A CLINICAL DECISION-MAKING APPROACH TO ADDRESS FUNCTIONAL LIMITATIONS IN AN INDIVIDUAL WITH KENNEDY'S DISEASE, A RARE NEUROLOGICAL DISORDER

Vance N1,2, Ulanowski E1, Barrett E1, Ryan D1, Danzl M1
1Bellarmine University, Physical Therapy, Louisville, United States, 2Norton Healthcare, Cressman Neurological Rehabilitation Center, Louisville, United States

Background: Kennedy's disease (KD) is a rare, progressive X-linked genetic motor neuron disease. Although there is literature available describing the etiology of KD there is little to no evidence supporting physical therapy interventions. Muscular endurance, task specific training, strength training and energy conservation is effective for other progressive neurological diseases such as Myasthenia Gravis (MG), Lower Motor Neuron disorders, Parkinson disease (PD), Huntington's disease (HD), and Multiple Sclerosis (MS). The literature in these diseases could help inform a physical therapy plan of care development and intervention selections for patients with KD.

Purpose: The purpose of this case report is to describe a clinical decision-making approach and selection of therapeutic interventions to meet patient specific goals, improve function, and reduce general fatigue in a person with KD.

Methods: The patient in this case is a 51-year-old male diagnosed with KD who attended a pro-bono physical therapy clinic 2 times a week for 12 weeks. The patient presented with weakness (core, upper extremities, lower extremities), fatigue, and activity limitations such as ascending and descending stairs, sit to stands, and long-distance ambulation. The plan of care and physical therapy interventions were informed by a variety of literature in other progressive neurological diseases including task specific training in PD and HD literature, fatigue management in MS literature, and recommendations for strengthening from MG and lower motor neuron literature. A plan of care included task-specific training encompassing core strengthening exercises, postural awareness, functional training (sit to stands, floor transfers, turns, stairs, over ground and body weight support gait training), and balance exercises with progression of these over the intervention period. Outcome measures utilized pre and post intervention were the Functional Gait Assessment (FGA), 5 time Sit-to-Stand Test (FTSTS), Six Minute Walk Test (6MWT), Timed Up and Go (TUG), 10 Meter Walk Test (10MWT), and Single Leg Stance (SLS).

Results: The patient stated decreased fatigue with post-testing. The TUG improved beyond the falls risk cut-off score (pre 14.5 seconds, post 11.2 seconds). Results exceeding the minimally clinically important difference included the FGA (pre 14/30; post 20/30) and 6MWT (pre not tested; mid 987 feet; post 1160 feet). The 10MWT and FTSTS results improved but not significantly (10MWT self-selected improved by 0.02 m/s; FTSTS improved by 14.09 seconds; SLS improved from ‹3 seconds bilaterally to 5 seconds right lower extremity and 10.1 seconds left lower extremity).

Conclusion(s): Outcome measure findings indicate improvements across multiple fronts with utilization of task specific training during strengthening, functional training, and balance training. This clinical decision-making approach could be applied when developing treatment plans for other patients with KD as well as other rare neurological diagnoses.

Implications: This case indicates that physical therapy interventions with a focus on improving fatigue and task specific training including core strengthening, postural awareness, functional training, and balance exercises enhances the functional ability in a patient with KD. Clinicians can trial this clinical decision-making model of drawing upon the literature in other progressive yet more common neurological disorders and applying that evidence to patients with rare neurological disorders.

Keywords: Kennedy''s Disease, Physical Therapy, Clinical Decision-Making

Funding acknowledgements: None

Topic: Neurology; Service delivery/emerging roles; Professional practice: other

Ethics approval required: No
Institution: Bellarmine University
Ethics committee: Bellarmine University Institutional Review Board
Reason not required: This is existing data that is not part of a process of generating new, generalizable knowledge, but is rather presented as a single patient case report for discussion. Nothing in the data indicates that human subjects are identifiable.


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