitudinal cohort study conducted in 2000 mea- prescribing P&O than did West Coast residents sured overall preparedness of pediatric residents in (means: South: 3.96; East Coast: 3.62; West Coast: Ͻ core clinical areas, and evaluated these same indi- 2.87; P 0.03). Southern residents believed they viduals after 5 yrs of clinical practice.6 In general, were more prepared than did East Coast residents ϭ it was found that the residents’ perceptions of pre- (means: South: 4.36; East Coast: 3.59; P 0.006) paredness at the time of graduation were similar to in spinal cord injury medicine. retrospective assessments 5 yrs later. With respect When asked to note future career paths, most to subspecialties in which there were differences in residents indicated they planned to pursue inter- perceived preparedness, practicing physicians felt ventional procedures and musculoskeletal medi- they may have been more prepared than they ini- cine (Table 5). Fifty-two respondents (56%) indi- tially thought they were at the time of residency cated that they planned to pursue a fellowship after completion. Similarly, a 2005 survey of recently graduation from residency. Of the residents sur- graduated obstetric–gynecology residents found veyed, 95.3% of those pursuing careers in interven- that overall levels of confidence in managing clin- tional procedures practice (significance: two sided, Ͻ ical problems remained constant after graduation 0.001, with forward exact test) planned to pursue in core areas, although it was noted that periodic fellowships to continue their training. review of graduates’ perception of training could be used to adjust residency curriculum relative to DISCUSSION future clinical needs.7 Self-assessed measures of The results of this study indicate that resident confidence in outpatient and inpatient competen- confidence and preparedness generally correlated cies, as well as procedural ability, have already been

1004 Raj and Rintala Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 implemented to evaluate specific areas during fam- in other procedural areas or whether individuals ily practice residency,8 and have even been used as with an interest in procedural based medicine seek an internal gauge for these residencies to evaluate out these procedures. However, according to the their overall effectiveness in teaching ACGME core data collected relevant to career paths, procedure- competencies to their trainees.9 Finally, though based practice will play an important role in the resident perception of comfort is multifactorial, a clinical work of future physiatrists. Further analy- 2006 survey of internal medicine residents found sis of resident exposure to these procedures may that self-perceived levels of comfort in performing help determine optimal exposure during residency clinical procedures was associated with the number to provide clinical competence after graduation. of procedures performed.10 Furthermore, such information may provide fur- Despite well-documented criteria required by ther insight as to what types of procedures will the ACGME for clinical education of residents, it yield the most effective results in preparing a res- has been noted that some difficulty exists in assess- ident for clinical practice. This seems to be of ing and measuring overall resident competence, particular importance given the percentage of res- and identifying appropriate methods of evaluation idents from this study that plan to incorporate for residency programs.11 The most recent pro- procedures in their future work. gram requirements for residency education estab- Residency education and experiences may sig- lished by the residency review committee of the nificantly differ according to the prevalence of cer- ACGME state that residents should have the follow- tain clinical diagnoses within various geographic ing clinical experiences before graduation: at least areas. If a residency program’s clinical experience 12 mos of direct responsibility for the complete is biased secondary to its geography, it may not management of inpatients; at least 12 mos in the have appropriate resources to provide exposure in care of outpatients, with significant emphasis on other facets of physiatry. This, in turn, could account patients with musculoskeletal disorders; and approx- for variable levels of confidence and preparedness imately 200 electromyographic consultations.12 Be- between regions. With further geographic informa- yond these requirements, residency programs have a tion, residency programs may be able to actively ad- great deal of the flexibility to design their curricula just clinical experiences to provide a well-rounded when incorporating the clinical competencies re- physiatric education. quired for a broadly based and complete physiatric The questions posed in this survey yield inter- clinical exposure. This flexibility may account for the esting results regarding resident self perception of great variability between residency programs in de- clinical skills, time devoted to clinical areas during signing their clinical and didactic schedules. residency education, and career and fellowship In addition to the SAE, scheduled resident and plans for graduating residents. However before program director meetings, and annual program in- generalizing the results of this study, potential ternal reviews, the measures used in this study may sources of error must be examined. A major threat provide a supplemental resource to help monitor the to the external validity of this study was the rela- progress of trainees. With further studies, correla- tively low response rate of 24%. Because of the tions may be shown between resident self perception potential for response bias, caution must be used and success on board certification or subspecialty when interpreting the data. More than 40% of the certification examinations. Quantitative analysis respondents to this survey stated they planned to could also be conducted to determine the appro- pursue interventional physiatry practice after grad- priate number of patient encounters or months of uation, and more than 30% planned to pursue clinical exposure, as they relate to reaching appro- musculoskeletal medicine. It is possible that resi- priate confidence and preparedness levels for pro- dents with these career plans were more likely to fessional success. As an adjunct method of evalua- respond to the survey. There is also potential bias tion, these data may assist residency programs for the results of this study to be skewed according when designing clinical and didactic components to the viewpoints and experiences of individuals to focus on the core rehabilitation disciplines and pursuing interventional, as well as musculoskele- newly evolving clinical and procedure-based expe- tal, based practice. Similar considerations for bias riences that trainees will need for future subspe- must be made when evaluating the geographic data cialization. in this study, because a majority of respondents Similar analyses could be used to evaluate the hailed from the East Coast and South. Though procedure-based curriculum in residency pro- producing graduates with increased beliefs of com- grams. This study indicates that there are positive petence may be a desired outcome of residency correlations between perceived preparedness levels education,13 confidence and perceived prepared- among different physiatric procedures. It is unclear ness in this study have not been validated with whether perceived preparedness in one procedural more objective measures, such as the SAE or area tends to generalize to perceived preparedness ABPMR Part I Certification Examination. In addi-

December 2007 Preparedness for Physiatry 1005 tion, months of training are traditionally used by preparedness measures, similar studies may provide residency programs to determine adequate resident residency programs an additional resource to evalu- exposure to clinical areas of training, but it is not ate their trainees. clear whether months of training, vs. specific patient REFERENCES encounters, is the appropriate method to quantita- 1. DeLisa JA, Jain SS, Yablon SA: Resident interest in physical tively evaluate clinical experience. Finally, the use of medicine and rehabilitation fellowship. Results of a survey. open-ended questions to describe fellowship plans Am J Phys Med Rehabil 1991;70:290–3 and career choices yielded a diverse array of re- 2. Fish DE, Radfar-Baublitz L, Choi H, Felsenthel G: Correla- sponses. A possible source of error was the subse- tion of standardized testing results with success on the 2001 American Board of Physical Medicine and Rehabilita- quent interpretation and assignment of these re- tion part 1 board certification examination. Am J Phys Med sponses into more traditional categorizations. Rehabil 2003;82:686–91 To counter the issue of low response rate, a 3. Anderson JM, Felsenthel G: Residency training in physical repeat study should be conducted to further in- medicine and rehabilitation I: clinical and didactic experience. Arch Phys Med Rehabil 1990;71:372–5 crease both regional and national response rates. 4. Kerrigan DC, Janes WW, Martin WA, Roe TJ: Physical med- With the assistance and cooperation of residency icine and rehabilitation residents’ educational needs assess- program directors, graduating residents could ment. Arch Phys Med Rehabil 1993;74:687–90 complete the survey either in paper form or with 5. Hart KA, Kevorkian G, Rintala DH: Continuing medical education: interests of former and current residents of a an Internet-based questionnaire. The survey could physical medicine and rehabilitation residency program. be administered at the time of the resident exit Am J Phys Med Rehabil 1999;78:561–70 interviews for graduation to ensure that the data 6. Roberts KB, Starr TG, DeWitt S: Resident preparedness for collected reflect the most comprehensive evalua- practice: a longitudinal cohort study. Ambul Pediatr 2002; 2:132–5 tion of experiences. With confidential coding of 7. Schneider KM, Monga M: Former-resident self-assessment: individual residents completing the surveys, corre- a tool for residency program curriculum evaluation in ob- lations could then be established between their stetrics and gynecology. J Reprod Med 2005;50:821–3 subjective answers of perceived confidence and pre- 8. Edwards F, Frey K: The future of residency education: implementing a competency-based educational model. Fam paredness with objective scores on either the SAE Med 2007;39:116–25 or ABPMR Part I Certification Examination. Fur- 9. Frey K, Edwards F, Altman K, Spahr N, Gorman RS: The thermore, the use of a more detailed survey with ‘collaborative care’ curriculum: an educational model ad- closed-ended questions could eliminate the ambi- dressing key ACGME core competencies in primary care residency training. Med Educ 2003;37:786–9 guity of open-ended responses regarding future 10. Huang GC, Smith CC, Gordon CE, et al: Beyond the com- career plans. fort zone: residents assess their comfort performing inpa- In conclusion, this survey presents interesting tient medicine procedures. Am J Med 2006;119:e17–24 data pertinent to the evaluation of resident experi- 11. Hassett JM, Zinnerstrom K, Nawotniak RH, Schimpfhauser ences and career choices. Future studies are needed F, Dayton MT: Utilization of standardized patients to evaluate clinical and interpersonal skills of surgical residents. with larger sample sizes to generalize results to the Surgery 2006;140:633–8 overall graduating resident population, specifically 12. Accreditation Council for Graduate Medical Education Web with respect to overall levels of confidence and pre- site. Available at: http://www.acgme.org. Accessed August paredness in various physiatric specialties, geo- 23, 2007 13. Butler DJ, Holloway RL, Gottlieb M: Predicting resident graphic differences between resident perceptions, and confident to lead family meetings. Fam Med 1998;30: future career plans. With validation of confidence and 356–61

1006 Raj and Rintala Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 Authors: Katherine L. Salter, BA Robert W. Teasell, MD Outcomes Norine C. Foley, MSc Jeffrey W. Jutai, PhD

Afﬁliations: Department of Physical Medicine and REVIEW & ANALYSIS Rehabilitation, St. Joseph’s Healthcare London, London, Canada (KLS, RWT, NCF, JWJ); Schulich School of Medicine, University of Western Ontario, London, Canada Outcome Assessment in (RWT, JWJ); and Lawson Health Research Institute, London, Canada Randomized Controlled Trials of (RWT, JWJ). Stroke Rehabilitation Correspondence: All correspondence and requests for reprints should be addressed to ABSTRACT Katherine Salter, c/o Dr. R. Teasell, Dept. of Physical Medicine and Salter KL, Teasell RW, Foley NC, Jutai JW: Outcome assessment in randomized Rehabilitation, St. Joseph’s controlled trials of stroke rehabilitation. Am J Phys Med Rehabil 2007;86: Healthcare London, Parkwood Site, 1007–1012. 801 Commissioner’s Road E., London, Ontario, Canada, N6C 5J1. The lack of a unified approach to outcome assessment in stroke rehabilitation limits our ability to interpret evidence provided by randomized controlled trials 0894-9115/07/8612-01007/0 (RCTs). The purpose of this review was to identify outcomes and assessment American Journal of Physical Medicine & Rehabilitation tools reported in RCTs of stroke rehabilitation interventions as a first step toward Copyright © 2007 by Lippincott consistent assessment of outcomes. Given that the validity of research is linked Williams & Wilkins to reliability and validity of measurement, the relationship between the use of previously developed outcome measures and the methodological quality of RCTs DOI: 10.1097/PHM.0b013e3181587b3d was explored. Electronic literature searches identified RCTs examining stroke rehabilitation therapies from 1968 to 2005. The Physiotherapy Evidence Database (PEDro) scale was used to assess methodological quality. Cited outcomes were recorded and assessment tools identified as previously published or study specific. Four hundred ninety-one RCTs cited the assessment of 1447 outcomes using 489 measurement tools. Two hundred fifty-four of these were previously published, and 235 were study specific. A core of 30 frequently cited tools was identified. The use of previously published assessment tools to evaluate primary study outcomes was associated with higher PEDro scores. Significant heterogeneity in outcome assessment was demonstrated, although a core of 30 frequently cited tools could be identified. Appropriate evaluation and selection of outcome measures would enhance the methodological quality of randomized controlled trials. Key Words: Outcome Assessment, Evidence-Based Medicine, Reliability, Validity, Stroke Rehabilitation, Measurement, Randomized Controlled Trials

December 2007 Outcomes Assessment in RCTs in Stroke 1007 evaluating the effectiveness of interventions in stroke Stroke has a broad impact on individuals, fam- rehabilitation were selected for inclusion. ilies, and the communities in which they live. Nu- merous assessment tools have been created to as- Data Abstraction and Quality sess the complex and heterogeneous consequences Assessment of stroke and the effect of stroke rehabilitation Two abstractors, each blinded to the other’s interventions on stroke outcomes. However, there results, reviewed each article independently. The is no consensus regarding what and how outcomes review process consisted of two parts. In the first should be measured to create a comprehensive part, data pertinent to the study were collected, description of individual functioning and health including study methodology, cited outcomes, and and to facilitate our ability to make comparisons means by which study outcomes were assessed. 1,2 The current across interventions and studies. After this, the study was assigned a quality-rating lack of a unified approach to assessment of out- score derived using the Physiotherapy Evidence come limits our ability to interpret the evidence Database (PEDro) scale. provided by randomized controlled trials (RCTs) in The PEDro scale, developed by the Centre for a clinically meaningful way.3–5 Evidence-Based Physiotherapy (CEBP) in Australia Our ability to interpret study results is also lim- (www.pedro.fhs.uwyd.edu.au), was created to as- ited by the validity of the research itself, which, in sess bibliographic details and abstracts of RCTs, turn, is limited by the reliability of outcome assess- quasi-randomized studies, and systematic reviews ment.6 Ideally, researchers should use, whenever pos- in physiotherapy.11 Based on a set of core criteria sible, measurement tools that have been previously for quality assessment in randomized controlled evaluated for use in similar subject populations and trials derived by expert consensus,11,12 the scale have demonstrated acceptable reliability, validity and consists of ten items (Table 1). Each item receives 6,7 The revised CONSORT guidelines8 responsiveness. a rating of either yes (1) or no (0). Item ratings are recommend the use of previously developed and validated tools to improve quality of measurement and facilitate between study comparisons. As a first step toward developing consensus TABLE 1 The Physiotherapy Evidence around consistent assessment of stroke rehabilitation Database (PEDro) scale outcomes, an examination of the outcome assessment tools that have been reported in the stroke 1. Subjects were randomly allocated to groups (in a crossover study, subjects rehabilitation literature was conducted. Given that were randomly allocated an order in the interpretability and utility of research results is which treatments were received) associated with the validity of outcome measurement, 2. Allocation was concealed the association between the use of previously devel- 3. Groups were similar at baseline oped outcome measures and the methodological regarding the most important prognostic indicators quality of RCTs was examined. 4. There was blinding of all subjects 5. There was blinding of all therapists who METHODS administered the therapy 6. There was blinding of all assessors who Literature Search measured at least one key outcome 7. Adequacy of follow-up (could all of the The present study arose from the Evidence- 9 subjects originally randomized be Based Review of Stroke Rehabilitation. Search strat- accounted for at the end of the egies included the following databases: MEDLINE, study?). This item was modified from CINAHL, EMBASE, MANTIS, PASCAL, Psych-Info the original item in the PEDro scale, and Sci Search from 1968 to 2005. The literature which stipulated measurement of the main study outcome in at least 85% search, study selection, and data abstraction strate- of subjects) gies employed for that review process have been de- 8. Intention-to-treat analysis scribed in detail elsewhere.9,10 For the present study, 9. Results of between-group statistical randomized controlled trials (RCTs) examining pri- comparisons were reported for at least mary or secondary stroke prevention treatments were one outcome 10. Study provides both point measures and excluded, as were review articles, meta-analyses, or measures of variability for at least one systematic reviews. RCTs were included if they cited key outcome the use of a measurement tool or scale to assess at Eligibility criteria were included in the original PEDro least one of the study outcomes. Studies assessing scale. Because subject selection pertains more directly to only discrete, counted outcomes (e.g., mortality, external rather than internal study validity, it was not in- length of stay, occurrence of a stroke event, etc.) were cluded in the present review.9,10 excluded. On the basis of these criteria, 491 RCTs

1008 Salter et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 summed to provide a total score. Unlike checklists scale. The remaining 1447 cited study outcomes such as the Downs and Black,13 the PEDro scale were assessed using 489 assessment tools. Of these, does not include evaluation of study outcomes and, 254 had been previously published, and 235 therefore, provides an evaluation of methodologi- (48.1%) were study specific. cal quality unbiased by the selection of outcome Of the 254 previously published scales, only 30 measures. Four of the PEDro items have been were cited more than ten times each. These 30 most empirically validated (randomization, concealed al- frequently cited measurement tools accounted for location, blinding, and adequacy of follow-up), and approximately 50.1% (725) of all outcome citations. A the remaining items have face validity.11 Reported list of the most frequently cited assessment instru- intraclass correlation coefficients for interrater re- ments is provided in Table 2. liability for the PEDro scale range from 0.5614 to One hundred fifty-two studies cited the use of 0.91.15 A moderate correlation (r ϭ 0.59) between one or more study-specific measures to assess out- PEDro scores and ratings of methodological quality comes. Of these, 66 cited the use of a study-specific using the Jadad scale has also been reported.16 Two measure to assess the primary study outcome. independent raters, blinded to each other, assigned Mean (ϮSD) and median PEDro scores as well as a PEDro score to each study. A third reviewer re- mean ranks are provided in Table 3. solved any scoring disagreements. A detailed descrip- Although the median PEDro score in the tion of this application of the PEDro scale has been group of studies including study-specific assess- published previously.9,10 ment tools is the same as the studies using only For each study included in the review, all cited outcomes were recorded. Primary outcomes were identified. For our purposes, an outcome was con- TABLE 2 Frequently cited outcome measures sidered to be primary if it was explicitly named as Most Frequently Cited Outcomes No. of the primary outcome by the study author(s) or if it 1968–2005 Citations was listed first in the study methodology. Each 22 measurement tool cited was categorized as previ- Barthel index 123 Timed walk assessments (varying 72 ously published or study specific. Tools were clas- times and distances)23,24 sified as previously published if prior publication of Fugl–Meyer assessment of stroke 58 the tool along with published, evaluation(s) of re- recovery25 liability or validity or responsiveness could be iden- Modified ashworth scale26,27 43 28 tified from the information provided by the study Functional independence measure 38 Mini-mental state examination29 27 authors. Study-specific tools were those developed Action research arm test30 25 for the project at hand; they had no previously Frenchay activities inventory31 22 published evidence of reliability, validity, and/or Nottingham health profile32 22 responsiveness cited by the study authors. Medical outcomes study short 22 33,34 Mean (ϮSD) and median (interquartile range) form-36 Nottingham extended activities of 21 PEDro scores were calculated for studies citing daily living35 previously published outcome measures and also Motor assessment scale36 21 for studies citing the use of study-specific tools. In Hospital anxiety and depression 17 addition, mean and median PEDro scores were scale37 38,39 calculated for studies using previously published or Berg balance scale 16 Rankin handicap/modified Rankin/ 16 study-specific tools to assess the primary study Oxford handicap40 outcome. Given that the PEDro scale yields non- Nine-hole peg test41 15 parametric data, median scores for studies using Hamilton rating scale for 15 previously published measures were compared depression42 43 with those citing the use of study-specific measures Motricity index 14 General health questionnaire-2844 14 using the Mann–Whitney U test (a nonparametric Rivermead mobility inventory45 13 alternative to the t test). An alpha level of Ͻ0.05 Rivermead motor assessment46 13 was considered to be statistically significant. All VAS–pain47,48 12 statistical calculations were performed using SPSS Porch index of communicative 12 49 for Windows (version 12.0). ability Motor activity log50 12 Brunnstrom scale51 11 RESULTS Caregiver strain index52 11 One thousand seven hundred twenty-one out- Timed up and go53 10 come citations were recorded from 491 identified Functional ambulation categories54 10 55 RCTs. Of these, 274 represented assessments of Scandinavian stroke scale 10 Weschler memory subtests56,57 10 clinical variables, which were described but made no reference to the use of a specific measurement

December 2007 Outcomes Assessment in RCTs in Stroke 1009 TABLE 3 Mean and median Physiotherapy Evidence Database (PEDro) scores for studies using previously published vs. study-specific measures Mean Mean SD Median IQR Rank

All studies (n ϭ 491) Cited only previously published measures (n ϭ 339) 6.04 1.4 6.0 2 256.5 Included study-specific measures (n ϭ 152) 5.70 1.5 6.0 2 222.5 Studies including study-specific measures (n ϭ 152) Used previously published measure to assess primary outcome (n ϭ 86) 5.97 1.4 6.0 2 84.47 Used study-specific measure to assess primary outcome (n ϭ 66) 5.35 1.6 5.0 2 66.12

previously published measures, both the mean effects in favor of treatment when compared with PEDro scores and mean rank reflect a significant control conditions. The authors suggest several difference in distribution toward lower PEDro possible explanations for why unpublished scales scores in the group using study-specific measures could be a source of bias: (i) unpublished scales (z ϭϪ2.51, P ϭ 0.012). Among the subset of tend to be used by small trials of poorer quality, (ii) studies citing the use of study-specific measures, selective reporting of data obtained from unpub- those studies using a previously published measure lished scales, eliminating nonsignificant results, or to evaluate the primary study outcome obtained a (iii) ad hoc adjustment to the content of unpub- higher median and mean PEDro score than did the lished scale to present the most favorable data. group of studies using a study-specific measure for Although this bias has not been demonstrated in this purpose. Use of a previously published mea- other branches of medicine, it is recommended that sure to assess primary study outcomes was signif- investigators refrain from using unpublished assess- icantly associated with higher PEDro scores (z ϭ ment tools.20 Indeed, it is generally accepted that Ϫ2.6, P ϭ 0.009). reliable and valid measurement is essential in the production of meaningful and replicable data, and, to DISCUSSION that end, researchers should endeavor to use mea- As in previous examinations of RCTs in stroke sures that are known to be reliable and valid.6,7 research, considerable heterogeneity of measure- The present study compared the methodolog- ment was noted.18,19 Four hundred ninety-one ical quality of trials citing the use of previously RCTs cited the use of 489 measurement tools in the published measures with those using study-specific assessment of 1447 study outcomes. This variabil- tools. Although use of a previously published mea- ity and inconsistency in the selection and assess- sure, particularly in the assessment of the primary ment of outcomes limits our ability to interpret study outcome, was associated with superior meth- individual study data and make appropriate com- odological quality, the difference between groups parisons between trials of similar interventions.5,19 was approximately equivalent to a single point on However, a core of 30 frequently cited, previously the PEDro scale. Total PEDro scores provide an published assessment tools was identified. These indication of which trials are more likely to be valid tools were used in the assessment of a wide range and contain sufficient statistical information to en- of patient outcomes along the continuum from hance interpretability.11 A difference of a single body structure and function assessments to health- point may represent an important deficiency in related quality of life and could, together with methodology, particularly if the item were consid- rigorous examination of their psychometric prop- ered to be a significant source of bias, such as erties, provide a starting point around which a concealed allocation, blinding of patients or ther- consensus for outcome assessment in stroke reha- apists, or intention-to-treat analysis. bilitation trials could begin. The apparent association between the use of Despite the availability of a wide selection of previously published measurement tools and the previously published measures, almost one half methodological quality of RCTs signals the impor- (48%) of all measurement tools identified in the tance of outcome assessment to research. The se- present review were study specific. Marshall et al.20 lection of outcome measures may be as important have identified the use of unpublished rating scales a component to the overall quality of an RCT as are as a major source of bias in randomized controlled methods for blinding, concealed allocation, ade- trials of nonpharmacological treatments of schizo- quacy of follow-up, and other components of re- phrenia, such that trials using unpublished scales search design. The selection of appropriate, reli- were significantly more likely to report significant able, and valid measurement tools should be more

1010 Salter et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 formally recognized and consistently reported in a Jutai is also a member of the Consortium for Assistive standardized manner, as recommended by Dijkers Technology Outcomes Research (CATOR) (http://www. et al.6 and Moher et al.21 atoutcomes.com).

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1012 Salter et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 Authors: John A. Schuchmann, MD Barry A. Browne, PharmD Hiccups Afﬁliations: Departments of Physical Medicine and Rehabilitation (JAS) and Medicine (BAB), Scott and White REVIEW & CASE REPORTS Clinic, Scott, Sherwood, and Brindley Foundation, Temple, Texas; and Texas A&M University College of Medicine, College Station, Texas (JAS, BAB). Persistent Hiccups During Correspondence: Rehabilitation Hospitalization All correspondence and requests for reprints should be addressed to John Three Case Reports and Review of the Literature A. Schuchmann, MD, Department of Physical Medicine and Rehabilitation, Scott & White Clinic, 2401 South 31st St., Temple, TX 76508. ABSTRACT 0894-9115/07/8612-01013/0 Schuchmann JA, Browne BA: Persistent hiccups during rehabilitation hospital- American Journal of Physical ization: three case reports and review of the literature. Am J Phys Med Rehabil Medicine & Rehabilitation 2007;86:1013–1018. Copyright © 2007 by Lippincott Williams & Wilkins Persistent hiccups have been reported to occur occasionally during rehabilitation hospitalizations. Hiccups can interfere with patient participation and progress, and DOI: 10.1097/PHM.0b013e3181152152 this can lengthen and complicate the rehabilitation hospitalization. Chlorproma- zine, the only Food and Drug Administration–approved agent specifically indicated for hiccups, is often chosen as a first-line treatment. However, chlorpromazine does not always provide favorable results. The intent of this paper is to (1) review hiccups and implications of persistent hiccups in the rehabilitation setting, (2) provide additional evidence that chlorpromazine is often not the best treatment choice for hiccups in rehabilitative patients, (3) report the effectiveness and favorable tolerability of modest doses of gabapentin in a small case series of three patients, and (4) review potentially effective treatment approaches for hiccups. Key Words: Hiccups, Hiccoughs, Singultus, Gabapentin, Stroke, Postoperative

OVERVIEW OF HICCUPS Hiccups1,2 (also hiccoughs or singultus) involve sudden, abrupt inspiration resulting from sudden, involuntary contraction of the diaphragm. Closure of the glottis then halts the incoming air. The column of air strikes the closed glottis to produce the characteristic sound. Hiccups most often involve unilateral contraction of the diaphragm (the left more frequently than the right). The reflex arc for hiccups includes an afferent limb (including the vagus and phrenic nerves and the sympathetic chain), a central mediator, and an efferent limb (including the phrenic nerve, plus efferents to the glottis and inspiratory intercostal muscles). The reflex pathways are similar to those that produce coughing, sneezing, swallowing, and vomiting. Hiccups do not seem to have any physiologic purpose or function after birth, but speculation exists that intrauterine hiccups permit training of the diaphragm without aspiration of amniotic fluid. Most cases of hiccups are of short duration, lasting less than 48 hrs. No gender difference seems to exist in the occurrence of transient hiccups. Brief bouts of hiccups do not require medical intervention.

December 2007 Persistent Hiccups During Rehabilitation 1013 Transient hiccups are benign in nature and ited participation in rehabilitative therapies. Drug occur nearly universally in all individuals from discontinuation was required. Hiccup resolution time to time. Such events are often spontaneous was ultimately achieved with baclofen in one case, but can be related to gastric overdistention (such haloperidol in the second, and carbamazepine in as overeating, drinking carbonated beverages, the third. aerophagia, and gastric insufflation during endos- The above reports highlight that hiccups can copy), sudden changes in ambient or gastrointes- occur in individuals with stroke usually affecting tinal temperature, alcohol use, tobacco use, sudden the brainstem. Complications can occur and can excitement, or emotional distress. protract the rehabilitation length of stay. Chlor- Intractable hiccups occur more frequently in promazine was poorly tolerated, and various other men, and about 4000 hospitalizations per year oc- agents (baclofen, haloperidol, and carbamazepine) cur in the United States for hiccups. The longer the provided resolution of the hiccups. duration of hiccups, the more likely an organic cause exists. Hiccups occurring at night also have CASE REPORTS a greater chance of having an organic vs. a psycho- Three cases are reported in which persistent genic cause. Numerous potential causes of medi- hiccups interfered with rehabilitation program par- cally significant hiccups have been described,1,2 ticipation and in which prompt hiccup resolution most commonly including central nervous system was achieved with the use of gabapentin. disorders, lesions of the vagus and/or phrenic nerve, toxic–metabolic disorders, adverse events Case 1 associated with pharmacological agents, and psy- A 60-yr-old male was admitted to a medical chological disorders. Hiccups are also not uncom- unit after the acute onset of ataxia, dizziness, and mon in the postoperative state. decreased coordination in the right upper extrem- The longer hiccups persist, the more difficult ity. Hiccups at a rate of 10–15/min began 15–20 treatment becomes. Persistent hiccups can prolong mins after onset of the neurological symptoms. A hospitalizations by contributing to debilitation magnetic resonance imaging scan showed the pres- with diminished nutritional and fluid intake, aspi- ence of a lateral medullary infarct. The patient was ration, insomnia, mental stress, and even wound admitted to rehabilitation 9 days after onset, still dehiscence. with hiccups. His hiccups caused persistent nausea, associated with emesis, with resultant poor REVIEW OF THE REHABILITATION fluid and nutritional intake. Blood urea nitrogen LITERATURE and creatinine had increased from 16 and 0.9 mg/dl Two published articles concerning hiccups at time of acute care admission to 47 and 1.5 mg/dl, were identified in the rehabilitation literature. respectively, during the sixth day of his stay on the Nickerson et al.3 report that hiccups developed in a rehabilitation unit. Baclofen was initially used 69-yr-old male with lateral medullary syndrome while on the medical unit at a dose of 5 mg orally during his acute care stay. He was given chlor- three times a day, then increased to 10 mg orally promazine, promethazine, and prochlorperazine, three times a day on the rehabilitation unit, with- without success. On admission to rehabilitation, out success. Chlorpromazine at a dosage of 25 mg his main complaints were of hiccups at a rate of orally three times a day was substituted for the 25/min, associated with nausea. At the rehabilita- baclofen. The patient’s hiccups improved, but he tion facility, baclofen (5 mg orally, three times a developed worsening dizziness and orthostatic hy- day) was initiated, and the hiccups resolved within potension, precluding participation in rehabilita- 48 hrs. tion therapies. Gabapentin (100 mg orally, four Kumar and Dromerick4 describe three patients times a day) was then substituted for the chlor- with strokes in the pontine region who developed promazine, with prompt resolution of hiccups, diz- intractable hiccups while participating in rehabili- ziness, nausea, and emesis, and normalization of tation programs. Complications seen in these pa- laboratory studies; blood urea nitrogen and creat- tients included dysphagia requiring enteral feed- inine values had decreased to 15 and 1.0 mg/dl, ing, aspiration pneumonia requiring intravenous respectively, at a follow-up visit 3 wks later. antibiotics, weight loss, respiratory depression, and esophagitis. The authors report that the hiccups Comments on Case 1 contributed to extending the rehabilitation lengths Hiccups previously have been described as as- of stay to 27 days, compared with an average of 16.6 sociated with lateral medullary infarction (also days for their other stroke patients. The authors known as posterior inferior cerebellar syndrome, chose chlorpromazine as their first-line therapy; or Wallenberg syndrome). Park et al.5 present a chlorpromazine provided benefit in two of the series of 51 patients with lateral medullary infarc- three patients, but it led to drowsiness, which lim- tion; magnetic resonance imaging scanning within

1014 Schuchmann and Browne Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 3 days of onset confirmed the diagnosis. Seven of cervical collar was used to stabilize her cervical these 51 patients developed hiccups in addition to spine because of the presence of the hematoma and the more typical symptoms of vertigo, dizziness, because she complained of dizziness with just min- nausea, vomiting, and dysphagia. imal neck movements. She slowly, steadily im- Pelin et al.6 describe four patients with lateral proved to the point where she was able to become medullary syndrome who also presented with hic- independent with ambulation and self-care activi- cups. These patients were treated with varied doses ties. She was discharged home to live alone after a and combinations of chlorpromazine, valproic acid, 22-day rehabilitation unit stay. Follow-up com- and baclofen. Full resolution of hiccups was ob- puted tomographic scan of the cervical spine before served in all patients. discharge demonstrated nearly complete resolu- Moretti et al.7 describe the use of gabapentin tion of the hematoma. in 15 patients (nine men and six women; mean age, Interestingly, at the time of admission to re- 69.1 yrs) who experienced hiccups caused by lateral habilitation, the patient indicated that she was ex- medullary infarcts. The authors report success in periencing persistent hiccups occurring at a fre- all of the patients with use of gabapentin, as was quency of two to three per minute; she reported the case with our patient. Two years after a stroke, that the hiccups had been present for 3 days and one patient had a recurrence of hiccups that again had begun about 4 days after the worsening of her responded to gabapentin. Follow-up neurologic ex- right hemiparesis. She stated that the hiccups were amination in this patient remained stable, and re- annoying to her and that they interfered with eat- peat magnetic resonance imaging demonstrated ing and drinking activities. She was prescribed the previous stroke, but no acute findings were chlorpromazine (25 mg orally, three times a day), detected. Moretti described the following gabapen- and the hiccups greatly improved; however, she tin protocol: 400 mg three times a day for 3 days, complained of dizziness, and she developed ortho- then 400 mg daily for 3 days, and then discontin- static hypotension, which precluded participation uation of gabapentin. No significant adverse effects in her rehabilitation program. The chlorpromazine were reported from the gabapentin. was discontinued, and gabapentin (100 mg orally, four times a day) was initiated, with total resolu- Case 2 tion of the hiccups and dizziness. The gabapentin A 72-yr-old female was initially seen with com- was tapered and discontinued without any recur- plaints of headache and clumsiness in the right rence of hiccups. lower extremity. She was brought to the emergency department. Computed tomographic scan of Comments on Case 2 the head was negative. Concerns were expressed Hemorrhage within the spinal canal is a rare but about the upper cervical spine (possible calcifica- serious complication of anticoagulation therapy, de- tion vs. cavernous angioma vs. small area of hem- scribed most often in individuals in the sixth or sev- orrhage). She was scheduled for a follow-up clinic enth decade of life. Most cases reported in the litera- appointment. ture seem to be spontaneous, but others are described Four days later, she developed increased weak- as secondary to trauma (epidural catheters, spinal ness in the right upper and lower extremities, and manipulation, snowboarding, nose blowing), small- she fell in her bathroom. She was brought to the cell lung cancer, metastatic hepatocellular cancer, or emergency department and was admitted. Coagu- coagulopathies. Patients described in many of the lation studies showed an international normalized reported cases had been anticoagulated within the ratio of 3.5. therapeutic range. The diagnosis should be consid- Her past history was significant for hyperten- ered if a patient taking anticoagulant medication sion, breast cancer, paroxysmal atrial fibrillation, complains of local or referred spinal pain and has and remote deep venous thrombosis with pulmo- associated limb weakness, sensory deficits, or urinary nary embolism 20 yrs previously (after bilateral retention. Anticoagulation should be reversed mastectomy for breast cancer). She had been on promptly. Emergency laminectomy and decompres- chronic anticoagulation with warfarin, and a sion of the spinal cord are often recommended Greenfield filter had been placed previously. (Pullarkat et al.8 describe these measures as “manda- Computed tomography of the cervical spine tory”), but cases in which the patient is already im- soon after admission demonstrated a hematoma proving rapidly and steadily and is under close obser- localized to the right side of the cervical spinal cord vation could be treated nonoperatively. (C2 and above), extending to the pontomedullary Hemorrhage adjacent to the spinal canal is junction. Anticoagulation was reversed with fresh- sufficiently rare; only limited numbers of cases frozen plasma. After normalization of her interna- have been described in the literature. This is the tional normalized ratio, she was admitted to reha- first case reported in which hiccups were present in bilitation, with persistent right hemiparesis. A association with such a hemorrhage. The location

December 2007 Persistent Hiccups During Rehabilitation 1015 of the hemorrhage—from the pontomedullary TABLE 1 Folk remedies and other junction descending to C2—is similar in location nonpharmacological treatments of to the lesions seen with lateral medullary stroke, hiccups implicating similar neurological factors in producing the hiccups. Breathing into a bag Drinking from opposite side of glass Case 3 Swallowing granulated sugar A 65-yr-old type 2 diabetic male was admitted Drinking or gargling ice water Forcible traction on tongue to rehabilitation 6 days after bilateral total-knee Biting on a lemon arthroplasties. After surgery, he had redness and Eating a spoonful of peanut butter swelling around his left leg that were thought to be Compression of the eyeballs attributable to cellulitis. He had been maintained Carotid massage on deep venous thrombosis prophylaxis. Doppler ul- Rectal massage Catheter or swab stimulation of the trasound studies for deep venous thrombosis were naso-oropharynx negative. The cellulitis was treated with cephalexin, Valsalva maneuver with improvement in the redness, swelling, and pain. Breath holding His overall rehabilitation course was slow because of Fright pain and decreased active knee motion. Hydrocodone Noxious odors (inhaling ammonia) Gastric lavage was used for pain control. He also had hypoalbumin- Modified Heimlich maneuver: three emia (2.6 g/dl) and required psychosocial encourage- moderate-pressure thrusts at 10-sec ment and supplements to boost his nutrition. intervals The patient developed hiccups (3–5/min), which Sexual intercourse persisted during his last day on the surgical service and for his first day on rehabilitation; the hiccups aggravated his postsurgical pain. Gabapentin (100 mg orally, three times a day) was initiated, with prompt A careful history, thorough physical examina- resolution of the hiccups. After 8 days on the reha- tion, and studies, as appropriate, should be per- bilitation unit, he had improved to the point where he formed to help identify conditions or disorders that was ready for hospital discharge with outpatient ther- might be producing or perpetuating the hiccups. apy. Hiccups did not recur. The list of potential causes of hiccups is long, including central and peripheral neurological le- Comments on Case 3 sions, head and neck lesions, cardiothoracic and Souadjian and Cain1 analyzed 220 patients abdominal disorders, infectious diseases, toxic/ with intractable hiccups seen at the Mayo Clinic. metabolic causes, postoperative status, and psycho- Risks produced by intractable hiccups were re- genic causes. ported as severe debilitation, dehydration, insom- For persistent hiccups, the patient should be nia, protracted mental stress, wound dehiscence, questioned about any medication that might be and, possibly, even death. Eighty-two percent of producing or aggravating the hiccups. Medication the patients were male. Forty patients (18% of the cessation or a drug “holiday” should be considered total group) were reported to have postsurgical (if possible) if a potentially offending medication is hiccups. Hiccups usually appeared 1–4 days post- identified. A number of different medications have operatively and were generally severe and inter- been implicated in producing or aggravating hic- fered with sleep. Initial abortive measures failed in cups (see Table 2). 65% of cases, and pharmacological measures were Review of treatment options should take into usually required to resolve the hiccups. consideration the potential precipitating or aggra- In our patient, an elderly diabetic male, gaba- vating causes of the hiccups. For example, if hic- pentin was chosen as the initial treatment for his cups are related to gastroesophageal reflux disease, hiccups. He had a prompt, complete resolution of a proton pump inhibitor might be quite effective in hiccups on low-dose gabapentin, and he tolerated relieving symptoms. this medication without adverse effects. Often, no obvious cause is identified to account for the hiccups. In these cases, pharmacologic treat- REVIEW OF TREATMENT APPROACHES ment is often needed. Initially, monotherapy is rec- FOR PERSISTENT HICCUPS ommended, but occasionally a combination of agents A variety of folk remedies and physical maneu- is required to treat persistent hiccups. vers have been described as helpful in aborting Chlorpromazine is the only medication specif- hiccups (see Table 1). These measures seem to be ically approved by the Food and Drug Administra- most effective in transient hiccups and are less tion for the treatment of hiccups. Use for hiccups effective with persistent hiccups. was first described by Friedgood and Ripstein9 in

1016 Schuchmann and Browne Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 combinations of these medications are necessary. TABLE 2 Medications implicated as possibly causing or aggravating persistent Other treatment approaches for resistant cases in- hiccups clude hypnosis, acupuncture, cervical epidural block, phrenic nerve block, crushing or pacing, and Alpha methyldopa general anesthesia with positive pressure ventila- Short-acting barbiturates tion and muscle relaxants. Corticosteroids, including dexamethasone and Gabapentin was found to be well tolerated and intravenous methylprednisolone Benzodiazepines, including diazepam and symptomatically useful for our three patients. chlordiazepoxide Gabapentin is a Food and Drug Administration– Chemotherapeutic agents, including cisplatinum approved anticonvulsant that is also frequently Opioids, including hydrocodone used for neuropathic pain, including diabetic neu- Nonsteroidal antiinflammatory drugs Antidopaminergic agents, including perphenazine ropathic pain and postherpetic neuralgic pain. The Antibiotics, including beta-lactams, macrolides, specific anticonvulsant mechanism of action is un- and fluoroquinolones clear; the agent is structurally related to gamma- Cardiovascular agents, including digoxin aminobutyric acid (GABA), but it does not interact Progesterone Anabolic steroids with GABA receptors, it is not metabolically con- verted into GABA or a GABA agonist, and it is not an inhibitor of GABA uptake or degradation. Som- nolence, dizziness, ataxia, and fatigue are the most 1955. The authors reported a series of 50 patients frequent adverse effects. 10 (46 men and 4 women, 26–80 yrs of age) with Hernandez et al. report a case of a 71-yr-old intractable hiccups treated with intravenous, male with Guillain–Barre syndrome and metastatic 50-mg injections of chlorpromazine, with the dose gastric neoplasm who presented with intractable repeated once, 2–4 hrs later. Successful hiccup hiccups. The neoplasm had infiltrated the anterior resolution was reported in 41 of the 50 cases. Five pillar of the diaphragm at the time of surgery. patients had hiccups recurrence, whereas four pa- Omeprazole (20 mg daily) was initiated along with tients showed no response to the medication. Re- chlorpromazine (25 mg, three times a day). Hic- ported adverse effects included a “depressive and cups, as well as emesis, persisted. Chlorpromazine sedative reaction,” and dermatitis in one case. The was discontinued and baclofen was added, initially authors also reported that older, more fragile pa- 5 mg three times a day, and progressively increas- tients had a more profound response to the medi- ing to 15 mg three times a day. The patient still cation. The dose was, therefore, decreased in el- failed to improve. Gabapentin was then added at a derly patients. Chlorpromazine, if chosen for dosage of 300 mg three times a day. Within 24 hrs, intravenous use, must be infused slowly for several the hiccups ceased. One week later, hiccups re- hours to prevent the development of hypotension. curred and resolved within 48 hrs after resumption Oral chlorpromazine (25–50 mg, three to four of gabapentin at a dosage of 400 mg daily. times a day, for 7–10 days) also has been shown to 11 3 Porzio et al. describe gabapentin as being be effective. More recent reports indicate that for useful in treating hiccups in patients with cancer. elderly and potentially neurologically impaired A 63-yr-old male with metastatic liver cancer de- rehabilitation patients, chlorpromazine is best veloped chronic hiccups, nausea, and fatigue. Met- avoided because of the problematic adverse effects oclopramide and dexamethasone prescribed by his of dizziness with orthostatic hypotension and primary care physician were ineffective. Chlor- drowsiness, which can preclude participation in promazine (25 mg intravenously, twice daily) eased active rehabilitation programs. Two of the patients the hiccups but caused postural hypotension and in this study experienced adverse responses to chlorpromazine, providing further evidence that severe drowsiness. Chlorpromazine was discontin- chlorpromazine is often not the most appropriate ued, and gabapentin (300 mg, three times daily) treatment choice for hiccups in rehabilitative pa- was initiated. The gabapentin was well tolerated tients. and provided effective relief from the hiccups. Two Numerous other medications have been re- other cases—a 43-yr-old male with pancreatic can- ported as effective for treating hiccups. Omepra- cer, and a 51-yr-old male with metastatic small-cell zole has been found helpful in patients in whom lung cancer—were reported to obtain relief of per- the hiccups seem to be related to the presence of sistent hiccups with 300–400 mg of gabapentin gastroesophageal reflux disease. Baclofen and anti- three times a day. The authors caution about the convulsants (phenytoin, valproic acid, carbamaz- use of baclofen in elderly patients because of the epine, and gabapentin) also can be effective in some frequent adverse effects of sedation, insomnia, diz- cases. Occasionally, in resistant cases of hiccups, ziness, weakness, ataxia, and confusion.

December 2007 Persistent Hiccups During Rehabilitation 1017 SUMMARY 2. Cymet TC: Retrospective analysis of hiccups in patients at a community hospital from 1995–2000. J Natl Med Assoc Most cases of hiccups are transient and benign. 2002;94:480–3 Home treatments are initially reasonable and are of- 3. Nickerson RB, Atchison JW, Van Hoose JD, Hayes D: Hic- ten effective. The longer hiccups persist, the more cups associated with lateral medullary syndrome. Am J Phys resistant to treatment they become. Intractable hic- Med Rehabil 1997;76:144–6 cups can be associated with a number of pathologic 4. Kumar A, Dromerick AW: Intractable hiccups during stroke conditions. A detailed history and physical examina- rehabilitation. Arch Phys Med Rehabil 1998;79:697–9 tion with appropriate testing should be performed on 5. Park MH, Kim BJ, Koh SB, Park MK, Park KW, Lee DH: patients who are experiencing intractable hiccups. Lesional location of lateral medullary infarction presenting Chlorpromazine, despite Food and Drug Admin- hiccups (singultus). J Neurol Neurosurg Psychiatry 2005; 76:95–8 istration approval for hiccups, is often poorly tolerated and is best avoided for older rehabilitative pa- 6. Pelin Z, Bozluolcay M, Erkol G, Bugay G, Denktas H: Hiccups as a sign of lateral medullary syndrome: reports of tients, especially those with debility, acute stroke, or four cases. Cerrahpasa J Med 2001;32:115–9 neurological events. Baclofen can be helpful in pro- 7. Moretti R, Torre P, Antonello RM, Ukmar M, Cazzato G, moting hiccup resolution, but it should be used cau- Bava A: Gabapentin as a drug therapy of intractable hiccup tiously in elderly patients because of the potential because of vascular lesion: a three-year follow up. Neurol- adverse effects of sedation, insomnia, dizziness, weak- ogist 2004;10:102–6 ness, ataxia, and confusion. 8. Pullarkat VA, Kalapura T, Pincus M, Baskharoun R: In- Gabapentin at modest doses, in this case series traspinal hemorrhage complicating oral anticoagulant therapy: an unusual case of cervical hematomyelia and a and others, has been found to provide prompt relief review of the literature. Arch Intern Med 2000;160:237–40 of hiccups, and it is usually well tolerated. A short 9. Friedgood CE, Ripstein CB: Chlorpromazine (thorazine) in course often can provide long-term relief. Mea- the treatment of intractable hiccups. J Am Med Assoc 1955; sures combining pharmacotherapy with more in- 157:309–10 vasive treatment are occasionally indicated for 10. Hernandez JL, Pajaron M, Garcia-Regata O, Jimanez V, more resistant cases of hiccups. Gonzalez-Macias J, Ramos-Estebanez C: Gabapentin for intractable hiccup. Am J Med 2004;117:279–81 REFERENCES 11. Porzio G, Aielli F, Narducci F, et al: Hiccup in patients with 1. Souadjian JV, Cain JC: Intractable hiccup. Etiologic factors advanced cancer successfully treated with gabapentin: re- in 220 cases. Postgrad Med 1968;43:72–7 port of three cases. N Z Med J 2003;116:U605

1018 Schuchmann and Browne Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 Authors: Xiulu Ruan, MD John Patrick Couch, MD Hiccup Rinoo Shah, MD Frank Wang, MD Hai Nan Liu, MS

Afﬁliations: CASE REPORT From Physicians’ Pain Specialists of Alabama, Mobile, Alabama (XR, JPC); Guthrie Clinic, Sayre, Pennsylvania (RS); School of Medicine, University of South Alabama, Mobile, Alabama Persistent Hiccup Associated with (FW); and Department of Urology, Qilu Hospital, Shandong University, Intrathecal Morphine Infusion Pump Jinan, China (HNL). Therapy Correspondence: All correspondence and requests for reprints should be addressed to Xiulu ABSTRACT Ruan, MD, Physicians’ Pain Specialists of Alabama, 2001 Ruan X, Couch JP, Shah R, Wang F, Liu HN: Persistent hiccup associated with Springhill Ave., Mobile, AL 36607. intrathecal morphine infusion pump therapy. Am J Phys Med Rehabil 2007;86: 0894-9115/07/8612-01019/0 1019–1022. American Journal of Physical Intraspinal drug-delivery therapy has been increasingly used in patients with Medicine & Rehabilitation intractable nonmalignant pain syndromes during the past two decades. Morphine, Copyright © 2007 by Lippincott Williams & Wilkins the only FDA-approved opioid for intrathecal administration, has been the principle agent for such therapy. Although intrathecal morphine infusion can produce DOI: 10.1097/PHM.0b013e31815206c8 profound spinal analgesia, it may also cause some untoward side effects. We describe the first case of persistent hiccup caused by intrathecal morphine infusion therapy. Key Words: Hiccup, Intrathecal Opioid Infusion Pump, Intrathecal Morphine, Intrathe- cal Hydromorphone

Hiccup (singultu, hiccough) is a common phenomenon that involves sudden inspiration, immediately followed by active closure of glottis.1 It is an involuntary, reflex-like activity that begins with contraction of the diaphragm, quickly terminated by the abrupt closure of the glottis. Hiccups are experienced at one time or another by almost everyone and, when transient, are usually harmless. Despite a common occurrence, the cause, mechanism, and significance of hiccups are still incompletely understood.2 Although the causes are often considered idiopathic, more than 100 known organic causes have been identified.3 The most common causes are gastrointestinal, such as gastric distention or gastroesophageal reflux disease.4 Metabolic derangements, as well as drugs, are also frequently implicated as probable causes.1,4 We report the first case of intractable hiccups presumably caused by intrathecal morphine infusion.

CASE REPORT A 65-yr-old female, referred to our multidisciplinary pain center, with a 5-yr history of severe low-back pain and left-sided leg pain from degenerative lumbar disc disease, lumbar spinal stenosis, and lumbar radiculitis. The patient also had a history of lumbar laminectomy with decompressive fusion at L4–L5 and L5–S1, about 2 yrs ago after she had undergone multimodality treatment, including medication trials, physical therapy, and spinal interventional treat-

December 2007 Persistent Hiccup and Intrathecal Morphine 1019 ment, including epidural steroid injections, lum- did not experience any hiccup during this epidural bosacral transforaminal steroid injections, lumbar morphine-infusion trial. She subsequently con- facet blocks, and lumbar facet radiofrequency rhi- sented and was scheduled for permanent intrathe- zotomy, with only short-term efficacy. The patient cal morphine pump implantation. The intrathecal described her low-back pain as being about as both- catheter was inserted at the left paramedian L2–L3, ersome as her left-leg pain. Her pain level was with the catheter tip located at T12, as confirmed usually at 8–9/10 on a numeric pain scale of 0–10. under fluoroscopy. A nonprogrammable Codman Her low-back pain generally worsened by sitting 3000 continuous-rate infusion pump was placed in and standing, whereas her left-leg pain, which she the right-middle quadrant below the right rib cage described as starting from the posterior thigh, ex- and above the right iliac crest. The intrathecal tending down to the posterior calf, and then to the infusion consisted of 1.0 mg/day of preservative- bottom of her left foot, significantly worsened by free morphine. walking. The only relieving factor was frequently The patient started experiencing hiccups about changing positions. She had no significant past 12 hrs after the intrathecal pump implantation. medical history or surgical history, except for lum- She did experience satisfactory analgesia of her bosacral decompressive fusion about 2 yrs before usual back and leg pain, and there was no other presenting to our clinic. Her family history and intolerable side effect, except for the hiccups. These review of systems were noncontributory. Her med- occurred at a frequency of four to six per minute. ications included extended-release morphine (30 The patient was instructed to try different maneu- mg, thrice daily), oxycodone/acetaminophen (10/ vers, including holding her breath, drinking water, 325, thrice daily, as needed), gabapentin (800 mg, etc., without efficacy. The patient then tried chlor- thrice daily), tizanidine (4 mg, twice daily), and promazine as needed, which did help her slightly. meloxicam (7.5 mg, twice daily). She had been on She was able to sleep at night, and her hiccups these drugs for more than 6 mos. Her lumbar MRI became less forceful. She also noted that the inter- with and without contrast revealed broad-based val between episodes of hiccups was increased. Yet, bulging at L3–L4, with degenerative changes of the she still experienced episodic hiccups while taking facet joints and bilateral neuroforaminal narrow- chlorpromazine. Six days after the initial implan- ing, more so on the left side. Postsurgical changes tation, the decision was made to change her intra- were at L4–L5 and L5–S1 with bilateral laminec- thecal infusion from morphine to hydromorphone tomy defects and compression plates with interpe- at 0.3 mg/day. Her hiccups resolved completely 16 dicular screws at L4, L5, and S1. hrs after intrathecal morphine had been changed Because she had failed all other conventional to hydromorphone. She has not experienced recur- treatment, we considered her for intraspinal drug- rence of her hiccups since switching to hydromor- delivery therapy. After a psychological evaluation phone. She has been on intrathecal hydromorphone confirmed her candidacy, she underwent an outpa- for almost 3 yrs now. Her current intrathecal hydro- tient controlled continuous epidural morphine morphone-infusion rate is 2.5 mg/day. trial. A tunneled lumbar epidural catheter was placed at L3–L4, with the catheter tip advanced to DISCUSSION L1 under fluoroscopic guidance. Satisfactory cath- The discovery of highly specific opioid recep- eter placement was confirmed by epiduragram. The tors in the central nervous system, especially the proximal tip of the catheter was then tunneled spinal cord in 1970s, made it possible for spinal subcutaneously and was connected to a Microject application of opioids to obtain spinally mediated PCEA pump (Codman, Raynham, MA) and a reser- antinociception. Intraspinal drug-delivery therapy voir bag containing 0.4 mg/ml of preservative-free has been used increasingly since the 1980s in pa- morphine. The pump was programmed to deliver a tients with intractable nonmalignant pain who basal rate of 0.5 ml/hr. The bolus dose was 0.2 ml, have failed to respond to conventional treatment or with a 60-min lockout interval. The patient was who could not tolerate systemic opioid therapy be- then instructed how to use the pump and was cause of side effects.5–9 By infusing small amounts of discharged home. During the trial, the infusion opioid directly into cerebrospinal fluid in close rate was further increased to 1 ml/hr, with her proximity to the receptor sites in the spinal cord, bolus dose increased to 0.4 ml. The infusion trial one can achieve spinally mediated analgesia, spar- lasted 7 days and was beneficial in controlling her ing some of the side effects caused by systemic pain. The patient reported more than 80% pain opioids. In our own practice, most of our patients reduction, with improved distance for ambulation. who ended up requiring intrathecal opioid infusion She did experience initial, transient itching, which pumps for better pain control were those with completely resolved. She did not feel the need to intractable low-back pain and/or leg pain from use any extra boluses while on epidural morphine failed back surgery syndrome, with/or without ad- infusion at 0.4 mg/hr (0.4 mg/ml at 1 ml/hr). She hesive arachnoiditis. Morphine, the only FDA-ap-

1020 Ruan et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 proved opioid for intrathecal administration, has afferent or efferent limb of the reflex arc triggers been found to be associated with numerous side hiccups. effects.10,11 The most common ones are pruritus, Multiple causes have been attributed to induce nausea and vomiting, urinary retention, water re- hiccups. The most commonly recognized ones are tention, constipation, and sexual dysfunction.10–12 gastrointestinal, such as gastric distention or gas- Less common ones include mental status change, troesophageal reflex disease. Multiple pharmaco- central nervous system excitation, hyperalgesia, logical agents,3,19 including barbiturates, benzodi- neurotoxicity, and respiratory depression.10,11 To azepines, methyldopa, and steroids, have been the best of our knowledge, hiccups induced by reported to cause hiccups. More than 100 known intrathecal morphine infusion have never been re- organic causes of hiccups have been identified.21,22 ported. No record of any publications in English on Local anesthetics given epidurally have been hiccups associated with intrathecal opioid use implicated as a cause of hiccups. McAllister et al.2 could be found after Medline search using hic- report one case of recurrent persistent hiccups cup(s) and intrathecal opioid(s). Hydromorphone after epidural steroid and bupivacaine injections in is considered a safe, alternative opioid for long- a 65-yr-old man with lumbar spinal stenosis. Epi- term intrathecal use when morphine fails because dural bupivacaine, rather than the steroid, was felt of pharmacological side effects or inadequate pain to be the culprit for causing hiccups, because hic- relief.13,14 Systemic opioids have been implicated cups occurred on three separate occasions after in causing hiccups, although there is a paucity of lumbar epidural injections with bupivacaine and data in the English literature. Wilcox15 has re- triamcinolone, whereas hiccups did not occur after ported one case of a 63-yr-old man with severe a fourth lumbar epidural injection with the same neck pain from squamous cell carcinoma of right amount of triamcinolone, with normal saline in- tonsil, experiencing persistent hiccups after taking stead of bupivacaine. Interestingly, to the contrary, slow-released oral morphine. Hiccups resolved intravenous and nebulizer lidocaine have been only after cessation of oral morphine, with no re- used as a therapeutic modality for persistent or current hiccups after substitution with a fentanyl intractable hiccups.23,24 Lower thoracic or high patch and oxycodone. Throughout the period when lumbar epidural local anesthetics may cause sympa- he was experiencing hiccups, the patient had no thetic blockade, resulting in contracted bowel attrib- other apparent side effects that would suggest an utable to parasympathetic dominance.25 However, opioid overdose. Lauterbach16 has reported a case this seems to be in contrast to the gastric distention of a 55-yr-old man who experienced persistent hic- that is thought to be frequently implicated in causing cups for 4 days after receiving hydrocodone (5 mg, hiccups.4 Diaphragmatic function can also be affected every 4 hrs) after dental procedures, resolving after by epidural blockade. Increased electrical diaphrag- hydrocodone cessation. Bagheri et al.17 have shown matic activity was noted with thoracic epidural anes- that opioids accounted for 6% of the 53 cases of thesia for upper abdominal or thoracic surgery.26,27 drug-induced hiccups in France between 1985 and The most likely explanation for thoracic epidural an- 1997. However, because of the small sample size, esthesia-related increases in diaphragmatic activity 6% of 53 cases would correlate to a total of three seems to be the disruption of the inhibitory reflex of cases in 12 yrs in France. phrenic nerve motor drive, caused by either direct Transient hiccups are common and usually deafferentation of visceral sensory pathways or by harmless. Hiccups lasting more than 48 hrs are diaphragmatic load reduction as a result of increased considered persistent; those lasting more than 2 abdominal compliance.25 mos are considered intractable.18 Persistent or in- Slipman et al.28 report a case of persistent tractable hiccups can lead to fatigue, sleep distur- hiccups associated with transforaminal thoracic bance, dehydration, and wound dehiscence in the epidural steroid injection with 3 ml of betametha- postoperative period.4 sone and 1 ml of 1% lidocaine in separate occasions The exact mechanisms of hiccups are still not in a 31-yr-old man with thoracic discogenic pain. completely understood. The hiccups lasted 15 and 18 hrs, respectively, and It is believed that the hiccup reflex consists of an were unresponsive to conventional maneuvers, in- afferent and efferent limb. The afferent limb com- cluding drinking water, holding breath, and initial prises the phrenic and vagal nerves as well as the chlorpromazine (10 mg). The hiccups were finally sympathetic chain arising from thoracic segments aborted after four doses of metoclopramide (10 mg, T6–T12.3,19 The central connection is thought to every 6 hrs). involve interactions among the brainstem; the mid- The authors attribute the hiccups to the epi- brain, including the respiratory center, phrenic nerve dural steroid, rather than lidocaine, because they nuclei, medullary reticular formation, and hypothal- believe that the injectate flow was confirmed in the amus; and unidentified areas in the cervical spinal epidural space, dorsal to the vertebral body, and cord between C3 and C5.20 Stimulation of either the that it did not, therefore, reach the sympathetic

December 2007 Persistent Hiccup and Intrathecal Morphine 1021 chain that lies along the anterolateral aspects of for treatment of chronic low back pain: report from the T6–T12 vertebral bodies. However, lower thoracic National Outcomes Registry for Low Back Pain. Pain Med 2004;:6–13 or high lumbar epidural local anesthetics may 25 8. Kumar K, Kelly M, Pirlot T: Continuous intrathecal mor- cause sympathetic blockade, simply because sym- phine treatment for chronic pain of nonmalignant etiology: pathetic blockade may still occur with epidural Long-term benefits and efficacy. Surg Neurol 2001;55: local anesthetic, but at more a proximal anatomic 79–88 location instead of at the sympathetic chain. 9. Anderson VC, Burchiel KJ: A prospective study of long-term intrathecal morphine in the management of chronic non- In our patient, hiccups occurred only after maligmant pain. Congress Neurol Surg 1999;44:289–300 intrathecal morphine infusion with no local anes- 10. Ruan XL: Drug-related side effects of long-term intrathecal thetic infusion. She had been on oral morphine morphine therapy. Pain Physician 2007;10:357–65 (extended-release morphine, 30 mg, thrice daily) 11. Chaney MA: Side effects of intrathecal ad epidural opioid. and oxycodone/acetaminophen (10 mg, thrice Can J Anaesth 1995;42:891–903 daily, as needed) for more than 6 mos without 12. Abs R, Verhelst J, Maeyaert J, et al: Endocrine consequences of long-term intrathecal administration of opioids. J Clin experiencing any hiccups. The 1-wk, epidural mor- Endocrinol Metab 2000;85:2215–22 phine-infusion trial went well, with good analgesia 13. Anderson VC, Cooke B, Burchiel KJ: Intrathecal hydromor- and without any hiccups or other intolerable side phone for chronic nonmalignant pain: a restrospective effects. The hiccups persisted for 6 days despite study. Pain Medicine 2001;2:287–97 treatment, only to resolve after the patient was 14. Hassenbusch SJ, Portenoy RK, Cousins M, et al: Polyanal- gesic Consensus Conference 2003: an update on the man- switched to an intrathecal infusion of hydromor- agement of pain by intraspinal drug delivery—report of an phone. It is not clear why intrathecal morphine expert panel. J Pain Symptom Manage 2004;27:540–63 induces hiccups, whereas intrathecal hydromor- 15. Wilcox SK: Persistent hiccups after slow-release morphine. phone does not. Indeed, it is puzzling because Palliat Med 2005;19:568–9 hydromorphone, a hydrogenated ketone analog, 16. Lauterbach EC: Hiccup and apparent myoclonus after shares similar properties to morphine, except for hydrocodone: review of the opiate-related hiccup and myoclonus literature. Clin Neuropharmacol 1999;22:87–92 being more lipophilic than morphine and about 29 17. Bagheri H Cismondo S, Montastruc JL: Drug-induced five to seven times more potent than morphine. hiccup: a review of France pharmacological vigilance data- Because of ethical concerns, we did not replace base [in French]. Therapie 1999;54:35–9 intrathecal morphine infusion with normal saline 18. Souadjian JV, Cain JC: Intractable hiccup: etiologic factors and rechallenge the patient later on with intrathe- in 220 cases. Postgrad Med 1968;43:72–7 cal morphine to further confirm causality of hic- 19. Kolodzik PW, Eilers MA: Hiccups (singultus): review and approach to management. Ann Emerg Med 1991;20:565–73 cups by intrathecal morphine infusion. 20. Bilotta F, Pietropaoli P, Rosa G: Nefopam for refractory In summary, we present a rare case of persis- hiccups in the palliative care population. Am J Hosp Palliat tent hiccups after intrathecal morphine pump Care 2003;20:149–54 placement. The exact mechanism of intrathecal 21. Harley C: Extradural corticosteroid infiltration: a follow-up morphine, not hydromorphone, inducing hiccups study of 50 cases. Ann Phys Med 1967;9:22–8 needs further investigation. It is important to rec- 22. Abram SE, O’Connor TC: Complications associated with epidural steroid injections. Reg Anesth 1996;21:149–62 ognize this possible adverse event so that proper 23. Cohen SP, Lubin E, Stojanovic M: Intravenous lidocaine in treatment can be initiated. the treatment of hiccup. South Med J 2001;94:1124–5 REFERENCES 24. Neeno TA, Rosenow EC 3rd: Intractable hiccups: consider nebulized lidocaine. Chest 1996;110:1129–30 1. Launois S, Bizec JC, Whitelaw WA, et al: Hiccup in adults: 25. Cousins MJ, Veering BT: Epidural Neural Blockade. Neural an overview. Eur Respir J 1993;6:563–75 Blockade in Clinical Anesthesia and Management of Pain. 2. McAllister RK, McDavid AJ, Meyer TA, Bittenbinder TM: Philadelphia, PA, Lippencott-Raven, 1998 Recurrent persistent hiccups after epidural steroid injection and analgesia with bupivacaine. Anesth Analg 2005;100: 26. Mankikian B, Cantineau JP, Bertrand M, et al: Improvement 1834–6 of diaphragmatic function by a thoracic extradural block after upper abdominal surgery. Anesthesiology 1988;68: 3. Rousseau P: Hiccups. South Med J 1995;88:175–81 379–86 4. Smith HS, Busracamwongs A: Management of hiccups in 27. Pansard JL, Mankikian B, Betrand M et al: Effects of tho- the palliative care populations. Am J Hosp Palliat Care racic extradural block on diaphragmatic electrical activity 2003;20:149–54 and contractility after the upper abdominal surgery. Anes- 5. Krames E: Intraspinal analgesia for nonmalignant pain, in thesiology 1993;78:63–71 Waldman SD: Interventional Pain Management, ed 2. Phil- 28. Slipman CW, Shin CH, Patel RK, et al: Persistent hiccup adelphia, PA, Saunders, 2001, pp 609–619 associated with thoracic epidural steroid injection. Am J 6. Raphael JH, Southall JL, Gnanadurai TV, Treharne GI, Kitas Phys Med Rehabil 2001;80:618–21 GD: Long-term experience with implanted intrathecal drug 29. Mahajan G, Fishman SM: Major opioids in pain manage- administration systems for failed back syndrome and me- ment, in Benzon HT, Raja SN, Molloy RE, et al. (eds): chanical low back pain. BMC Musculoskeletal Disord 2002; Essentials of Pain Medicine and Regional Anesthesia,ed2. 3:17 Philadelphia, PA, Elsevier Churchill Livingstone, 2005, pp 7. Deer T, Chapple I, Classen A, et al: Intrathecal drug delivery 94–112

1022 Ruan et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 Authors: Kristi L. Kirschner, MD Rebecca Brashler, LCSW Ethics Teresa A. Savage, PhD, RN

Afﬁliations: From the Donnelley Family Disability Ethics Program, Rehabilitation COMMENTARY Institute of Chicago, Chicago, Illinois (KLK, RB, TAS); Departments of Physical Medicine and Rehabilitation and Medical Humanities & Bioethics, Northwestern University Feinberg Ashley X School of Medicine, Chicago, Illinois (KLK, RB); and Department of Maternal Child Nursing, University of Illinois at Chicago, Chicago, Illinois ABSTRACT (TAS). Kirschner KL, Brashler R, Savage TA: Ashley X. Am J Phys Med Rehabil Correspondence: 2007;86:1023–1029. Kristi L. Kirschner, MD, The recent case of Ashley X has sparked much recent public debate and Rehabilitation Institute of Chicago, 345 E. Superior St., Room 1136, controversy, and raises critical questions for physiatrists and rehabilitation pro- Chicago, IL 60611. fessionals. The case came to light when Gunther and Diekema published an article in the October 2006 issue of Archives of Pediatric and Adolescent 0894-9115/07/8612-01023/0 Medicine describing a novel growth attenuation treatment for Ashley X, a 6-yr-old American Journal of Physical girl with developmental disabilities. Her parents also published a blog about Medicine & Rehabilitation Ashley, with detailed explanations for their rationale in choosing this treatment on Copyright © 2007 by Lippincott Williams & Wilkins behalf of Ashley which involved beliefs about her future and quality of life. Ashley’s parents refer to the series of interventions as the Ashley Treatment in DOI: 10.1097/PHM.0b013e31815b77b2 their blog, and this phrase has also been adopted by the popular press and others who have commented on the case. In this article we present an analysis of the Ashley X treatment and use a disability ethics approach to examine the perspectives of various stakeholders involved, including Ashley and other girls with extensive disabilities, parents, physicians, and bioethics committees. We conclude with critical questions for physiatrists and other disability specialists who are in a unique position to examine medical controversies involving people with disabilities. Key Words: Disability, Ethics, Evidence-Based Medicine, Pediatrics

Recently the parents of a young girl with disabilities sought controversial medical interventions to attenuate her growth.1 This case, described by Gunther and Diekema2 in an article published in the October 2006 issue of Archives of Pediatric and Adolescent Medicine, touches on many concerns central to the practice of rehabilitation medicine. The following commentary on the Ashley X treatment uses a disability ethics approach3 to examine the perspectives of various stakeholders involved. Such an approach opens up alternative ways to frame the issues, various treatment options, and considerations for the role of health care providers as moral agents facilitating difﬁcult decisions on behalf of children with signiﬁcant cognitive and developmental disabilities.

BACKGROUND According to her physicians, Ashley X is a disabled girl with a diagnosis of static encephalopathy, the etiology of which is unknown.2 She was born at full term after an unremarkable pregnancy and uncomplicated birth. She has two healthy siblings and lives with her parents, both of whom have college educa-

December 2007 Commentary 1023 tions.2 She is described as being dependent in all Simplistically stated, the medical model of disabil- activities of daily living, and completely immobile, ity characterizes disability as a defect intrinsic to hence the parents’ term “Pillow Angel”—that is, the individual.5 The primary goal in the medical she stays where they put her.1 She is nonverbal and model is to cure or normalize the person when receives all of her nutrition via a feeding tube. possible, to treat disease and relieve pain. In the Ashley X responds to others in her environment via social model, disability is seen as inherently neu- smiles and vocalizations. Her developmental stage tral, and a product of the interactions between the is described as that of a 3-mo-old infant, and her person and the environment.5 Neither model fully physicians have indicated that they do not expect explains all aspects of disability. More nuanced this to change. models see disability as a “complex interaction of When Ashley X was 6 yrs 7 mos old, and biological, psychological, cultural and sociopoliti- beginning to show signs of early puberty, her par- cal factors which cannot be extricated except with ents approached a pediatric endocrinologist for an imprecision.”6 (p38) In the analysis of the Ashley evaluation. In the 6 mos before the evaluation with Treatment that follows, we have a clear illustration the endocrinologist she had advanced from the of just how complex these interactions are and the 50th to the 75th percentile for length. The parents challenges they pose for healthcare providers. “particularly feared that continued growth eventu- ally would make it untenable for them to care for Ethical Issues for Stakeholders: Ashley their daughter at home, despite their strong desire and Other Young Girls with Cognitive todoso....After extensive consultation between and Physical Disabilities parents and physician, a plan was devised to atten- In December 2006, the United Nations General uate growth by using high-dose estrogen.”2 (1014) Assembly adopted the Convention on the Rights of To avoid complications from high-dose estrogen, Persons with Disabilities, stating that “every per- Ashley also underwent a hysterectomy and breast son with disabilities has a right to respect for his or bud removal. It is this combination of interven- her physical and mental integrity on an equal basis tions that the parents later refer to as the Ashley with others.”7 The American College of Obstetrics Treatment. and Gynecology (ACOG) came to a similar conclu- The parents’ blog provides additional informa- sion in their position paper on the “Sterilization of tion about their rationale and states that Ashley women, including those with mental disabilities.”8 requires all the care of a baby.1 The parents felt that They concluded that hysterectomy should not be they were approaching the limits of their ability to used as the primary and initial treatment for deal- lift Ashley at 65 lbs. Other than her parents, the ing with menstruation, or for nonvoluntary steril- grandmothers were her only care providers and ization for girls with disabilities. “In disabled they were beginning to have difficulty lifting Ash- women with limited functional capability, indica- ley. They felt that by accelerating the closure of her tions for major surgical procedures remain the growth plates it would be easier to care for her in same as in other patients. In all cases, indications the home and would improve her quality of life by for surgery must meet standard criteria, and the facilitating mobility as well as community par- benefits of the procedure must exceed known pro- ticipation. Ashley’s parents were also concerned cedural risks.”8 The concerns raised by caregivers about the potential for sexual abuse if she were for Ashley regarding menstrual cramps and hy- to develop secondary sex characteristics, discom- giene issues are only speculative. It is critical to fort from menstruation, large or fibrocystic breasts assess if there are less risky and reversible treat- (which were common in her family)and difficulty ments available that might be equally effective be- with wheelchair harness fitting. A number of other fore embracing riskier and permanent interven- secondary benefits and concerns were raised in- tions. cluding the prevention of skin breakdown, infec- ACOG further notes that using medical treat- tions, and ease in bathing. Quoting from the par- ments for social or quality-of-life reasons can be ents’ blog, they also state that “given Ashley’s problematic. Many of the concerns raised in sup- mental age a nine and a half year old body is more port of the Ashley Treatment—such as Ashley’s appropriate and more dignified than a fully grown future care, risk of sexual abuse, wheelchair fitting, female body.”1 and community participation—cannot be resolved simply by medical efforts to keep her small, by DISABILITY ETHICS performing an hysterectomy or mastectomy. On Disability ethics incorporates a disability stud- the other hand, physicians are often in a position to ies approach to traditional clinical medical ethics. make referrals to other specialists (e.g., indepen- Disability studies is an emerging interdisciplinary dent living centers, rehabilitation professionals, field that is grounded in the lived experience of etc.) when it is clear that the problems identified disability and in the social model of disability.4 extend beyond medical and biological factors to

1024 Kirschner et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 environmental and social concerns. The ACOG and tations for the future and ignores Ashley’s capacity UN perspectives can both be summarized as a right for social and emotional development. Develop- to bodily integrity for people with cognitive and mental skills and abilities of children with perva- developmental disabilities. Involuntary (against the sive needs, in the absence of known progressive person’s wishes) or nonvoluntary (for those such as neurological disorders, can change with time, nur- Ashley who cannot participate in the decision mak- turance and stimulation, just as they can change ing) medical interventions and surgical procedures for children without disabilities. in general should only be provided when there are clear health benefits. Ethical Issues for Stakeholders: Ashley’s Furthermore, people with cognitive and devel- Parents and Other Caregiving Family opmental disabilities have been harmed in the past Members by controversial and experimental medical treat- The lack of adequate social supports, including ments and need added protections. The history of reliable attendant care, quality respite services and medicine is replete with examples of harm to people affordable community-based living programs for peo- with disabilities by well-intentioned doctors, using ple with disabilities remains problematic. Parents popular but misguided and inadequately tested treat- would not need to seek risky medical interventions to ments such as prefrontal lobotomies.9,10 New treat- minimize difficulties with caregiving if they were ments for those with disabilities must undergo the offered reasonable alternatives. If Ashley’s parents same scrutiny and rigorous research to elucidate the were convinced that she would always receive loving actual benefits and risks as do new medical interven- and capable care, either in their home or in alterna- tions for the general population. Many people with tive community-based programs, regardless of her cognitive and developmental disabilities are unable to size and sexual maturation, their concerns could have provide informed consent and rely on others to make been mitigated. This appalling lack of support lies at decisions on their behalf. They are particularly vul- the center of this debate and cannot be ignored or nerable and therefore require additional safeguards. fixed with medical interventions such as surgery and Ashley and other children with cognitive and drugs. developmental disabilities have interests and needs It is also true that parental caregivers have apart from those of their parents. It is difficult to been harmed by judgmental health care profession- separate Ashley’s interests from those of her par- als who have incomplete appreciation of their lived ents—clearly her future well-being will largely be experience as caregivers. There has been a ten- determined by the ability of her family to continue dency in medicine to criticize and pathologize fam- to provide or arrange for a loving home and 24-hr ily members who seek solutions to caregiving chal- care. Children with developmental disabilities who lenges. Parents are often viewed as selfish if they are dependent upon their parents for all of their institutionalize their children, yet overprotective if daily care and who may never be able to commu- they keep them at home without outside assis- nicate their opinions and desires are often not seen tance. Parents are also often left with inadequate as having distinct interests. Yet, it is precisely be- resources and information when making decisions cause of such dependencies and vulnerability that on behalf of their children. When analyzing the health care professionals need to be aware that treatment options for Ashley, rehabilitation profes- society has placed some parameters around the sionals need to be careful not to contribute to this types of decisions parents can and cannot make. pattern by blaming her parents for their efforts to For example, it is no longer acceptable that parents seek unconventional solutions in the absence of of newborns with Down syndrome can refuse life- quality resources for families of children with dis- saving surgeries for treatable esophageal atresias or abilities. heart anomalies, or for parents to refuse blood trans- It is also important to note that in most in- fusions or high benefit life-saving interventions for stances parents are in the best position to make their children based upon the parental religious be- decisions on their child’s behalf. Parents are im- liefs (e.g., Jehovah’s Witnesses, or Christian Sci- bued with the legal and moral responsibility to care ence)11,12 There are also limits in place regarding for their children.15 In making medical decisions, parental decisions for pediatric sterilization including they are expected to use a “best interest” standard, state laws such as the one in Washington where weighing the proposed benefits and burdens of Ashley had her surgery.13,14 In rare instances, the each decision.16 Some healthcare professionals and state is empowered to step in and take over the care bioethicists argue that considering the child’s “best of a child under the parens patriae power when the interests” in isolation is unrealistic given that par- parents or guardians are unable or unwilling to act in ents also have to balance the needs and interests of the best interest of the child. other family members.17,18 The lives of physicians, Finally, being described as a girl with the men- health care professionals and other stakeholders tal age of an infant may unnecessarily limit expec- are not affected by these decisions in the same way

December 2007 Commentary 1025 as the lives of Ashley’s family members. Therefore found that these children with CP, though often the parents’ decisions, while not without limits, smaller than their nondisabled peers, tended to must carry significant weight. have better health and social participation if they had better growth.24 Clearly much is not known Ethical Issues for Stakeholders: about the effects of growth on health and social Physicians and Healthcare Professionals participation for children with significant develop- People with disabilities are high users of med- mental disabilities. ical services, and most physicians are not trained to Though in general, medical professionals do deal with the complex issues that accompany dis- not offer treatments where the risks exceed the ability. Patients with permanent disabilities have expected or known benefits, exceptions do occur. historically posed particular challenges for health An example might be when a disease process, such care professionals. People with disabilities often as cancer, may result in death without treatment. require health interventions to prevent diseases and There may even be times when some risky treat- complications from occurring, but invariably have ments—such as mastectomy—are initiated pro- environmental and social components that compli- phylactically, because the risk of developing breast cate their degree of function, social participation, cancer is believed to be very high (such as for a and quality of life. Many physicians have never woman who carries the BRCA genes). The decision heard of the social model of medicine, or George to perform surgery prophylactically vs. increasing Engel’s biopsychosocial model.19 Nor are they fa- the breast cancer screening protocols is not uni- miliar with disability resources and specialists versally accepted, though, and the fact that a (such as physiatrists, independent living centers, woman (i.e., the decision is not offered to children) etc.). Finally, numerous studies have demonstrated can weigh the risk and benefits and make her own that health care professionals are not very accurate decision based on her values is a critical variable in at predicting what life with disability is like and, the decision making. indeed, consistently underestimate the quality of There are also examples of prophylactic in- life as expressed by people who live with disabilities terventions that are widely adopted on behalf of and chronic conditions.20–22 Such a dearth of in- children—like immunization practices. These formation and experience places both families and are practices in which the benefits are believed to physicians at a great disadvantage when attempting be very high (though the actual risk of infectivity to make decisions on behalf of a child with exten- may be low) and the risk from the treatment is also sive disabilities. believed to be very low. Ashley does not easily fit Physicians have responsibility to frame treat- into either category. One could argue that she does ment options based on principles of evidence-based not have a serious disease that warrants risky med- medicine (EBM). EBM involves “the conscientious, ical interventions, and as a prophylactic interven- explicit, and judicious use of current best evidence tion, the risk of the Ashley Treatment is substantial in making decisions about the care of individual and not proportionate to the identified problem. patients. The practice of evidence-based medicine Others may believe, as Ashley’s physician’s con- means integrating individual clinical expertise with cluded, that “these risks do not seem to be un- the best available external clinical evidence from reasonable and are not out of line with the risks systematic research.”23 Gunther and Diekema2 ac- of other medical interventions these children knowledge that there were few available data to undergo.”2 (1016–1017) The final risk/benefit anal- guide them in their analysis of risks, benefits and ysis is contingent upon how the problem is de- efficacy of the Ashley Treatment for patients with fined, how the risk is assessed and how alterna- Ashley’s condition. Though data on the risks of tive options are identified. general anesthesia and surgery are available, the data on the risks, benefits, and efficacy of high-dose Ethical Issues for Stakeholders: estrogen treatment are sketchier. In the end, Ash- Bioethics Committees and/or ley’s physicians extrapolated from studies on the Consultants use of high dose estrogen treatment with constitu- Ethics committees and consultation services tionally tall adolescent girls in the 1950s to support were established as advisory bodies to provide a their belief that the treatment would be safe and forum for discussion of complex issues in clinical possibly efficacious.2 care. Since Karen Ann Quinlan, the courts have It is also interesting to consider the assump- asked that hospital-based ethics committees be tions of benefit made in light of more recent data formed and serve as the primary resource to fam- on growth curves for children with moderate to ilies and health care providers for ethical dilem- severe cerebral palsy (CP), a diagnosis that is more mas, rather than the courts. “[This would allow] akin to Ashley’s condition than the study group of some much needed dialogue regarding these issues nondisabled, tall adolescent girls. The researchers and [force] the point of exploring all of the options

1026 Kirschner et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 for a particular patient.”25 It is now a requirement or holds the same opinion. How should the dis- of the Joint Commission for Accreditation of ability representative be selected? And can a cog- Healthcare Organizations (JCAHO) that all hos- nitively intact adult with a disability represent pitals have some ethics mechanism26 Organiza- Ashley’s interests any better than her parents or tions such as the American Society for Bioethics others on the committee? Certainly, every hos- and Humanities have worked out recommenda- pital ethics committee must think critically tions for core competencies for those who provide about these questions, the issue of representa- ethics consultation.27 Critical to a robust discus- tion, and the group process. sion of complex ethics issues is ensuring that the Whereas some have critiqued the ethics “right” composition of stakeholder perspectives is committee proceedings, others within the bio- represented. Due diligence—the gathering of per- ethics community have supported the process tinent information and relevant perspectives—is that took place in Washington and lauded essential to this process. Diekema and Gunther for publishing this case. Disability organizations have raised concerns Three bioethicists writing in Scientific Ameri- that the ethics committee process in the Ashley case can.Com (Joel E. Frader, MD, Norman Fost, MD, prevented the sort of due process protections that the MPH, and Benjamin Wilfond, MD) “came down judicial system would have provided. Not Dead Yet, a firmly on the side of the parents and the decision disability activist organization opposed to assisted of the original ethics board” with a few reserva- suicide and euthanasia, issued a statement condemn- tions.32 Norm Frost writes, “whatever disagree- ing the ethics committee and viewed the Ashley ments critics may have, it is not possible to say Treatment as doing an “end-run” around Ashley’s this decision was made casually or quickly, with- 28 constitutionally guaranteed right of due process. out careful consideration for the relevant facts The Disability Rights Education and Defense Fund and arguments.”32 They believe that the child’s believes Ashley was denied her “basic human rights best interests remained the focus of the ethics through draconian interventions to her person” and committee’s decision and that going to court for the conduct of the physicians and ethics committee permission on every invasive procedure involv- 29 should be “widely questioned.” Three other groups ing children would be untenable. who have long-served children with disabilities issued statements. The American Association on Intellectual Ethical Issues for All Stakeholders and Developmental Disabilities categorizes the Ashley Treatment as “unjustifiable nontherapy” and asks A critical question in the Ashley case is why an independent legal advocate or expert in dis- whether it is ever justified to surgically alter the ability rights was not part of the review process.30 body of an otherwise healthy and nonconsenting United Cerebral Palsy and The Arc, in their joint child? Quality-of-life criteria were the foundation statement, believed the counsel on behalf of the eth- for the arguments put forward by the parents and 1,2 ics committee to the parents and physicians was physicians. Although there are examples of qual- “insufficient... since they failed to account for Ash- ity-of-life criteria justifying surgical interventions, ley’s fundamental rights as a person with intellectual such as in esthetic surgery, an important criteria is and developmental disabilities...consideration of who defines the “quality-of-life” goal of treatment. Ashley and her stake in her own civil and human People who elect to have esthetic surgery make the rights was absent.”31 The Disability Rights Washing- decision for themselves based on their assessment ton (formerly the Washington Protection and Advo- of quality-of-life benefits and medical risks. Quality- cacy System) critiqued the Ethics Committee process of-life criteria have also been used in decisions to further, agreeing that a court order was legally re- treat children, such as the use of synthetic growth quired before the hysterectomy could be performed.14 hormone for boys who are constitutionally short As part of the settlement agreement with Seattle (but not growth hormone deficient), or high-dose Children’s Hospital they recommended that a disabil- estrogen for girls who were constitutionally tall ity rights advocate be included in future deliberations (ironically the only limited experience with this regarding the Ashley Treatment or growth limiting treatment as applied in the Ashley case), as short interventions for children with developmental dis- boys and tall girls have been considered socially abilities.14 and culturally undesirable.33,34 The use of medical Stakeholder representation is critical but treatments for such nonmedical, quality-of-life in- this recommendation is not without complexity. dications have been considered controversial, and Somebody who has a lived experience of disabil- in general, have fallen out of favor. ity may be able to authentically represent a dif- Medical historian, Alice Dreger,35 (p261) who ferent perspective than the professionals who has studied conjoined twins and people with inter- typically sit on hospital ethics committees. How- sex conditions offers the following advice to par- ever, not everybody with a disability thinks alike ents contemplating such surgeries:

December 2007 Commentary 1027 Make sure you ask yourself and your surgeon fortable to others? And if not, should there be prohi- bitions on certain types of medical treatments for more than once: Given the risk, should you children and those who cannot consent for themselves? ever choose, on behalf of someone else (even your own child), a surgery offered purely for SUMMARY Ashley’s treatment undoubtedly encompasses psychological and social reasons? Surgeons issues related to her biology, her psychological well-being, her cultural/familial environment and often tell me ‘you can’t change society so you sociopolitical factors. Her impairments, the re- have to change children’s bodies,’ but clearly in stricted options available to her family, the limits of medicine and the history of discrimination against many cases we do try to change society. We’ve children with cognitive/developmental delays all come together to make this case particularly poi- tried to eliminate sexism that tries to keep girls gnant and compelling. Given our unique training and experience, it is imperative that physiatrists, as down just because their bodies are different well as other specialists in disability, address these than boys, we’ve tried to eliminate racism that concerns and, along with other stakeholders, be intimately involved in this ethics debate. keeps some kids down just because their bodies ACKNOWLEDGMENTS are darker than others. So consider the The authors would like to thank Debjani Mukherjee PhD, Carmen Cicchetti MA, Elliot J. possibility that, instead of changing your Roth MD, and Raymond H. Curry MD for their child’s body, we should start expecting others critical review of the manuscript. to change their minds. REFERENCES 1. The “Ashley Treatment”: Toward a better quality of life for “Pillow Angels.” Available at: http://ashleytreatment.spaces. live.com. Accessed March 6, 2007 The use of quality-of-life criteria when making 2. Gunther DF, Diekema DS: Attenuating growth in children decisions on behalf of people who cannot speak for with profound developmental disability. Arch Pediatr Ado- themselves is further problematic because of the lesc Med 2006: 1013–7 limited ability of third parties to predict what con- 3. Savage TA, Gill CJ, Kirschner KL: (Special section intro- stitutes a “good” quality of life for others.36 The duction on disability ethics. J Clin Ethics 2004;15:256–63 social model of disability would question whether 4. Albrecht GL, Seelman KD, Bury M: The Formation of Dis- ability Studies. In Albrecht GL, Seelman, KD, Bury M, eds. the identiﬁed problem in this case is intrinsic to Handbook of Disability Studies. Thousand Oaks, California: Ashley’s body or embedded in a lack of environ- Sage Publications;2001: 1–8 mental supports, negative social attitudes and the 5. Oliver M: The Politics of Disablement. London: Macmillan; way others see her or frame her possibilities. Evi- 1990 dence for this concern is implicit—both in the 6. Shakespeare T: Disability Rights and Wrongs. London: Routledge; 2006 parents’ blog and public commentaries, one of 7. United Nations General Assembly: Convention on the which states, “given Ashley’s mental age a nine and Rights of Persons with Disabilities. Available at: http://www. a half year old body is more appropriate and more un.org/disabilities/convention/conventionfull.shtml. Accessed digniﬁed than a fully grown female body.”1 In ap- March 6, 2007 parent support of this position Ashley’s parents 8. Committee on Ethics, American College of Obstetrics and Gynecology: Sterilization of Women, including those with quote George Dvorsky, a member of the board of Mental Disabilities. Available at http://www.acog.org/from_ directors for the Institute for Ethics and Emerging home/publications/ethics/ethics056.pdf. Accessed March 6, Technologies, “The estrogen treatment is not what 2007 is grotesque here. Rather, it is the prospect of 9. Kopell BH, Rezai AR: Psychiatric neurosurgery: a historical perspective. Neurosurg Clin N Am 2003:181–97 having a full-grown and fertile woman endowed 1 10. Krugman S: The Willowbrook hepatitis studies revisited: with the mind of a baby.” ethical aspects. Rev Infect Dis 1986;8:157–62 What if the fundamental issue driving the Ashley 11. American Academy of Pediatrics Committee on Bioethics: Treatment is this sentiment—the fact that society is Ethics and the care of critically ill infants and children. uncomfortable with adults who have cognitive dis- Pediatrics 1996;98:149–52 abilities? If so, should health care professionals ever 12. American Academy of Pediatrics Committee on Bioethics: Religious objections to medical care. Pediatrics 1997;99: sanction or be complicit in using medical treatments 279–80 to alter the bodies of people with disabilities to make 13. Committee on Bioethics: Sterilization of minors with de- them more acceptable, more lovable, or more com- velopmental disabilities. Pediatrics 1999;104:337–40

1028 Kirschner et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 14. Carlson DR, Dorfman DA: Investigative report regarding the Comprehensive Accreditation Manual for Hospitals: The Offi- “Ashley Treatment.” Washington Protection & Advocacy Sys- cial Handbook. Oakbrook Terrace, IL, Joint Commission on tem, 2007. Available at: http://www.disabilityrightswa.org/ Accreditation of Healthcare Organizations, 2006 news-1/Investigative%20Report%20Regarding%20the% 27. American Society for Bioethics and Humanities: Core Com- 20Ashley%20Treatment.pdf petencies for Health Care Ethics Consultation. Glenview, 15. Miller PS: Toward truly informed decisions about appear- American Society for Bioethics and Humanities, 1998 ance-normalizing surgeries, in Parens E (ed): Surgically 28. Not dead yet: statement on “growth attenuation” experi- Shaping Children. Baltimore, Johns Hopkins University mentation. Available at: http://www.notdeadyet.org/docs// Press, 2006, pp 211–26 Growth_AttenuationPR0107.html. Accessed March 10, 16. President’s Commission for the Study of Ethical Problems 2007 in Medicine and Biomedical and Behavioral Research: De- 29. Disability Rights Education & Defense Fund: Modify the ciding to Forego Life-Sustaining Treatment. Washington, system, not the person. Available at: http://www.dredr.org/ DC: U. S. Government Printing Office, 1983, pp 214–29 news/ashley.shtml. Accessed March 6, 2007 17. Ross LF: Children, Families, and Health Care Decision- Making. New York, Clarendon Press, 1998 30. Board of Directors of the American Association on Intellec- tual and Developmental Disabilities: Unjustifiable non- 18. Kopelman LM: The best-interests standard as threshold, therapy: a response to Gunther & Diekema (2006), and to ideal, and standard of reasonableness. J Med Phil 1997;22: the issue of growth attenuation for young people on the 271–89 basis of disability. Available at: http://www.aaidd.org/Policies/ 19. Engel G: The need for a new medical model. Science 1977; board_positions/growth.shtml. Accessed March 6, 2007 196:129–36 31. UCP & The Arc joint statement on “Ashley’s Treatment.” 20. Albrecht GL, Devlieger PJ: The disability paradox: high Available at: http://www.ucp.org/ucp_generaldoc.cfm/1/9/ quality of life against all odds. Soc Sci Med 1999;48:977–88 10020/10020-10020/7108?PrintFriendlyϭYes. Accessed March 21. Blaymore Bier J, Liebling JA, Morales Y, Carlucci M: Par- 8, 2007 ents’ and pediatricians’ views of individuals with meningo- 32. Mims C: The pillow angel case—three bioethicists weigh in. myelocele. Clin Pediatr 1996;35:113–7 Scientific American Com January 5, 2007 22. Gerhart KA, Koziol-McLain J, Lowenstein SR, Whiteneck 33. Gill DG: “Anything you can do, I can do bigger?”: the ethics GG: Quality of life following spinal cord injury: knowledge and equity of growth hormone for small normal children. and attitudes of emergency care providers. Ann Emerg Med Arch Dis Child 2006;91:270–2 1994;23:807–12 34. Barnard ND, Scialli AR, Bobela S: The current use of estro- 23. Sackett DL, Rosenberg WMC, Muir Gray JA, et al: Evidence gens for growth-suppressant therapy in adolescent girls. based medicine: what it is and what it isn’t. BMJ 1996;312: J Pediatr Adolesc Gynecol 2002;15:23–6 7023 35. Dreger AD: What to expect when you have the child you 24. Stevenson RD, Conaway M, Chumlea WD, et al: Growth and weren’t expecting, in Parens E (ed): Surgically Shaping health in children with moderate-to-severe cerebral palsy. Children. Baltimore, Johns Hopkins University Press, 2006, Pediatrics 2006;118:1010–7 pp 211–26 25. In re Quinlan, 70 N.J. 10, 355 A. 2d 647, cert denied, 429 36. Kothari S, Kirschner KL: Abandoning the golden rule: the U.S. 922 (1976) problem with putting ourselves in our patient’s place. Top 26. Joint Commission on Accreditation of Healthcare Organizations: Stroke Rehabil 2006;13:68–73

CME Self-Assessment Exam Answers American Journal of Physical Medicine & Rehabilitation Vol. 86, No. 12 • December 2007

CME Article Number 2: J. A. Saveika, et al.

1. B

2. A

3. A

4. A

5. D

December 2007 CME Self-Assessment Exam 1029 VISUAL VIGNETTE

Exacerbation of Habitual Dislocation of Ulnar Nerve by Concurrent Dislocation of Triceps Muscle: Complementary Role of Dynamic Ultrasonography to Electrodiagnosis Myung Jae Yoo, MD, Dennis DJ Kim, MD, and Mooyeon Oh-Park, MD From the Department of Rehabilitation Medicine, Albert Einstein College of Medicine, Monteﬁore Medical Center, Bronx, New York. FIGURE 1 Ultrasonography of the left elbow in extended posi- 0894-9115/07/8612-1030/0 tion. Markedly enlarged and hypoechoic ulnar nerve American Journal of Physical Medicine & Rehabilitation Copyright © 2007 by Lippincott Williams & Wilkins (n) in retrocondylar groove. Medial epicondyle (m). DOI: 10.1097/PHM.0b013e31815b7d07

A 50-yr-old woman with past medical history of left elbow fracture in childhood presented with 3 mos of numbness and tingling of the left ring and little fingers. Physical examination was remarkable for cubitus varus deformity, atrophy and weakness of the left hand intrinsic muscles, and a positive Tinel sign over the left retrocondylar groove. During elbow flexion, snapping of a cordlike structure was noted bilaterally over the medial epicondyle. Electrodiagnosis demonstrated markedly slow conduction velocity of the left ulnar nerve across the retro- FIGURE 2 condylar groove. Fibrillation potentials and polyphasic mo- Ultrasonography of the left elbow in flexed position. Ulnar nerve (n) and medial head of triceps tor unit action potentials were noted in the left first dorsal muscle (t) are dislocated over the apex of medial interosseous, abductor digiti quinti, flexor digitorum pro- epicondyle (m). fundus, and flexor carpi ulnaris muscles. These findings were compatible with entrapment neuropathy of left ulnar operative management, including transposition of the ulnar nerve at the retrocondylar groove. nerve and medial triceps, as well as corrective valgus osteot- To evaluate the “snapping” structure over the medial omy of the distal humerus. epicondyle, dynamic ultrasonography was performed using a A transient snapping sensation during elbow flexion can broadband linear transducer with medium frequency of 10 be caused by either dislocation of the ulnar nerve or the MHz (LogiqE, General Electric, Inc). The left ulnar nerve was medial portion of the triceps muscle.1 In this case, a palpable markedly enlarged and hypoechoic compared with the con- snap over the medial epicondyle was initially interpreted as a tralateral side (Fig. 1). At 70 degrees of elbow flexion, dislo- habitual dislocation of the ulnar nerve. Dynamic ultrasonog- cation of the left ulnar nerve over the medial epicondyle was raphy differentiated concurrent dislocation of the triceps demonstrated; at 90 degrees of elbow flexion, sonographic muscle as well as the ulnar nerve on the symptomatic side imaging revealed concomitant dislocation of the triceps mus- from isolated dislocation of the ulnar nerve on the asymp- cle pushing the ulnar nerve and producing an appreciable tomatic side.2 This case illustrates the complementary role of snap (Fig. 2). Ultrasonography of the contralateral elbow ultrasonography to electrodiagnosis in evaluating underlying revealed habitual dislocation of the ulnar nerve, without clin- anatomic structures of entrapment neuropathy such as snap- ical or electrodiagnostic findings of nerve dysfunction. On the ping triceps syndrome, posterior interosseous nerve syn- basis of the electrodiagnostic and ultrasonographic findings, drome, or tarsal tunnel syndrome. snapping triceps syndrome with resulting ulnar nerve entrap- REFERENCES ment at the elbow was diagnosed. The patient was referred for 1. Spinner RJ, Goldner RD: Snapping of the medial head of the triceps and recurrent dislocation of the ulnar nerve. Anatomical and dynamic factors. J Bone Joint Surg Am 1998;80:239–47 All correspondence and requests for reprints should be addressed to Mooyeon 2. Jacobson JA, Jebson PJ, Jeffers AW, et al: Ulnar nerve dislocation and Oh-Park, MD, Department of Rehabilitation Medicine, Albert Einstein College of snapping of triceps syndrome: diagnosis with dynamic sonography— Medicine, Montefiore Medical Center, 111 E 210th Street, Bronx, NY 10467. report of three cases. Radiology 2001;220:601–5

1030 Yoo et al. Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 LETTERS TO THE EDITOR

Re: Introduction to Nanotechnology: Potential to engineered nanomaterials in air, water, and terrestrial Applications in Physical Medicine and ecosystems. Because nanotechnologies are diverse and ex- Rehabilitation posures to nonmaterial will vary widely, assessing exposure To the Editor: We find the article titled “Introduction and potential impacts on health or the environment will to nanotechnology: potential applications in physical med- require a multidisciplinary approach for assessments. icine and rehabilitation” by Gordon and colleagues (March Among the large array of nanomaterials and processes, 2007) interesting and timely.1 Scientist now attempt to the quantum dots are of particular importance for their manipulate matter at the scale of atoms and molecules, possible therapeutic and diagnostic medical applications. using the term nanotechnology to revolutionize manufac- These nanoparticles consist of a metalloid core and a shell turing across all industry sectors, including tissue engi- that surrounds the core and renders the quantum dots’ neering, drug delivery, imaging, diagnostics, surface tex- bioavailability. The further addition of a specific coating turing, and biointerfaces that could impact the practice of can give the quantum dots a desired bioactivity, which is physical medicine and rehabilitation in the future. Gov- essential for promising medical applications such as tar- ernments and conglomerates must establish strict codes of geted drug delivery and in vivo biomedical imaging. Hu- ethics for nanotech products to avoid future mismanage- man exposure to quantum dots may result from environment of this novel technology. Lack of appropriate ethical mental, workplace, and therapeutic exposure. There may codes of conduct in this science may lead to disasters to be a risk of bioaccumulation of these materials within mankind as well as nature. Perhaps it is time to remember organs and tissues (e.g., in lungs) with still unexplored or that natural organisms harbor excellent nanotech protec- underexplored health risks. Introduction of quantum dots tion shaped by millions of years of natural selection—lotus into the environment may occur via waste streams from leaves possess micro- and nanotextures on the surface. industry, research, and clinical settings. The persistence of Similarly, animals from bees to birds have unique nano- these materials in the environment may be quite long, and texturing that engenders water repellency and lack of ad- the environmental exposure will depend on the partition of hesion. Prehistoric cave painters apparently used nature’s quantum dots between water, air, and various soil types.4 nano materials in their painting processes. Particular impacts of nanomaterials are foreseen on the Globally, industry and governments spend nearly $10 ecology of soils, which often already contain and move billion on nanotech research and development annually around small particles. Nanoparticles could easily be ab- (Table 1). The National Science Foundation in the United sorbed by earthworms, possibly allowing them to move up States predicts that nanoscale technology will capture a $1 the food chain and reach humans.5 trillion market by 2011. Thousands of tons of nanomaterials are already being produced each year, and more than Global understanding of nanotechnology-specific risks 300 claimed nanotechnology products have surfaced on is essential if large and small industries are to operate on the market.2 Nano particles like nano paint come when the a level playing field and if developing economies are not to general public has great concern regarding genetically be denied essential information on designing safe nano- modified organisms and toxic chemical residues in human technologies. The ethical issues fall into the areas of eq- and pet foods. Thus, the public in developed and develop- uity, privacy, security, environment, and metaphysical ing nations may undoubtedly demand from their govern- questions concerning human–machine interactions. A ments more transparency and safer nanotechnology.3 wide debate is needed on the implications and democratic Unfortunately, there are no ethical and professional regu- control of nanotechnologies worldwide, to restrict nano- lations for large- or small-scale nanotech industries, and technology before it becomes a lawless science. The debate no impact studies have been mandated to assess exposure should not be restricted to meetings of expert scientists or targeted on human/environmental health and safety issues. Ethical implications of nanotechnology must be addressed by people from all walks of life. TABLE 1 Government R&D spending in nanotechnology ($U.S., in millions) Govindasamy Agoramoorthy Department of Pharmacy Region 2001 2005 Tajen University European Union ϳ225 ϳ1050 Yanpu, Taiwan Japan ϳ465 ϳ950 United States ϳ465 1,081 Others ϳ1535 ϳ4081 Chiranjib Chakraborty Department of Biotechnology Source: National Nanotechnology Initiative (United States). College of Engineering & Technology Greater Noida, India

December 2007 Letters to the Editor 1031 REFERENCES 3. Renn O, Roco MC: Nanotechnology and the need for risk governance. 1. Gordon AT, Lutz GE, Boninger ML, Cooper RA: Introduction to J Nanoparticle Res 2006;8:153–91 nanotechnology: potential applications in physical medicine and reha- 4. Mehta MD: From biotechnology to nanotechnology: what can we learn bilitation. Am J Phys Med Rehabil 2007;86:225–41 from earlier technologies? Bull Sci Tech Soc 2004;24:34–9 2. The Nanotechnology Consumer Products Inventory. Washington, DC, 5. Reynolds GH: Nanotechnology and regulatory policy: three futures. Woodrow Wilson International Center for Scholars, 2006 Harv J Law Technol 2003;17:179–205 3. Maynard AJ, Aitken RJ, Butz T, et al: Safe handling of nanotechnology. Nature 2006;444:267–9 DOI: 10.1097/01.phm.0000297450.49425.d4 4. Hardman RA: A toxicological review of quantum dots: toxicity depends on physico-chemical and environmental factors. Environ Health Per- Re: Sacroiliac Joint Pain: Anatomy, spect 2006;114:165–72 Biomechanics, Diagnosis, and Treatment 5. Brumfiel G: Nanotechnology: a little knowledge. Nature 2003;424: To the Editor: Foley and Buschbacher1 are to be com- 246 mended on their informative as well as thorough review of DOI: 10.1097/01.phm.0000297451.49425.9d the sacroiliac joint (SIJ) as a pain generator (December 2006). As they readily acknowledge, “the literature on SIJ pain is Response Letter complex and controversial.” Three decades ago, when we evaluated the SIJ as a source of lumbosacral and pelvic pain, The Authors Respond: We thank Drs. Agoramoorthy it was also underappreciated, as it remains so even today.2 and Chakraborty for their letter in which they express con- Although Dreyfuss et al.3 state in their review “that no cern for safety and regulation of nanotechnology and the need physical examination test [has] demonstrated diagnostic for shared international information. In our article (March value and that SIJ pain is resistant to identification by 2007), we raise safety issues by citing known toxicity of historical and physical examination,” our experience in nanoparticles to animals in reference citations 122–126.1 We this regard would suggest otherwise. Although we would agree with Drs. Agoramoorthy and Chakraborty about the agree there is no one clinical or laboratory test that is importance of environmental impact studies, and we are pathomonic of SIJ dysfunction, an appropriate history and encouraged by the initial steps taken to address these issues physical examination buttressed by dynamic pelvic x-rays (references 127 and 128 in our article). and an immediate reduction in pain in response to an The issue of nanotechnology regulation has received anesthetic injection of the joint can be diagnostic. In the supine position, an asymmetric restriction of hip external a great deal of international attention among materials rotation on the symptomatic side associated with increased scientists, legal scholars, and government agencies. The pain over the SIJ as it is passively rotated into abduction is reader is referred to several review articles that carefully also a frequent clinical finding (Fig. 1). A standing pelvic discuss the sharing of resources and expertise, company x-ray followed by alternate-leg weight bearing may also dem- protectionism, trade conflicts, and models for transna- onstrate excessive instability of the pubic symphysis, often tional regulation based on international agreements on exceeding 3 mm (Fig. 2). Usually, this slip is greater on the environmental pollutants, arms proliferation, and global side of the symptomatic SIJ. Symphyseal instability mirrors ethics.2–5 Substantive progress will be limited, however, by similar rotary luxation at the symptomatic SIJ as this artic- an inadequate framework for discussion and implementa- ulation responds to shifts in the center gravity horizontally as tion of policy, combined with a scientific uncertainty re- well as vertically during normal ambulation (Fig. 3). garding toxicity and bioaccumulation of nanomaterials. Of An immediate reduction in SIJ pain after a combined note, the first law regarding nanotechnology regulation injection of a steroid and Xylocaine is also diagnostic. has already been instituted in the United States. In December, 2006, the city of Berkeley amended its hazardous materials law to include nanostructures, requiring researchers and manufacturers to report what materials they are working with and how they are handling their disposal. The “Berkeley Nanotechnology Regulation” represents an initial step and demonstrates that oversight is achievable. Assaf T. Gordon, MD Department of Physical Medicine and Rehabilitation Rusk Institute, New York University New York, New York

REFERENCES 1. Gordon AT, Lutz GE, Boninger ML, Cooper RA: Introduction to FIGURE 1 Asymmetric restriction of hip external rotation on nanotechnology: potential applications in physical medicine and rehabilitation. Am J Phys Med Rehabil 2007;86:225–41 the symptomatic side is associated with increased 2. Marchant GE, Sylvester DJ: Transnational models for regulation of pain over the sacroiliac joint as it is passively nanotechnology. J Law Med Ethics 2006;34:714–25 rotated into abduction.

1032 Letters Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 medical history and physical examination in diagnosing sacroiliac joint pain. Spine 1996;21:2594–602

DOI: 10.1097/01.phm.0000297443.85757.8a

Response Letter The Authors Respond: The authors appreciate Dr. LaBan’s thoughtful comments. We agree that although no single physical exam test is particularly predictive, a panel of three or more tests can be extremely helpful. The tests described by Dr. LaBan may prove useful to those examining patients with sacroiliac joint pain. Hopefully, FIGURE 2 Standing pelvic x-ray showing excessive instabil- further research will be performed to test those hypotheses. ity of the pubic symphysis. They make sense to the authors, but they have not yet been validated in the literature. Dr. LaBan’s caveat regarding other regional pain generators that masquerade as SIJ pain is a good one that should be heeded by all practicing clinicians. Brian S. Foley, MD Ralph M. Buschbacher, MD Department of PM&R Indiana University School of Medicine Indianapolis, Indiana

DOI: 10.1097/01.phm.0000297442.78134.fe

Re: The Myofascial Trigger Point Region: Correlation Between the Degree of Irritability and the Prevalence of Endplate Noise FIGURE 3 Diagram showing rotary luxation at the symptom- To the Editor: The article “The myofascial trigger atic sacroiliac joint during normal ambulation. point region: correlation between the degree of irritability and the prevalence of endplate noise” by Kuan et al.1 (March 2007) is a valuable addition to the current Except in the presence of extreme obesity, this procedure body of knowledge surrounding myofascial pain. The can easily be accomplished without x-ray guidance. En- authors suggest a possible mechanism of action pro- trance to the articulation itself can be assured by noting posed originally by Simons et al.2 in which hypoxia and restricted side-by-side movement of the syringe as limited ischemia are related to local release of inﬂammatory by the congruent joint surfaces As a caveat, it is important substances, which may sensitize muscle nociceptors. We to note that SIJ may not be the primary pain generator. would like to add that our recent study3 in 2005 also Instead, it may be a referred site of discomfort, both via its supports this contention. direct innervation and secondary to more remote mechan- We used a novel microdialysis technique to sample ical problems—among others, a degenerative disc at muscle with and without myofascial trigger points for L5–S1 and/or spinal stenosis, as well as degenerative dis- levels of protons, inﬂammatory mediators, cat- ease of the hip. echolamines, neuropeptides, and cytokines. The results Myron M. LaBan, MD, MMSc, FACP, FAAPMR show distinct local biochemical differences between sub- William Beaumont Hospital jects with a spontaneously painful myofascial trigger point Royal Oak, Michigan (active MTrP) in the upper trapezius muscle compared with those with a nonpainful trigger point (latent MTrP) and those without a trigger point. Analysis for pH demon- REFERENCES strated that active MTrPs possessed relatively higher acidic 1. Foley BS, Buschbacher RM: Sacroiliac joint pain: anatomy, biomechanics, diagnosis and treatment. Am J Phys Med Rehabil 2006;85:997–1006 levels (lower pH) than the other muscle groups, which could be associated with conditions attributed to hypoxia and 2. LaBan MM, Meerschaert JR, Taylor RS: Symphysis and sacroiliac joint pain associated with pubic symphysis instability. Arch Phys Med Re- ischemia. Similarly, levels of bradykinin, serotonin, norepineph- habil 1978;56:470–2 rine, substance P, CGRP, TNF-␣, and IL-1␤ were all at relatively 3. Dreyfuss P, Michaelsen M, Pauza K, McLarty J, Bogduk N: The value of higher concentrations in active MTrPs.

December 2007 Letters to the Editor 1033 We feel that the combination of these two studies 3. Simons DG: Myofascial pain syndrome due to trigger points, in Goodgold provides good support to the proposed mechanism of myo- J (ed): Rehabilitation Medicine. St. Louis, Mosby, 1988, pp 686–723 4. Simons DG, Hong C-Z, Simons LS: Prevalence of spontaneous elec- fascial trigger point pain. trical activity at trigger spots and at control sites in rabbit skeletal Jay P. Shah, MD muscle. J Musculoskelet Pain 1995;3:35–48 Rehabilitation Medicine Department 5. Simons DG: Clinical and etiological update of myofascial pain from National Institutes of Health trigger points. J Musculoskelet Pain 1996;4:93–121

Bethesda, Maryland DOI: 10.1097/01.phm.0000297444.62886.41 Sagar Parikh, MS IV University of Medicine and Dentistry of New Jersey Re: Characteristics and Treatment of New Brunswick, New Jersey Headache After Traumatic Brain Injury To the Editor: The authors of “Characteristics and Jerome Danoff, PT, PhD Treatment of Headache After Traumatic Brain Injury” (Lew Department of Exercise Science 1 George Washington University Medical Center et al., July 2006) are to be complimented on their com- Washington, DC prehensive review of “posttraumatic headache (PTH).” They readily acknowledge the present confusion inherent Lynn H. Gerber, MD in both the “accepted terminology” and the exact patho- College of Health and Human Services George Mason University genesis of “cerviogenic headache” pathology when both Fairfax, Virginia intracranial and cervical spine trauma have occurred si- multaneously. REFERENCES Although controversy in this regard persists, in our 1. Kuan T-S, Hsieh Y-L, Chen S-M, Chen J-T, Yen W-C, Hong C-Z: The own 40-yr clinical experience, posttraumatic occipital neu- myofascial trigger point region: correlation between the degree of ralgia (PTON) is all too often the principle causal agent in irritability and the prevalence of endplate noise. Am J Phys Med Rehabil 2007;86:183–9 cases of persistent PTH. The discomfort of PTON is located 2. Simons DG, Travell JG, Simons LS: Travell & Simons’s Myofascial primarily in the occipital area, often radiating to the vertex Pain and Dysfunction: The Trigger Point Manual, vol 1, ed 2. Balti- as a direct consequence of injury to the greater and/or more, Williams & Wilkins, 1999 lesser occipital nerves at their C1–C2 spinal roots of origin. 3. Shah JP, Phillips TM, Danoff JV, Gerber LH: An in vivo microanalytical When digital pressure is applied to these nerves over technique for measuring the local biochemical milieu of human skeletal muscle. J Appl Physiol 2005;99:1977–84 the occipital notch, the patient may experience either hyper- and/or hypoesthesia in the occipital nerve’s distri- DOI: 10.1097/01.phm.0000297445.62886.08 bution. Thereafter, an occipital nerve block can be both Response Letter diagnostic and therapeutic in confirming the origin of the headache by alleviating the symptoms of the PTON. The Authors Respond: We highly appreciate the com- A 25-gauge needle in this procedure is inserted 3.5 cm ments by Shah et al. We also regret that we have not cited their inferolaterally to the occipital protuberance, and a combi- paper1 as one of the references in our article.2 Both their work1 nation of both bupivacaine hypochloride (Marcaine) and a and ours2 have definitely contributed substantially to increas- corticosteroid (1 ml of each) is injected. In a review of ten ing the credibility of myofascial trigger point (MTrPs). The PTH patients, Lew report a good response to a greater former has further confirmed the energy crisis hypothesis,3 occipital nerve block in eight patients and, in an additional two patients, a partial response.2 and the latter has proven the correlation between MTrP and Additional symptoms often accompanying PTON in- endplate noise.4,5 The great pioneer in MTrP, David G. clude, among others, a “light and sound hypersensitiv- Simons, developed these two important theories more than ity,” as well as vertigo, nausea, and facial hyperesthesia. 10 yrs ago. At the upper spinal cord levels, the fibers of the descend- Chang-Zern Hong, MD ing tract of CN V, accompanied by the tracts of CN VII, Department of Physical Therapy IX, and X, interdigitate with the ganglion cells of CI and HungKuang University CII. Subsequently, these tracts cross the midline to- Sha-Lu, Taichung, Taiwan gether and progress retrogradely as the ascending tract Ta-Shen Kuan, MD of CN V. At the C1–C2 spinal levels, this tract is vulner- Department of Physical Medicine and Rehabilitation able to trauma, with the potential of inciting brainstem National Cheng Kung University symptoms. Tainan, Taiwan As the authors themselves conclude, “headaches are multifactorial and involve a combination of central and REFERENCES peripheral mechanisms.” We would agree that a definitive 1. Shah JP, Phillips TM, Danoff JV, Gerber LH: An in vivo microanalytical technique for measuring the local biochemical milieu of human skel- diagnosis as related to intracranial and/or extracranial pa- etal muscle. J Appl Physiol 2005;99:1977–84 thology remains paramount to efficient and appropriate 2. Kuan T-S, Hsieh Y-L, Chen S-M, Chen J-T, Yen W-C, Hong C-Z: The treatment. myofascial trigger point region: correlation between the degree of irritability and the prevalence of endplate noise. Am J Phys Med Myron M. LaBan, MD, MMSc Rehabil 2007;86:183–9 Royal Oak, Michigan

1034 Letters Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 REFERENCES retrospective review and report of ten patients. J Head Trauma Rehabil 1. Lew HL, Lin P-H, Fuh J-L, Wang SJ, Clark DJ, Walker WC: Charac- 2004;19:58–71 teristics and treatment of headache after traumatic brain injury. A focused review. Am J Phys Med Rehabil 2006;85:619–27 DOI: 10.1097/01.phm.0000297446.70510.d4 2. Hecht JS: Occipital nerve blocks in posterior concussive headaches: a respective review and report of ten patients. J Head Trauma Rehabil Re: Shoulder Impingement Syndrome: 2004;19:58–71 Relationships Between Clinical, Functional, and Radiologic Findings DOI: 10.1097/01.phm.0000297448.64672.d1 To the Editor: We read with interest the article by Ardic and colleagues.1 The authors detected the relation- Response Letter ship between clinical, functional, and radiologic diagnostic The Authors Respond: The authors appreciate the in- (ultrasonography, magnetic resonance imaging [MRI]) mea- sightful comments by Dr. Myron M. LaBan regarding our sures in patients with subacromial impingement syndrome recent article on headache after traumatic brain injury (TBI).1 (SIS) and compared the diagnostic performances of ultrasonography and MRI. In this study, MRI has been used as a Incidentally, after publication of this manuscript, we have gold standard. However, MRI mainly evaluates the anato- received feedback from private practitioners of different spe- mopathologic conditions in the rotator cuff (RC) and sur- cialties, as well as academicians, who have echoed our per- rounding structures. It has also been shown that a significant ception that most headaches involve a combination of central number of asymptomatic shoulders may reveal abnormal and peripheral mechanisms. They also share our observations internal signals and even complete ruptures of RC tendons, regarding the heterogeneity in terminology and the paucity of by MRI.2,3 These tears were increasingly frequent with ad- well-controlled studies in this field. The consensus is that vancing age and were compatible with normal subjects. MRI clinicians should perform a comprehensive and systematic evaluation of elder asymptomatic patients (60 yrs of age and evaluation before initiating appropriate treatment. What we above) showed an incidence of 28% full-thickness tears.3 4 propose in Figure 2 is one possible algorithm that can be Frost et al. report that age was related to MRI based on expanded on, according to the local resources and level of supraspinatus pathology more so than clinical signs of im- expertise of the individual clinician. As described by Dr. La- pingement. In addition, Neer5 and Hawkins–Kennedy6 impinge- Ban, occipital nerve block is one example of a dual diagnostic ment tests are not enough for diagnosis of SIS. These tests and therapeutic approach that is relatively time-efficient to may be positive in other shoulder disorders. SIT should be perform and that could prove to be useful to patients. Like all performed as a reference test for differential diagnosis of other methods we have alluded to, this procedure deserves SIS.5,7–9 Neer5,7 identifies SIT in his reports as a most useful formal evaluation in the specific context of headache after method of separating impingement lesions from other causes TBI. Retrospective case review and report2 can certainly proof shoulder pain. SIT does not investigate the anatomopatho- vide impetus for further studies. Hopefully, with increasing logic condition in RC, but it depresses the pain generators in interest and awareness in the field, more prospective, ran- the subacromial area where the impingement occurs. Obvi- domized, controlled trials can be conducted to enhance the ous relief of pain and almost total improvement in passive standard of care for headache management in patients with and/or active shoulder range of motion after SIT suggest that TBI. the test is positive and discriminates SIS from other shoulder pathologies. Henry L. Lew, MD, PhD As a result, MRI is not the gold standard for diagnosis Stanford University School of Medicine of SIS. SIS is a clinical diagnosis that is done with SIT and PM&R Service, VA Palo Alto Health Care System Palo Alto, California impingement tests. Ilknur Aktas, MD David J, Clark MD, PhD Department of Physical Medicine and Rehabilitation Stanford University School of Medicine Saygi Hospital Anesthesiology Service, VA Palo Alto Health Care System Marmara University Institute of Neurological Sciences Palo Alto, California Istanbul, Turkey Kenan Akgun, MD William C. Walker, MD Department of Physical Medicine and Rehabilitation Virginia Commonwealth University Cerrahpasa Medical Faculty PM&R Service, McGuire VAMC University of Istanbul Richmond, Virginia Istanbul, Turkey

REFERENCES REFERENCES 1. Ardic F, Kahraman Y, Kacar M, Kahraman MC, Findikoglu G, 1. Lew HL, Lin P-H, Fuh J-L, Wang SJ, Clark DJ, Walker WC: Charac- Yorgancioglu ZR: Shoulder impingement syndrome: relationships be- teristics and treatment of headache after traumatic brain injury. A tween clinical, functional, and radiologic ﬁndings. Am J Phys Med focused review. Am J Phys Med Rehabil 2006;85:619–27 Rehabil 2006;85:53–60 2. Hecht JS: Occipital nerve blocks in posterior concussive headaches: a 2. Chandnani V, Ho C, Gerharter J, et al: MR ﬁndings in asymptomatic

December 2007 Letters to the Editor 1035 shoulders: a blind analysis using symptomatic shoulders as controls. We selected patients that had no history of trauma and Clin Imaging 1992;16:25–30 had shoulder pain of Ͼ3 mos, were unresponsive to analgesic 3. Sher JS, Uribe JW, Posada A: Abnormal findings on magnetic resonance images of asymptomatic shoulders. J Bone Joint Surg Am medication after 3 wks, and had a diagnosis of suspected SIS 1995;77:10–5 waiting for physical therapy and shoulder rehab.1 It is ridic- 4. Frost P, Andersen JH, Lundorf E: Is supraspinatus pathology as defined ulous to make clinical and radiologic assessments for asymp- by magnetic resonance imaging associated with clinical sign of shoulder impingement? J Shoulder Elbow Surg 1999;8:565–8 tomatic shoulders. We carefully evaluate the patients with 5. Neer CS: Impingement lesions. Clin Orthop 1983;173:70–7 chronic shoulder pain demographically, clinically, and radio- 6. Hawkins RJ, Abrams JS: Impingement syndrome in the absence of logically for the treatment options. Diagnosis and treatment rotator cuff tear. Orthop Clin North Am 1987;18:373–82 should be based on combination of these data. 7. Neer CS: Cuff Tears, Biceps Lesions and Impingement. Shoulder Reconstruction. Philadelphia, Saunders, 1990, pp 41–142 As we state in our article, “MRI has been used as the 8. Calis M, Akgun K, Birtane M, Karacan I, Calis H, Tuzun F: Diagnostic gold standard because our patients did not want arthro- values of clinical diagnostic tests in subacromial impingement syn- scopic evaluation.” In addition, MRI has been accepted as drome. Ann Rheum Dis 2000;59:44–7 the “best imaging tool” in many shoulder problems, in- 9. Birtane M, Calis M, Akgun K: The diagnostic value of magnetic resonance imaging in subacromial impingement syndrome. Yonsei Med cluding RC, labrum, capsule, biceps tendon/anchor, osse- 2001;42:418–24 ous structures, arthritis, and neural impingement.5,6 It is DOI: 10.1097/01.phm.0000297449.72296.3d well known that MRI is the only technique that can accurately image the early changes of this condition, in partic- Response Letter ular bursal thickening and effusion (subacromial bursitis), The Authors Respond: Many thanks for your interest edema, and inflammatory changes of the rotator cuff and to our article.1 First of all, our studies were done by an its tendons. SIS is a clinical diagnosis, and MRI provides us upper-extremity rehabilitation team consisting of experts with critical information regarding the size and location of in this area using standardized evaluation and therapy a tear, the specific tendons involved, the degree of muscu- systems/algorithms. lar atrophy and tendon retraction, and the quality of the We have to use comprehensive diagnostic and thera- torn edges.5,6 peutic tools for patients’ referral to shoulder rehab, as we Neer’s7 impingement concept depicts the syndrome as mentioned before.1 Special shoulder tests commonly per- a lifelong process with three stages. Stage I impingement formed by upper-extremity professionals can be divided for consists of reversible edema and hemorrhage typically in instability, impingement, labral lesions, scapular stability, active patients Յ25 yrs. Stage II implies fibrosis and ten- muscle tendon pathology, brachial plexus, and thoracic donitis. Stage III represents degeneration and rupture of- outlet syndrome.2,3 Common shoulder impingement tests ten associated with osseous changes most commonly in are the Neer–Walsh, Hawkins–Kennedy, and posterior inpatients Ͼ40 yrs.5 Therefore, early diagnosis and treat- ternal impingement tests.3 We used the first and the sec- ment are critical to prevent progression and improve ond impingement tests because the third is found primar- shoulder function. Frequently, however, clinical signs and ily in overhead athletes and workers.3 symptoms are nonspecific, and the diagnosis is often de- The positive Neer–Walsh impingement test suggests layed until a full-thickness defect in the RC has developed.6 rotator cuff (RC) pathology like the positive Hawkins– Symptomatic elderly patients may have stage III SIS 2 Kennedy impingement test. If one of these tests elicits mainly treated by shoulder rehab. However, asymptomatic pain, subacromial impingement test (SIT) with 10 ml of individuals are not our interest area. 0.1% lidocaine to the subacromial space will eliminate the Finally, there were very limited numbers of random- 2 pain of repeat testing. Because of medicolegal issues ized controlled trials for SIS using clinical and MRI and the approval procedure of this study by the IRB, we variables. MR/MR arthrography are still the best imag- didn’t choose the subacromial impingement test to as- ing tools in many shoulder problems. MRI has proved to sist in the diagnosis. It is not clear that these injections be highly sensitive and accurate for the diagnosis of have proven useful in directing care toward successful stage II–III SIS, with excellent correlation of arthro- outcomes, possibly because of the confounding placebo scopic and/or miniopen surgical findings. Specified clin- factor. Actually, Henkus et al.4 report that injections in ics in shoulder rehab should use comprehensive diag- subacromial bursa were inaccurate, with false-positive nostic and therapeutic work-ups. and -negative results in their randomized controlled MRI study with level II evidence. We didn’t prefer SIT, because it F. Figen Ayhan, MD Z. Rezan Yorgancioglu, MD is invasive; it requires patient’s approval and is only 66% 1,4 Ankara Education and Research Hospital accurate. Our opinion for the diagnosis of subacromial Department of PM&R impingement syndrome (SIS) was noninvasive clinical and Division of Hand and Upper Extremity Rehab radiologic work-ups. Ankara, Turkey

1036 Letters Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12 REFERENCES acromial injections: a prospective randomized MRI study. Arthroscopy 1. Ardic F, Kahraman Y, Kacar M, et al: Shoulder impingement 2006;22:277–82 syndrome: relationships between clinical, functional, and radiologic 5. Stoller D, Tirman P, Bredella M, et al: Diagnostic Imaging Orthopae- ﬁndings. Am J Phys Med Rehabil 2006;85:53–60 dics. Utah, Amirsys, 2004 2. Braddom RL: Physical Medicine & Rehabilitation. China, Saunders 6. Greenspan A: Orthopedic Imaging: a practical approach. Philadelphia, Elsevier, 2007 Lippincott Williams & Wilkins, 2004 3. Magee DJ: Orthopedic Physical Assessment. Philadelphia, Saunders, 7. Neer CS II: Impingement lesions. Clin Orthop 1983;173:70–7 2002 4. Henkus HE, Cobben LP, Coerkamp EG, et al: The accuracy of sub- DOI: 10.1097/01.phm.0000297455.15615.5e

December 2007 Letters to the Editor 1037 Erratum

In the article by Ersoz et al., published in the September 2007 issue of the American Journal of Physical Medicine & Rehabilitation, Tables 1 and 4 should appear as follows:

TABLE 1. Comparison of the stroke subgroups with respect to demographic and clinic parameters for the frequencies of symptomatic urinary tract infection Factor Frequency (%) P

Age, yrs 0.067 Ͻ65 11/56 (19.6) Ն65 19/54 (35.2) Gender 0.147 Male 10/49 (20.4) Female 20/61 (32.8) Level of education 0.055 Ungraduated 18/46 (39.1) Primary school 9/44 (20.5) Secondary/high school or university Type of lesion 0.858 Hemorrhagic 8/28 (28.6) Ischemic 22/82 (26.8) Side of lesion 0.354 Right 6/29 (20.7) Left 24/81 (29.6) Bladder emptying method 0.041 Spontaneous 23/96 (24.0) Indwelling catheter Residual urine, Ͼ50 ml 0.039 Absent 15/77 (19.5) Present 8/19 (41.2) Functional ambulation class 0.147 Յ2 20/61 (32.8) Ͼ2 10/49 (20.4) Brunnstrom stage class of upper extremity 0.012 1––2 23/59 (39.0) 3––4 3/26 (11.5) 5––6 4/25 (16.0) Brunnstrom stage class of lower extremity 0.055 1––2 16/39 (41.0) 3––4 9/44 (20.5) 5––6 5/27 (18.5)

TABLE 4. Microorganisms isolated in urine (Ն105 CFU/ml) and their frequencies in all stroke patients, in patients who are voiding spontaneously, and in patients with indwelling catheters Spontaneous Indwelling Catheter All Patients (43 ؍ n) (%) (11 ؍ n) (%) (32 ؍ n) (%)

Escherichia coli 19 (59.4) 6 (54.5) 25 (58.1) Klebsiella pneumoniae 7 (21.9) 0 (0) 7 (16.3) Pseudomonas aeruginosa 0 (0) 4 (36.4) 4 (9.3) Staphylococcus aureus 3 (9.4) 1 (9.1) 4 (9.3) Proteus mirabilis 3 (9.4) 0 (0) 3 (7.0)

REFERENCE 1. Ersoz M, Ulusoy H, Oktar MA, Akyuz M: Urinary tract infection and bacteriuria in stroke patients: Frequencies, pathogen microorganisms, and risk factors. Am J Phys Med Rehabil 2007;86:734–41

1038 Erratum Am. J. Phys. Med. Rehabil. ● Vol. 86, No. 12