Case Study

A Multi-Modal Treatment Approach for a Young Adult with Friedreich’s : A Case Report

Megan Siler, PT, DPT; Bill Andrews, PT, MS, EdD, NCS Elon University – Elon, NC

ABSTRACT

Background: FRDA is a progressive and degenerative autosomal recessive disorder affecting the neuromuscular, endocrine, cardiovascular, and musculoskeletal systems. The literature is limited regarding physical therapy intervention strategies specifically for patients with FRDA. Case Description: The participant in this case report was a nineteen-year-old female who had been diagnosed with FRDA two and a half years earlier. At the initial examination, the participant presented with muscle weakness, difficulty with walking, decreased balance, and increased ataxia. Interventions focused on coordinative, balance, and strength training activities as well as aquatic therapy. Outcomes: The patient demonstrated a six-point increase on the Berg Balance Scale, a 0.14m/s increase in gait speed with a rollator, and a fifteen-second decrease in time for the five times sit-to-stand test upon completion of the eight-week period. The participant reported that she felt stronger and steadier with activities of daily living performance and had decreased the use of her rollator inside her apartment. Conclusion: A multi-modal treatment approach comprised of coordinative, balance, and strength training interventions along with aquatic therapy demonstrated positive gains for this patient with FRDA. Further research involving larger sample sizes is needed in order to verify the effectiveness of these interventions for other patients with FRDA.

Background increases the amount of cell death. The cardiac, pancreatic, and nervous systems Friedreich’s Ataxia (FRDA) is a progressive have cells that require higher energy and degenerative autosomal recessive demands and consequently are susceptible to disorder affecting multiple systems of the cellular damage from free radicals, thus body, including the cardiovascular, explaining the more affected body systems 1 musculoskeletal, endocrine, and in FRDA. The typical age of onset is 3 neuromuscular systems.1 The estimated between five and twenty-five years , with prevalence of FRDA is 1:50,000 in Europe symptoms usually appearing between the and the United States, affecting males and ages of five to fifteen years. Disease females equally, and is the most common manifestations earlier than five years of age autosomal recessive ataxia in the Caucasian and after the third to fourth decades of life population.1,2 FRDA is caused by a mutation are less frequent. Diagnosis of FRDA is in the FXN gene, particularly an increase in currently confirmed by genetic testing in the number of GAA trinucleotide repeats conjunction with the presence of clinical 1,4 which leads to a partial loss of frataxin. symptoms and family history. Frataxin is a protein that is linked to mitochondrial iron homeostasis.1,3 The loss The most common clinical features of of frataxin leads to a buildup of free radicals FRDA and the most prominent neurological which damages cells and subsequently symptoms are gait and limb ataxia, which

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Friedreich’s Ataxia 19 are typically first to appear. Ataxia is progression to surgical correction. An broadly defined as “motor disturbances additional musculoskeletal impairment is exhibited by people with cerebellar malalignment of the feet, usually pes cavus, dysfunction.” 5 However, “in-coordination which can lead to decreased mobility and balance dysfunction in movements secondary to having an altered weight- without muscle weakness” is a more precise bearing surface. Another system affected is and descriptive definition of ataxia.6 A the endocrine system, in which 10-20% of cerebellar ataxic gait is described as a gait persons with FRDA have clinically apparent with “increased step width, variable foot diabetes and another 30% have impaired placement, irregular foot trajectories, and a glucose tolerance.1 resulting unstable stumbling walking path with a high risk of falling.”7 The type of FRDA is a progressive disease though ataxia is based on the primary regions disease progressions are individualized. An affected. In FRDA, there is a loss of cells in average of 15.5 (±7.4) years after onset, a the dorsal root ganglia leading to patient’s primary means of mobility is degeneration in the posterior columns and typically a wheelchair, while also needing spinocerebellar tracts from the spinal cord to assistance for walking, standing, and even the cerebellum.1 Therefore, FRDA is most sitting.1,3 Such mobility impairments are similar to other spinocerebellar as a caused by the ataxia and balance deficits degenerative cerebellar disease. The associated with the amount of degeneration cerebellum is responsible for motor and at the cerebellar and corticospinal levels. postural control, as well as motor learning.5 Muscle strength is comparatively preserved, Other neurological symptoms that are but unable to be utilized secondary to the common, but not present in every patient aforementioned deficits. Females with with FRDA, are in all extremities, FRDA progress to using an assistive device diminished tendon reflexes, spasticity, or wheelchair for mobility more quickly , increased postural sway, than males when compared across age of dysarthria, , sensory onset and disease severity.1 neuropathy, and weakness. The weakness begins in the pelvic girdle and progresses to There currently is no cure for FRDA. the lower extremity (LE), then the upper Studies are focused on pharmacological and extremity (UE), and then trunk weakness antioxidant agents and research gains are appears later in the disease process.1,2 being made.3 The rehabilitation management of FRDA is similar to other degenerative Beginning with the cardiovascular system, neurological conditions including different persons diagnosed with FRDA typically types of ataxias, Parkinson’s disease, and have hypertrophic cardiomyopathy with or . Since ataxia is defined as without cardiac symptoms of heart disease an “incoordination and balance dysfunction such as dyspnea upon exertion. Having in movements without muscle weakness,” progressive cardiomyopathic symptoms can there is a multi-modal treatment approach lead to decreased cardiac function and thus that needs to be considered for each patient.6 impaired aerobic capacity and endurance.1,2 The primary focus of rehabilitation is The most common musculoskeletal teaching strategies and compensatory impairment in those with FRDA is scoliosis techniques for maintaining or improving a and for some the onset of scoliosis can person’s ability to participate at the level appear before ataxia or other neurological one wishes in one’s environment and role in symptoms.1,3 Depending upon the severity the community, as well as focusing on of the scoliotic curve, treatment varies coordination and balance training, sensation, between bracing and monitoring the curve’s and aerobic endurance. Despite the JOURNAL OF STUDENT PHYSICAL THERAPY RESEARCH | 2014 ⏐VOLUME 7, NUMBER 1, ARTICLE 3

Friedreich’s Ataxia 20 definition of ataxia including the phrase Since minimal studies have been performed “without muscle weakness,” patients can specifically regarding treatments for patients develop weakness due to a general with FRDA, this case report is important to deconditioning secondary to lack of activity, delineate the effectiveness of specific decrease in adherence to a home exercise treatment strategies. The multi-modal program (HEP), or as a result of a medical treatment approach for this nineteen-year- co-morbidity causing a change in medical old female included three 45-minute status. outpatient physical therapy sessions per week, comprising of balance and Also, as the disease progresses, slight coordinative training, general core and decreases in lower limb strength do occur. extremity strength training, aquatic therapy, However, weakness is not the cause for and the development of a HEP to be transitioning to using an assistive device for performed daily. This case report was ambulation; the progressive ataxia is the approved by the Elon University underlying cause. Patients with FRDA Institutional Review Board. demonstrate a typical progression of weakness regarding which lower limb Case Description muscles lose strength first. The first significant weakness is seen in hip extensor Patient Description muscles, and sometimes the combination of The patient is a nineteen-year-old female hip extensor and hip abductor weakness is 8 who was diagnosed with Friedreich’s Ataxia found. An overall treatment focus to two and a half years prior to participation in maintain joint and muscle integrity and this case report. The patient indicated general conditioning of muscles is important 1,3 symptoms of FRDA were present four years for the rehabilitation process. prior to her diagnosis. Pertinent past medical history includes a left ankle sprain about six Minimal research for rehabilitation in months ago and a left meniscus knee patients with FRDA is known. Treatments surgery. The patient was admitted to the focusing on coordinative training to improve hospital three months prior to beginning this motor performance have been shown to be bout of physical therapy for a effective in reducing ataxic symptoms up to gastrointestinal condition and reported eight weeks after intervention in patients increased weakness after discharge requiring with ; however, more the use of a rollator to ambulate. The distinct training effects were seen in patients patient’s most recent bout of outpatient who have primary cerebellar ataxia with no 7 physical therapy was about seven months afferent pathways affected. FRDA is a ago. primarily afferent ataxia and treatment effects were found, but the effects did not Referral for physical therapy was from her persist at long-term assessment of one year. physician for ataxia, specifically to address In patients with afferent ataxias, there is gait training and balance. Her chief greater loss of afferent information coming complaint upon evaluation was increased into the cerebellum, thus preventing some of muscle weakness and decreased balance the necessary inputs for adequate cerebellar since her recent hospitalization, and right processing. This study, through short- and knee pain secondary to increased weight long-term follow up, does show the benefits bearing on her right side. The subject is a of continuous coordinative training as part student at a four-year university, living in a of an intense home program for patients 9 dorm with elevator access and with with ataxia. roommates. She had a fear of falling and

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Friedreich’s Ataxia 21 injuring herself as a result of her decreased community dwellers with a balance disorder confidence in her walking abilities. The with a mean age of 48 years is 15.3 seconds patient’s primary goal of physical therapy (± 7.6 seconds).14 was to regain strength and not to use an assistive device for ambulation. Gait and gait speed were assessed through observation and by the ten-meter walk Examination Procedures (10mW) respectively. Normative data for On the initial examination, a brief history gait speed for a female in her twenties was taken and strength, range of motion walking at a comfortable pace is 1.41 m/s. 15 (ROM), balance, gait, and functional mobility were assessed. Range of motion Initial Findings was grossly assessed through observation in The patient demonstrated ROM within the seated and standing positions and functional limits during the initial strength was tested using Manual Muscle examination. MMT scores were as follows: Testing (MMT) in a seated position on the shoulder flexion, shoulder abduction and plinth. elbow flexion: 4/5 bilaterally, elbow extension: 4-/5 bilaterally, hip flexion 4-/5 Balance was assessed using a functional bilaterally, knee flexion and extension: 4/5 assessment tool, the Berg Balance Scale bilaterally, and ankle dorsiflexion: 4+/5 (BBS). The BBS was chosen secondary to bilaterally. A BBS score of 32/56 indicated having high inter-rater reliability and good that the patient was at high risk for falls. The specificity for identifying non-fallers. A patient’s 10mW gait speed was 1.0 m/s, systems assessment for balance was not indicating that the patient was likely a performed secondary to the nature of the community ambulator with an assistive patient’s diagnosis. For example, the device. The 5STS-test was completed in 36 Balance Evaluation Systems Test’s seconds, indicating that the patient was more (BESTest) goal as a systems assessment tool likely to have a balance dysfunction. The is to determine the underlying cause of the patient’s gait was observed during balance deficits.11 In this case, the patient’s ambulation with a rollator walker, principal cause of balance impairment is the demonstrating an ataxic and steppage gait progressive nature of FRDA and the primary pattern with increased hip and knee flexion symptom of ataxia. Also, the patient, at the for foot clearance, and decreased time of the evaluation was using an assistive dorsiflexion. The patient demonstrated device for ambulation, and the BESTest moderate postural sway during static contains ambulation components; therefore, standing with her UEs at high guard at the BBS was used. In patients with times. UE support was needed to take steps Parkinson’s disease, another progressive without the rollator throughout the neurological disorder, the mean BBS was examination. The patient was able to stand 40.22 ± 8.48 out of a total of 56, with a with a narrow base of support; however, she range of scores from 21-53.12 required UE support to attain the position.

Balance and lower extremity strength were Diagnosis and Prognosis assessed using a functional assessment tool, Functional limitations found from the initial the five times sit-to-stand test (5STS-test). evaluation included decreased safety during The 5STS-test has been validated in persons functional activities, impaired gait and with Multiple Sclerosis to be related to knee locomotion, and limitations in community flexor and knee extensor muscle strength in activities and performance in leisure the most affected lower extremity as well as activities, as determined through interview a balance performance tool.13 Data found for with the patient and observation. The JOURNAL OF STUDENT PHYSICAL THERAPY RESEARCH | 2014 ⏐VOLUME 7, NUMBER 1, ARTICLE 3

Friedreich’s Ataxia 22 American Physical Therapy Association opened and eyes closed with no UE support, (APTA) Guide to Physical Therapy practice standing in tandem stance with no UE pattern is 5E: Impaired Motor Function and support, and standing on small and large tilt Sensory Integrity Associated with boards. The patient was standing in front of Progressive Disorders of the Central a mirror for visual cues when her eyes were Nervous System.16 The patient’s prognosis open for balance activities. Coordinative was good based on the patient’s young age, exercises typically used in patients with motivation, and the support of her friends ataxia were introduced during sessions six and family. and seven after the development of LE strengthening and core stability/balance Procedures programs (see Appendix B Figures 6-7). The patient was scheduled to receive a total Coordinative training in quadruped of 24 physical therapy sessions over eight consisted of UE alternating flexion and LE weeks. Interventions to be included in alternating extension, and coordinative treatment were balance and coordinative training in sitting included sitting on an air- exercises, gait training, strengthening, and filled disc with alternating UE/LE flexion aquatic therapy, along with a HEP. (see Appendix B Figures 4-5).7,9 Aquatic therapy interventions included ambulating Intervention forwards and backwards, side stepping, Two sessions weekly of 40-45 minutes were standing hip strengthening exercises, and devoted primarily to strengthening and one core stability training in 4’6” water. session was devoted to balance and coordinative training, with aquatic therapy Outcomes added at session eight in exchange for one strengthening session. See Appendix A for a The patient participated in a total of 22 detailed summary of interventions for weeks sessions at the completion of her eight-week one through four. certification period, with the last session being a re-assessment of functional Each session began with performance on a outcomes. Two sessions were missed as a recumbent ergometer for ten minutes, result of the participant’s break from school. increasing the intensity from level 1 to level The participant scored 32/56 on the BBS on 4 over the course of the eight-week initial examination and at eight weeks she intervention period, with bilateral LE only scored 38/56 (Figure 1). for LE strengthening and aerobic 10 endurance. Strength exercises were The patient demonstrated difficulties with performed twice each week during weeks attaining feet together, tandem, and single one through three and once each week leg stance, alternating toe taps on four-inch during weeks three through seven. Strength step and turning in a 360° circle in each exercises utilized a leg press machine with direction. The patient did demonstrate single-leg leg press (working up to 35 improvements with the above-mentioned pounds) and seated LE strength exercises tasks upon re-assessment. The subject had which progressed to ankle weights and minimized postural sway in standing and resistance while sitting on an air-filled disc required minimal use of both UE for sitting to challenge core stability (see Appendix B and for maintaining static standing. Gait Figures 7-12). Balance training was speed during the 10mW with a rollator performed during sessions one and four, increased to 1.14 m/s upon eight-week before coordinative training began, and reassessment, from an initial speed of 1.0 again during week eight. Balance activities m/s with a rollator (Figure 2). The included standing on foam pad with eyes JOURNAL OF STUDENT PHYSICAL THERAPY RESEARCH | 2014 ⏐VOLUME 7, NUMBER 1, ARTICLE 3

Friedreich’s Ataxia 23 Figure 1. Berg balance scale scores

Figure 2. Ten-meter walk gait speed calculations

Figure 3. Five times sit to stand test

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Friedreich’s Ataxia 24 participant’s 5STS-test time decreased from gains during ADL. No minimal detectable 36 seconds at initial examination to 21.95 change (MDC) or minimal clinical seconds (Figure 3). At the initial evaluation, importance difference (MCID) has been the patient stated she had a fear of falling, established for younger populations, as the which was increased secondary to her recent BBS is primarily used in the elderly hospitalization. The patient reported one fall population. However, considering the during the eight-week period of this case similarity FRDA with Parkinsonism traits, study, during which she was standing with as part of another progressive neurological her backpack on using the rollator for disorder, the MDC on the BBS in patients support and had a loss of balance backwards with Parkinsonism is 5/56.17 and caught herself with her hands. She reported minimal injuries to her thumbs. At The BBS was used to assess balance the eight-week re-assessment, the patient capacities before and after coordinative stated that she felt stronger, had decreased training in patients with degenerative use of the rollator inside her apartment, and cerebellar diseases. At post-intervention she was able to perform the majority of her follow-up, the two groups, subjects with activities of daily living (ADL) with cerebellar ataxia and subjects with primary decreased unsteadiness. The patient afferent ataxias (including FRDA), showed continued with outpatient physical therapy significant improvement in BBS scores and at the completion of the case report on a upon one-year follow-up both groups’ twice-a-week basis, as a result of the gains scores were slightly higher than baseline, made at the three times per week frequency. but no longer significant.9 The decrease in both groups’ scores at one-year follow-up Discussion can be attributed to the specificity of coordinative training received through physical therapy while participating in the The purpose of this case report was to research. The quality and intensity of home determine the benefit of a multi-modal programs specifically for coordinative physical therapy treatment approach for a training is not reproducible secondary to patient with FRDA. One of the participant’s safety concerns and the supervision needed goals at the initial examination was to for techniques and the development of more ambulate safely without using the rollator. intense exercises, which can explain the The goal was considered ongoing secondary regression of scores.7 to the patient’s increased ability to perform activities around her apartment without the The subject’s 5STS-test scores decreased rollator; however, the patient reported from 36 seconds to 21.95 seconds upon re- holding onto walls and furniture to help assessment. Mean 5STS-test scores in maintain balance. Strength, balance, and younger subjects with a balance dysfunction coordination gains were made through the were noted at 15.3 seconds (±7.6 seconds), eight-week certification period. However, with a range of 6.4-56.6 seconds.14 The with the gains made the patient was more subject’s score was within the range noted. likely to be closer to her baseline before the In patients with Parkinson’s disease, the cut- hospitalization as her BBS score upon off score differentiating fallers from non- beginning her last bout of physical therapy fallers is 16.0 seconds; therefore, scoring about seven months ago was 38/56. >16.0 seconds indicates a risk of falls.18 No

MDC or MCID has been established for The patient’s six-point gain in the BBS was younger patients with or without balance also revealed by her subjective reports of disorders or for any progressive neurological increased functional mobility and balance diseases such as Parkinson’s. JOURNAL OF STUDENT PHYSICAL THERAPY RESEARCH | 2014 ⏐VOLUME 7, NUMBER 1, ARTICLE 3

Friedreich’s Ataxia 25 Ambulation was not specifically practiced along with general strength training, is vital during treatment sessions, but the focus was to patients with FRDA as the LE typically on requirements of ambulation: balance, lose strength before the core and UE.8 coordination, and strength. The patient did Lower extremity and core strength gains demonstrate improvements in gait speed were demonstrated through a decrease in with no marked observational changes. The time to complete the 5STS-test and a patient’s 10mW gait speed with a rollator decrease in postural sway with standing. In progressed from 1.0m/s to 1.14m/s. this case report, the patient’s follow-through Normative data relating to a comfortable with the HEP was poor secondary to the gait speed of females in their twenties with patient’s busy lifestyle as a college student. no balance disorder is 1.41 m/s.15 In patients A limitation of this case report was the with Parkinson’s disease, the MDC for patient’s decreased adherence to the HEP. comfortable gait speed is 0.18m/s.17 This Therefore, one can assume more gains could patient’s comfortable gait speed increased have been made with a more progressive by 0.14m/s during the eight-week strength and coordinative training program intervention period. that would have been performed as part of a daily HEP. Though this patient’s plan of care did not focus on improving aerobic capacity, it is Conclusion important for the overall maintenance of functional mobility for patients with FRDA. The outcomes from this case report are One study showed that improvements in optimistic. The participant who presented aerobic fitness can be made from initially with decreased coordination, participating in a stationary cycling program decreased balance, muscle weakness, and for 27 monitored visits of 20-25 minutes at difficulty with walking perceived 70-80% of maximum heart rate, preceded improvements in balance and strength. and followed with a stretching program in Research indicates that patients with FRDA patients with FRDA.10 Patients with FRDA will use an assistive device secondary to need to maintain a daily plan of care ataxia and not from muscle weakness including range of motion (muscle length resulting in difficulties with walking.8 This and soft tissue extensibility), a HEP for program focused on balance and strengthening and maintaining functional coordinative training as well as core and LE mobility, along with maintaining proper strengthening through land and aquatic biomechanical alignment.1,3 Adaptive interventions in hopes to return the patient to equipment can be utilized to address balance her prior level of functioning without the impairments that become a functional rollator. Integration with the available limitation for the patient.2 recreational facilities at her university was

implemented to continue with the patient’s Despite findings that ataxia causes greater progress and facilitate more compliance. mobility deficits than muscle weakness in Further research comprising of larger patients with FRDA, the patient’s plan of sample sizes will be needed for verification care was focused on muscle strengthening of the effectiveness of the interventions secondary to her hospitalization over the utilized in this case report for other patients summer that resulted in muscle weakness with FRDA. and not a sudden progression of ataxia symptoms.1,3 Focusing on LE strengthening

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Friedreich’s Ataxia 26 References 10. Fillyaw MJ, Ades PA. Edurance exercise training in friedreich ataxia. Arch Phys 1. Maring JR, Croarkin E. Presentation and Med Rehabil. 1989;70:786-788. progression of friedreich ataxia and 11. Mancini M, Horak FB. The relevance of implications for physical therapist clinical balance assessment tools to examination. Phys Ther. 2007;87:1687- differentiate balance deficits. Eur J 1696. Phys Rehabil Med. 2010;46:239-248. 2. Harris-Love MO, Siegel KL, Paul SM, 12. Qutubuddin AA, Pegg PO, Cifu DX, Benson K. Rehabilitation management of Brown R, McNamee S, Carne W. friedreich ataxia: Lower extremity force- Validating the berg balance scale for control variability and gait performance. patients with parkinson's disease: A key Neurorehabil Neural Repair. to rehabilitation evaluation. Arch Phys 2004;18:117-124. Med Rehabil. 2005;86:789-792. 3. Mancuso M, Orsucci D, Choub A, 13. Moller AB, Bibby BM, Skjerbaek AG, Siciliano G. Current and emerging et al. Validity and variability of the 5- treatment options in the management of repetition sit-to-stand test in patients friedreich ataxia. Neuropsychiatr Dis with multiple sclerosis. Disabil Rehabil. Treat. 2010;6:491-499. 2012;34:2251-2258. 4. Maring J, Croarkin E, Morgan S, Plack 14. Whitney SL, Wrisley DM, Marchetti M. Perceived effectiveness and barriers GF, Gee MA, Redfern MS, Furman JM. to physical therapy services for families Clinical measurement of sit-to-stand and children with friedreich ataxia. performance in people with balance Pediatr Phys Ther. 2013;25:305-313. disorders: Validity of data for the five- 5. Martin CL, Tan D, Bragge P, times-sit-to-stand test. Phys Ther. Bialocerkowski A. Effectiveness of 2005;85:1034-1045. physiotherapy for adults with cerebellar 15. Bohannon RW. Comfortable and dysfunction: A systematic review. Clin maximum walking speed of adults aged Rehabil. 2009;23:15-26. 20-79 years: Reference values and 6. Armutla K. Ataxia: Physical therapy and determinants. Age Ageing. 1997;26:15- rehabilitation applications for ataxic 19. patients. International Encyclopedia of 16. American Physical Therapy Association. Rehabilitation. 2013:September 22, 2013. Interactive guide to physical therapist http://cirrie.buffalo.edu/encyclopedia/en/ practice. . 2003. article/112. 17. Steffen T, Seney M. Test-retest 7. Ilg W, Synofzik M, Brotz D, Burkard S, reliability and minimal detectable Giese MA, Schols L. Intensive change on balance and ambulation tests, coordinative training improves motor the 36-item short-form health survey, performance in degenerative cerebellar and the unified parkinson disease rating disease. Neurology. 2009;73:1823-1830. scale in people with parkinsonism. Phys 8. Beauchamp M, Labelle H, Duhaime M, Ther. 2008;88:733-746. Joncas J. Natural history of muscle 18. Duncan RP, Leddy AL, Earhart GM. weakness in friedreich's ataxia and its Five times sit-to-stand test performance relation to loss of ambulation. Clin in parkinson's disease. Arch Phys Med Orthop Relat Res. 1995;311:270-275. Rehabil. 2011;92:1431-1436. 9. Ilg W, Brotz D, Burkard S, Giese MA, Schols L, Synofzik M. Long-term effects of coordinative training in degenerative cerebellar disease. Mov Disord. 2010;25:2239-2246. JOURNAL OF STUDENT PHYSICAL THERAPY RESEARCH | 2014 ⏐VOLUME 7, NUMBER 1, ARTICLE 3

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Appendix A. Summary of interventions for sessions 1-12

Session 1: Recumbent ergometer Level 1, step up/down to 4” step x 10 repetitions, neuromuscular re-education activities for balance training with no upper extremity (UE) support and eyes opened/ eyes closed on foam pad, on solid ground in tandem stance and on tilt board.

Session 2: Recumbent ergometer Level 1, LE strengthening exercises: bridges8 (3sets of 10), sit to stand (3 sets of 5), clam shells8 (3 sets of 10 each side), single-leg leg press 15 lbs. (3 x 10 each side).

Session 3: Recumbent ergometer Level 1, LE strengthening exercises: bridges (3sets of 10), sit to stand (3 sets of 5), clam shells (3 sets of 10 each side), and single-leg leg press 15 lbs. (3 sets of 10 each side). Clam shells and bridges for home exercise program to be performed daily8.

Session 4: Recumbent ergometer Level 1, on mat table: side bend onto wedge for oblique strengthening (2 sets of 10 each side), neuromuscular re-education activities for balance with no UE support with eyes open using a mirror for visual cues on foam pad and on solid ground in tandem stance.

Session 5: Recumbent ergometer Level 2, LE strengthening exercises: sitting on mat table with no UE support: alternating hip flexion with 2 lb. ankle weights, long arc quad (LAQ) with 2 lb. ankle weights, resisted knee flexion with yellow theraband (3 sets of 10 for all), single-leg leg press 20 lbs. (3 sets of 10 each side), lateral flexion on elbow to wedge (3 sets of 10 each side) and sit to stand (3 sets of 5).

Session 6: Recumbent ergometer Level 2, LE strengthening exercises: sitting on mat table with no UE support: alternating hip flexion sitting on air-filled disc, long arc quad (LAQ) with 2 lb. ankle weights, resisted knee flexion with yellow theraband (3 sets of 10 for all), single-leg leg press 20 lbs. (3 sets of 10 each side), lateral flexion on elbow to wedge (3 sets of 10 each side) and sit to stand (3 sets of 5).

Session 7: Recumbent ergometer Level 2, LE strengthening exercises: sitting on mat table on air-filled disc alternating hip flexion (3 sets of 10). Neuromuscular re-education: quadruped with alternating UE flexion (3 sets of 10), quadruped with alternating LE extension (2 sets of 10) with PT support for pelvis7,9, and balance activities on foam pad with no UE support with eyes open and eyes closed.

Session 8: First Aquatic Therapy session: Entered/exited pool through zero- entry ramp, in 4’6” water: walking forwards 4 lengths of pool, side stepping 4 lengths and backwards walking 2 lengths, standing hip strengthening: hip abduction, hip extension, hip flexion and standing plantarflexion 2 x 45 seconds for all exercises. Standing in water with bilateral UE in 90 degrees shoulder flexion moving arms into horizontal abduction/ adduction with hand cupped 2 bouts of 60-90 seconds, holding kickboard in front and resisting movements for 1 minute and sitting on 3 noodles under thighs while maintaining good posture for core stability training. Freestyle kicking on stomach with 3 noodles under stomach and kickboard in hands for 4 lengths of the pool.

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Session 9: Recumbent ergometer Level 2, LE strengthening exercises: sitting on air-filled disc on mat table with no UE support: alternating hip flexion sitting with 3 lb. ankle weights, long arc quad (LAQ) with 3 lb. ankle weights, resisted knee flexion with red theraband (3 sets of 10 for all), single-leg leg press 20 lbs. (3 sets of 10 each side), bridges with LE on air-filled (3 sets of 10).

Session 10: Recumbent ergometer Level 2, Neuromuscular re-education: quadruped with alternating UE flexion (3 sets of 10), quadruped with alternating LE extension (2 sets of 10) with PT support for pelvis.7,9 Seated on air-filled disc alternating UE flexion and hips flexion (2 sets of 20) and lateral flexion on elbow to mat table (10 repetitions).

Session 11: Entered/exited pool through zero- entry ramp, in 4’6” water: walking forwards 4 lengths of pool, side stepping 6 lengths and backwards walking 2 lengths, standing hip strengthening: hip abduction, hip extension, hip flexion and standing plantarflexion 2 x 45 seconds for all exercises. Standing in water with bilateral UE in 90 degrees shoulder flexion moving arms into horizontal abduction/ adduction with hand cupped 2 bouts of 60-90 seconds.

Session 12: Recumbent ergometer Level 2, LE strengthening exercises: sitting on air-filled disc on mat table with no UE support: alternating hip flexion sitting with 3 lb. ankle weights, long arc quad (LAQ) with 3 lb. ankle weights, resisted knee flexion with red theraband (3 sets of 10 for all), resisted plantarflexion with dorsiflexion stretch (2 sets of 10), single-leg leg press 20 lbs. (1 of 10 each side), single-leg leg press 30 lbs. (2 sets of 10 each side).

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Appendix B. Coordinative and strengthening exercises

Figure 5: Quadruped alternating UE flexion

Figure 4: Seated alternating hip and UE flexion on air-filled disc

Figure 6: Seated on air-filled disc side bend onto elbow

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Figure 7: Bridges with one –count hold at top

Figures 8-9: Single-leg leg press with preventative knee hyperextension assist

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Figure 11: Seated weighted alternating knee extension on air-filled disc Figure 10: Seated weighted alternating hip flexion on air-filled disc

Figure 12: Seated resisted ankle plantarflexion on air-filled disc

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