Multiple System Atrophy: A Case Study

Client Characteristics

Mr. L is a 62-year-old retired NASA astronaut. He is currently living in California with his husband of 20 years, who is also retired. Mr. L first presented to his family physician three years ago with issues of erectile dysfunction, in which his doctor believed to be result of aging. Over the next year, Mr. L began developing other symptoms seemingly unrelated to his previous health history. He presented with noted difficulty balancing, asymmetric postural tremor (right side), bradykinesia, rigidity, urinary incontinence, and minor slurring of speech – warranting a probable diagnosis of Parkinson’s disease. Mr. L was prescribed oral Levodopa (250 milligrams, four times a day). This medication eased his symptoms for four months, but gradually, he began noticing the return of his symptoms – warranting a reassessment. Despite increasing his medication dosage, Mr. L’s chief complaints were perpetuated. Additionally, he began experiencing gait ataxia with cerebellar dysarthria, action myoclonus, and cold hands and feet. After conducting a thorough physical assessment, it was noted that Mr. L presents with orthostatic hypertension (dropping from 120/94 mmHg in a recumbent position, to 90/68 mmHg, after prolonged standing). Based on the presenting factors, a diagnosis of Multiple System Atrophy-Parkinsonism (MSA-P) was established. The physician referred Mr. L to physiotherapy for gait and mobility training, pelvic floor training, and education on symptom management.

Examination Findings

Subjective

Patient Profile	62 y/o male
History of Present Illness	Diagnosis of probable MSA-P three weeks ago, right-sided postural tremor, difficulty balancing, bradykinesia, rigidity, urinary incontinence, slurred speech, cerebellar dysarthria, cold hands and feet, action myoclonus, and orthostatic hypotension.
Past Medical History	N/A
Medications	Levodopa (250 mg 4X/day, for 4 months) Levodopa (500 mg 4X/day, for 2 months) CBD for pain management (occasional)
Health Habits	Non-smoker, non-drinker, formerly very active (prior to symptom onset)
Family Health History	No family history of progressive neurological disorders
Psychosocial	Patient is experiencing marital issues due to the progressive nature of the disease. Patient also expresses concerns of loneliness, isolation, and depression due to fatality of the diagnosis. Mr. L also worries about burdening his partner and family as the disease progresses and he becomes less independent. Having been previously very active, Mr. L is having trouble coping with his new sedentary lifestyle. Previously, Mr. L volunteered weekly at his local YMCA, running a space exploration program for young children – bringing joy to his life. Due to cerebellar dysarthria, he is having trouble effectively communicating with others. Lastly, he used to really enjoy dancing with his husband but has not been able to do so recently because of his condition.
Living Environment	Two-story home with 14 stairs to the second floor. Bedroom located on second floor. Half bathroom on first floor, full bathroom on second floor. Second floor bathroom equipped with a spacious walk-in shower. Hardwood flooring throughout the home.
Functional History	Prior to probable MSA-P diagnosis: very active, running 15km a week and walking everywhere within the city. Drove when needed. Able to independently maintain his property and home chores. Able to complete all activities of daily living independently.
Current Functional Status	After probable MSA-P diagnosis: able to ambulate 200m independently, but very slowly and unsteadily. Beyond 200m, requires a rollator walker or assist X1. Requires assistance from his husband for ADLs (cooking, cleaning, bathing, shopping, etc.). Trouble controlling urination and bowel movements (requires protective underwear). Able to ascend and descend one flight of stairs with assist X1. Unable to drive. Fine motor activity is limited due to action myoclonus (unable to type). Trouble communicating due to dysarthria.
Imaging	MRI T2 weighted MRI revealed a hyperintense putaminal slit sign bilaterally
Precautions/Contraindications	Depression, lack of social interaction (decreased involvement with community), and trouble communicating due to dysarthria (no difficulties with comprehension). Otherwise, no contraindications to a physiotherapy program.

Objective

General	Minor hypomimia, postural AP tremor, action tremor
Posture	Standing Rounded shoulders, right shoulder depression, forward head posture, forward lean, wide base stance
	Seated Rounded shoulders, right shoulder depression, posterior pelvic tilt
Gait	Festination, shuffling (short stride) wide base gait, bradykinetic ambulation, reduced arm swing bilaterally, action myoclonus more prominent during ambulation
Tone	High tone on right side of body in comparison to the left
AROM	Upper Extremity Left Right Shoulder flexion 80° 65° Shoulder extension 40° 30° Shoulder abduction 150° 110° Shoulder adduction 75° 70° Elbow flexion 130° 120° Elbow extension 0° -5° Wrist flexion 90° 90° Wrist extension -10° -20°
	Trunk C-Spine T-Spine L-Spine Flexion 50° 30° 50° Extension 10° 10° 15° Side flexion L/R 30/25° 30/25° 25/15° Rotation L/R 70/65° 30/25° 0/0°
	Lower Extremity Left Right Hip flexion 115° 109° Hip extension -4° -4° Hip abduction 40° 38° Hip adduction 45° 42° Knee flexion 120° 115° Knee extension -5° -10° Ankle dorsiflexion 50° 50° Ankle plantarflexion 5° 0° Ankle inversion 34° 35° Ankle eversion 15° 5°
PROM	PROM limited by muscle rigidity and contracture
Strength	Respiratory MIP: 39.08% of normal values MEP: 49.59% of normal values
	Musculoskeletal Manual muscle testing revealed global muscle weakness (4/5)
Sensation	WNL
Neurological Testing	Upper Motor Neuron Testing Babinski sign with hyperreflexia Positive clonus test
	Lower Motor Neuron Testing Normal
	Myotomes and Dermatomes Myotomes: Abnormal Dermatomes: WNL
Self Reported Outcome Measures	Activities-Specific Balance Confidence Scale (ABC) – assesses balance (vestibular and non-vestibular, functional mobility) Score = 50% (between low and moderate level of physical functioning
	Short Form – 36 (SF36) - general assessment of overall health function and psychosocial quality of life Score = 12/36
Outcome Measures	Unified Multiple System Atrophy Rating Scale (UMSARS) Part 1 (historical review) Score = 26 Part 2 (motor examination scale) Score = 30 Part 3 (autonomic examination) 120/94 mmHg to 90/68 mmHg Heart rate 98 (supine), 104 (standing) Orthostatic symptoms = yes Part 4 (global disability) Score = 4 Overall = 60/104
	TUG Score = 19 Seconds
	Performance-Oriented Mobility Assessment (POMA) Score = 22/28
	Functional Reach Score = 6" bilaterally
	Single Limb Stance (SLS) Unable to stand on one limb