Australasian Journal of Neuroscience AJON

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ANNA Australasian Neuroscience Nurses’ Association

Registered by Australia Post Publication No. NGB9480 Australasian Journal of Neuroscience Volume 24 Ɣ Number 2 Ɣ October 2014

2 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 Australasian Journal of Neuroscience Australasian Journal of Neuroscience, the journal of the Australasian Neu- roscience Nurses Association, publishes original manuscripts pertinent to neuroscience nursing standards, education, practice, related paramedical fields and clinical neuroscience nursing research. Copyright ©2014 Aus- tralasian Neuroscience Nurses Association. All rights reserved. Reproduc- tion without permission is prohibited. Permission is granted to quote briefly in scientific papers with acknowledgement. Printed in Australia.

ANNA ANNA Executive Australasian Journal of President Neuroscience Nursing Sharryn Byers (Nepean Hospital) c/- PAMS, PO Box 193, Surrey Hills. [email protected] Victoria. 3127.

Tel: (+61 3) 9895 4461 Vice President

Fax: (+61 3) 9898 0249 Kylie Wright (Liverpool Hospital) [email protected] www.anna.asn.au Secretary [email protected] Journal Editor

Vicki Evans (RNSH) Treasurer [email protected] Sandra Krpez (Liverpool Hospital BIU) Editorial Board [email protected]

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3 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

5 Editorial -— Vicki Evans Guest Editorial — Ashleigh Tracey

7 Geriatric Trauma Patients: The Real Picture of Their Health May Be Blurred Mary King

12 Apathy a Common Feature in Many Neurological Conditions: Care and Management Mary Braine

23 Analysing the Present Situation of Protection Against Exposure to Temozolomide on a Japanese Neurosurgical Ward Tetsuya Isaji

25 Anti-N-methyl-D-aspartic Acid Receptor Encephalitis: A Critical Literature Review and Implications for Neuroscience Nurses Linda Nichols

34 The Tomorrow: Advanced Treatments for PD Does Not Necessarily Equate to Treatments for Advanced PD David Tsui

38 Defying the Odds of Gravity: The Case of the Sinking Skin Flap Rhiannon Carey (Tonnie Koenen Prize winner 2014 ANNA Conference)

42 Falls Prevention Strategies Among Acute Neurosurgical Inpatients: A Best Practice Implementation Project Kylie M Wright

52 Freezing of Gait: What to Do When the Parkinson’s Medications Do Not Work. Melissa Drury

55 Calendar of Upcoming Events WFNN News

56 Instructions for Authors

4 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

Editor - Vicki Evans Ashleigh Tracey

This edition sees many articles from local I am honoured to be a part of ANNA’s guest sources as well as overseas contributors. The editorial for October. Being a neuroscience first article is from the United States and dis- ICU nurse for the past ten years, I have had cusses the special considerations when car- the pleasure of working with some of the most ing for the growing population of elderly trau- talented and knowledgeable nurses, who ma patients, focusing on their aged-related have endlessly supported the growth of new changes and co-morbid conditions. nurses entering the unit because in Neurosci- ence you don’t just sit on the sideline! From the UK, this paper describes apathy as a significant neuro-behavioural condition fol- Walking into the Neuroscience ICU on my first lowing TBI. Documented as common amongst day as a 20yr old new graduate was a daunt- many neurological disorders, apathy is still not ing, terrifying yet exhilarating experience. I well understood, nor described in the litera- was preceptored with a CNS who I admire to ture. A multidisciplinary team approach is re- this day. Her clinical knowledge surpassed quired with an emphasis on the importance of even the most confident of neurosurgical reg- being motivated for maximum recovery. istrar and who I watched skillfully manage the most complex neurosurgical patients with a From Japan, implementation and education beautiful sense of ease and control, whilst strategies for the disposal of material from having the ability to guide and support fami- patients undergoing Temozolomide treatment lies at every turn. For her, not only was the is described, with special note to exposure patient her priority, but so too was my learning from the oral antineoplastic agents. opportunities. I had the opportunity to practice Anti-N-methyl-D-aspartic acid (anti-NMDA) and refine assessment techniques, practical Receptor Encephalitis is a devastating, com- skills and the general nursing ‘tricks of the plex disease that is eloquently described trade’ needed to competently and safely man- here. Like most things neuro, early recogni- age neuroscience patients. Whilst I strongly tion and rehabilitation is essential. believe classroom learning is the underpinning of strong theories and concepts, for me There are two articles relating to Parkinson’s as a new neuroscience nurse, watching my Disease, describing the important role that PD colleagues’ practice at the bedside and to Nurse Specialists play in caring for these pa- have been allowed to learn in a safe, non- tients. Discussion around freezing-of-gait and threatening clinical environment has resonat- future treatments for PD is also presented. ed the strongest with me, and why I advocate this for all nurses entering this highly special- The sinking skin flap is a refreshing article ised and intricate field. describing a complication following decompressive craniectomy in which the skin flap Clinical teaching and educational opportuni- sinks down causing direct pressure on the ties however, can prove difficult in some situa- cortex and hence, neurological symptoms. tions and are not without their challenges. Floor plan layouts including single rooms, Finally, the process of implementation of a increased staffing demands and increased falls system is described. There are many patient acuity all play a role in limiting oppor- relevant outcomes from this paper that can be tunities for clinical bedside teaching. There is implemented across all areas. an increased pressure placed on nurses due to overwork and stress, which is further com- Our Guest Editorial captures the essence of pounded by the need to maintain clinical neuroscience nursing—the teaching of others standards and patient safety. in a caring and compassionate manner in which the future of the specialty can flourish. A preceptorship model we are currently using, Cheers, pairs new nurses with skilled experienced Vicki neuroscience nurses and allows both nurses 5 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 to work the same 12 hour day and night shifts, from day one. This allows for continuity when requiring a resource person and provides the new nurses with an opportunity to practise and develop assessment skills and techniques in a safe, familiar environment. Added to this, practical application of skills assists with the consolidation of knowledge learnt at university and allows for constructive feedback and critical reflection, all essential com- ponents in improving professional development.

Neuroscience Intensive Care is an environment rich in learning and professional growth, and where the disciplines of critical care and neuroscience naturally co-exist. Whilst we need to maintain our focus on developing nurses to encompass both specialties, we need to encourage the professional qualifica- tion of neuroscience to ensure the continual development of highly skilled neuroscience nurses.

Ash Tracey Nursing Unit Manager, Neurosurgery ICU. Royal North Shore Hospital, Sydney.

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Trauma and the Elderly: The Real Picture of Their Health May be Blurred

Mary L. King

Abstract The growing elderly population is impacting health care world-wide. Studies have shown that people over age 65 years have an increased risk of death, even with injuries that may be considered ‘minor’. Overall survival rates in the elderly due to trauma are improving with advances in medical care; however, the incidence of co-morbidities are high in this age group thereby increasing lengths of stay, risk of complications, and the disproportionate amount of medical resources when compared to younger patients. While prompt and aggressive treatment of the elderly trauma patient decreases mortality, clinicians need to be aware that age-related insufficient physical reserves may mask underlying conditions and shock states that are difficult to identify. This article will discuss the hazards related to the elderly trauma patient and special considerations for health care professionals.

Keywords: elderly, trauma, base deficit, physical reserves

Introduction related to motor vehicle accidents, violence, and falls as the leading causes of death Aging (Sheetz, 2010). Among the elderly involved in The natural progression of aging is an ex- motor vehicle crashes (MVC), blunt chest inju- pected decline in multiple body systems. As ry is a frequent pattern of injury. In addition to these changes occur slowly over time, they this, the elderly also suffer a significant num- may not be evident until the elderly person ber of potentially fatal conditions such as spi- becomes ill or injured. Decreases in brain nal cord and thorax injuries or intracranial mass, cardiopulmonary function, gastric motil- hemorrhage (Sheetz, 2010). ity, renal function, and impaired blood flow are among the major systems affected with age. In patients over age 65 years with head injury, Degeneration of joints, decreased senses of a computed tomography scan (CT scan) is sight, smell, taste, and hearing, as well as recommended to improve diagnosis and out- neuropathies, loss of skin elasticity/turgor, comes (Weber, Jablonski, & Penrod, 2010). and an approximate 15 to 30% loss of total Osteoporosis and degenerative joint disease body fat contribute to the development of predispose the elderly to fractures, specifically complications with traumatic injury (Clement, cervical spine fractures. Plain films are usual- Tennant, & Muwanga, 2010). ly not adequate for identifying a cervical frac- ture in the elderly due to pre-existing degener- In the United States (U.S.) in 2006, the num- ative changes in the vertebrae that may mask ber of elderly (age 65 years and older) was high cervical (C1, C2) fractures. A CT scan of approximately 37 million, or about 12% of the the head and neck provide better visualisation overall population. By the year 2020, the of the bony and soft tissue structures making number of elderly is expected to double diagnosis more accurate (Weber, Jablonski, & (Weber, Jablonski, & Penrod, 2010). Data Penrod, 2010). from the World Health Organization (WHO) mirrors that of the U.S. showing a global in- There is no simple formula to determine the crease in the elderly population with injuries rate of deterioration in the elderly population. Each person is unique in their aging process Questions or comments about this article should be and their response to illness or injury. The directed to Mary King, CNS, Barrow Neurological Insti- functional status of older adults contributes to tute. Phoenix, Arizona at [email protected] their ability to survive an injury. Elders with Copyright©2014 ANNA high functional ability may have more physical reserves or resiliency to recover from an inju-

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ry that is not a reflection of their chronologi- Blood pressure is one of the parameters cal age (Min, Ubhayakar, Saliba, Kelley- under consideration with regard to the elder- Quon, Morley, Hiatt, Cryer, & Rillou, 2011). ly population. In general, a systolic blood Nurses are faced with the challenges asso- pressure (SBP) value of less than 90 mmHg ciated with caring for the elderly population is considered hypotensive in a normal adult. and must be aware that ‘normal’ is not al- Recent discussions are suggesting that a ways the true picture of the elderly patients’ higher SBP of 110 - 117 mmHg may be a health. Physiologic age-related changes better definition of hypotension for patients result in a decreased ability to effectively over age 65 years (Oyetunji, Chang, Cromp- compensate for effects on body systems ton, Greene, Efron, Haut, et al., 2011). One caused by the trauma; thus the laboratory study found that one-third of the elderly pa- values and vital signs in the elderly patient tients with normal blood pressure and con- may reflect seemingly normal values when sidered ‘stable’ died within 24 hours of car- the underlying picture is that of low oxygen- diac arrest due to age-related cardiac insuf- ation, poor cardiac output, and an overall ficiency as a result of hypo-perfusion and unstable condition (Sheetz, 2010). higher peripheral vascular resistance (Oyetunji et al., 2011). Blurred Data The under-detection of trauma in the elderly This age-related insufficiency in cardiac out- may be related to the use of trauma guide- put and hypo-perfusion results in a lower lines that may not accurately identify the heart rate and masks signs and symptoms extent of injury resulting in an inappropriate of metabolic deficiencies thereby making transport of the elderly patient to a non- shock more difficult to diagnose (Chang, trauma center. Standard Advanced Trauma Tsai, Su, Huang, Chang, Tsai, 2008). Re- Life Support (ATLS) protocols pertain to the search has demonstrated a tendency to un- elderly adult, but with precautions. This is of der-compensate in shock states in the elder- particular concern to nurses in the emergen- ly resulting in insufficient oxygen delivery cy department who may receive a “stable” and ‘oxygen debt’ resulting in higher mortali- elderly patient only to have the patient ty and poor outcomes (Chang et al., 2008). quickly deteriorate on arrival due to their injuries and co-morbid conditions. The el- Elderly trauma patients with higher peripher- derly with diabetes, for example, may have al vascular resistance and lower cardiac neuropathies and sensory deficits that affect output had significantly higher mortality accurate assessment (Neville, Nemtsey, rates than younger adults or the elderly pa- Manasrah, Bricker, & Brant, 2011). tient with higher peripheral vascular resistance without a lower cardiac output. The elderly patient is very vulnerable to res- The uninjured elderly patient is able to com- piratory compromise. One or two rib frac- pensate and remain stable at rest, but the tures may have significant implications and addition of trauma greatly complicates the healthcare providers should have a high picture as their physiologic reserves are suspicion for chest injury with the so-called drained and their ability to tolerate the stress ‘minor’ falls. Half of the elderly population of the injury is depleted (Oyetunji et al., who experience a ground-level fall will suffer 2011). rib fractures resulting in respiratory distress that is complicated by the effects of aging as The usual markers, arterial base deficit and evidenced by a loss of alveolar volume and serum lactate levels, are helpful in determin- atrophy of the chest wall and muscles ing the degree of shock in the elderly and if (Farhat, Velanovich, Falvo, Horst, & Swartz, the resuscitation efforts were adequate. 2012). These elderly patients need early Base deficit is the amount of base (in mmol) intervention and airway management is criti- that is needed to bring a liter of whole arteri- cal. If the patient is not able to take deep al blood to a pH of 7.40 and is considered a breaths, has low oxygen saturations (≤ standard assessment of metabolic acidosis. 90%), early intubation should be consid- Hypotension and hemorrhage is common in ered. The consequence of intubating an trauma patients and the degree of metabolic elderly patient with co-morbidities of heart acidosis on arrival to the emergency depart- failure or chronic obstructive pulmonary dis- ment (ED) often provides a means to predict ease (COPD) may be the inability to wean outcomes (Chawla, Nader, Nelson, Govindji, from mechanical ventilation or extubation Wilson, Szlyk, et al., 2010). Base deficit is (Farhat, et al., 2012). also considered a marker for severity of illness. When oxygen delivery is inadequate 8 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

and the body converts to anaerobic metabo- underlying shock, but damage to the intesti- lism the result is an excess amount of lactate nal wall or organs may not be evident for a and hydrogen ions which combine to create long time and will be more life-threatening lactic acid in the extracellular fluid. Monitoring (Catananti & Gambassi, 2010). serum lactate levels during resuscitation may be helpful in determining the effectiveness of The elderly patient is more likely to be on the interventions as decreasing lactate levels multiple medications (polypharmacy) espe- are predictive of improved survival rates cially those that are prescribed to treat cardi- (Wilson, Grande, & Hoyt, 2013). Since hypo- opulmonary conditions. Medications such as perfusion is often imperceptible in the elderly, beta-blockers have the potential to delay rap- examining serum markers of tissue oxygen id identification of problems as they may cov- deficit (lactic acid and base deficit) can help er up a loss of physical reserves or hypo- to detect underlying problems. Lactic acid perfusion making diagnosis and treatment and base deficit are among the measures more complicated. Often the elderly patient is employed to predict mortality in the elderly on an anticoagulant medication, i.e. warfarin patient with blunt trauma but who appears or aspirin that increases their risk of haemor- stable with a ‘normal’ blood pressure rhage resulting in a necessary blood transfu- (London, 2011). sion which further increases morbidity and mortality (Soles & Tornetta, 2011). Although the range of normal values may be the same for the elderly patient as in the Pain management is a challenge as the el- younger patient, the mortality rates are high- derly patient is often sensitive to opioids and er. For example, the elderly trauma patient due to age-related decline in renal function, with a base deficit over -10 had a mortality clearance from the kidneys is slowed. A com- rate of 80% and those with base deficits in mon caveat for treating pain in the elderly is the -6 to -9 or moderate range had a 60% ‘start low and go slow’ to mitigate potential mortality rate (Chang et al., 2008). An ele- complications of respiratory depression and vated serum lactate level is a factor in occult over-sedation. Elderly patients can be safely hypo-perfusion with the clearance rate direct- treated with opioid medications as long as ly associated with mortality as the elderly pa- health care providers order appropriately for tient is less able to compensate. A lactic acid age and physical condition (Weber, Ja- level greater than 22 mg/dl (or greater than blonski, & Penrod, 2010). 2.4 mmol/L) for longer than 12 hours is also associated with high mortality (Chang et al., Elderly Scoring 2008). Thus it is important to remember that Research has been aimed at identifying the specificity is more important than sensitivity; vulnerable elderly patients in order to focus if the serum markers are positive, the test is interventions to reduce complications and likely an accurate depiction of the patients’ promote better use of resources. The strong- condition. However, if the serum markers are est predictors of survival have shown to be negative, it may only indicate the elderly pa- age, sex, co-morbid conditions, severity of tient is in an early phase of decline and im- injury, and vital signs. One tool that has been mediate interventions are required (London, used is the Injury Severity Scoring (ISS) that 2011). measures the overall severity of injury in multiple body areas; however, it should be noted Changes in the tissues and organs of an el- that some trauma associations have not rec- derly patient can mask injuries that may be ommended the ISS as a triage tool as the full more evident in the younger patient. For ex- extent of the patients’ injuries are often un- ample, thinner eardrums and a decline in known (Min et al., 2011). hearing may prevent timely diagnosis of a ruptured eardrum. Changes in membranes Another study used the Vulnerable Elders within the eye and diminished vision may de- Survey-13 (VES-13) as a determinant of lay identification of injuries to the elderly per- complications and mortality in the injured el- sons’ eyes (Catananti & Gambassi, 2010). derly. This survey has been validated in the Abdominal pain may not be felt as acutely uninjured elderly population to predict the risk due to an age-related decrease in sensitivity of functional decline and death in acute care of pain receptors. The elderly patient may and ambulatory settings. One of the benefits have a reduction in the acute nociceptive of this survey is ease-of-use. A non-clinical sensation from the deep abdominal struc- person, i.e. caregiver can answer the survey tures. Clinicians may recognise and treat an questions via telephone, if necessary. The

9 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

survey may be completed by a medical or Invasive monitoring devices may help to di- nursing professional, or a non-licensed staff agnose occult hypoperfusion states/shock member without prior knowledge of the per- and reduce end-organ failure. The use of pul- sons’ pre-existing conditions. The VES-13 is monary artery catheters (PACs) has de- based on age and functional status and uses creased in the past few years; however, in a point-scoring system for age, activities of the elderly trauma patient hemodynamic daily living (ADLs), common physical tasks, monitoring allows for direct measurement of and self-rated health status (Min et al., 2011). cardiac function (London, 2011). Evidence This survey defines disability when the indi- has shown that outcomes are improved with vidual requires help or is unable to perform a targeted approach to optimizing cardiac the activity due to health reasons. Among function and oxygen consumption. Early in- the items included in the survey are shop- terventions, i.e. admission to an intensive ping, light housework, managing money, lift- care unit (ICU), goal-directed resuscitation, ing 10 pounds (4.5 kg), reaching above and accurate monitoring are recommended shoulder level, and writing. The self-rated to improve survival rates (London, 2011). health of the individual is compared with other persons of similar age. The study using Summary the VES-13 with injured elderly patients Care of the elderly trauma patient is a chal- found a potential use existed to predict com- lenge given the age-related changes and the plications in the hospitalised elderly adult suf- interaction of their co-morbid conditions and fering from traumatic injuries when used with medications with their injuries. Medical and the ISS (Min et al., 2011). nursing interventions aimed at resuscitation and stabilization of the elderly patient should Other studies with elderly trauma patients be implemented aggressively and in a similar (age 65 years or older) found the incidence of manner to younger patients. Evaluation of complications was similar to younger trauma laboratory values, specifically lactic acid and patients, but noted a trend in patients age 45 base deficit are critical in the early stages of years or older and an increased risk of com- resuscitation and may assist the healthcare plications and mortality (Adams et al., 2012). team to more accurately determine the pa- When the researchers examined lengths of tient’s condition. What may be considered stay, end-organ failure, coagulopathies, and ‘normal’ vital signs and laboratory values in a incidence of infectious complications there younger patient are not necessarily ‘normal’ was a distinct difference between those pa- when the patient is age 65 years or older. tients younger than age 45 years and pa- Physical reserves are easily depleted and tients age 45 years and older. Interestingly, changes in pain perception, cardiac function, infectious complications seemed to peak vision, and hearing may be further complicat- around age 65 years, then decline; however ed with the addition of medications such as urinary tract infections (UTI) increased in the beta-blockers or an anticoagulant, i.e. warfa- patients who are 74-85 years old. In this age rin. Elderly trauma patients have unique group it is difficult to diagnose infection due needs that require expert knowledge and to atypical presentation such as confusion as skills to improve outcomes. An awareness of a presenting symptom instead of fever the subtly in which the elderly trauma patient (Adams et al., 2012). presents and the motivation to ‘dig deeper’ when reviewing laboratory and radiology re- Healthcare Considerations sults, and accurate (and frequent) re- Given the multiple studies on elderly trauma assessment will offer the elderly patient the patients that demonstrate higher complication best chance for a meaningful recovery. The and mortality rates the questions arise wheth- elderly trauma patient may present with a er the elderly patient should be as aggres- stable, but blurred picture of health but with sively treated as the younger (less than age an understanding of the special considera- 45 years) population. Research on elderly tions for the elderly trauma patient the trauma patients has suggested that use of healthcare team can bring that picture into the ISS should be considered for pre-existing focus. disease processes. When complications are anticipated, the elderly trauma patient should References receive aggressive care as there is evidence ACEP News. (2011). http://www.acep.org that a reasonable recovery is possible (Bar- Adams, S, Cotton, B., McGuire, M., Dipasupil, E., Pod- bielski, J., Zaharia, A., Holcomb, J. (2012). Or, Salottolo, Orlando, Mains, Bourg, & The unique pattern of complications in elderly Offner, et al., 2013). trauma patients at a Level I trauma center. Journal of Trauma and Acute Care Surgery, 10 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

72(1), 1-17. http://dx.doi.org/10.1097/ Weber, J., Jablonski, R., & Penrod, J. (2010, January). TA.0b013e318241f073 Missed opportunities: Under-detection of trau- Bar-Or, D., Salottolo, K., Orlando, A., Mains, C., Bourg, ma in elderly adults involved in motor vehicle P. & Offner, P. (2013, August). Association crashes. Journal of Emergency Nursing, 36(1), between a geriatric trauma resuscitation proto- 6-10. http://dx.doi.org/10.1016/ col using venous lactate measurements and j.jen.2009.06.008 early trauma surgeon involvement and mortali- Wilson, W., Grande, C., & Hoyt, D. (2013). Trauma: ty risk. Journal of the American Geriatrics Emergency resuscitation, perioperative anes- Society, 61, 1358-1364. Retrieved from Jour- thesia, surgical management. Retrieved from nals@OvidFullText 1420052446, 9781420052442 Catananti, C., & Gambassi, G. (2010). Pain assessment in the elderly. Surgical Oncology, 19(3), 140- 148. http://dx.doi.org/10.1016/ j.suronc.2009.11.010 Chang, W., Tsai, S., Su, Y., Huang, C., Chang, K., & Tsai, C. (2008, March). Trauma mortality factors in the elderly population. International Journal of Gerontology, 2(1), 11-17. Retrieved from Ovid database Chawla, L., Nader, A., Nelson, T., Govindji, T., Wilson, R., Szlyk, S., Seneff, M. (2010). Utilization of base deficit and reliability of base deficit as a surrogate for serum lactate in the perioperative setting. BMC Anesthesiology, 10 (16). http://dx.doi.org/http:// www.biomedcentral.com/1471-2253/10/16 Clement, N., Tennant, C., & Muwanga, C. (2010). Poly- trauma in the elderly: Predictors of the cause and time of death. Scandinavian Journal of Trauma, Resuscitation, and Emergency Medi- cine, 18(26). Retrieved from http:// creativecommons.org/licenses/by/2.0 Farhat, J., Velanovich, V., Falvo, A., Horst, H., & Swartz, A. (2012). Are the frail destined to fall? Fraility index as predictor of surgical morbidity and mortality in the elderly. Journal of Trauma and Acute Care Surgery, 72(6), 1526-1530. Re- trieved from Ovid database London, S. (2011). Geriatric patients fare worse after trauma. Retrieved from https://www.acep.org/ Content.aspx?id=79746 Min, L., Ubhayakar, N., Saliba, D., Kelley-Quon, L., Mor- ley, E., Hiatt, J., & Tillou, A. (2011). The vulnerable elders survey-13 predicts hospital complications and mortality in older adults with traumatic injury: A pilot study. Journal of the American Geriatrics Society, 59, 1471-1476. http://dx.doi.org/10.111/j.1532- 5415.2011.03493.x Neville, A., Nemtsey, D., Manasrah, R., Bricker, S., & Brant, A. (2011, Oct). Mortality risk stratifica- tion in elderly trauma patients based on initial arterial lactate and base deficit levels. The American Surgeon, 77(10), 1337-1341. Re- trieved from ProQuest Central Oyetunji, T., Chang, D., Crompton, J., Greene, W., Efron, D., Haut, E., Haider, A. (2011, July). Redefining hypotension in the elderly: Normo- tension is not reassuring. Arch Surg. 2011, 146(7), 865-869. Retrieved from http:// archsurg.jamanetwork.com/ Sheetz, L. (2010). Prehospital factors associated with severe injury in older adults. Injury, 41(9), 886- 893. http://dx.doi.org/10.1016/ j.injury.2010.05.18 Soles, G., & Tornetta, P. (2011). Multiple trauma in the elderly: New management perspectives. Jour- nal of Orthopedic Trauma, 25, S61-66. Re- trieved from www.jorthotrauma.com/ovid database Somes, J., & Donatelli, N. (2014). Considerations when explosions involve geriatric patients. Journal of Emergency Nursing, 40, 70-81. http:// dx.doi.org/http://dx.doi.org/10.1016/ j.jen.2013.09.005 11 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 Apathy a common feature in many neurological conditions: care and management

Mary E. Braine

Abstract Apathy is associated with many neurological conditions including traumatic brain injury (TBI), stroke, Parkinson’s disease and dementia and is primarily a dysfunction of the frontal-subcortical circuit. Despite being common, it is often under recognised, not viewed as problematic, or misdiagnosed as depression. Various definitions of apathy are offered in the literature along with various assessment tools and prevalence amongst various neurological conditions. This paper discusses the diagnosis criteria along with the importance of an accurate and timely assessment in aiding healthcare practitioners in caring for patients with apathy, and can be a useful starting point for rehabilitative efforts. Apathy can be profoundly disabling for both the patient and their caregivers, and is associated with a number of adverse outcomes such as a negative impact on daily life and quality of life, potential for poorer rehabilitation outcomes and increased burden on caregivers. Areas for further research and development are also presented.

Keywords: Apathy, assessment, goal directed behaviours, traumatic brain injury, Parkinson’s disease, stroke, Alzheimer’s disease.

Introduction lence in many neurological conditions but Apathy is an important, debilitating and dis- because of the negative impact on both the tressing behavioural symptom that occurs in patient and their families and carers. This many neurological conditions and neuropsy- paper will discuss the various definitions and chiatric disorders. Despite its frequency, apa- criteria of apathy, describe assessment tools thy is often unrecognised, untreated and mis- and review associated neurological disorders. diagnosed and not viewed as a problem. Ap- The adverse effects on both the patient and athy may also occur in association with a va- the family are provided along with various riety of other clinical problems and may com- treatment options. plicate both assessment and treatment (Marin, 1990). Apathy is a significant and Definition and diagnostic criteria common neurobehavioural sequela following The term ‘apathy’ according to English lan- traumatic brain injury (Kant, Duffy & Pivovar- guage dictionaries is defined as a lack of in- nik, 1998) and is a common feature of sub- terest or concern. The word apathy consists cortical pathologies such as Parkinson’s dis- of the prefix ‘a’ meaning without and ‘pathos’ ease, Huntington’s chorea and Progressive the Greek word for passion. Apathy is there- Supranuclear Palsy (PSP). It is also a com- fore often described as a lack of emotion or mon behavioural disturbance following stroke interest. Nurses who come across apathetic (Mayo, Fellows, Scott. Cameron & Wood- patients may describe them as ‘lacking a Dauphinee, 2009) and in neurodegenerative spark’ or ‘drive’. However, consensus on its pathologies such as dementia (Mendez, Lau- definition in the field is still absent. terbach & Sampson, 2008). Despite apathy being prominent in many neurological and Two researchers, Robert Marin and Sergio psychiatric disorders, there is a paucity of Starkstein, have led the work in suggesting a nursing literature on the care and manage- diagnostic criterion to define this condition. ment for a patient with apathy. This is sur- The work of the American psychiatrist Robert prising not only because of its high preva- Marin in the early 1990’s initially led the interest, describing it as a syndrome of Questions or comments about this article should be di- “...diminished motivation and goal directed rected to Mary Braine, Senior Lecturer, School of Nurs- behaviour, not attributed to diminished level ing, Midwifery, Social Work and Social Sciences of consciousness, general cognitive impair- University of Salford. England. [email protected] ment or emotional distress.…therefore a state Copyright©2014 ANNA of primary motivational impairment” (Marin,

12 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

p.12:1990). According to Marin, Biedrzycki & resulted in a revised consensus criteria on Firinciogullari, (1991) apathy is a disorder of the diagnostic criteria for apathy (DCA) in drive and motivation, and characterised by Parkinson’s and Alzheimer’s disease three main domains: (Robert, Onyike, Leentjens, Dujardin, Aalten & Sarkstein, 2009) see Table 1. However, to x diminished goal-directed behaviours date there still remains no consensus on the lack of interest or concern for social diagnostic criteria for apathy as a syndrome, and personal activities and is mostly defined as a disorder of moti- x lack of responsiveness to positive and vation. negative events (emotional indifference) Despite the DCA offered by Robert et al., (2009), international documentations only Marin emphasised that the lack of motivation refer to apathy as a symptom of several con- is primary, and not secondary to intellectual ditions. The International Classification of impairment, emotional distress (e.g., depres- Diseases ICD-10 (WHO, 2010) refers to apa- sion), or impaired consciousness (e.g., deliri- thy under symptoms and signs involving um). Other researchers have conceptualised emotional state (R45.3 2010). More specifi- apathy as an absence of responsiveness to cally the ICD-10 Classification of Mental and stimuli (external or internal) and character- Behavioural Disorders (1992) make several ised by a lack of self-initiated action (Stuss, references to the apathy as a symptom of van Reekum & Murphy, 2000). Whilst Levy & several disorders; dementia, personality dis- Dubios (2006) conceptualised apathy is a “… orders, acute intoxication from substances quantitative reduction of self-generated vol- e.g. sedative-hypnotic drugs and schizophre- untary and purposeful behaviours” (Levy & nia. In the updated American Diagnostic and Dubois p.916:2006). The Australian psychia- Statistical Manual of Mental Disorders Fifth trist Sergio Starkstein (2000) modified and Edition (DSM-V-TR, 2013) apathy is only organised apathy into a standardised set of referred to under the newly named entity diagnostic criteria and specified the following Mild and Major neurocognitive disorder core features: diminished motivation, initia- (NCD). tive and interest and blunting of emotions. This criterion has recently been modified by Several experts within the field also argue a task force involving the French Association that apathy sits on a continuum of disorders for Biological Psychiatry, the European Psy- of decreased motivation with apathy being chiatric Association, and the European Alz- the less severe manifestation though to abu- heimer's Disease Consortium in 2008 and lia and ending with akinetic mutism, the most severe form (Marin 1990; Marin & Wilkosz

For a diagnosis of apathy the patient should fulfil the criteria A,B,C and D A Loss of or diminished motivation

B Presence for at least one symptom in at least two or three domains for a period of four week Domain B1 Lack of self-initiated behaviours Loss of environmental stimulated behaviours Domain B2 Loss of spontaneous ideas and curiosity Loss of environmental stimulated ideas Domain B3 Loss of spontaneous emotions Lack of emotional responsively to positive or negative events

C These symptoms A-B cause clinically significant impairment in social, occupational or other important functioning

D These symptoms A-B are not explained or due to physical disability, diminished levels of consciousness or direct physiological effects of substances (e.g., medication)

Table 1: (Above) Diagnostic criterion for apathy. (Robert et al., 2009) 13 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

Mild Degrees of severity of apathy Severe

Apathy Abulia Akinetic mutism

x Uninterested in their sur- x From the Greek ‘a- boule’ x A state of avolition in roundings meaning ‘without will’ which the patient has no x Unmotivated and lacking x The inability to initiate desire to think, speak or in enthusiasm and spon- behaviour lack of will or move. taneity in day-to-day ac- initiative and can be seen x Total absence of sponta- tivities such as chores as a disorder of diminished neous behaviour and and activities of daily motivation (DDM). speech, occurring in the living x Often used interchangea- presence of preserved x Diminished emotional bly with apathy but is re- visual tracking reactivity, and dimin- garded as the most ex- ished initiative treme form of apathy. x Lack of ability to decide Characterised by the inability to communicate, initiate, and self- regulate purposeful behaviours

Table 2: (Above) Apathy – degree of severity and symptoms

2005) see Table 2. cations have also been implicated in apathy and need to be ruled out. The commonly pre- Assessment scribed antidepressants selective serotonin A good starting point in the assessment pro- reuptake inhibitors (SSRI’s) have been re- cess, when caring for patients who might be ported to affect the balance between seroto- vulnerable to apathy, is for the nurse to con- nin and dopamine levels in the brain resulting sider some key questions that may identify in SRRI-induced apathy or as sometimes the need for a more detailed assessment referred to SSRI- induced indifference (Settle such as: 1998; Wongpakaran, van Reekum, x Is there a loss of interest or enthusi- Wongpakaran & Clarke, 2007; Sansone & asm in the world around him/her? Sansone 2010; Zahodne, Bernal-Pacheco & x Does he/she lack motivation for start- Okun, 2012). The effect of SSRI's exposure ing new activities? E.g. are they con- on the risk for apathy has not been well stud- tent just to sit in their chair or lie in their ied. This adverse effect is insidious in its on- bed? set, and dose-dependent i.e. the higher the x Is he/she more difficult to engage in dose the increased change of apathy. How- conversation or in doing activities of ever, it can be completely resolved with a daily living? dose reduction or discontinuation of the SSRI x Has their appearance changed e.g. (Sansone & Sansone 2010). Given that have they lost interest in their appear- SRRI’s are commonly-prescribed antidepres- ance? sants in patients with Parkinson’s Disease, in x Has the patient become indifferent and which the prevalence of depression is report- "cold" emotionally to events that would ed to vary from 2.7-90% (Rejners, Ehrt, We- normally give pleasure or cause for ber, Aarsland & Leentjens, 2008), this is an concern? importance consideration in the assessment process. Healthcare professionals need to be It is also important to rule out causative fac- aware, when caring for neuroscience patients tors in the initial assessment process by car- who are depressed and prescribed SSRI, of rying out a detailed patient history which in- this unwanted clinical side effect, and edu- cludes the pre-morbid personality and behav- cate the patients as well as their families to iours, and a thorough medical examination to the effect. Other notable medications which determine whether the apathy is as a result of can induce an apathetic state include neuro- psychosocial, medical or pharmacological leptics such as haloperidol and chlorproma- causative factors. A number of medical condi- zine. Neuroleptics are known to cause seri- tions may present with an apathy-like syn- ous disturbance of the signalling mechanism drome for example hyperthyroidism in the in the frontal limbic system and consequen- elderly (Lahey 1937). In addition some medi- tially a ‘blunting of emotional arousal’.

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Major Depression Disorder Apathy Syndrome x Presence of five of nine diagnostic symptoms with a minimum duration of 2 weeks x Loss of interest in daily life, situation or x Depressed mood e.g. sadness stimuli that would normally be motivating x Loss of interest or feeling of pleasure x Loss of motivation x Changes in weight or appetite x Lacking initiation and ability to sustain x Insomnia or hypersomnia activities x Psychomotor retardation or agitation x Reduced emotional engagement and flat- x Loss of energy or fatigue tened affect x Feelings of worthlessness/guilt x Blunted emotions (response to emotional x Trouble making decisions/thinking or con- situations poor or short lived) centrating x Indifference x Recurrent thoughts of death or suicide, or a suicide attempt

Table 3: (Above) Differences in the symptoms of depression and apathy symptoms (American Association of Psychiatry (DSM-5) 2013; Levy et al., 1998; Levy and Dubois, 2006) It is important to understand that the assess- resulting from brain-related pathology. Stark- ment process may be complicated by the stein, Mayberg, Preziosi, Andrezejewski, overlap between the symptoms of apathy Leiguarda & Robinson, (1992) later devel- and depression as this may cause diagnostic oped an abridged and modified version of the confusion (Levy, Cummings, Fairbanks, Mas- AES and publishing the Apathy Scale (AS). terman, Miller & Craig, 1998). Levy et al Several other scales have been developed (1998) also suggest that much of this overlap since then most have been validated in Alz- is compounded by the use of depression heimer’s disease and Parkinson’s disease. scales which contain apathy items. It is im- Given that in the acute stage of injury or ill- portant to understand that apathy and de- ness neurological patients are often identified pression may coexist, and that apathy is reas being at risk of developing depression and lated to, but different from major depression apathy it would seem pertinent for healthcare from the viewpoint of its causes and treat- professionals to assess for these conditions. ment. There is also evidence that separate Early identification may minimise the conse- brain circuits are involved in apathy and de- quences of misdiagnosis, under treatment or pression, adding to the view that the two are overlooking apathy and aid in the caring of different neuropsychiatric disorders. Table 3 the patient with apathy. illustrates the main differences between the two. Neurobiology Although the neurobiological basis of apathy Despite the recent consensus criteria for di- is not fully understood, research has shown agnosis there is no gold standard to assess several neuronal networks that are associat- apathy. However, a number of assessment ed with apathy. These networks form parallel tools have been developed specifically to segregated specific circuits connecting se- rate the severity of apathy, and there are lected cortical areas in the frontal lobes with some instruments that assess apathy as part the basal ganglia and thalamus (Levy & Du- of a broader behavioural symptom assess- bois, 2006; Bonelli & Cummings, 2007). ment see Table 4. Due to the unavailability of These circuits are also referred to as the pre- structured instruments to diagnose apathy frontal cortical - basal ganglia functional axis these severity assessment tools have been (Levy & Dubois, 2006). According to Bonelli used to diagnose apathy using arbitrary cut & Cummings (2007) three main frontal- off scores on the various severity rating subcortical circuits are implicated in apathy scales. Indeed most definitions of apathy these include: have been derived from severity assessment scores and have helped facilitate and pro- x dorsolateral prefrontal circuit allows mote the research into apathy over recent the organisation of information to facili- years. tate a response, x anterior cingulate circuit is required for Marin (1991) was the first to develop a spe- motivated behaviour, cific scale, the Apathy Evaluation Scale x orbitofrontal circuit allows the integra- (AES), as a method for measuring apathy tion of limbic and emotional infor-

15 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 Assessment Scale Authors Application in clinical practice Apathy Evaluation Scale (AES) Marin et al. The reliability and validity of this scale in different 18-item (1991) patient groups has been extensively evaluated in- Multiple rater sources cluding; Parkinson’s disease, Alzheimer’s disease, AES-I (informant) Stroke & Traumatic Brain Injury. AES-S (self-rated) AES-C (clinician) The Apathy Scale (Starkstein Starkstein et Generally reliable and valid instrument, especially Apathy Scale (SAS)) 14-item al. (1992) for use in patients with Parkinson’s disease, Alz- Scored by the patient’s relative or heimer’s disease or Stroke caregiver Apathy Inventory (AI) Robert et al. Specifically designed and validated to assess apathy Caregiver and patient-based ver- (2002) in Parkinson’s disease. High internal consistency, sions item reliability, and inter-rater reliability in patient with cognitive impairment, Parkinson’s disease, Alzheimer’s disease (Robert et al. 2002)

Lille Apathy Rating Scale Sockeel et al. Validated in Parkinson’s disease & has the ability (LARS) Semi-structured patient (2006) to distinguish apathy from depression interview with 33-item divided up into 9 domains. LARS-i caregiv- Zahodne et al. er version (2009)

Neuropsychiatric Inventory (NPI) Cummings et Widely used and developed to evaluate behavioural 12 subscales structured interview al. (1994) dysfunction including apathy in dementia patients conducted by the clinician. Developed to assess the presence and severity of behavioural specific domains; apathy being one of 10 Administered to caregivers Frontal Systems Behaviour Scale Grace and A reliable and valid measure developed as a meas- (FrSBe) 46-item with three sub- Malloy (2001) ure of behaviour associated with damage to the scales: frontal systems of the brain. apathy (14 items), disinhibition (15 items), executive dysfunction (17 items) Versions include self-rating or rating by a caregiver

Table 4: (Above) Apathy measurement/assessment tools

mation into behavioural responses. role in our emotions was later elaborated by Many researchers have focused on these the American physician, James Papez in fronto-subcortical circuits, given the exten- 1937 in the seminal paper titled ‘A proposed sive interconnection between basal ganglia mechanism of emotion’. Papez proposed and the prefrontal cortex to explain behav- that these limbic structures formed a circuit ioural abnormalities such as apathy. It is the or emotional system between the hypothala- dysfunction of these circuits that is thought to mus and the cerebral cortex and became give rise to apathy. Neurobiological history known as the Papez circuit, and was thought can provide some insight and understanding to be related to the regulation of emotion. of the cingulate cortex and its role in motiva- Papez (1937) also noted that tumours press- tion. In 1879 Pierre Broca, a French physi- ing on the cingulate cortex produced “loss of cian, surgeon, and neuroanatomist, applied spontaneity in emotion, thought and activity.” the term “limbic” (from the Latin limbus for The limbic lobe or circuit largest component border) to the curved rim of the cortex which is the cingulate cortex and functionally forms includes the cingulate and the parahippo- part of the limbic system. The anterior cingu- campal gyri. This area of the brain and its late cortex (ACC), the frontal part of the cin-

16 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

gulate cortex, is also referred to as the cingu- (Mendez et al., 2008). The prevalence of apa- late circuit (Habib, 2004; Bonelli & Cummings, thy in Alzheimer’s disease ranges from 25 to 2007), and is situated deep in the cerebrum 88% with a trend to increase with disease and surrounds the corpus callosum. More severity (Starkstein, Jorge, Mizrahi & Robin- recently studies using modern imaging tech- son, 2006). In light of the aging population niques have demonstrated the involvement of worldwide, global estimates of Alzheimer’s the anterior cingulate cortex (ACC) in motiva- Disease, the commonest subtype of demen- tion and associated with apathy (Zamboni, tia, is generally estimated to increase from the Huey, Krueger, Nichelli & Grafman, 2008), estimated 35.6 million to 65.7million in 2030, and decreased spontaneous goal-directed. and by 2050, to 115.4 million (World Alz- heimer Disease 2009). Moreover within this Prevalence of apathy in neurological population apathy is frequently seen as an conditions early presentation and often described before Despite being under diagnosed and untreated the memory deficits become apparent (Bruen, apathy is particularly prominent in neurologi- McGeown, Shanks & Venneril, 2008). This cal disorders that affect the frontal lobes, the raises the issue that apathy may be an early basal ganglia, or the links between them. diagnostic indicator of the disease. These include acquired brain injury, in particular Traumatic Brain Injury involving the Apathy is one of the most prevalent neurobe- frontal lobes, stroke, subcortical pathologies havioral symptoms in Huntington's disease mainly affecting the basal ganglia, such as (HD), occurring in approximately 70% of the Parkinson’s disease (PD), Huntington’s cho- symptomatic HD population (Krishnamoorthy rea and Progressive Supranuclear Palsy & Crauford, 2011) and occurs in 91% in Pro- (PSP), and dementias such as Alzheimer’s gressive Supranuclear Palsy (PSP) (Litvan, disease, vascular dementia and fronto- Mega, Cummings & Fairbanks, 1996). temporal dementia. Individuals suffering from acquired brain injury Although there are no authoritative estimates in particular stroke and traumatic brain injury of its frequency in the general population commonly manifest apathy. In traumatic brain some idea can be gleaned by looking at its injury (TBI), varying prevalence rates are re- prevalence in specific neurological conditions. ported. Kant, Duffy & Pivovarnik, (1998) one Studies have demonstrated that in sub- of the first researchers to study apathy in the cortical disorders apathy results from the dis- TBI population reported a rate of 71.08% us- ruption of ascending and descending projec- ing a self-report version of the AES, whereas tions to and from the frontal lobes. It occurs in Marin & Wilkosz (2005) reported that some 20–70% of patients with Parkinson’s disease 50% of adults suffering from TBI are affected (PD) (Pedersen, Alves, Larsen & Aarsland, with apathy. Similarly van Reekum, Stuss & 2009a; Oguru, Tachibana, Toda, Okuda & Ostrander, (2005) reported rates of 61.4%, Oka, 2010), depending upon the instrument whereas Al-Adawi, Dorvio, Burke, Huynh, Ja- for assessment and the samples examined. cob, Knight, Shah & Al-Hussaimi, (2004) According to available statistics, an estimated Omanian study reported much lower rates of 6.3 million people have Parkinson’s worldwide 20%. They attributed this to the sample being (Baker & Graham 2004). Thus the number of relatively older than survivors in other popula- Parkinsonian patients with apathy could be tions and the sample was small and heteroge- between 4.41 and 1.26 million. Furthermore neous with variations in duration and severity this figure is likely to increase given that the of TBI. number of people over age 60 is increasing worldwide and unless a prevention or cure is Stroke survivors are commonly described as identified, the number of people with PD will having lost interest, seem unmotivated and also grow substantially over the next 25 unable to get going. Whilst the prevalence of years. Apathy has been shown to be associ- depression post-stroke has been extensively ated with or without depressive symptoms in studied few have studied apathy. Existing PD and is associated with increased severity studies yields a varying prevalent rate of apa- of depression (Pedersen, Larsen, Alves & thy post-stroke between 20% (Mayo et al., Aarsland, 2009b). 2009) and 55% (Yamagata, Yamaguchi & Kobayashi, 2004). In Mayo and col- Similarly, apathy is also prevalent and persis- leagues’ (2009) Canadian longitudinal study tent in the dementia population in particular the authors found that the extent of apathetic fronto-temporal dementia (FTD with reported behaviour remained fairly stable over the first prevalence rates ranging from 62 to 89% year after stroke. Similarly Santa, Sugimori, 17 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

Kusuda, Yamishita, Ibayashi & Iida, (2008) be of significance in the field of neuroscience, found 20% of patients with apathy by patient given that medication adherence is a crucial report but also suggested that apathy tends to area of many neurological conditions such as occur more frequently in first time stroke pain Parkinson’s disease and is warrants inves- tients who are older and more cognitively im- tigation. paired. In Hama, Yamishita, Shigenobu et al., (2007) Japanese study a higher rate of apa- Apathy can interfere with a patient’s ability to thy 40% was reported by patients, but a lower fulfil their full recovery potential and act as a rate of 19 % was reported by structured inter- barrier to participating in rehabilitation activi- view of the caregiver. A more recent meta- ties (Resnick, Zimmerman, Magaziner & analysis of 24 studies found that apathy oc- Adelman, 1998). In patients with TBI apathy curs in 29.5 - 40.2 % of patients after stroke, has also been observed to contribute to the and is typically associated with worse disabil- unsatisfactory integration into home and com- ity and enduring cognitive deficits (van Dalen munity activities (Cattelani, Roberti & Lombar- et al. 2013). In stroke, apathy has been found di, 2008). Whilst in post-stroke patients apa- to be associated with damage or reduced thy is also reported to lead to less recovery in blood flow to prefrontal cortex and basal gan- activities of daily living (ADL’s) (Mikami, Ri- glia (Okada, Kobayashi, Yamagata, cardo, Moser, Jang & Robinson, 2013), and a Takahashi & Yamaguchui, 1997). Other re- predictor of poor functional recovery (Hama et searchers have demonstrated reduced real., 2007). In a more recent American study gional cerebral blood flow in the basal ganglia Harris and colleagues (2014) found that post- for the apathetic patient when compared with stroke apathy resulted in an increased the non-apathetic patients (Onoda, Kuroda, chance of being admitted to a nursing home Yamamoto, Abe, Oguro, Nagai, Bokura & and scoring below the mean FIM score. Yamaguchi, 2011). Apathy not only impacts negatively on the The variation in prevalence figures may in patient but also on their family and carers. part be attributed to the utilisation of varying Apathy can disrupt the quality of life for both assessment measurements across the stud- patients and caregivers (van Reekum et al., ies, sample sizes and study population. In 2005). Gerritsen et al. (2005) study found that addition studies that rely on the apathetic pa- apathy in nursing home residents, with rela- tient rating of their severity of apathy may tively good cognition, was found to be associ- underestimate their severity. Within neurosci- ated with lower quality of life (QoL). Howev- ence care this may be an important consider- er, in those with severe cognitive impairment ation as many stroke and TBI survivors lack apathetic behaviour was associated with high cognitive abilities or insight to be able to rec- QoL. Apathy has also been found to be asso- ognise that they have feelings or behaviours ciated with increased caregiver burden and that can be attributed to apathy. reported to be a significant source of caregiver stress. In a UK study by Leroi et al (2012) Adverse effects a significantly greater burden was seen in Several studies have documented the ad- carers of PD participants with apathy when verse consequences that apathy can have on compared with carers of PD participants with- interpersonal relationships, daily functioning out such behavioural disturbances. In con- and recovery outcomes. Apathy may also trast, caregivers of those with Alzheimer’s have a direct adverse impact on self-care disease who suffer apathy found apathy to be leading to failure to recognise early symptoms less burdensome than more active behav- of impending medical problems. For example iours such as executive dysfunction and disin- in a prospective multi-centre study of 686 Alz- hibition (Davis & Tremont 2007). heimer’s disease suffers, the presence of apathy was significantly associated with poor The full impact of apathy on families and car- nutritional status (Benoit, Andrieu & ers is unknown. Additional research is espe- Lechowski, 2008). Apathy has also been cially warranted to evaluate the impact due to shown, although limited, to impact negatively societal increases in both care giving and the on medication adherence. In the American prevalence of apathy in many neurological study involving 81 male patients with diabetes disorders. Padala, Desouza, Almeida et al., (2008), showed that 61% were apathetic and that Nursing Care they were less likely to adhere to exercise Once the reversible causes of apathy have plan or insulin regimen. Although no studies been excluded, and the diagnosis of apathy have been conducted on the subject this may has been made, interventions need to be con- 18 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

sidered to prevent any adverse consequenc- behaviour. Interventions should also be spe- es. Despite the increasing number of pub- cifically and appropriately geared to the limi- lished paper on prevalence rates of apathy in tations of the patient’s condition. many neurological conditions there is very little published literature on the treatment or Several studies have demonstrated the im- management of apathy. Nurses can contrib- portance of the environment in the manage- ute to the management of apathy in a num- ment of apathy, although this is mainly found ber of ways. By adopting an individualist ap- in the literature on dementia care. Under proach to their management, centred on the stimulation or a lack of appropriate stimula- individual behaviour that involves the family tion in such an environment may lead to and caregiver, then the apathy potentially is boredom in older adults, particularly for those treatable. Nurses’ interventions should be with dementia (van Reekum et al., 2005). tailored initially to the suspected causes of Multisensory stimulation also referred to apathy, which have been identified via a de- Snoezelen therapy has been shown to re- tailed assessment. Furthermore regular as- duce apathy compared with activity therapy sessment may be useful as a means of moni- or no therapy in people with moderate to se- toring especially in rehabilitation. vere dementia (Verkaik et al., 2005). Holmes, Knights, Dean et al. (2006) demonstrated Many of the non-pharmacological interven- that live music had a positive engagement tions are subject to a limited number of stud- effect in dementia patients with apathy. The ies and most have been carried out in the usefulness of stimulation such as multisenso- dementia population. Brodaty & Burns (2012) ry stimulation and music in other neurological argue that despite the lack of methodological disorders is unknown. A systematic review by rigor, non-pharmacological interventions in Lane-Brown & Tate (2009) evaluated the ef- dementia patients have the potential to re- fectiveness of non-pharmacological interven- duce apathy. They further argue in their sys- tions available in the TBI population and con- tematic review that therapeutic activities, par- cluded that there was not robust evidence for ticularly those provided individually such as the interventions reviewed. creative activities, cooking and gardening have the best available evidence. Critical to There is very little evidence to guide the the implementation of any non- pharmacological management of apathy. pharmacological approach for an apathetic Drijgers, Aalten, Winogrodzka, Verhey & patient is the development of an individual- Leentjens, (2009), in their systematic review ised plan that involves input from the multi- concluded that there was insufficient evi- disciplinary team. dence for the pharmacological treatment of apathy in patients with neurodegenerative As apathy may result in a lack of or failure to disease. As the dopaminergic system is im- engage in goal direct-behaviour, motivation is plicated in the pathophysiology of apathy this a very important facet of the recovery and has increasingly been the target for modula- rehabilitation process. If apathy is believed to tion in the treatment of motivational deficit be related to a lack of motivation, factors in- behaviours such as apathy. fluencing the appraisal of the situation, such as past experiences and beliefs and previous Nurses need to acknowledge the role the interests may help to predict individual strate- family and carers can play in caring for the gies for engaging with the apathetic patient. apathetic patient. By being aware of the im- This may involve providing external clues and pact of apathy on families and carers nurses prompting the patient. Exploring what the can help to improve support and coping patient wants and why they want it, and ex- mechanisms especially in the in-patient plaining what might realistically be expected phrase when much of the family and social may assist in establishing attainable goals. adaptation occurs. Helping the family and Although the apathetic patient may not be caregiver understand their family members’ able to initiate activities the nurse can help by behaviour, (i.e that they are not just being initiating or suggesting an activity that the lazy or difficult) may prevent resentment and patient enjoys and encouraging them to par- difficult interactions between themselves and ticipate. Nurses may, if available, want to the patient. Additionally, it may also help to consider group activities rather than activities alleviate caregiver frustration, stress and bur- that the patient is expected to do on their den. Family and carer involvement may also own. In addition offering choices to enhance be effective in reducing apathy and increase self-determination and rewards frequently, functioning and outcomes of the patient. especially early on, may help to sustain the Nurses can play a crucial role in involving 19 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

and educating family members and caregiv- recognition that apathy is present in signifi- ers about apathy. This should include: provid- cant proportions in the neuroscience popula- ing information about their family members’ tion, by participating in the assessment pro- condition and apathetic behaviour; involving cess and contributing to the patients’ man- the family and carers in multi-disciplinary agement through non-pharmacological inter- team meetings, goal setting and decision- ventions and education and involvement of making; and targeting interventions that in- the family and caregivers. volve the family and carers. References The apathetic patient may depend more on Al-Adawi S, Dorvlo ASS, Burke DT, Huynh CC, Jacob L, their families and carers to provide care, re- Knight R, Shah MS, Al-Hussaimi AA (2004) Apa thy and Depression in Cross-Cultural Survivors sulting in a loss of skill and function in activi- of Traumatic Brain Injury. Journal of Neuropsy ties of daily living. Thus it is vitally important chiatry Clinical Neuroscience, Vol.16, No 4: pp. when planning care activities that the family 435-442. or caregiver does not cause more apathy American Psychiatric Association (2013) Diagnostic and Statistical Manual of Mental Disorders, Fifth seeking active engagement, rather they pro- Edition (DSM-5). Washington, DC: American mote physical and cognitive stimulation. Psychiatric Association. Baker MG, Graham L. (2004) ‘The journey: Parkinson’s In summary apathy care and management disease.’ British Medical Journal, Vol. 329, No 7466: pp.611-614. requires a multidisciplinary approach that Benoit M, Andrieu S, Lechowski L, et al. (2008) Apathy considers careful assessment, environmental and depression in Alzheimer’s disease are asso modifications, and individual interventions ciated with functional deficit and psychotropic that consider the apathetic person as well as prescription. International Journal of Geriatric Psychiatry, Vol. 23: pp. 409–414. their family and carers. Although non- Bonelli RM, Cummings JL (2007) Frontal-subcortical pharmacological interventions may be of lim- circuitry and behavior. Dialogues Clinical Neuro ited effectiveness they are low risk and science, Vol. 9, No 2: pp.141-51. should be a considered in the nursing man- Bruen PD, McGeown WJ, Shanks MF, Venneri1 A (2008) Neuroanatomical correlates of neuropsy agement of apathy. chiatric symptoms in Alzheimer's disease. Brain, Vol.131 No 9: pp.2455-2463. Conclusion Brodaty H, Burns K (2012) Nonpharmacologcial Man Apathy is a debilitating, under-recognised agement of Apathy in Dementia: A Systematic Review. The American Journal of Geriatric Psy and under-studied multidimensional syn- chiatry, Vol. 20, No 7: pp. 549-564. drome that affects many neurological condi- Cattelani R, Roberti R, Lombardi F (2008) Adverse ef tions, and results in functional impairment fects of apathy and neurobehavioral deficits on among patients and causes stress among the community integration of traumatic brain injury subjects. European Journal of Physical their caregivers. Despite the prevalence of Rehabilitation Medicine, Vol. 44, No 3: pp.245– apathy in varying neurological conditions 251. there remains a lack of a clear definition and Cummings JL, Mega M, Gray K, Rosenberg-Thompson a consensus on diagnostic standards. A vari- S, Carusi DA, Gornbein J. (1994) The Neuropsy chiatric Inventory: comprehensive assessment of ety of assessment tools have been devel- psychopathology in dementia. Neurology, Vol. oped over the years but there is no gold 44, No 12: pp.2308–2314. standard by which to assess apathy. Early Davis JD, Tremont G (2007) Impact of Frontal Systems identification of patients at risk of developing Behavioral Functioning in Dementia on Caregiv er Burden. Journal of Neuropsychiatry Clinical apathy through timely and accurate assess- Neuroscience, Vol. 19, No1: pp.43-49. ment and the development of targeted inter- Drijgers RL, Aalten P, Winogrodzka A, Verhey FR, ventions may help to prevent misdiagnosis, Leentjens AF (2009) Pharmacological treatment improve long-term outcomes and the quality of apathy in neurodegenerative diseases: a sys tematic review. Dementia and geriatric cognitive of life for the patient and their carers. There disorders, Vol. 28, No1: pp.13-22 is a paucity of research to guide healthcare Gerritsen DL, Jongenelis K, Steverink N, Ooms ME, professionals in the management and treat- Ribbe MW (2005) Down and drowsy? Do apa ment of apathy. High quality, methodologi- thetic nursing home residents experience low quality of life? Aging and Mental Heath, Vol. 9. cally rigorous treatment studies of apathy is No 2: pp.135-141 required along with studies that validate the Grace J Malloy PF (2001) Frontal Systems Behavior use of an assessment scale across varying Scale Professional Manual Lutz FL. Psycholog pathological conditions. Given the importance cial Assessment Resources. Habib M (2004) Athymhormia and disorders of motiva of motivation in achieving goals in rehabilitation in Basal Ganglia disease. Journal of tion and during the recovery process follow- Neuropsychiatry Clinical Neuroscience, Vol. 16 ing illness and injury, apathy deserves more No 4: pp.509-524. focused attention. Neuroscience nurses can Hama S, Yamashita H, Shigenobu M, et al. (2007) De pression or apathy and functional recovery after play a major part in this process by early 20 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

stroke. International Journal of Geriatric Psychia maguchui S. (1997) Poststroke Apahty and Re try, Vol. 22, No 10: pp.1046–1051. gional Cerebral Blood Flow. Stroke, Vol. 28: Harris A L, Elder J, Schiff ND, Victor JD and Goldfine AM pp.2437-2441 (2014) Post-Stroke Apathy and Hyersomnia Onoda K, Kuroda Y, Yamamoto Y, Abe S, Oguro H, Lead to Worse Outcomes from Acute Rehabilita Nagai A, Bokura H, Yamaguchi S. (2011) tion. Translational Stroke Research, Vol. 5: Post-Stroke Apathy and Hypoperfusion in Basal pp.292-300. Ganglia: SPECT Study. Cerebrovascular Holmes C, Knights A, Dean C, et al. (2006) Keep music Disease, Vol.31, No1: pp.6- 11. live: Music and the alleviation of apathy in de Padala PR, Desouza CV, Almeida S, et al. (2008) The mentia subjects. International Psychogeriatric, impact of apathy on glycaemic control in diabe Vol. 18: pp.623–630. tes: A cross-sectional study. Diabetes Research Kant R, Duffy JD, Pivovarnik A (1998) Prevalence of and Clinical Practice, Vol. 79: pp.37–41. apathy following head injury. Brain Injury, Vol. 12 Papez J. (1937) A proposed mechanism of emotion. No1: pp.87-92. Arch. Neurol. Psychiatry, Vol. 38: pp.725-743. Krishnamoorthy A1, Craufurd D. (2011) Treatment of Pedersen KF, , Alves G, Larsen JP, Aarsland D (2009a) Apathy in Huntington's Disease and Other Move Occurrence and risk factors for apathy in Parkin ment Disorders. Current Treatment Options in son Disease: a four year prospective longitudinal Neurology, Vol.13 No5: pp.508-19. study. Journal of Neurology, Neurosurgery and Lahey FA. (1937) Non activated (apathetic) type of hy Psychiatry, Vol. 80, No 11: pp.1279-1282. perthyroidism. New England Journal of Medi Pedersen KF, Larsen JP, Alves G, Aarsland D (2009b) cine, Vol. 204:pp.747. Prevalence and clinical correlates of apathy in Lane-Brown A, Tate R (2009) Interventions for apathy Parkinson’s Disease. Parkinson and Related after traumatic brain injury. Cochrane Database Disorders 15: 295-299. of Systematic Reviews 2009, Issue 2. Art. No.: Rejners JS, Ehrt U, Weber WEJ, Aarsland D and Leent CD006341. jens AFG (2008) A Systematic Review of Preva DOI: 10.1002/14651858.CD006341.pub2 lence Studies of Depression in Parkinson Dis Leroi I, Harbishettar V, Andrews M, McDonald K, Bryne ease. Movement Disorder, Vol. 23: pp.183-189 EJ, Burns A (2012) Carer burden in apathy and Resnick B, Zimmerman SI, Magaziner J, Adelman A impulse control disorders in Parkinson’s disease (1998) Use of apathy evaluation scale as a International Journal of Geriatric Psychiatry, Vol. measure of motivation in elderly people Rehabili 27: pp.160–166. tation Nursing, Vol. 23, No3: pp.141-147. Levy ML, Cummings JL, Fairbanks LA, Masterman D, Robert PH, Clairet S, Benoit M, Koutaich J, Bertogliati C, Miller BL, Craig A.H et al. (1998) Tible O, et al. (2002) The apathy inventory: Apathy is not depression. The Journal of Neuro assessment of apathy and awareness in Alzhei psychiatry and Clinical Neurosciences, Vol. 10, mer's disease, Parkinson's disease and mild No 3: pp.314-319. cognitive impairment. International Journal of Levy R and Dubois B (2006) Apathy and its functional Geriatric Psychiatry, Vol. 17, No12: pp.1099- anatomy of the prefrontal cortex-basal 1105. ganglia circuits Cerebral Cortex, Vol. 16, No 7: Robert P, Onyike CU, Leentjens A F, Dujardin K, Aalten pp.916-928. P, Starkstein S et al. (2009) Proposed diagnostic Litvan I, Mega MS, Cummings JL, Fairbanks L (1996) criteria for apathy in Alzheimer's disease and Neuropyschiatric aspects of progressive supra other neuropsychiatric disorders. European Psy nuclear palsy. Neurology, Vol. 47: pp.1184-1189. chiatry, Vol. 24, No2: pp.98–104. Marin RS (1990) Differential Diagnosis and Classification Santa N, Sugimori H, Kusuda K, Yamashita Y, Ibayashi of Apathy. The American Journal of Psychiatry, S, Iida M (2008) Apathy and functional recovery Vol.147, No 1: pp. 22-30. following first-ever stroke. International Journal Marin RS, Biedrzycki RC, Firinciogullari S (1991) Relia of Rehabilitation Research, Vol. 31, No 4: pp.321 bility and Validity of the Apathy Evaluation Scale. -326 Psychiatry Research, Vol. 38: pp.143-162. Sansone RA, Sansone LA. (2010) SSRI-induced indiffer Marin RS, Wilkosz P (2005) Disorders of diminished ence. Psychiatry, Vol. 7: pp.14-18. motivation. Journal of Head Trauma Rehabilita Settle EC Jr. (1998) Antidepressant drugs: disturbing tion, Vol. 20, No. 4: pp.377-88. and potentially dangerous adverse effects. Marsh NV, Kersel DA, Havill JH, et al. (1998) Caregiver Journal of Clinical Psychiatry, Vol. 59, Suppl 16 : burden at 1 year following severe traumatic brain pp.25-30. injury. Brain Injury, Vol.12: pp.1045-1059. Sockeel P, Dujardin K ,Devos D, Denève C, Destée A, Mayo NE, Fellows LK, Scott SC, Cameron J, Wood- Defebvre L (2006) The Lille apathy rating scale Dauphinee S (2009) A Longitudinal View of Apa (LARS), a new instrument for detecting and thy and Its Impact After Stroke. Stroke, Vol. 40: quantifying apathy: validation in Parkinson's pp.3299-3307. disease. Journal of Neurology Neurosurgery Mendez M F, Lauterbach E C, Sampson S M. (2008) An Psychiatry, Vol. 77, No5: pp.579–584. evidence-based review of the psychopathology Starkstein SE, Mayberg HS, Preziosi TJ, Andrezejewski of frontotemporal dementia: a report of the P, Leiguarda R, Robinson RG. (1992) Reliability, ANPA Committee on Research Journal of Neu validity, and clinical correlates of apathy in Park ropsychiatry and Clinical Neurosciences, Vol. 20, inson's disease. Journal of Neuropsychiatry No. 2: 130-149. Clinical Neuroscience Vol. 4 No 2: pp.134-139. Mikami K, Ricardo EJ, Moser DJ, Jang M, Robinson RG Starkstein SE, Jorge R, Mizrahi R, and. Robinson R G, (2013) Incident Apathy During the First Year (2006) “A prospective longitudinal study of apa After stroke and Its Effect on Physical and Cog thy in Alzheimer’s disease,” Journal of Neurolo nitive Recovery. The American Journla of Geri gy, Neurosurgery & Psychiatry, Vol. 77, No1: pp. atirc Psychiatry, Vol. 21, No 9: pp.848- 854. 8–11. Oguru M, Tachibana H, Toda K, Okuda B, Oka N. (2010) Stuss DT, Van Reekum R, Murphy KJ (2000) Differentia Apathy and depression in Parkinson disease. tion of states and causes of apathy. In: J Borod Journal of Geriatric Psychiatry and Neurology, (editor): Neuropsychology of Emotion. New York: Vol. 23, No1: pp.35–41. Oxford University Press. Okada K, Kobayashi S, Yamagata S, Takahashi K, Ya van Reekum R, Stuss D T, Ostrander L (2005) Apathy: 21 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

Why care? Journal of Neuropsychiatry & Clinical sation. http://apps.who.int/classifications/icd10/ Neurosciences Vol. 17, No1: pp. 7–19. browse/2010/en (accessed 1 March 2015) van Dalen JW, van Charante EPM, Nederkoorn PJ, van Yamagata S, Yamaguchi S, Kobayashi S (2004) Im Gool WA, Richard E. (2013) Poststroke Apathy. paired Novelty Processing in Apathy After Sub Stroke,Vol 44, No 3: pp.851-60 cortical Stroke. Stroke, Vol. 35: pp.1935-1940 Verkaik R, van Weert JCM, Francke, AL (2005) The Zahodne LB, Young S, Kirsch-Darrow L, Nisenzon A, effects of psychosocial methods on depressed, Fernandez HH, Okun MS, Bowers D (2009) aggressive and apathetic behaviors of people Examination of the Lille Apathy Rating Scale in with dementia: a systematic review. Internation Parkinson disease. Movement Disorder, Vol. 24, al Journal of Geriatric Psychiatry, Vol. 20, No 4: No 5: pp.677–683 pp. 301-314. Zahodne LB, Bernal-Pacheco O, Okun, MS (2012) Are Wongpakaran N, van Reekum R, Wongpakaran T, Selective Serotonin Reuptake Inhibitors Associ Clarke D. (2007) Selective serotonin reuptake ated With Greater Apathy in Parkinson’s Dis inhibitor use associates with apathy among de ease? The Journal of Neuropsychiatry and Clini pressed elderly: A case-control study. Annuals cal Neuroscience, Vol. 24, No 3: pp.326-330. of General Psychiatry 6:7. doi:10.1186/1744- Zamboni G, Huey E D, Krueger F, Nichelli P F, Graf 859X- 6-7. man, J (2008) Apathy and disinhibition in fronto World Alzheimer Report (2009) Alzheimer’s Disease temporal dementia: Insights into their neural International World Alzheimer Reports Editors correlates. Neurology, Vol. 71, No10: pp.736- Prince M & Jackson J London accessed 742. 11/3/2014 http://www.alz.co.uk/research/files/ WorldAlzheimerReport-ExecutiveSummary.pdf World Health Organization. (1992) The ICD-10 classifi cation of mental and behavioural disorders: clini cal descriptions and diagnostic guidelines. Ge neva: World Health Organization. World Health Organisation (2010) International Statisti cal Classification of Diseases and Related Health Problems, ICD-10 Version: 2010 Online Version for 2010 Geneva: World Health Organi

The Louie Blundell Prize

This prize is in honour of our colleague Louie Blundell and will be awarded for the best neuroscience nursing paper by a student submitted to the Australasian Neuroscience Nurses Association (ANNA) for inclusion in the Australasian Journal of Neuroscience by the designat- ed date each year. The monetary value of the prize is AUD$500.

Louie Blundell, was born in England, and although she wanted to be a nurse she had to wait until after World War II to start her training as a mature student in her late twenties. Later she and her family moved to Western Australia in 1959. She worked for a General Practice surgery in Perth until a move to the Eastern Goldfields in 1963. Subsequently, she worked at Southern Cross Hospital and then Meriden Hospital. During this time she undertook post basic education to maintain her currency of knowledge and practice, especially in coronary care.

Louie was also active in the community. She joined the Country Women’s Association and over the years held branch, division and state executive positions until shortly before her death in 2007. She was especially involved in supporting the welfare of students at secondary school, serving on a high school hostel board for some time.

She felt strongly that education was important for women and was a strong supporter and advocate of the move of nursing education to the tertiary sector, of post graduate study in nursing and the development of nursing scholarship and research, strongly defending this view to others over the years.

For further details and criteria guidelines please visit the ANNA website at www.anna.asn.au

22 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 Analysing the Present Situation of Protection Against Exposure to Temozolomide on a Japanese Neurosurgical Ward Tetsuya Isaji

Abstract Temozolomide was approved as an oral alkylating agent against glioma in 2006 in Japan. The nurse gives Temozolomide through the stomach tube with medicine suspension method for patients with a disturbance of consciousness and a dysphagia. Exposure of antineoplastic agents has been a focus in recent years in Japan. Nurses use protection goods when they give Te- mozolomide; however, they did not protect themselves from exposure of the antineoplastic agent when they disposed of used materials and excrement. Therefore, there is the need to educate and evaluate protection materials.

Keywords: Temozolomide, Antineoplastic agents, exposure, personal protective equipment, Ja- pan.

Background and fully understand the safety precautions Temozolomide was approved as an oral alkyl- for themselves when handling excrement. ating agent against glioma in 2006 in Japan. Seventeen patients with a glioma were pre- Methodology scribed Temozolomide at the Kizawa Memori- The present situation of handling and giving al Hospital in 2012. Although Temozolomide Temozolomide was understood. Best prac- is an oral antineoplastic agent, not all patients tice was researched and discussions were can take it by themselves. The nurse gives held with the pharmacist about the best way Temozolomide through the stomach tube via to safety handle the giving of Temozolomide medicine a suspension method for patients and the consequences of handling patients’ with a disturbance of consciousness and a waste products afterwards. dysphagia. It was found that nurses suffered from alkylating agent due to giving Te- Results mozolomide from stomach tube. Exposure of Nurses wore vinyl gown, surgical mask and antineoplastic agents has been a focus for nitrile gloves in medicine the Japanese medical staff in recent years. suspension method and Thus, many nurses have studied about expo- giving Temozolomide from sure of antineoplastic agents in the hospital stomach tube (Fig. 1). since 2010. Although nurses take care of exposure from injections of antineoplastic Figure 1: (Left) Nurses use agents, it was found that they did not worry vinyl gown, surgical mask and about exposure from the oral antineoplastic nitrile glove for preparing and giving Temozolomide. agents.

Objective In the medicine suspension method, nurses The purpose of this study was to analyse the put Temozolomide in syringe and they used present situation of preparation and giving apple juice as a solution in order to keep the Temozolomide and handling of excrement in stability of Temozolomide (Fig.2). Nurses put the neurosurgical ward in order that nurses the syringe in hot water. Then, they put the can give patients the prescribed treatment cap on the syringe because it is possible that the apple juice-melted Temozolomide leaks Questions or comments about this article should be without the cap (Fig.3). After warming up the directed to Tetsuya Isaji, Certified Nurse Specialist syringe, nurses stirred the Temozolomide Cancer Nursing, Kizawa Memorial Hospital, Gifu, Japan solution by shaking the syringe. They then at [email protected] discarded the used syringe and other goods Copyright©2014 ANNA without any protection from exposure (Fig.4,5).

23 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

posed to urine containing Temozolomide easily. In addition, vinyl gloves cannot protect from antineoplastic agents. Also, the washed bottles are placed on newspaper to dry. The newspaper gets wet with Te- mozolomide-contaminated water.

Figure 2: (Above) Syringe Figure 3: (Above) Using Temozolomide is an alkylating agent. Alkyl- in a medicine suspension cap for preventing leaking. ating agents act on DNA; thus, it has an af- method. fects on cancer cells but normal cells are also affected. Temozolomide is excreted in the urine within 8hours and metabolites of Te- mozolomide remain for over 24 hours (Baker et al.,1999). Approximately 75% of nurses in the neurosurgical ward are in the 20-39 year- age-group. This is important and safety must be considered due to the opportunity of preg- Figure 4: (Above) discard Figure 5: (Above) nurses nancy within this age group. There is a law to with used goods. do away with used syringe protect people from exposure of radiation but without protection. the Japanese government has not made a When handling waste, nurses wore a vinyl national policy against exposure of antineo- apron without sleeve, surgical mask and vinyl plastic agents. Although the guideline was glove. This is the same as for patients who made by the Japanese Society of Hospital do not take Temozolomide (see Figure 6). If Pharmacists in 1992, the nurses have not the patients who take Temozolomide had a been educated to this guideline. So, only few urethral catheter, nurses wore only vinyl nurses know about exposure of antineo- gloves. Used bottles for collecting urine are plastic agents. Hence, it is important to edu- washed with detergent. This is the same as cate and protect the nurses from exposure. other patients who do not take Te- Therefore, we need to prepare and evaluate mozolomide (see Figures 7 & 8). Nurses protection materials. This is an ongoing edu- discarded used diapers without protection cation initiative for the Japanese nurses. from exposure. Conclusion Discussion After this study, all nurses now have a better Nurses in the neurosurgical ward used pro- understanding of the consequences of Te- tection goods when they give Temozolomide. mozolomide delivery and the precautions that However, they did not protect themselves must be taken when delivering this type of from exposure of the antineoplastic agent medication. For our staff, this is an ongoing education matter and there will be follow up evaluations of the content and protection methods available.

References Baker, SD, Wirth, M, Statkevich, P, Reidenberg, P, Al ton, K, Sartorius, SE, Dugan, M, Cutler, D, Batra, V, Grochow, LB, Donehower, RS and Rowinsky, Figure 6: (Above) Figure 7: (Above) Wash- EK (1999) ‘Absorption, Metabolism, and Excre Wearing style when ing used bottle. tion of 14C-Temozolomide following Oral Admin handling excrement. istration to Patients with Advanced Cancer’, when they disposed of Clinical Cancer Research, vol. 5, No. 2: pp. 309- 317. used materials and excrement. The method of discarding used materials is very dangerous because nurses do not protect them- Figure 8: (Above) selves. Therefore, it is Storing washed bottles. possible that Te- mozolomide adheres to the nurses’ hands. Nurses who wear vinyl aprons and vinyl groves for handling excrement can be ex- 24 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 Anti- N-methyl-D-aspartic acid Receptor Encephalitis: A Critical Literature Review and Implications for Neurosci- ence Nurses

Linda Nichols

Abstract Anti- N-methyl-D-aspartic acid (anti-NMDA) Receptor Encephalitis is a devastating disease that is linked with alarming rates of misdiagnosis and clinical management conundrums. As a relatively new disease process the literature is limited to case studies and small case series, generally focusing on the natural history of the disease, clinical management and medical interventions. This has resulted in a dearth of literature related to the neuroscience nursing care of individuals diagnosed with anti-NMDA receptor encephalitis. Nurses play a vital role in caring for individuals diagnosed with anti-NMDA receptor encephalitis and thus it is vital that this role is explored.

Keywords: anti-NMDA receptor encephalitis, paraneoplastic syndrome, autoimmune disease, neuroscience nurses.

Let me introduce myself including psychotic manifestations, autonomic You don’t know me yet, but by the time you do abnormalities and catatonia (Sansing et al., You will wish we had never met 2007). Whilst there has been limited oppor- I will take your memory tunity to analyse anti- N-methyl-D-aspartic I will take your speech acid receptor encephalitis (anti-NMDA recep- I will make you see, hear, horrible things tor encephalitis) from a historical perspective You will no longer be able to eat (Day, High, Cot, & Tang-Wai, 2011; Hughes You will no longer be able to sleep et al., 2010), there has been noteworthy ref- You will no longer be able to breathe erence in the literature prior to this of similar I will play with your heart, and just for fun descriptors of the disease process. First rec- I may even make it stop ognised as a paraneoplastic syndrome in Your arms and legs will be at my command young women with ovarian teratomas, anti- And these are just a few of the things I can do NMDA receptor encephalitis is a member of You will be mine, all mine the neuro autoimmune syndromes that make Let me introduce myself antibodies against synaptic proteins in re- My name is Anti-NDMA Receptor Encephalitis sponse to either a neoplasm or another potential trigger (Dalmau et al., 2008). This pa- Figure 1: (Above) Poem by Nesrin Shaheen (2008). per critically explores the literature focusing on the implications for neuroscience nurses, Introduction exploring the key neuroscience nursing and This beautiful poem was created by Nesrin treatment interventions that require consider- Shaheen. Her daughter Sonia suffered from ation when caring for individuals diagnosed anti-NMDA receptor encephalitis. It's a very with anti-NMDA receptor encephalitis. good description of what this illness does to its sufferers. Anatomy and Pathophysiology Anti-NMDA receptor encephalitis is an auto- Identified in 2005 and officially named in immune disease that results in the production 2007, anti-NMDA receptor encephalitis is en- of antibodies against the glutamate binding cephalitis associated with antibodies against site of the NR1 subunit of N-methyl-D- the N-methyl-D-aspartic acid receptors and aspartic acid (NMDA) receptors. The receptors are located throughout the central nerv- Questions or comments about this article should be di- ous system with concentrations in the fore- rected to Linda Nichols, Lecturer Unit Coordinator Neuro- brain, limbic system and hippocampus, which science Nursing, School of Health Science, University of Tasmania at [email protected] in some part explains the clinical features of the disease (Ferdinand & Michelle, 2012). presentsCopyright©2014 with distinct ANNA clinical characteristics The receptors are made up of two subunits;

25 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

NR1, which binds glycine and NR2, which tion of a differential diagnosis and it is not binds glutamate (Dalmau, et al., 2008). The uncommon for individuals to experience an receptors are ligand-gated cation channels, exhaustive range of medical evaluations and which play a role in synaptic transmission investigations before a diagnosis is made. and plasticity, acting as the cellular sub- There are many published case studies and strates for learning and memory (Chapman & cases presented in the media describing Vause, 2011). For a receptor to function, both young girls who were initially identified as subunits must be bound, thus dysfunction of psychiatric cases only to be diagnosed with one leads to a failure of synaptic transmis- anti-NMDA receptor encephalitis. Despite sion (Verhelst et al., 2011). Transmission this, it is often invaluable to have ongoing between the receptors is important and it is psychiatric input as individuals will often pre- hypothesised that hypoactivity plays a role in sent with a complex range of psychiatric the pathogenesis of schizophrenia with hy- symptoms and the management of these peractivity linked to conditions such as de- symptoms can improve recovery time. mentia (Goff & Coyle, 2001). In the case of anti-NMDA receptor encephalitis the body Antibody testing can often be delayed, due to forms antibodies as an immune response or the limited number of pathology laboratories against neuroplastic cells contained within a accredited in Australia to test for NMDA re- tumour and in turn this is replicated with the ceptor assay and diagnostic delays. Cerebro- receptor resulting in damage to the ion chan- spinal fluid (CSF) analysis is integral to diag- nels in the post-synaptic membrane (Day, et nosing a multitude of cerebral nervous sys- al., 2011). The antibodies decrease the num- tem conditions. Abnormal CSF analysis is ber of cell surface NMDA receptors and reported in 80% of affected individuals NMDA receptor clusters in the postsynaptic (Dalmau, et al., 2008), presenting with in- dendrites, reducing neuronal signals creased oligoclonal bands or bands of immu- (Chapman & Vause, 2011). However, in most noglobulins and leukocyte counts. Over 90% cases the disease is reversible irrespective of of individuals will demonstrate lymphocytic the duration of symptoms, supporting an im- pleocytosis during the course of their illness mune mediated neuronal dysfunction rather (Consoli et al., 2012; Johnson, Henry, Fess- than irreversible degeneration of the neurons ler, & Dalmau, 2010). However, protein and (Ferdinand & Michelle, 2012). Despite this glucose levels can present as normal, poten- reversibility inflammatory processes can lead tially delaying further analysis of the CSF. to scaring and a prolonged or an incomplete The diagnosis of anti-NMDA receptor en- recovery for individuals, particularly those cephalitis is confirmed with specific testing for who experience delays in diagnosis and sub- antibodies to the NR1/NR2 heteromers of the sequent treatment. NMDA receptor in both CSF and serum samples (Iizuka et al., 2008). Whilst serum anti- Diagnosis bodies to the NMDA receptor can be detect- Described by some as being “driven insane ed via a cell-based assay, the levels are typi- by your own immune system”, anti-NMDA cally lower than those in the CSF, with Vin- receptor encephalitis is linked to an alarming- cent & Bien (2008) suggesting that CSF lev- ly high incidence of misdiagnosis and inap- els can be up to 10 times higher than serum propriate admission to psychiatric units levels. (Lebon et al., 2012; Maneta & Garcia, 2014; Turkdogan, Orengul, Zaimo, & Ekinci, 2014). Electroencephalograph presentation is usual- Regularly misdiagnosed as a psychiatric ly slow non-specific dis-organised activity presentation, there is a need to consider anti- with occasional electrographic seizures. The NMDA receptor encephalitis as a differential rhythm change can occur relatively unilateral- diagnosis when individuals present with the ly (Day, et al., 2011). There is generally little constellation of acute onset psychiatric symp- evidence of limb paralysis and deep tendon toms, autonomic dysfunction and altered reflexes are generally normal. Imaging, in- muscle tone (Chapman & Vause, 2011). cluding computed tomography, plays a lim- Rapid referral to psychiatric teams for as- ited role in the diagnostic period with magnet- sessment and diagnosis has long been criti- ic resonance imaging (MRI) generally pre- cised for its ethical abuses and the ability that senting as normal on admission (Gable et al., psychiatry has to ultimately cancel all other 2009). However, during the course of the dis- medical interventions and investigations ease MRI may show meningeal enhance- (Rutkowski & Gordon, 1994). This initial mis- ment, and periventricular white matter chang- diagnosis and incarceration into psychiatric es similar to demyelination (Hughes, et al., units has historically limited further investiga- 2010; Tuzun et al., 2013). Once the diagno- 26 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

sis of anti-NMDA receptor encephalitis is sus- behaviours at home with limited support. pected, a prompt and thorough investigation for neoplasm is vital, including imaging of the The non-responsive phase presents with abdomen, chest and pelvis. Dalmau, Lancas- cognitive decline, anorexia, incontinence, ter, Martinez-Hernandez, Rosenfeld & Balice- disorganised language progressing to linguis- Gordon (2011) suggest that the younger an tic regression and mutism, finally leading to individual is, the less likely that a tumour will catatonia (Gable, et al., 2009). Individuals will be detected. In the absence of an identified often present with their eyes open, yet unre- malignancy, authors such as Florance et al., sponsive to visual threats, and absent or lim- (2009), suggest that periodic ultrasound of ited responses to painful stimuli (Chapman & the abdomen should be continued for up to Vause, 2011; Iizuka, et al., 2008). During two years post diagnosis. this phase there is often evidence of increased muscle tone with dystonic and cata- Phases of Anti-NMDA Receptor Encepha- leptic posturing. An estimated 76% of individ- litis uals will experience seizures (Dalmau, et al., Anti-NMDA receptor encephalitis differs from 2008), and seizure activity can progress to limbic encephalitis in that it normally devel- status epilepticus. The hyperkinetic phase ops in five distinct phases. In children and can present as a separate phase or can over- infants the symptoms can differ with the lap with the unresponsive phase. It presents prominent presenting symptom being mood as increased muscular activity resulting in or behavioural changes including temper tan- excessive and abnormal movement of the trums, disintegrative disorders and autistic limbs and body. Dalmau et al., (2008) sug- like behaviours (Ben Achour et al., 2013; gests that up to 86% of individuals will experi- Creten et al., 2011; Tsutsui et al., 2012). This ence dyskinesia with individuals presenting is followed by a period hyperactivity and irri- with increased restlessness, flexing of the tability rather than psychosis, often delaying limbs and facial twitches (Day, et al., 2011). the diagnosis (Dalmau, et al., 2011). Whilst Movements generally include the chorea-like adults are more likely to present with psycho- rapid uncontrolled movements and athetosis logical disturbances and children with neuro- or slower twisting movements. Orofacial dys- logical disturbances, the symptoms generally kinesia is identified as a key feature, with in- merge within a month to a similar presenta- dividuals exhibiting lip smacking and tongue tion (Ingram & Robertson, 2013; Titulaer et twitching (Dalmau, et al., 2008). An estimated al., 2013). 69-89% of individuals will also experience dysautonomia, with autonomic instability evi- The first phase is the prodromal phase where denced by fluctuation in temperature, blood up to 70% of individuals experience symp- pressure, heart rate, hyper-salivation and toms similar to those of a viral infection hypoventilation (Chapman & Vause, 2011; (Dalmau, et al., 2011). However, this prodro- Gable, et al., 2009). During the course of the mal phase may go unnoticed, as symptoms disease more that 60% of individuals will ex- are often mild and non-specific including perience hypoventilation and will require res- headache, malaise and fever. These symp- piratory support (Dalmau, et al., 2008). toms generally last a week and can occur up (Ingram & Robertson, 2013) purport that up to two weeks prior to the second phase to 75% of individuals require admission to an (Ferdinand & Michelle, 2012). The second intensive care unit at some point during the phase is the psychiatric phase, where individ- trajectory of the disease for respiratory sup- uals present with an acute onset of psychiat- port (Lim et al., 2013). ric manifestations with an estimated 68% of individuals exhibiting psychotic symptoms With treatment, individuals will enter a gradu- (Dalmau, et al., 2008; Gable, et al., 2009). al recovery phase however this may include Symptoms can include progressive confu- relapses, with up to 20-25% of individuals sion, agitation, hallucinations and suicidal experiencing at least one clinical relapse ideation, fear, personality changes, mood (Dalmau, et al., 2011). Recovery may take up disturbances, depression, twitching, paranoid to three years and it is a multistage process delusions and violent episodes (Gable, et al., that usually occurs in the reverse of the 2009; Sonn & Merritt, 2010). It is important to symptom presentation. Social behaviour and note that this phase can be a particularly diffi- executive functions are the last to normalise cult for parents and loved ones; there are and recall of the trajectory of the illness is often numerous presentations to hospital and normally poor as persistent amnesia is a psychiatric admissions and reports of parents characteristic feature of anti-NMDA receptor struggling to manage increasingly difficult encephalitis (Dalmau, et al., 2008). 27 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

Incidence and Aetiology tions, (Dalmau, et al., 2011) suggest that The exact incidence of anti-NMDA receptor these events only act as an adjuvant of the encephalitis remains difficult to identify. autoimmune response in individuals with a When it was first identified in 2005, four wom- racial or genetic predisposition to autoim- en with ovarian teratomas were described. mune diseases. By 2007 when the NMDA antibodies were detected over 420 cases were known Treatment and Interventions (Dalmau, et al., 2011), and the number of The care of individuals diagnosed with anti- cases continue to grow suggesting that anti- NMDA receptor encephalitis is complex and NMDA receptor encephalitis is perhaps not challenging and the clinical presentation pre- such a rare disease. Gable, Sheriff, Dalmau, sents a conundrum in terms of what type of Tilley & Glaser (2012) suggest that anti- ward setting is most beneficial and the safest NMDA receptor encephalitis occurs four option for individuals. Individuals generally times more frequently than viral encephalitis. require hospitalisation for several months, Whilst anti-NMDA receptor encephalitis af- followed by long-term physical and cognitive fects both genders, there is a predominate rehabilitation. The complex neuro-psychiatric predilection for females. This is shown in presentation may lead to the consideration of Dalmau et al., (2011) case series of over 400 a psychiatric unit as a potential admission individuals identifying that 80% of those diag- option. However, psychiatric units are limited nosed were female. The estimated age in their ability to facilitate medical interven- range spans from 8 months to over 80 years tions and to facilitate regular monitoring of (Armangue et al., 2013; Day, et al., 2011; individuals in terms of autonomic instability. Wong-Kisiel et al., 2010) with 65% of cases Likewise, whilst a neurology setting may be occurring in individuals aged 18 years or considered appropriate in terms of managing younger (Dalmau, et al., 2008; Gable, et al., seizures, these units are often ill-equipped to 2012). Individuals generally present with an manage the behavioural and psychiatric unremarkable medical and psychiatric history needs of individuals diagnosed with anti- (Mann et al., 2012) and no history of ciga- NMDA receptor encephalitis (Chapman & rette, alcohol, or drug use (Barry et al., 2011). Vause, 2011). Neurosurgical units are often Individuals are often not sexually active and considered the best option for the acute man- have no history of oral or genital herpes agement of individuals diagnosed with anti- (Hegarty & Mikli, 2013). NMDA receptor encephalitis (Lim, et al., 2013). Neurosurgical wards, particularly Anti-NMDA receptor encephalitis is generally those that specialise in traumatic brain inju- associated with ovarian teratomas that al- ries, are often staffed at higher levels and most always contain central nervous system can provide the constant supervision together tissue (Day, et al., 2011). However, anti- with an environment that is usually secure NMDA receptor encephalitis has also been and organised to accommodate confused associated with breast cancer, neuroblasto- individuals. More importantly, the nursing mas and Hodgkin's lymphoma (Lebas, Hus- staff are competent in managing conditions son, Didelot, Honnorat, & Tardieu, 2010; such as post traumatic amnesia and auto- Zandi et al., 2009). Within the male popula- nomic dysfunction. The symptoms of auto- tion, anti-NMDA receptor encephalitis has nomic dysfunction can interfere with rehabili- been linked to a number of malignancies in- tation due to their unpredictability and physi- cluding testicular seminoma and lung cancer cal nature that adversely affect progress. (Dalmau, et al., 2008) although it is estimated Treatment is focused on a symptomatic ap- that less than 5% of male individuals will pre- proach and symptoms are treated inde- sent with an underlying malignancy (Finne pendently as there is no specific treatment for Lenoir et al., 2013). Dalmau et al., (2008) and the underlying cause. Gable et al., (2009) suggest a predilection in Hispanics, African Americans, Asian and Pa- Initially treatment is focused on the identifica- cific Islanders, thereby linking with an in- tion and removal of any tumour. However this creased incidence of ovarian teratomas in stage can often be delayed due to prece- individuals with dark skin pigmentation. How- dence to treat psychiatric symptoms. The ever, it is suggested that up to 40% of individ- surgical removal of malignancies, particularly uals do not present with an identifiable tu- ovarian teratomas, is associated with im- mour (Dalmau, et al., 2008). Whilst there proved outcomes and a reduction in the se- have been some reports in the literature link- verity and duration of symptoms (Ferdinand ing anti-NMDA receptor encephalitis to vac- & Michelle, 2012; Uchino et al., 2011). The cinations and non-specific systemic infec- use of steroidal and immunosuppressive 28 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

therapy has proved vital in the management cesses (Ahmed, Spigelman, Cavacini, & Pos- of symptoms, although there have been no ner, 2006). Immunotherapy is associated with large studies or randomised control trials that fewer relapses when used for anti-NMDA have evaluated the management and treat- receptor encephalitis (Gabilondo et al., 2011; ment effect on anti-NMDA receptor encepha- Titulaer, et al., 2013). Treatment is generally litis (Hegarty & Mikli, 2013; Ingram & Robert- continued until there has been clinical im- son, 2013). Treatment decisions are general- provement and a reduction in antibody levels. ly based on personal preferences and clinical However treatment can be extended for up to symptoms rather than antibody titers 1 year to prevent clinical relapses (Dalmau, (Titulaer, et al., 2013). et al., 2011; Ferdinand & Michelle, 2012).

The first line of treatment is usually a high Symptomatic treatment includes seizure dose five-day course of intravenous management, where multiple agents may be methylprednisolone and or intravenous im- required, with some individuals requiring ben- munoglobulin therapy. As a plasma product zodiazepine infusions for refractory seizures derived from the immunoglobulin antibodies, (Maramattom, Philip, & Sundaram, 2010). intravenous immunoglobulin slows the body’s Seizure activity can often be a presenting natural immune response and binds the over- symptom or can develop during the early active antibodies, in this case the N1 subu- stages of the disease (Dalmau, et al., 2011). nits of the NMDA receptors (Pham, Daniel- Seizure activity can be difficult to distinguish Johnson, Stotler, Stephens, & Schwartz, from hyperkinetic movements and this has 2011). Individuals generally begin to demon- resulted in both an under recognition of sei- strate improvement with 48 hours of com- zure activity for some individuals, or in con- mencing immunoglobulin therapy. However trast, an unnecessary escalation of anti- this may involve individuals experiencing a convulsant medications for others progression in reverse order through each of (Bayreuther et al., 2009). The use of continu- the clinical phases (Day, et al., 2011). Plas- ous video electroencephalography is recom- ma exchange or plasmapheresis is an alter- mended as it allows for simultaneous video native option to immunoglobulin therapy, recording of clinical manifestations to be cor- where the offending antibodies can be re- related with electroencephalography activity. moved from circulation in an attempt to tem- This approach, and clear descriptions of be- per the disease process. The use of plasma haviours by staff, can ensure that there is a exchange can be limited in cases of auto- clear differentiation between seizure activity nomic instability, or in children and uncooper- and abnormal movements. ative individuals (Dalmau, et al., 2011). Whilst plasma exchange can remove the of- Psychiatric drugs do not stop the develop- fending antibodies, the success is dependent ment or progression of the disease. However on adjunct treatments that are focused on the they do play an important role in reducing the suppression of the immune system, including psychiatric symptoms. All features of psycho- treatment with rituximab. sis should be managed in the conventional manner, as treatment will often unmask other Immunotherapy is generally considered as symptoms that can then be treated either an adjunct or second line therapy. It is (Ferdinand & Michelle, 2012). Supportive considered when there has been a delayed treatment is vital as individuals often respond diagnosis or limited response to other inter- to treatment environments such as those pro- ventions, and for those without an identifiable vided for post traumatic amnesia, including a malignancy (Ferdinand & Michelle, 2012; darkened room, minimal distractions and rou- Lim, et al., 2013). Immunosuppressive treat- tine sleep wake cycles. Individuals often re- ment options include rituximab, azathioprine quire physical and chemical restraint to main- and cyclophosphamide. Rituximab (or tain their own safety, particularly during peri- Mabthera as it is commonly known) is a ther- ods of violence, self-harm and agitation apeutic monoclonal antibody that is used to (Zehry, Matrunola, & Hyde, 2011). Sedation treat common forms of Non-Hodgkins lym- reduces elevated sympathetic tone and the phoma (NHL) and is often the drug of choice relaxed state often facilitates easier manage- as it has demonstrated positive results in the ment of blood pressure and intracranial pres- treatment of multiple autoimmune diseases. sure. It is vital that oxygen saturations are Rituximab and immunoglobulin therapy are monitored at all times. It is also important that often used in combination due to their syner- nurses support families when making this gistic effects and are associated with long- decision and that hospital policies and proto- term remission for a number of disease pro- cols are followed at all times. Ongoing reas- 29 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

surance to family members is vital as the to a minimum. Activities of daily living and symptoms that individuals can experience care interventions, such as taking observa- can be distressing. tions, can be difficult to undertake as they can increase an individual’s agitation and Neuro-rehabilitation consultants play a vital anxiety, triggering and elevating behaviours role in facilitating the recovery of individuals and autonomic instability. Thus careful plan- post anti-NMDA receptor encephalitis. Indi- ning and alternative options often need to be viduals respond to similar medications and considered, which also includes regular toilet- care regimes used for post traumatic amne- ing regimes to assist in regaining continence. sia. When managing agitation, it is important The majority of individuals will also require to consider non-pharmacological approach- nutritional support, and coupled with this is es. Individuals should be nursed in a low the need to maintain glycaemic control. Hy- stimulation environment, and provided with perglycaemia leads to hypercatabolic state ongoing reorientation and reassurance. Tar- and muscle protein breakdown. Tight glycae- geted strategies should also be employed to mic control has become a part of the routine address short-term memory impairment management of individuals diagnosed with (Chapman & Vause, 2011). This can include anti-NMDA receptor encephalitis. interactive white boards and clear care plans. Initial goals of treatment include increasing Recovery and rehabilitation can also involve individuals’ responses to external stimuli in- speech therapy for both language and com- cluding pain, touch, light and verbal stimula- munication assistance as well as for swallow- tion. Following this, there is a focus on chan- ing assistance. This is because a significant neling excess energy into functional activities proportion of individuals will develop swallow- that will help in reestablishing a normal rou- ing issues at some stage during the course of tine again. More than 85% of individuals will the disease, and will require enteral feeding still be experiencing significant psychiatric due to decreased level of consciousness symptoms when discharged (Kayser, Kohler, and/or reduced motor control. Individuals re- & Dalmau, 2010), thus making family and quire close monitoring due to the impulsive caregiver education a high priority. nature of the disease and activities should be broken down into smaller tasks to assist in Individuals also face the challenges of im- maintaining attention. Interventions need to paired executive functioning including re- be focused on reducing the risk of general- duced impulse control, loss of inhibitions, ized physical deconditioning, and where pos- poor attention spans, disorganisation and sible, maintaining independence with activi- impaired planning skills (Chapman & Vause, ties of daily living. This requires a multidisci- 2011). Given the high incidence of frontal plinary approach including nurses, speech lobe dysfunction associated with anti-NMDA therapists, occupational therapists and physi- receptor encephalitis, long-term rehabilita- otherapists. At all times individuals need to tions, particularly specialised neuro rehabili- be nursed in a safe environment that includes tation, is central to improved outcomes. As bed rails, padding and floor mats to protect individuals pass through the initial phase of individuals during the hyperkinetic phase. anti-NMDA encephalitis they often begin to Central cardiac monitoring also needs to be exhibit a range of symptoms, including a in place to monitor for respiratory distress range of functional changes. These changes and supported by regular observations. can affect an individual’s thinking; memory When available, a 1:1 nursing staff ratio is and reasoning skills; language skills including also suggested. communication, understanding and expres- sion and sensations may be altered including Prognosis and Outcomes taste, touch and smell. Perhaps one of the The prognosis and outcome for individuals is most difficult challenges is when an individu- generally improved when there has been a al’s emotions are affected resulting in anxie- recognised malignancy and diagnosis has ty, depression, aggressive and or socially been prompt. Mortality rates range from 4- inappropriate behaviour. 10% with the average time to death 3.5 months (Dalmau, et al., 2011; Gable, et al., The establishment of a sleep-wake cycle is 2009). Post diagnosis mortality is linked to vital to assisting individuals to re-orientate events including sepsis, cardiac arrest, sei- and still have the energy to undertake activi- zure activity and respiratory distress. Morbidi- ties of daily living. Nursing staff should have ty rates are variable and the recovery pro- clear care plans in terms of nocturnal activi- cess can take many years. In Dalmau, et al., ties and noise and stimulation should be kept (2008) series of 100 individuals, close to 50 30 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

made a full recovery, 28 experienced mild disorder in a 13-year-old girl. Turkish Journal long-term deficits and 18 experienced severe of Psychiatry, 24(2), 145-147. Chapman, M. R., & Vause, H. E. (2011). Anti-NMDA deficits. Of these deficits, frontal lobe dys- receptor encephalitis: diagnosis, psychiatric function was the most prominent with 85% of presentation, and treatment. American Journal individuals in this, and subsequent series, of Psychiatry, 168(3), 245-251. doi: 10.1176/ experiencing some degree of dysfunction appi.ajp.2010.10020181 Consoli, A., Raffin, M., Laurent, C., Bodeau, N., Campi- (Dalmau, et al., 2008; Finke et al., 2012). The on, D., Amoura, Z., . . . Cohen, D. (2012). goals of treatment are to improve concentra- Medical and developmental risk factors of tion, judgment and problem-solving abilities. catatonia in children and adolescents: a pro- At this level, individuals are involved in their spective case-control study. Schizophr Re- search, 137(1-3), 151-158. doi: 10.1016/ care and goal setting and activities can be j.schres.2012.02.012 focused around tasks of daily living such as Creten, C., van der Zwaan, S., Blankespoor, R. J., Maat- accessing phone numbers, shopping and kamp, A., Nicolai, J., van Os, J., & Schieveld, returning to their former employment or J. N. (2011). Late onset autism and anti- NMDA-receptor encephalitis. Lancet, 378 study. As individuals progress, their level of (9785), 98. doi: 10.1016/s0140-6736(11)60548 responsibility increases and in most cases -5 they are able to return home with only mini- Dalmau, J., Gleichman, A. J., Hughes, E. G., Rossi, J. mal supervision. E., Peng, X., Lai, M., . . . Lynch, D. R. (2008). Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lan- Conclusion cet Neurology, 7(12), 1091-1098. doi: 10.1016/ The management of individuals diagnosed s1474-4422(08)70224-2 with anti-NMDA receptor encephalitis re- Dalmau, J., Lancaster, E., Martinez-Hernandez, E., Rosenfeld, M. R., & Balice-Gordon, R. (2011). mains unclear. As there are few guidelines, Clinical experience and laboratory investiga- care and interventions are currently focused tions in patients with anti-NMDAR encephalitis. on medical interventions with limited literature Lancet Neurology, 10(1), 63-74. doi: 10.1016/ to support the role that neuroscience nurses s1474-4422(10)70253-2 Day, G. S., High, S. M., Cot, B., & Tang-Wai, D. F. play in people with this condition. This paper (2011). Anti-NMDA-receptor encephalitis: case demonstrated the vital role that neuroscience report and literature review of an under- nurses do play in caring for individuals diag- recognized condition. Journal of General Inter- nosed with anti-NMDA receptor encephalitis. nal Medicine, 26(7), 811-816. doi: 10.1007/ s11606-011-1641-9 It is vital that neuroscience nurses under- Ferdinand, P., & Michelle, L. (2012). Anti-NMDA enceph- stand the natural history, available treatment alitis. Clinical, Cellular & Molecular Biology, options and interventions required for individ- S10, 1-6. uals diagnosed with anti-NMDA receptor en- Finke, C., Kopp, U. A., Pruss, H., Dalmau, J., Wanding- er, K. P., & Ploner, C. J. (2012). Cognitive cephalitis. deficits following anti-NMDA receptor encephalitis. Journal of Neurology, Neurosurgery, and References Psychiatry 83(2), 195-198. doi: 10.1136/jnnp- Ahmed, A. R., Spigelman, Z., Cavacini, L. A., & Posner, 2011-300411 M. R. (2006). Treatment of pemphigus vulgaris Finne Lenoir, X., Sindic, C., van Pesch, V., El Sankari, with rituximab and intravenous immune globu- S., de Tourtchaninoff, M., Denays, R., & lin. 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Anti- -piano playing motions and nocturnal hypoven- N-methyl-D-aspartate receptor encephalitis. tilation in anti-NMDA receptor encephalitis: American Journal of the Medical Sciences, response to prompt tumor removal and immu- 345(6), 491-493. doi: 10.1097/ notherapy. Internal Medicine, 50(6), 627-630. MAJ.0b013e3182760e3b Verhelst, H., Verloo, P., Dhondt, K., De Paepe, B., Men- Maneta, E., & Garcia, G. (2014). Psychiatric manifesta- ten, B., Dalmau, J., & Van Coster, R. (2011). tions of anti-NMDA receptor encephalitis: neu- Anti-NMDA-receptor encephalitis in a 3 year robiological underpinnings and differential old patient with chromosome 6p21.32 micro- diagnostic implications. Psychosomatics, 55 deletion including the HLA cluster. European (1), 37-44. doi: 10.1016/j.psym.2013.06.002 Journal of Paediatric Neurology, 15(2), 163- Mann, A., Machado, N. M., Liu, N., Mazin, A. H., Silver, 166. doi: 10.1016/j.ejpn.2010.07.004 K., & Afzal, K. I. (2012). A multidisciplinary Vincent, A., & Bien, C. G. (2008). Anti-NMDA-receptor approach to the treatment of anti-NMDA- encephalitis: a cause of psychiatric, seizure, receptor antibody encephalitis: a case and and movement disorders in young adults. review of the literature. Journal of Neuropsy- Lancet Neurology, 7(12), 1074-1075. doi: chiatry & Clinical Neurosciences, 24(2), 247- 10.1016/s1474-4422(08)70225-4 254. doi: 10.1176/appi.neuropsych.11070151 Wong-Kisiel, L. C., Ji, T., Renaud, D. L., Kotagal, S., Maramattom, B. V., Philip, C., & Sundaram, P. S. (2010). Patterson, M. C., Dalmau, J., & Mack, K. J. Idiopathic anti-NMDA-receptor encephalitis in (2010). Response to immunotherapy in a 20- a young Indian girl. Neurology India, 58(4), month-old boy with anti-NMDA receptor en-

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cephalitis. Neurology, 74(19), 1550-1551. doi: 10.1212/WNL.0b013e3181dd41a1 Zandi, M. S., Irani, S. R., Follows, G., Moody, A. M., Molyneux, P., & Vincent, A. (2009). Limbic encephalitis associated with antibodies to the NMDA receptor in Hodgkin lymphoma. Neurol- ogy, 73(23), 2039-2040. doi: 10.1212/ WNL.0b013e3181c55e9b Zehry, K., Matrunola, A., & Hyde, C. E. (2011). Violence, malignant catatonia or anti-NMDA receptor encephalitis: a case report. Journal of Psychi- atric Intensive Care, 8(1), 55.

33 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 The Tomorrow: Advanced Treatments in Parkinson’s Disease Does Not Necessarily Equate to Treatments in Advanced Parkinson’s Disease David Shek-Yan Tsui

Abstract A person with Parkinson’s disease (PD) can have variations in the severity of Parkinsonian symptoms; this is dependent on the stage of their medication cycle. Variations in dopamine plasma levels cause erratic changes in severity of symptoms, known as ‘motor fluctuations’. Apomorphine infusion, Duodopa® and Deep brain stimulation are the three current advanced treatments for people with PD. The goal of therapy is to replicate the normal function of the neurotransmitter dopamine by providing continuous stimulation in an effort to minimise motor fluctuations. Although these advanced treatment options seem like the ideal solutions for people with PD, awareness and knowledge is relative low amongst the neuroscience community.

Advanced PD treatments have the potential to improve the quality of life in people who are battling this chronic and complex disease. The aim of the presentation is to increase awareness amongst neurosciences nurses of modern treatments available to people with PD, enabling neuroscience nurse to advocate for their patients. This presentation will also highlight the role of the Parkinson’s Disease Nurse Specialist in the assessment, diagnosis, management and ongoing follow-up of these patients who require specialised expertise.

Keywords: Parkinson’s disease, tremor, deep brain stimulation, dopaminergic, movement disorders, Parkinson’s disease nurse specialist, motor fluctuations, “OFF” period, “ON” period, dyskinesia.

Traditional Thinking Versus Contemporary ro-transmission and stimulation to the motor Thinking cortex causing motor dysfunction (McCance & Parkinson’s Disease (PD) was first described Heuther, 2006). by James Parkinson in 1817 in the paper ‘An essay on the Shaking Palsy’. He was able to However, the more contemporary thinking document some key features of PD which are which is beginning to be more wildly accepted still relevant to this day. Some of the key fea- is known as the Braak’s Hypothesis. The tures he described include the chronic degen- Braak’s hypothesis proposes that there are 6 erative nature of the disease, the predomi- stages of PD. Stages 1 and 2 are the pre- nance of unilateral symptoms, tremors and motor or prodromal phase of PD which sug- the latency in tremors, akinesia and bradyki- gests the pathophysiology of PD begins in the nesia, rigidity, stooped posture, postural insta- gut causing gastrointestinal symptoms com- bility, gait disturbance and freezing of gait and monly manifesting as long standing constipa- even non-motor symptoms of the disease tion years before the diagnosis of PD. The such as sleep disturbance, speech disturb- progression of the pathophysiology into the ance and constipation (Parkinson, 1817 & medulla causes the autonomic dysfunctions 2002). such as temperature imbalance, postural hypotension, urinary and erectile dysfunction. The traditional understanding of the patho- Further progression to the pons causes the physiology of PD begins with the destruction sleep disturbances such as REM sleep be- of dopamine producing neurons in the sub- haviour disorders, insomnia and day time stantia nigra located in the midbrain. These somnolence (Braak, Del Tredici, Rüb, de Vos, structures are part of the basal ganglia which Jansen Steur & Braak, 2003). is responsible for initiation and controlling vol- untary movements. The reduced production Stages 3 and 4 are classed as the motor or and action of dopamine lead to reduced neu- clinical phase of PD when the midbrain is affected causing the manifestations of the motor Questions or comments about this article should be symptoms such as tremor, rigidity, akinesia directed to David Tsui Clinical Nurse Consultant in Parkinson’s Disease & Movement Disorders , Westmead and postural imbalance which allows for the Hospital. [email protected] clinical diagnosis of PD to be made (Braak et. al. 2003). Copyright©2014 ANNA

34 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

Stages 5 and 6 are caused by further pro- Contemporary Treatments gression of the pathophysiology into the fore- Despite the advancement in technology and brain and the rest of the cortex which leads to the development of advanced treatments, the cognitive impairments such as dementia, some of the older treatments used in PD are hallucinations and psychosis and can exacer- still currently in use today. Oral Levodopa, in bate pre-existing anxiety and depression the form of Sinemet® (Levodopa/Carbidopa) (Braak et. al. 2003). or Madopar® (Levodopa/Benserazide) continue to be the gold standard treatment in PD The ‘OFF’ & ‘ON’ Phenomenon and since the 1960’s (Hornykiewicz, 2010). The Dyskinesias introduction of dopamine agonists such as People with Parkinson’s disease may have Sifrol® and Cabaser® (Cabergoline) and en- different phases that their body is in depend- zyme inhibitors such as Comtan® ing on the stage of their medication cycle. (Entacapone) and Azilect® (Rasagiline) have This is commonly known as motor fluctua- assisted in maintaining and/or prolonging tions. In their “OFF” period or pre-medicated ‘ON’ periods and better management of PD state, the person may have typical motor symptoms (Hayes et. al. 2010). symptoms of PD and in their “ON” period or their medicated state, the same person may The development of combination drugs such well be functioning close to the level of a nor- as Stalevo® (Levodopa/Carbidopa/ mal person with minimal interruptions from Entacapone) and once daily extended re- their Parkinson’s symptoms due to having lease tablets such as Sifrol ER® optimised dopaminergic plasma levels. (Pramipexole Extended Release) have en- There is also a third state when the dopamin- hanced simplicity and convenience in the ergic plasma levels peak and break through patient’s often complex medication regimen the therapeutic threshold of the “ON” state (Hayes et. al. 2010). causing excessive chorea-like movements. These movements are involuntary, twisting or Delayed gastric emptying in patients with PD writhing movements which can affect any often leads to sporadic absorption of oral PD part of the body. This is known as peak-dose medications which can worsen motor fluctua- dyskinesia (Frabbrini, Brotchie, Grandas, tions. As a result, more contemporary treat- Nomoto & Goetz, 2007). ments attempt to bypass the gastrointestinal tract. The Neupro® (Rotigitine) patch, for ex- Contemporary Concepts of Treatments in ample, is a form of dopamine agonist made PD – Continuous Dopaminergic into a daily 24 hour patch and is the only Stimulation transdermal treatment currently available in The control and balance of motor fluctuations the management of PD (Hayes et. al. 2010). within the therapeutic threshold is dependent on accurate titration of the individualised The Advanced Treatments in PD medication regimen, strict compliance and Currently there are three advanced treat- accurate timing of medication administration. ments in Parkinsonǯs Disease based upon However, when the symptoms are refractory the concepts of providing stable and continu- to these strategies, advanced treatments in ous dopaminergic stimulation to prevent the PD are considered (Hayes, Fung, Kimber, & sudden and unexpected fluctuations in dopa- O’Sullivan, 2010). The common mispercep- minergic plasma levels. These treatments tion of advanced treatments for PD is that include Apomorphine, Duodopa® and Deep they are usually reserved for patients in their Brain Stimulation (DBS) (Hayes et. al. 2010). later or advanced stages of the disease process. However, these advanced treatments Apomorphine is a subcutaneous injection and can be implemented in the earlier stages of is the only injectable treatment available to PD and patients can benefit from earlier inter- manage PD. Apomorphine is a dopamine ventions. agonist that works to stimulate the dopamine receptors in the substantia nigra, more spe- The concept behind the advanced treatments cifically the D1 and D2 receptors. It can be in PD is referred to as ‘continuous dopamin- given as an intermittent rescue injection us- ergic stimulation’ where medication or stimu- ing a heparin/insulin syringe or via continu- lation is administered in a constant fashion to ous subcutaneous infusion. Although it is de- provide stability and continuity to prevent the rived from the morphine molecule it has no sudden and pulsatile fluctuations in dopamin- narcotic, analgesic or addictive properties. ergic plasma levels (Nutt, 2007). The dilution and infusion rates on the pump are pre-set and individualized to each patient 35 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

by a neurologist and commonly in conjunc- health professionals of the multidisciplinary tion with a Parkinson’s Disease Nurse Spe- team to manage the multifaceted aspects of cialist (PDNS) (Hayes et. al. 2010). . PD.

Duodopa® is an intestinal gel of Levodopa/ The Future of the Parkinson’s Disease Carbidopa (20mg/5mg/ml) enclosed in a 100 Nurse Specialist Role ml cassette delivered via an infusion pump With the growing development of technology for the treatment of Parkinson’s Disease. The and advanced treatments, there is a growing Duodopa® pump infuses the medication di- need for the expertise of Parkinson’s Disease rectly into the duodenum or jejunum via a Nurse Specialists. However, the majority of Percutaneous Endoscopic Gastrostomy PDNS in New South Wales are funded only (PEG) tube with a Percutaneous Endoscopic at a part time capacity and majority by phar- Jejunostomy (PEJ) threaded through it. The maceutical companies and/or research effect is superior to oral medications because grants and therefore ongoing funding cannot the absorption of the medication is optimal in always be guaranteed. Formal education pro- the duodenum or jejunum. The infusion rates grams or courses are in the development on the pump are also pre-set and titrated in- process but still in preliminary stages and dividually for each patient (Hayes et. al. there is a need for accreditation programs to 2010). ensure that PDNS are practicing according to a standardised and evidence based guide- Deep brain stimulation is a neurosurgical op- lines. tion in the treatment of Parkinsonǯs Disease. The treatment involves stereotactic insertion An audit performed by the World Health Or- of electrodes into target sites in the brain, ganisation (WHO) in collaboration with Euro- usually the subthalamic nucleus or globus pean Parkinson’s Disease Association pallidus, and connecting the electrodes to a (EPDA) and International Council of Nurses pacemaker to control the abnormal firing of (ICN) revealed similar responses from cur- neurons by delivering steady and continuous rent PDNS practicing in numerous different electrical impulses to the target sites (Hayes countries. They conclude with a consensus et. al. 2010). view that there is a lack and a need for uni- formity in formal education courses and es- The Role of the Parkinson’s Disease tablished career pathways for the develop- Nurse Specialist ment of a PDNS. A good model for the With the development of advanced treat- Australian PDNS to follow would be to exam- ments in PD, there is growing complexity and ine the role of the PDNS in the United King- subspecialisation of these treatments. Nurs- dom of which the role was first described in ing assessments and interventions are vital in the early 1990’s (European Parkinson’s Dis- the initiation, implementation and ongoing ease Association, 2009). management of advanced treatments in PD. Pre and post treatment assessments includ- Conclusion ing the use of the Unified Parkinson’s Dis- The field of Parkinson’s Disease and ease Rating Scale (UPDRS), cognitive as- Movement Disorders are growing in sessments, quality of life assessments and complexity and specialty as technology and other psychosocial assessments require the the development of advanced treatments expertise, detailed attention and dedicated become more prominent and accessible. time of a specialised PDNS. With the thor- The role of the PDNS and the multidiscipli- oughness of the nursing assessment, the nary team are a vital part of this process and PDNS becomes the ideal person to deter- there is a need for more stable government mine and profile which patient is appropriate funding and accredited education programs for which advanced treatment based on treat- for the development of the role of the PDNS. ment goals, lifestyle wants or needs and comorbidities. References Braak, H., Del Tredici, K., Rüb, U., de Vos, R., Jansen The PDNS is also in the perfect position to be Steur, E. & Braak, E. (2003), ‘Staging of brain pathology related to sprodic Parkinson’s Dis the patient’s point of contact and liaison with ease’, Neurobiology of Aging, Vol. 24, No. 2, pp. the neurologist for medical and psychosocial 197-211 support and also for follow up purposes. Frabbrini, G., Brotchie, J., Grandas, F., Nomoto, M. & These may include issues such as trouble- Goetz, C. (2007), ‘Levodopa-Induced Dyskine sias’, Movement Disorders, Vol. 22, No. 10, pp. shooting device malfunctions, side effects 1379-1389 monitoring and coordinating with other allied Hayes, M., Fung, V., Kimber, T. & O’Sullivan, J. (2010), 36 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

‘Current concepts in the management of Parkin son disease’, Medical Journal of Australia, Vol. 192, No. 3, pp. 144-149 Hornykiewicz, O. (2010), ‘A brief history of levodopa’, Journal of Neurology, Vol. 257, No. 2, pp. 249- 252 McCance, K. & Huether, S. (2006), Pathophysiology: The Biologic Basis for Disease in Adults and Children (5th ed.). United States of America: Mosby European Parkinson’s Disease Association (EPDA) (2009), ‘Parkinson’s Disease Nurse Specialist Core Competencies’, European Parkinson’s Disease Association, Kent, United Kingdom Nutt, J (2007), ‘Continuous Dopaminergic Stimulation: Is it the Answer to the Motor Complications of Levodopa’, Movement Disorders, 22(1): 1-9 Parkinson, J (2002), ‘An essay on the shaking palsy. 1817’, The Journal of Neuropsychiatry & Clinical Neurosciences, Vol. 14, No. 2, pp. 223-236 [Parkinson’s essay was originally published as a monograph by Sherwood, Neely & Jones (London, 1817)].

37 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 Defying the Odds of Gravity: The Case of the Sinking Skin Flap Rhiannon Carey

Abstract For many years, neurosurgeons have performed decompressive craniectomies for the relief of intracranial hypertension. It involves temporarily removing a large segment of skull in order to accommodate for the rising pressure inside the head. Many studies have shown that the procedure is effective in reducing intracranial pressure (ICP) and improving cerebral blood supply and the flow of cerebrospinal fluid (CSF). Nurses are aware that all surgical procedures carry a degree of risk of complications to occur. When thinking about craniectomy, the most common complication would probably be infection.

However, what of the sinking skin flap? The Sinking Skin Flap Syndrome (SSFS) is a complication from decompressive craniectomy characterised by symptoms such as headache, seizures, motor deficits and paralysis. It occurs when the skin covering the segment of missing skull literally sinks down onto the brain tissue and causes direct pressure on the cortex. Theories for why this condition occurs are endless; however studies have shown that the neurological symptoms associated with SSFS vastly improve when a cranioplasty is performed.

Neuroscience nurses are constantly on the lookout for infection and preventing injury to the unpro- tected brain tissue when their patient has had a decompressive craniectomy. However, how aware are they about the complication of the sinking skin flap and its’ potential to cause neurological deterioration? This literature review will explore the case of the sinking skin flap and highlight the need for nurses to be more aware of its’ potential impact on an individual’s recovery.

Keywords: sinking skin flap syndrome, decompressive craniectomy, syndrome of the trephined, cranioplasty, complications.

Introduction theorised that by the look of the scar tissue The decompressive craniectomy was first car- surrounding the holes in these skulls, each of ried out in approximately 4000BC, when the the people to whom they belonged had lived pre-Colombian Incas performed trepanation considerably past the time that the holes were on infants that were epileptic (Kakar, Nagaria actually made (Gross, 1999). As a member of & Kirkpatrick, 2009). The word “trepanation” the Paris Surgical Society, Mr Broca brought originates from the Greek word “Trypanon”, his theory forward. Unfortunately, other mem- meaning “to bore”. The concept of trepanation bers of the Society could not comprehend that has been the interest of brain surgeons since such primitive man could trephine the skull of the early 1860’s (Finger & Clower, 2001). another living human being and not cause death in the process. Overall, it took seven Craniectomies were first performed using a years for Broca’s theory to be proved (Gross, piece of equipment called a Trephine; “a cylin- 1999). drical or crown saw used in the removal of a disc or bone” (Gadde, Dross & Spina, 2012, Alongside Paul Broca was a man named Vic- pg 213). The first trephined skulls were dis- tor Horsley, who also took interest in the con- covered in Peru in the 1860’s and studied by cept of trephining. Whilst Broca’s interest in a man named Paul Broca, the same man that the concept was life-long, Horsley’s interest discovered the area of speech in the brain. He was more of a passing craze. Either way, the contribution of theories put forth by Horsley Questions or comments about this article should be was well regarded and, between the two men, directed to Rhiannon Carey, Registered Nurse Educator, the surgical procedure of craniectomy came St Vincent’s Private Hospital, Melbourne at about (Gross, 1999). [email protected]

38 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

who has an 11 year affiliation with the neuro- to appear, she was to lie flat again thus reliev- surgical department of a large teaching hospi- ing pressure and lessening the hemiplegic tal. Mrs V was first admitted in 2002 with a large intracerebral haemorrhage in her right fronto-parietal lobe. Treatment for this involved a craniectomy and a lengthy stay in hospital, followed by a cranioplasty later the same year.

In 2010 Mrs V was readmitted with complications of hydrocephalus, for which a Ventriculo -Peritoneal (VP) shunt was inserted. At the same time, a reconstructive cranioplasty was performed. Then late in 2013 Mrs V was readmitted for a third time due to an infected shin, which spread to her cranioplasty site and in- Figure 1: (Above) Compression of the right cerebral hemi- fected the bone flap. Mrs V underwent a third sphere, due to the skin flap sinking inward. (Compliments craniectomy to remove the infected bone. of St Vincent’s Public Hospital CMMI Melbourne). Due to the infection testing positive for Methi- cillin-Resistant Staphylococcus Aureus symptoms. A cranioplasty was the required (MRSA), performing a cranioplasty was not treatment as this would allow relief from the appropriate at this time. Instead, Mrs V was compression on the brain tissue. A synthetic commenced on antibiotics, fitted with a pro- bone plate was ordered, antibiotics were com- tective helmet and transferred back to her menced and the warfarin was ceased. Sur- nursing home. gery was planned for 1 week’s time. Post surgery, Mrs V showed significant im- In early 2014, Mrs V was readmitted to the provement. Her left-sided paralysis was be- neurosurgical department for a fourth time. ginning to resolve and she was capable of The handover from her nursing home was performing activities of daily living that she that she had developed a left-sided weakness was unable to do prior to the cranioplasty. A approximately 6 weeks prior, which was worsening over time. On initial examination, Mrs V was lying in a supine position and was able to spontaneously move both her left arm and leg. She had full flexion in both the knee and elbow. However, when trying to sit Mrs V up, she immediately developed a left hemipare- sis. Mrs V was incapable of holding herself up. All that could be elicited from Mrs V’s left side was a response to painful stimuli. Mrs V scored a Glasgow Coma Scale (GCS) of 14/15 and had a systolic blood pressure of 110mm/Hg. She was noted to have an exten- Figure 2: (Above) Post cranioplasty- a synthetic bone sive cardiac history and was currently taking plate now resides where the missing bone once was. warfarin. A Computed Tomography (CT) scan Swelling on the outer edge of the plate is noted. was immediately undertaken. The result of (Compliments of St Vincent’s Public Hospital CMMI the scan showed slight mid-line shift however, Melbourne). the cause for concern was the area where the CT (see Figure 2) showed that Mrs V’s brain bone flap had previously been removed. A tissue was much less compressed and the two-degree compression of Mrs V’s right midline shift had resolved. Despite there be- hemisphere had occurred due to the skin flap ing some swelling and slightly more gliotic, sinking down onto it non-functioning tissue than was present be- (see Figure 1). fore the surgery, Mrs V recovered well and was able to transfer back to her nursing Mrs V was diagnosed with “Sinking Skin Flap home. Syndrome”, otherwise known as the “Syndrome of the Trephined”. Mrs V was to Discussion be managed lying flat, as per her surgeon’s The Syndrome of the Trephined is a delayed orders. She was allowed to sit for meals; how- complication of a craniectomy. Generally, it ever as soon as the left-sided paralysis began will take place more than 8 weeks post the 39 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 procedure (Staykov, Huttner, Kollmer, followed 108 patients who underwent a crani- Ganslandt, Doerfler, Schwab & Kohrmann, ectomy post head injury. Of those, 14 (13%) 2010). There are a number of symptoms one were later diagnosed with the Syndrome of may experience with this condition and quite the Trephined. The study found that older pa- often they may go unnoticed. In many instanc- tients and those with more severe head trau- es, symptoms may be mistaken for those re- ma were at a higher risk for developing the lated to the underlying brain insult for which condition. A second study conducted by Sarov the craniectomy was first indicated. Such et al., (2009), analysed 27 patients undergo- symptoms include dizziness, headache, irrita- ing a craniectomy post hemispheric infarction. bility, seizures, depression, behavioural 3 (11%) of those were later established to changes and focal deficits (Staykov et al., have sinking skin flaps. 2010). It has been shown that the symptoms often worsen when a person is upright and It is important to understand the symptoms of ease off slightly when a person lies flat. It has a sinking skin flap because, if left untreated, it been suggested that the Syndrome of the Tre- can go on to cause “Paradoxical Herniation”. phined is more common in stroke victims, This condition is described when the brain however due to the fact that craniectomies are herniates down through the tentorial notch as mainly performed on individuals who have had a consequence of extremely low pressures a stroke or endured a traumatic brain injury, it inside the skull (Staykov et al., 2010). Man- is difficult to know if it would occur in those agement of herniating brain tissue is generally with other conditions for which craniectomy is achieved by draining excess CSF, hyperventi- not indicated (Staykov et al., 2010). lating the patient and administering Mannitol - all indicators for a high pressure state. Howev- Syndrome of the Trephined occurs because er, paradoxical herniation is a low pressure the atmospheric pressure exceeds intracranial state, therefore management would involve pressure (Sarov, Guichard, Chibarro, Guet- preventing CSF drainage, hydrating the pa- tard, Godin, Yelnik, George, Bousser & Vahe- tient and lying the patient down to try and nor- di, 2009). Increased pressure outside the skull malise the pressures (Gadde et al., 2012). creates a force that pushes down on the skin flap post craniectomy, causing it to collapse Ultimately, the only way to treat the Syndrome down onto the underlying brain tissue. Wheth- of the Trephined is to perform a cranioplasty. er an individual experiences symptoms de- Conservative management alone has been pends on the amount of pressure being exert- found to be largely ineffective (Bhat, Kirmani, ed (Sarov et al., 2009). Nizami, Kumar & Wani, 2011). Mokri (2010) proposes that the symptoms of the Syndrome Research suggests two consistent theories of the Trephined will completely resolve after supporting why a sinking skin flap can cause a cranioplasty is performed. Trnovec, neurological deficits. Kemmling, Duning, Halatsche, Behnke-Mursche & Mursche Lemcke, Niederstadt, Minnerup, Wersching & (2009), agree that neurological improvement Marziniak (2010), suggest that when an area is frequently observed after bone flap re- of brain tissue becomes compressed, the implantation occurs. Furthermore, increased amount of blood flow to that area is restricted. cerebral blood flow post cranioplasty suggests This leads to a decrease in oxygen supply and that lack of blood flow may be an underlying less blood entering the venous system, hence mechanism for why a sinking skin flap causes a reduction in neurological function. Gadde, neurological deterioration (Sarov et al., 2009). Dross & Spina (2012), support the notion that defects in the skull create a siphoning or tur- The Syndrome of the Trephined has a large bulent effect on the flow of CSF. The change negative impact on patients who are trying to in CSF flow then affects the amount of blood rehabilitate after a craniectomy. Many patients supply to the area, leading to a decrease in do not regain full physical and/or cognitive neurological functioning. Symptoms of a sink- function after the procedure, which in the long- ing skin flap can be aggravated by CSF diver- term, creates problems with the psychosocial sion, dehydration and/or changes in position impact of recovery (Hossain-Ibrahim, Tarnaris (Sarov et al, 2009). & Wasserberg, 2011). The challenge of rehabilitation post craniectomy is hard enough, There are minimal statistics available indicat- without having to endure the effects of a sinking the incidence and prevalence of the Syn- ing skin flap. Archavlis & Nievas (2011), report drome of the Trephined, but what is available that the rehabilitation of someone who has appears to be low. A study carried out by suffered a sinking skin flap is significantly pro- Yang, Wen, Shen, Li, Lou, Liu & Zhan (2008), longed because they must undergo periods of 40 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 immobility which potentially could lead to in- impairment. BMC Neurology, 80(10), 1-4. creased rates of thrombolytic events and pulmo- Mokri, B. (2010). Orthostatic headaches in the syndrome of the trephined: resolution following cranioplasty. The nary infection. A lack of knowledge of the Syn- Journal of Head and Face Pain, 1206-1211. drome of the Trephined by healthcare workers Sarov, M.S., Guichard, J.P., Chibarro, S., Guettard, E., means that the symptoms of the condition often Godin, O., Yelnik, A., George, B., Bousser, M. & go unnoticed (Kemmling et al., 2010). If aware- Vahedi, K. (2009). Sinking skin flap syndrome and paradoxical herniation after hemicraniectomy for ness of the condition can be raised, patients may malignant hemispheric infarction. Stroke, 41, 560- be treated more quickly and more accurately, 562. minimising the delay in their rehabilitation Staykov, D., Huttner, H.B., Kollmer, R., Ganslandt, O., (Janzen, Kruger & Honeybul, 2012). Doerfler, A., Schwab, S. & Kohrmann, M. (2010). When decompressive craniectomy does not lead to decompression: sinking skin flap syndrome after in Conclusion tracerebral haemorrhage. European Neurology, 64, The Syndrome of the Trephined is a delayed 319. complication of craniectomy, generally occurring Trnovec, S., Halatsch, M.E., Behnke-Mursch, J. & Mursch, K. (2009). An ultrasound study of brain tissue biome more than eight weeks post procedure. The con- changes in patients following craniectomy. dition is largely characterised by headache, dizzi- Neurorehabilitation, 24, 267-271. ness, mood or behavioural disturbance and focal Yang, X.F., Wen, L., Shen, F., Li, G., Lou, R., Liu, W.G. & deficits. Changing position, particularly into an Zhan, R.Y. (2008). Surgical complications secondary to decompressive craniectomy in patients with a upright position, appears to make the symptoms head injury: a series of 108 consecutive cases. Acta worse. It is also suggested that there is a higher Neurochir, 150, 1241-1248. rate of incidence in the older patient and those with larger craniectomy sites. The neurological deficits associated with a sinking skin flap are a result of poor blood flow and oxygen supply to the craniectomy site and the only way to treat the deficits is to perform a cranioplasty. The Syn- drome of the Trephined is not well known or understood; therefore the delays in recovery associated with the condition are extensive. Prevention of such setbacks in patient rehabilitation is to raise awareness of the complication of the sinking skin flap and ultimately improve care.

References Archavlis, E. & Nievas, M.C.Y. (2012). The Impact of timing of cranioplasty in patients with large cranial defects after decompressive hemicraniectomy. Acta Neuro chir, 154 (1), 1055-1062. Bhat, A.R., Kirmani, A.R., Nizami. F., Kumar, A. & Wani, M.A. (2011). “Sunken brain and scalp flap” syndrome fol lowing decompressive “extra-craniectomy”. Indian Journal of Neurotrauma, 8(2), 105-108. Finger, S. & Clower, W.T. (2001). Victor Horsley on Trephining in Pre-historic Times. Neurosurgery, 48 (4), 911-918. Gadde, J., Dross, P. & Spina, M. (2012). Syndrome of the trephined (sinking skin flap syndrome) with and with out paradoxical herniation: a series of case reports and review. Delaware Medical Journal, 84(7), 213- 218. Gross, C.G. (1999). A Hole in the Head. The Neuroscientist, 5 (4), 263-269. Hossain-Ibrahim, M.K., Tarnaris, A. & Wasserberg, J. (2011). Decompressive craniectomy- friend or foe? Trauma, 14(1), 16-38. Janzen, C., Kruger, K. & Honeybul, S. (2012). Syndrome of the trephined following bifrontal decompressive craniectomy: implications for rehabilitation. Journal of Brain Injury, 26(1), 101-105. Kakar, V., Nagaria, J. & Kirkpatrick, P.J. (2009). The current status of decompressive craniectomy. British Journal of Neurosurgery, 23(2), 147-157. Kemmling, A., Duning, T., Lemcke, L., Niederstadt, T., Minnerup, J, Wersching, H. & Marziniak, M. (2010). Case report of MR perfusion imaging in sinking skin flap syndrome: growing evidence for haemodynamic

41 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 Falls Prevention Strategies among acute Neurosurgical inpatients: a Best Practice Implementation Project Kylie M Wright

Abstract Patient falls are a significant problem and one of the most common adverse events experienced in hospitals. Falls are considered an indicator of the quality of nursing care that requires uncompromising attention and they have been recognised by the Australian Commission on Safety and Quality in Health Care as a National Standard.

This project aimed to conduct an audit of in-hospital falls-prevention practices, to implement evidence-based best practice recommendations and to increase staff compliance within an acute neurosurgical unit. The Joanna Briggs Institute’s Practical Application of Clinical Evidence System and Getting Research into Practice audit tool for promoting change in healthcare practice was used. A baseline audit was conducted measuring eight best practice recommendations, followed by the implementation of targeted strategies and follow-up audits.

The baseline audit revealed large gaps between current practice and best practice and overall performance was poor. Barriers for implementation of best practice falls prevention strategies were identified by the project team and numerous strategies were implemented. There were improved outcomes in the follow-up audits.

The findings showed how audits may be used to promote best practice in healthcare and that fo- cussed education and provision of relevant resources can have an immediate impact on clinical practice. By the end of the project, attitudes to falls prevention had been ‘transformed’ from passive acceptance of falls to active engagement in falls prevention. Future audits are planned to ensure sustainability.

Keywords: falls prevention; best practice; acute neurosurgical inpatients

haemorrhages, fractures and in some cases Background can lead to permanent disability or death. Patient falls are a significant problem and one Falls rates in Australian acute care settings of the most common adverse events experi- are reported to range from 2-5% per 1000 enced in hospitals. Evidence-based best patient separations (Kannus, Khan, and Lord, practice guidelines for preventing falls are 2006), with one health service area reporting available and provide specific information for more than 22,000 falls resulting in patient Australian hospital settings. Most hospitals harm in a one year period, representing a rate have fall prevention policies that include the of 2.5 falls per 1000 patient separations, with use of falls risk assessment tools. However, a higher rate in public hospitals (3.3) than in despite access to these resources, many pre- private hospitals (1.3) (Australian Institute of ventable falls continue to occur in Australian Health and Welfare , 2012). hospitals. The impact of falls on patients and the added Patients admitted to hospital often have costs to the healthcare system are significant. changes in physical or cognitive condition, Patients may experience decreased physical and when combined with unfamiliar surround- activity related to the fear of further falls ( Su- ings present a high risk of falling. Injuries re- zuki, Sonoda, Misawa, Saitoh, Shimizu, and sulting from falls can range from minor bruis- Kotake, 2005), decreased falls self-efficacy ing to serious injuries such as intracranial (the belief that one can independently ambu- Questions or comments about this article should be late without falling), a diminished sense of directed to Kylie M Wright, CNC Neurosurgery, Liverpool dignity (Rapport, Hanks, Millis, and Desh-

Hospital at [email protected] pande, 1998), an increased length of hospital stay, a reduced quality of life, and emotional Copyright©2014 ANNA distress (Department of Health and Aging, 42 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

2013). Furthermore, the cost to the community has a strong commitment to teaching and re- and increase on the demand for health ser- search across a wide range of disciplines and vices for falls-related injuries is considerable. serves between 1.3 and 1.4 million people in It has been predicted that unless effective pre- the South West of Sydney, with the most cul- ventative strategies are utilised, the cost at- turally diverse population in the state,39% of tributable to falls-related injury will increase people from non-English speaking back- three-fold to $1375million per annum by 2051 grounds (NESBs).The hospital has well estab- (Department of Health and Aging, 2013). lished policy directives on falls prevention and management for inpatients including a falls Multiple risk factors that can contribute to in- risk assessment tool embedded into the elec- hospital falls have been identified in the litera- tronic medical record, patient/carer falls bro- ture and include patient characteristics, staff chures in six different languages, established behaviour and the hospital environment (Stern ‘falls champions’ in clinical areas, patient falls and Jayasekara, 2009). Patient risk factors risk assessment guidelines and prevention include advanced age, muscle weakness, gait strategies endorsed by the New South Wales or balance problems, visual impairment, al- (NSW) Clinical Excellence Commission tered bowel or bladder elimination patterns, (CEC). Despite a culture of falls prevention dizziness or vertigo, depression, cognitive awareness and established resources, the deficits, impaired activities of daily living, use 2012 falls rates for the neurosurgical unit was of psychotropic medications and a history of 6.44 per 1000 beds (Liverpool Hospital. Inci- falls (Hendrich, Bender, and Nyhuis, 2003; dent Management System). Rubenstein and Josephson, 2002; Oliver, Brit- ton, Seed, Martin, and Hopper, 1997; Leipzig, Not only are patient falls one of the most com- Cumming, and Tinetti, 1999; Tinetti and Ku- mon adverse events experienced in hospitals, mar, 2010). Neurosurgical patient groups ex- but such incidences are considered an indica- perience many of these identified risk factors, tor of the quality of nursing care that require with falls rates of 4-12 per 1000 bed days in uncompromising attention. The Australian patients over the age of 65 years and more Commission on Safety and Quality in Health than 40% of patients with specific clinical neu- Care (ACSQHC) has recognised the preven- rological problems experiencing one or more tion of falls and harm from falls as a National falls during their hospital admission Standard (Australian Commission on Safety (Australian Commission on Safety and Quality and Quality in Health Care,. 2012). In brief, in Health Care, 2009). this standard requires that health service or- A large number of interventions for preventing ganisations have governance structures and in-hospital falls have been recommended in systems in place to reduce falls and minimise the literature. These include early detection harm from falls; that patients on presentation, and treatment of conditions such as inconti- during admission and when clinically indicated nence, eyesight problems and delirium, re- are screened for risk of a fall and the potential viewing medication regimes, providing safe to be harmed from falls; and that patients and non-slip footwear to patients and modifying carers are informed of the identified risks from the environment with things such as handrails falls and are engaged in the development of a and non-slip flooring (Stern and Jayasekara, falls prevention plan. Overall, the aim of this 2009). Current literature recommends a com- standard is to reduce the incidence, or num- prehensive and multi-factorial methodology to ber, of patient falls and minimise harm from falls prevention, involving the use of risk as- falls when they occur, hence in-patient falls sessment tools and targeted interventions prevention is very much a priority of modern (Stern and Jayasekara, 2009; Australian Com- Australian healthcare. mission on Safety and Quality in Health Care, 2009; Cameron, Gillespie, Robertson, Murray, Objectives Hill, Cumming, et al, 2012; Oliver, Healy, and This project aimed to conduct an audit of in- Haines,2010). hospital falls prevention practices, to implement evidence-based best practice and as- This project aimed to conduct an audit of in- sess the effects of these strategies at minimis- hospital falls prevention practices, to imple- ing in-hospital falls in a neurosurgical unit in a ment evidence-based best practice and as- large tertiary hospital. The overall purpose of sess the effects of these strategies at minimis- the project was to increase staff compliance ing in-hospital falls. The project was undertak- with falls prevention best-practice to prevent in en in the neurosurgical unit of a large 855 bed -hospital falls amongst at-risk patients. Objec- public, tertiary referral hospital and major trau- tives included: ma centre in Sydney, Australia. The hospital 43 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 x To improve the local practice of com- throughout this project. The eight identified pleting patient falls risk assessments criteria for data collection were divided into appropriately, accurately and in a timely assessment, education and intervention cate- manner. gories and were measured as follows: x To ensure health care professionals Assessment have been educated regarding falls as- 1. Fall risk assessment is done upon ad- sessment and prevention strategies and mission. targeted interventions have been imple- This criterion was considered met if the case mented. notes/electronic medical record (EMR) showed a risk assessment completed within x To ensure patient and family education four hours of admission. regarding falls is conducted. 2. Fall risk assessment is done upon An evidence-based practice approach under- transfer pins the entire implementation project. This criterion was considered met if the case notes/EMR for patients that have been trans- Methods ferred (intra-hospital transfer) show a risk as- The project used the Joanna Briggs Institute sessment completed within four hours of (JBI) Practical Application of Clinical Evidence transfer. System (PACES). JBI PACES is an online tool for health professionals and/or researchers to 3. Reassessment occurs when there is a use for collection and comparison of data and change in condition or following a fall. to conduct efficient audits in small or large This criterion was considered met if the case healthcare settings. PACES has been de- notes/EMR for patients who have had a signed to facilitate the use of audits to imple- change in clinical condition (that affects their ment evidence based health practice and in- falls risk status) or experienced a fall, included cludes the Getting Research into Practice a reassessment performed within four hours (GRIP) framework that may be used to help of this event. identify factors underpinning gaps between practice and best practice and strategies to 4. Patients who have experienced a fall overcome them. The project involved three are considered at high risk for future falls phases as follows. This criterion was considered met if it was documented in the case notes/EMR for pa- Phase 1—Baseline audit tients who have had a history of falls, are as- A baseline audit of in-hospital falls prevention sessed as high risk for future falls according to practices was conducted. A multidisciplinary the risk assessment. team of key stakeholders was formed to sup- 5. Fall risk assessment is done accurately port the work of this project. The lead author using a falls assessment tool of this article, is the neurosurgical clinical This criterion was considered met if the case nurse consultant at the hospital where the notes suggest the fall risk assessment was project was implemented, and led the project done accurately. If the accuracy of the risk as part of the JBI Clinical Fellowship program assessment is not clear from the notes, then (Joanna Briggs Institute, Adelaide, South Aus- the patient can be visited to determine the tralia). Involvement of the project team was in accuracy of the assessment. varying capacities of support, data collection, data entry and/or participation. Patients/ Education consumers were also inclusive of the team at 6. Healthcare professionals have received a participatory level. education regarding falls assessment and prevention strategies The objectives of the baseline audit were to This criterion was considered met if staff establish the size and nature of the gap be- members in the participating wards report that tween practice and best practice in falls pre- they have received education in the last two vention strategies on the neurosurgical unit. years. Question: “Have you received educa- The JBI best practice recommendations, relat- tion regarding falls assessment and preven- ed to falls assessment and preventative inter- tion strategies in the last two years?” This is ventions, are based on a structured search of by convenience sampling. Sample: 30 the literature and selected evidence-based healthcare staff. health care databases. Eight criteria based on 7. Patient and family education is carried these recommendations were audited out for patients at risk of falls 44 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

This criterion was considered met if from the each best practice criterion was reviewed and case notes, for patients at risk of falls, there is strategies for improved compliance were dis- documentation that patient and family education cussed. Furthermore, potential barriers and strat- has occurred. egies to overcome such barriers, as well as resources required to implement change strategies Intervention were identified, discussed and formally docu- 8. Targeted interventions are implemented mented. according to risk factors A GRIP report matrix was generated which kept This criterion was considered met if it is docu- the project team informed, as well as provided a mented in the case notes for patients assessed means of gathering and recording their opinions as at risk, that there has been implementation of and clearly outlining the implementation plan and targeted interventions to address every identified the team involvement. As described further in the risk factor. results section, a major strategy identified to close the gap between practice and best practice The baseline audit was conducted over a 4 week was educating clinicians on best practice falls period. To assess the compliance of each audit prevention strategies. This education, along with criterion, the case notes/EMR of 30 neurosurgi- other strategies, was implemented during Phase cal inpatients were examined. To assess compli- 2 of the project which was conducted over a 4 ance for the audit criteria regarding staff educa- month period. tion, 30 clinicians working on the neurosurgical ward were interviewed by the project lead. Phase 3—Follow-up audits- Cycle 1 & Cycle 2 The objectives of the follow-up audits were to Phase 2 – GRIP (Getting Research Into Prac- assess whether there had been improvement in tice) Strategy compliance with best practice; to establish if im- The objectives for the second phase of the pro- provements, if any, had been sustained, and ject were to gain an understanding of the barriers identify remaining areas where further improve- underpinning gaps between practice and best ments are required. Cycle 1 and Cycle 2 post- practice found in the baseline audit and to imple- implementation audits and collection of data ment tailored strategies to close gaps and ad- were repeated using the same eight criteria de- dress barriers. Using the PACES program, base- fined in Phase 1. There were no variations to the

Figure 1: (Above) Baseline audit results for the topic, the criteria, the sample size, the character- neurosurgical ward istics or location of the project during the follow- line audit results were collected for analysis and up cycles. discussion by the project team and proposed The follow-up data was entered into the PACES strategies for improving compliance of falls pre- program and data analysis comparing follow-up vention best practice were identified. results with those of the baseline audit were undertaken to examine any change in compliance Open communication and engagement with all rates. Phase 3 was conducted over a 4 week stakeholders was maintained and welcomed at period on both occasions. all times throughout the project and provided the platform to suggest and discuss strategies for The project received formal approval by the improvement. Via a series of face-to-face meet- South Western Sydney Local Health District Hu- ings using practice development principles and e man Research and Ethics Committee (NSW). -mail correspondence between the project team,

45 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 Results—Baseline Audit ity and the most feasible strategy to imple- The percentages for compliance with each ment as a means of bringing best practice audit criterion in the baseline audit for the neu- falls prevention strategies more in line with rosurgical ward are shown in Figure 1. The actual practice. A working party was formed, best baseline performance was found for crite- led by the project leader, to collaboratively rion 4, which measured that patients who had develop, distribute and deliver a ‘Falls Preven- experienced a fall were considered at high risk tion and Management’ education package for future falls. In 52% of cases there was evi- incorporating best practice strategies and dence of this practice. practical application.

Performance emerged as very poor in the To further build upon falls prevention aware- baseline audit for the remaining criteria. Only ness, an overview of falls risk factors, impact 30% of occasions showed evidence that the of falls, and falls incidents (case studies) that fall risk assessment was done upon patient had occurred over the past 2 years were pre- transfer (Criterion 2) and/or when there was a sented to nursing staff via formal presenta-

Figure 2: (Above) Baseline and Follow-up Cycle 1 audit results for the neurosurgical ward. change in condition or following a fall tions and analysis of falls incident reports (Criterion 3). There was evidence that only were discussed at the ward level. The project three patients had targeted interventions im- team also engaged with clinicians and ena- plemented according to risk factors (Criterion bled essential practice changes to the docu- 8), and one healthcare professional, a medical mentation of targeted falls prevention strate- officer, had received education regarding falls gies via implementation of a Falls Risk As- assessment and prevention strategies, result- sessment Management Plan (FRAMP) Form ing in very low compliance of 3% (Criterion 6). (Clinical Excellence Commission, 2013). Of most concern, was that upon review of 30 patient medical records there was no docu- In addition, the hospital had patient/carer falls mented evidence that patient and family edu- brochures in six different languages, however cation was carried out for patients at risk of they needed updating and were very low on falls (Criterion 7). stock. The project leader sourced, copied and distributed the NSW CEC endorsed falls edu- GRIP Strategy cational materials and engaged consumer representatives regarding appropriateness of Table 1 shows the barriers to best practice this material. As a result, multi-lingual patient/ falls prevention strategies that emerged from carer falls education material encompassing the project team discussions of the Phase 1 17 different falls related topics were made results. It also shows resources identified as available to the bedside clinicians. The ‘Falls being required to implement the strategies Prevention and Management’ education pack- and the outcomes. age informed staff of the range and location of educational material. Clinicians were encour- Providing education to the clinicians was se- aged to distribute such material to patients lected by the project team as the highest prior- and families as a strategy to provide educa-

46 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

Barriers Strategies Staff nurses’ lack of awareness Overview of falls risk factors, impact of falls, falls incidents over the past 2 years and understanding of the extent (analysis of SAC1 & SAC 2 incidences) to be presented formally to nursing of the ‘problem’ of inpatient staff. falls and its importance in rela- Analysis of falls incident reports at ward level – present & communicate analysis tion to the National Standards. to core ward groups and discuss findings. Build awareness by—Displaying falls rates “running tally”, Commencement of ward “measles chart” Participate in interactive discussion & open communication Staff nurses lack awareness Build/promote awareness by—Education sessions/handouts; Signs in clinical are- and understanding of the evi- as; Distribution of baseline audit results; Focus groups; Environmental check- dence base for falls prevention lists strategies among in-patients Identify and engage ward based Falls Champions Promote awareness of JBI Best Practice Implementation Project- initiate as a regular agenda item on all ward based meetings. Participate in interactive discussion & open communication No existing standardised hospi- Engage hospital Falls Reference group & Falls Prevention Committee and collabo- tal wide falls prevention educa- rate with key stakeholders for input into education session content. tion package Develop, distribute and deliver a ‘Falls Prevention and Management’ education package incorporating—Falls risk assessment; Management strategies; Post fall management including a case study to highlight each section. Gain Executive support for mandatory nursing attendance at educational session. Engage key stakeholders to deliver education package A culture of ‘weekly’ falls risk Reinforce via education session situations when the falls risk assessments should assessments were embedded be completed and re-assessed and in what time frame including use of the into clinical practice due to an over-ride option existing weekly “falls score Perform an audit from the EMR looking at compliance of falls risk assessments on compliance” audit occurring all days of the week (not just Wednesdays), and analysis of times the assess- routinely on Wednesdays. ments are being completed. Provide feedback on appropriate completion of falls risk assessments and use of the over-ride option. Existing method of “flagging” Implementation of ‘green’ initiatives including—High risk fall sign above bed; high risk falls patients was Inserts to place in bedside folders; Post fall stickers to be placed in patient often invisible and ineffective records Documentation of falls risk assessment score on electronic handover Falls assessment score included in all clinical handovers Agreement of dedicated “High visibility beds” – with placement of “flagged” high risk patients into such beds Development of a ward based “Falls Resource Manual” Provide feedback on appropriate “flagging” of high risk patients Limited range of educational Source, copy and distribute falls educational material and engage consumer repre- materials for falls prevention sentative regarding appropriateness of material. strategies available for patients Source multi-lingual patient/carer falls education material. and carers Inform nurses of range of educational material and encourage distribution to patients/carers as an education strategy. Development of a ward based “Falls Resource Manual” No appropriate falls risk pre- Make essential practice changes to documentation via implementation of a Falls vention strategy / management Risk Assessment & Management Plan (FRAMP) form - falls care plan that plan documentation structure highlights individual patient risk factors and records actions implemented to record targeted interventions To provide nurses with education regarding use of newly implemented FRAMP in patient health care records Development of a ward based “Falls Resource Manual” Provide feedback on appropriate completion of FRAMP Reluctance of nurses to partici- Engage the multidisciplinary team pate in the project due to in- Use a practice development approach, to explore staff opinions and use their ad- creased workload and compet- vice and ideas regarding implementation of best practice/patient centered ing pressures care. Provide positive feedback and encouragement to improve fall prevention practices Make communication channels available Participate in interactive discussion & open communication Provide feedback on ward based falls rates per month

Table 1: (Above) – GRIP (Identified barriers to best practice and strategies to overcome them)

47 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

Barriers Strategies Continued lack of Overview of falls risk factors, impact of falls, falls incidents and phase 3-cycle 1 results, awareness and under- and FRAMP utilisation to be presented formally to staff standing across some Build and promote awareness via continued education sessions provided to—Allied health care staff of the Health; Night duty & weekend staff; Non-clinical staff (targeted at ward orderlies, evidence base for inward clerks, technicians etc); Consumer representatives; Medical Grand Rounds patients falls prevention (targeted at medical students and all medical staff) strategies Analysis of falls incident reports at ward/organisational level – present & communicate analysis to widespread committee groups and discuss findings & ongoing strategies. Participate in interactive discussion , open communication & action planning

Table 2: (Above) GRIP (Identified barriers to best practice and strategies to overcome them). tion regarding falls prevention. 97%. Table 2 shows the barriers to best practice Follow-up Audits—Cycle 1 falls prevention strategies that emerged from The percentage of compliance for the audit the project team discussions after the phase 3 criteria found in the follow-up Cycle 1 audit, - cycle 1 results were analysed. together with the results from the baseline audit is displayed in Figure 2. Looking at the Supplying further education to the clinicians results of the follow up audit, compared with as well as education to non-nursing staff those in the baseline audit, there has been an groups was selected by the project team as overall improvement in compliance of imple- the next step and a more hospital wide ap- menting best practice falls prevention recom- proach was implemented in bringing best mendations. practice falls prevention strategies in the facility more in line with best practice. The results suggest that whilst there has been an emphasis on staff education, this translat- Building further upon falls prevention aware- ed only partly into implementation in practice ness, an overview of falls risk factors, impact for some criteria. The criterion for performing of falls, and falls incidents (case studies), and a falls risk assessment upon admission results of the Phase 3-Cycle 1 results were (Criterion 1) remained reasonably static; how- presented via a series of inservices and to a ever the remaining seven criteria showed im- hospital wide nursing audience via the facility provements. The criteria measuring if Nursing & Midwifery Grand Rounds. In addi- healthcare professionals had received education, specific education was delivered to Clini- tion regarding falls assessment and preven- cal Nurse Educators (CNEs) and nominated tion strategies (Criterion 6), if there was docu- ward Falls Champions in the form of a master mented evidence that patient and family edu- class. The project team also continued en- cation was carried out for patients at risk of gagement with clinicians and enabled essen- falls (Criterion 7), and if there was evidence tial practice changes to the documentation of that patients had targeted interventions imple- targeted falls prevention strategies via implemented according to risk factors (Criterion 8) mentation of the Falls Risk Assessment Man- showed the largest increase in compliance agement Plan (FRAMP) Form (Clinical Excel- over baseline with improved changes of up to lence Commission, 2013).

Figure 3: (Above) Baseline and Follow-up Cycle 2 audit results for the neurosurgical ward

48 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

Furthermore night duty and weekend nursing widespread impact on falls prevention and staff, as well as medical, allied health, non- management across the organisation because clinical staff groups, and consumer represent- strategies were adopted and supported across atives were also provided with targeted edu- the hospital and had the underpinning support cation. of the Executive and Governance structures. Medical Governance was obtained and was An analysis and discussion of falls incidences imperative to the success of this project, par- were broadened beyond the neurosurgical ticularly in regards to medication reviews, di- ward to an organisational wide perspective. agnosis and management of delirium and Falls incidences and prevention and manage- treatment of orthostatic hypotension. This sup- ment strategies were regular agenda items port is reflected in the improvement in compli- tabled at all committee meetings throughout ance with the audit criteria and changes in the hospital. practice. Cycle 2 The ‘Falls Prevention and Management’ edu- The percentage of compliance for the audit cation package incorporating best practice criteria found in the follow-up Cycle 2 audit strategies and practical application was an together with the results from the baseline effective strategy. The content of the package audit is displayed in Figure 3. Looking at the was divided into three sections and included results of the follow up Cycle 2 audit, com- principles in line with the best practice recom- pared with those from the baseline audit, over- mendations including how and when to do the all improvement in compliance of implement- falls risk assessment on the EMR, implemen- ing best practice falls prevention recommen- tation and documentation of targeted manage- dations has been maintained for most criteria. ment strategies via the FRAMP (Clinical Ex- cellence Commission, 2013), engaging in pa- The Cycle 2 follow up results suggest that tient education and post fall management. It improvements in compliance demonstrated in also included a case study to highlight each the Cycle 1 follow up audit were maintained, section of the education package which could or further improved upon, for most criteria be modified to suit any clinical specialty. The when the re-audit occurred 3 months later. package gave the staff the knowledge needed The criterion measuring if fall risk assessment to prevent falls and to help their patients stay was done accurately using a falls assessment safe and was designed to be administered as tool (Criterion 5), and the criterion measuring a presentation in a one hour block, or could be if healthcare professionals had received edu- given section by section in 20 minute cation regarding falls assessment and preven- timeframes for clinical areas that had difficulty tion strategies (Criterion 6) remained static. releasing staff for extended education. Execu- However the criteria for reassessment of falls tive support was obtained, attendance was risk occurring when the patient has been deemed mandatory and the education pack- transferred (Criterion 2) and when there is a age was delivered across the facility initially change in condition or following a fall over six consecutive sessions capturing 500 (Criterion 3) slightly decreased. The remaining nursing staff. four criteria including performing the fall risk assessment upon admission (Criterion 1), Due to difficulties capturing all staff, CNE’s deeming patients who have experienced a fall within the facility were engaged in the educa- considered at high risk for future falls tion process and were given the education (Criterion 4), if there was documented evi- package as a PowerPoint presentation so it dence that patient and family education was could be delivered to after-hours and weekend carried out for patients at risk of falls (Criterion staff. In addition, they were empowered to 7), and if there was evidence that patients had take informal and formal opportunities to teach targeted interventions implemented according their ward colleagues to amplify and personal- to risk factors (Criterion 8) showed further in- ise the learning and make it ward specific. As creases in compliance in the cycle 2 follow up a result, clinical staff across the facility re- audit with improved changes further increas- ceived tailored education and are now more ing up to 23%. aware of the best practice recommendations for falls risk management and how to incorpo- Discussion rate this into their clinical practice. Further This best practice implementation project education strategies and continued education achieved improvements in compliance in all at a ward and organisational level resulted in eight best practice recommendations in the an additional 500 staff undergoing falls pre- neurosurgical ward over the nine month peri- vention and management education, hence by od. These practices, however had a more 49 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 the conclusion of the project 1000 staff had ed via this project that clinicians may have received targeted falls education. improved their practice in relation to falls management strategies, but do not always docu- Improvements in practice is evidenced by ment their care hence the practice change is weekly “snap shot” falls risk assessment com- not recognised at any point of the audit. A lack pliance scores across the organisation im- of documentation does not necessarily indi- proving from 76% at the beginning of the pro- cate a lack of care, skill or knowledge and a ject to an all-time high of 93.5% compliance future strategy will be to raise awareness of within one month of the education package falls prevention related documentation and delivery. Furthermore falls rates for the neuro- reporting of practice in the patients’ EMR. surgical ward have remained static throughout The reduction and prevention of falls is a qual- the study period however severity assessment ity initiative that is directly related to the Na- codes of falls have improved across this high tional Standards. It is a priority for the facility risk inpatient area. to keep the focus on falls in its efforts to improve patient safety and continue to meet this The successful implementation of the FRAMP standard. Implementation of the evidence (Clinical Excellence Commission, 2013), im- through this project will be sustained by con- pacted targeted falls prevention interventions tinued analysis and distribution of falls data, organisation-wide. This tool incorporates a falls tally boards, environmental checks and care plan that highlights individual patient risk ‘measles charts’ in clinical units. Facility poli- factors and systematically records and evalu- cies and procedures have been revised to ates the targeted actions that have been im- reflect changes in practice as a result of this plemented. This meant that many more pa- project and multiple dedicated “Falls Champi- tients received individual assessments and ons” who have undergone specific falls cham- care that is essential to preventing falls in hos- pion training via a one day workshop have pital. The FRAMP (Clinical Excellence Com- been appointed in each ward. Ongoing en- mission, 2013), and its utilisation was intro- gagement with the Falls Prevention Commit- duced to clinicians via the ‘Falls Prevention tee will continue and the audit will be repeated and Management’ education package as well annually. as one to one education administered at the bedside. Despite initial resistance and com- Conclusion ments such as “not more paperwork”, it was It is indisputable that patient falls and patient embraced by all clinical areas once its useful- fall related injuries are considered an indicator ness had been established and the form utili- of inadequate nursing care and are currently sation has now become embedded into the one of the most worrying clinical issues nursing admission process and ongoing prac- amongst clinicians. Preventing falls and harm tice. from falls has been recognised as a standard that must be met by the National Safety and Some best practice recommendations im- Quality Health Service (NSQHS) Standards. A proved to a lesser degree in the follow up au- high priority needs to be given to the preven- dits than others. The performance of a falls tion of in-hospital falls in order to avoid poor risk assessment upon transfer of a patient, patient outcomes. and change in condition warrants ongoing attention. The primary resource required to The purpose of this project was to increase improve these areas of practice is clinician staff compliance with falls prevention best- time for re-assessment, and hence, this may practice within an acute neurosurgical setting. partly be explained by clinicians feeling that This included an audit of in-hospital falls pre- their workload is too high to spend the addi- vention practices, implementation of evidence- tional time required to repeat the falls risk as- based best practice, and assessment of the sessment when it may have only been recent- effects of implemented strategies in a Neuro- ly performed. Falls prevention is one of a surgical ward in a large tertiary hospital. The number of initiatives competing for staff and project succeeded in achieving the objectives managerial time and with large, busy wards as all criteria used to audit practice improved with a high turnover of patients and staff, such after a ‘Falls Prevention and Management’ as the neurosurgical ward, problems with ful- education package incorporating best practice filling some of the best practice criteria were recommendations and various other strategies challenging for the bedside clinicians. Further- were implemented. Whilst it is suggested that more, documented evidence is required for the implementation of evidence based best some criteria to be achieved. It is thought that practices will improve patient care and out- through the awareness strategies implement- comes, this cannot be assured on the basis of 50 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 this project alone. Some criteria measured in this Kannus, P, Khan KM and Lord SR (2006) ‘Preventing falls project did not improve to a great degree with among elderly people in the hospital environment’, Medical Journal of Australia, 184 (8): 372-3. increases in compliance minimal, leaving plenty Leipzig, RM, Cumming, RG and Tinetti, ME (1999) ‘Drugs and of room for improvement. By the end of the pro- falls in older people: a systematic review and meta- ject however, attitudes to falls prevention on the analysis:1 Psychotropic drugs’, Journal of American neurosurgical ward and across the facility had Geriatric Society, 47 (1): 30-39. Liverpool Hospital. Incident Management System. been ‘transformed’ from passive acceptance of Oliver, D, Britton, M, Seed, P, Martin, FC and Hopper AH falls, to active engagement in falls prevention and (1997) ‘Development and evaluation of evidence minimisation of injury. based risk assessment tool (STRATIFY) to predict which elderly inpatients will fall: case-control and Identification of time limitations within a nurse’s cohort studies’, British Medical Journal, 315 (7115): daily role and often poor documentation practices 1049-53. Oliver, D, Healy, F and Haines, TP (2010) ‘Preventing falls were two of the main barriers underpinning the and fall-related injuries in hospitals’, Clinic Geriatric gaps between best practice recommendations Medicine, 26 (4): 645-92. and actual practice. Identification of these barri- Rapport, LJ, Hanks, RA, Millis, SR and Deshpande, SA ers facilitated understanding why for some of the (1998) ‘Executive functioning and predictors of falls in the rehabilitation setting’, Archives of Physical audit criteria performance improved only minimal- Medicine and Rehabilitation, 79: 629-33. ly. This has highlighted the importance of future Rubenstein, LZ and Josephson, KR (2002) ‘The epidemiology education initiatives targeted at clinicians includ- of falls and syncope’, Clinical Geriatric Medicine. ing a focus on this aspect of care. 2002, 18 (2): 141-58. Stern, C, and Jayasekara, R (2009) ‘Interventions to reduce Although it is acknowledged that a focus on falls the incidence of falls in older adult patients in acute- tends to increase the number of reported falls on care hospitals: a systematic review’, International Journal of Evidence Based Healthcare, 7 (4): 243-9. a ward, potential long term benefits such as a Suzuki, T, Sonoda, S, Misawa, K, Saitoh, E, Shimizu, Y and reduction in the neurosurgical ward falls rates will Kotake, T (2005) ‘Incidence and consequence of falls be measured over a longer study period. Future in inpatient rehabilitation stroke patients’, audits are planned to ensure changes are sus- Experimental Aging Research, 31: 457-469. Tinetti, ME and Kumar, C (2010) ‘The patient who falls: “it’s tained and improved with the aim that the neuro- always a trade-off”’, Journal of American Medical surgical ward and wider hospital not only pre- Association. 303 (3): 258-66. vents falls and harm from falls but can give a patient centred approach and instil confidence in our patients and their carers that we are doing all we can to prevent such events. Acknowledgements The author wishes to acknowledge the JBI for fostering and facilitating this project through the Clinical Fellowship Program.

References Australian Commission on Safety and Quality in Health Care (2009) ‘Preventing falls and harm from falls in older people: best practice guidelines for Australian Hospitals’. Australian Commission on Safety and Quality in Health Care (2012) ‘Safety and Quality improvement guide Standard 10 – Preventing Falls and Harm from Falls’: 66-76. Australian Institute of Health and Welfare (2012). ‘Australian Hospital Statistics 2010-2011’. In: Australian Government, editors: 376-7. Cameron, ID, Gillespie, LD, Robertson, MC, Murray, GR, Hill, KD, Cumming RG, et al (2012) ‘Interventions for pre venting falls in older people in care facilities and hos pitals’, Cochrane Database Systematic Reviews, 12 Clinical Excellence Commission (2013) Falls Risk Assess- ment and Management Plan (FRAMP), SMR060.912, Sydney: State Health Forms, NSW Health. Department of Health and Aging (2003) ‘Projected costs of fall related injury to older persons due to demographic change in Australia’, In: Australian, Government, editors. Hendrich, AL, Bender, PS and Nyhuis, A (2003) ‘Validation of the Hendrich II fall risk model: a large concurrent case/control study of hospitalised patients’, Applied Nursing Research, 16 (1): 9-21. 51 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 Freezing of Gait: What to do when the Parkinson’s Medications do not work

Melissa Drury

Abstract Parkinson’s Disease (PD) is the second most common neurological disorder affecting around 80,000 Australians. One of the most troublesome and incapacitating motor symptoms affecting people with PD is freezing of gait (FoG). FoG has been recognised to be a major cause of falls, hospitalisation, decreased quality of life and loss of independence. FoG is described as a transi- ent start and turning hesitation, where the patient’s feet appear to be glued to the ground. The relationship between FoG and PD medication remains complicated, as FoG sometimes does not respond to PD medication or in rare cases; the medication can actually cause FoG. It is important to implement other interventions such as auditory/visual cueing, physiotherapy as well walking aids with auditory/visual cueing devices. The role of the Parkinson’s Disease Nurse Specialist (PDNS) also plays an important role in educating the patient with Parkinson’s and their family as well as identifying the stage/s of FoG. Further research and interventions are required to provide a better quality of life for people with this debilitating motor disease.

Keywords: Freezing of Gait, Parkinson disease

Introduction early stage disease which increased to 60% Parkinson’s Disease (PD), is the second most in more advanced patients with Parkinson’s; common neurological condition affecting Aus- Heremans, Nieuwboer & Vercruysse (2013) tralians, second only to Alzheimer’s. Currently reported 26% in the earlier stages and 70% in there are approximately 80,000 Australians the later stages of PD. who have been diagnosed with this slow, chronic and non-curable disease (Parkinson’s FoG most commonly occurs on initiation of Australia, 2011). PD is characterised by both walking, walking in a straight line, passing motor and non-motor symptoms resulting through narrow passages, turning and on from the loss of dopaminergic neurons in the reaching the destination (Nutt et al, 2011). basal ganglia (Vandenbossche, Deroost, FoG can last from a few milliseconds to over Soetens, Coomans, Spildooren, Vercruysse, 30 seconds. Certain circumstances such as Nieuwboer & Kerckhofs, 2013). One of the narrow doorways, dual tasking, mental dis- most troublesome and incapacitating motor tractions and crowed places have been iden- symptoms affecting people with PD is freez- tified to exacerbate FoG (Nutt et al, 2011). ing of gait (FoG). FoG has been recognised However, Nutt et al (2011) also reported that to be a major cause of falls, hospitalisation, emotional excitement, auditory cueing at the decreased quality of life and loss of inde- proper pace, targets for stepping and climb- pendence (Panisset, 2004). ing stairs can ameliorate FoG. Levodopa therapy has been identified as the FoG is defined as “brief, episodic absence or gold standard of treatment for people with marked reduction of forward progression of Parkinson’s (PWP); however the relationship the feet despite the intention to walk” (Nutt, between FoG and PD medication remains Bloem, Giladi, Hallett & Horak, 2011). Pa- complicated. FoG typically occurs when the tients with PD describe the sensation of FoG PWP is in the pre-medicated, “OFF” state. as if their feet are glued to the floor. The prev- FoG is abolished when the person reaches alence of FoG varies between reports: Con- their optimal medicated, “ON” state. Howev- treras & Grandas (2012) found FoG in 7% in er, PD medications do not always alleviate FoG and the freezing continues into the “ON” Questions or comments about this article should be state. There is also a rarer phenotype in directed to Melissa Drury, CNS PD & Movement Disorders, Westmead Hospital at which the PD medications induce the “ON” [email protected] state FoG, even when FoG is absent in the “OFF” state (Espay, Fasano, van Nuenen, Copyright©2014 ANNA Payne, Snijders & Bloem, 2012). These rare

52 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

FoG conditions can create a troublesome and ethnic background, age, disease onset and complicated symptom that is refractory to gender have not been associated with the medical treatment. likelihood of developing FoG (Panisset, 2004). However, it has been identified that Pathophysiology longer disease duration, more advanced PD, The pathophysiology of FoG is largely un- greater disability from PD symptoms, pro- known and seems to differ from that of the longed treatment with PD medications and cardinal features of Parkinsonism (tremor, depression are associated with increased risk bradykinesia and rigidity). This is not surpris- of developing FoG (Panisset, 2004). ing as FoG occurs in other conditions such as Normal Pressure Hydrocephalus (NPH), De- Intervention mentia with Lewy Body (DLB), Progressive Since not all forms of FoG are responsive to Supranuclear Palsy (PSP) and Vascular Par- PD medications, it is important to implement kinsonism (VP) (Nutt et al., 2011). After re- other therapeutic options to help with mobility. viewing the literature from 1966 to 2011, Nutt Physiotherapy and exercise assist in walking, et al (2011) proposed five directions for devel- falls prevention and strengthening and are oping a hypothesis on the pathophysiology of identified as an important aspect of treatment FoG. More recent publications (reviewed by of FoG (Nutt et al, 2011). Heremans et al (2013) support these five theories: Auditory and visual cueing has been identified 1. Abnormal Gait Pattern Generation to be an important intervention for FoG The person with FoG also exhibits a progres- (Delval, Moreau, Bleuse, Tard, Ryckewaert, sive shortening of step-length prior to the Devos & Defebvre, 2013). Cueing acts to freezing episode. This form of spatial- substitute the failed automation of walking to temporal incoordination would suggest a dis- a conscious learnt activity. Visual cues that rupted pattern generation. alleviate FoG include lines on the floor and 2. A problem with Central drive and other visual targets which stimulate the PWP Automaticity of Movement to walk – including LASER projections onto In FoG, there is an impairment of the auto- the floor from a specially designed walking mated tasks such as posture and gait. With aid (stick or walker). Auditory cues include this impairment the normally automated tasks walking to a metronome beat or music, count- demand increased attention. This explains ing numbers, singing a rhythm out loud or why freezing is exacerbated when attending simply reminding the patient to adopt a to dual tasks and how a decrease in FoG is ‘marching’ (high-stepping) gait. derived from external cues (which provide a drive to the patients’ stepping patterns). The PDNS plays an important role in the management of a PWP with FoG. A dose 3. Abnormal Coupling of Posture with cycle (a trial of the clinical response to either Gait levodopa or apomorphine) is a helpful diag- The upper body propels forward in anticipa- nostic and investigative tool to correlate the tion to start walking but a miscommunication persons FoG in relation to their medication causes difficulty in the lower limbs initiating cycle. It involves a comprehensive motor as- forward progression, such that the lean and sessment of the patient’s FoG in at least the limb movements are not well coordinated. three medication states: the “OFF” state, “ON” state and the “Supra-ON” state where 4. A Perceptual Malfunction the person is given a double dose of their Perceptual malfunctions causing difficulty in regular PD medication to distinguish which interpreting length, width and distance of the state/s FoG occurs (Espay et al, 2012). The environment may lead to FoG. This is seen in PDNS also has an important role in educating worsening of FoG through doorways, other the PWP and their carers on the nature of narrow constraints and by environmental ob- FoG, strategies to manage it and equipment stacles. available to combat this troublesome symptom. 5. Frontal Executive Dysfunction There is increasing evidence that patients With the advent of cheaper and more ad- with FoG show greater dysfunction in execu- vanced electronic technology, there have tive control compared with patients without been multiple products recently developed to FoG. The severity of the patient’s executive assist in people with FoG. The U-Step Walk- dysfunction correlates with freezing severity. er® is a four-wheeled walking frame which Demographic factors such as family history, has both a device that makes rhythmical mu- 53 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014 sic for auditory cueing as well as a laser line that 2nd July 2013, http://www.parkinsonsnsw.org.au/about shines on the ground and serves as a visual cue -parkinsons-disease/parkinsons-disease/ Vandenbossche, J, Deroost, N, Soetens, E, Coomans, D, for the person to walk over. Other laser beam de- Spildooren, J, Vercruysse, S, Nieuwboer, A and vices include the Agilitas® - a circular electronic Kerckhofs, E (2013) ‘Impaired Implicit Sequencing device with a motion sensor that is worn on the Learning in Parkinson’s Disease Patients with Freez person’s belt and automatically activates a laser ing of Gait’, Neuropsychology, Vol. 27, No 1: pp.28- beam on the floor when the person is in a freezing 36. episode. In the future, mobile phone applications might be used to trace a PWP’s freezing episodes during the day and provide on demand visual cues, for example through Google Glass® eyewear. The voice activated pair of glasses is linked to the In- ternet and can remind the patient when to take their PD medications. However, most PWP tend to be relatively elderly and may not have the fi- nancial ability or the technological wherewithal to afford or function complex electronic devices.

Conclusion FoG remains a mysterious phenomenon that may ultimately be one of the most debilitating motor symptoms of PD. When PD medications do not alleviate FoG, it is important to identify and implement other therapeutic interventions such as education in using auditory/visual cues, implementation of walking devices and/or physiotherapy to ensure that PWP suffering from FoG can sustain a good quality of life and reduce the risk of unnecessary FoG-associated falls and hospital admissions. The PDNS plays an important role in identifying the nature of a PWP’s FoG through dose cycle assessment and can provide important education to both the PWP and their carers. Further research is required into the pathophysiology of FoG, however, in the interim, such interventions can assist PWP in providing a better quality of life.

References Contreras, A and Grandas, F (2012) ‘Risk factors for freezing of gait in Parkinson Disease’, Journal of the Neurolog ical Science, Vol. 320:pp. 66-71. Delval, A, Moreau, C, Bleuse, S, Tard, C, Ryckewaert, G, Devos, D and Defebvre, L (2013) ‘Auditory cueing if gait initiation in Parkinson’s disease patients with freezing of gait’, Clinical Neurophysiology, Vol. 125, No. 8:pp1675-1681. Espay, A J, Fasano, A, van Nuenen B F L, Payne, M, Snijders, A H and Bloem, B R (2012) ‘”On” Freezing of Gait in Parkinson Disease: a paradoxical levodopa- induced complication’, Neurology, Vol. 78: pp. 454- 457. Heremans, E, Nieuwboer, A, and Vercruysse, S (2013) ‘Freezing of gait in Parkinson’s Disease: Where are we now?’, Current Neurology Neuroscience Reports, Vol. 13:pp. 350-358. Nutt, J, Bloem, B, Giladi, N, Hallett, M and Horak, F (2011) ‘Freezing of Gait: moving forward on a mysterious clinical phenomenon’, The Lancet Neurology, Vol. 10, No. 8: pp.734-744. Panisset, M (2004) ‘Freezing of Gait in Parkinson’s Disease’, Neurologic Clinics, Vol. 22: pp.53-62. Parkinson’s Australia (2011) Living with Parkinson Disease-update, Deloitte Access Economics, viewed

54 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

Northern Californian Meeting: 26-27 September, 2015. University of California, Davis. Sacramento, USA. Join us for a two day meeting covering topics such as Correlative Neuro Anatomy & Physiology, Neuro Assessment and Traumatic Brain Injury. Details, including registration, will be available soon on the WFNN website www.wfnn.org

The 2017 WFNN Congress— Opatija, Croatia. September 17-21. This event planning is well underway. Please begin to think about submitting an abstract. Watch the WFNN website www.wfnn.org for further information.

2015:

x Macquarie Neurosurgery 2015 Cerebrovascular and Skull Base Tumours with Dr. Robert Spetzler, from the Barrow Neurological Institute, Phoenix, Arizona. USA. Dates: 20-12 February at The Lang- 2014: ham, Kent Street, Sydney. Information from — x BANN Conference [email protected] Palace Hotel. Manchester, England Date: 17-18 October x American Association of Neuroscience Nurses Conference 21-24 March Nashville Convention Centre, Nashville, Tennessee. USA . www.aann.org

x European Association of Neuroscience Nurses Congress Post Scholarship Requirements Belgrade, Serbia. Successful applicants presenting an oral Dates: 13-16 May paper must submit their written paper to www.eann2015.rs be published in the Australasian Journal of Neuroscience as part of their award x WFNN Northern California Meeting requirements. Speaker names will be for- UC Davis, Sacramento warded to the Journal Editor for follow-up. Dates: 26-27 September www.wfnn.org

55 Australasian Journal of Neuroscience Volume 24 ● Number 2 ● October 2014

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