What are the management strategies for a patient with Multiple System Atrophy (MSA)-cerebellar (MSA-C)?

Management of Multiple System Atrophy - Cerebellar Type (MSA-C)

For MSA-C, prioritize aggressive symptomatic management of autonomic dysfunction—particularly orthostatic hypotension—over parkinsonian features, as autonomic failure drives morbidity and mortality in this rapidly progressive disease with median survival of only 6 years. 1

Immediate Diagnostic Confirmation

• Obtain MRI brain without contrast to confirm diagnosis, looking specifically for cerebellar and pontine atrophy, the pathognomonic "hot cross bun" sign in the pons, and middle cerebellar peduncle hyperintensity on T2/FLAIR sequences 2, 1
• Document baseline autonomic dysfunction with lying and standing blood pressure measurements to quantify orthostatic hypotension 3, 4

Treatment Algorithm by Priority

1. Orthostatic Hypotension (First Priority - Prevents Falls and Syncope)

Non-pharmacological interventions (initiate immediately):

• Compression garments (abdominal binders and thigh-high stockings) 1
• Physical counter-pressure maneuvers (leg crossing, squatting) 1
• Acute water ingestion (500 mL rapidly) before standing 1
• Increased salt intake (6-10 grams daily) 1
• Elevate head of bed 30 degrees to reduce nocturnal hypertension 3, 4
• Avoid large meals, hot environments, and prolonged standing 3, 4

Pharmacological therapy (add when non-pharmacological measures insufficient):

• Midodrine (alpha-1 agonist): Start 2.5-5 mg three times daily, titrate to 10 mg three times daily 1
• Droxidopa (synthetic norepinephrine precursor): Alternative or adjunct to midodrine 1
• Fludrocortisone (mineralocorticoid): 0.1-0.2 mg daily, increases plasma volume 1

2. Genitourinary Dysfunction (Second Priority - Major Quality of Life Impact)

Urinary symptoms affect 83% of MSA patients and require aggressive management: 5

• For urinary retention: intermittent self-catheterization or indwelling catheter 3, 4
• For urinary incontinence: anticholinergics (oxybutynin, tolterodine), though monitor for worsening orthostatic hypotension 3, 4
• For erectile dysfunction: sildenafil or other PDE-5 inhibitors, but use cautiously as they may worsen orthostatic hypotension 3, 4

3. Parkinsonian Features (Third Priority - Limited Response Expected)

Trial levodopa/carbidopa despite limited efficacy:

• Start levodopa/carbidopa 25/100 mg three times daily, titrate to 200/2000 mg daily over 2-3 months 3, 4
• Only 40-60% of MSA-P patients show partial response; MSA-C patients respond even less frequently 3, 4, 6
• Dopamine agonists and amantadine are no more effective than levodopa and should not be first-line 3, 4
• Do NOT use deep brain stimulation—it is contraindicated in MSA 1

4. Cerebellar Ataxia (Fourth Priority - No Effective Treatment)

No pharmacological therapy improves ataxia in MSA-C: 6

• Physical therapy focused on gait training and fall prevention 6, 7
• Assistive devices (walker, wheelchair) as disease progresses 7
• Speech therapy for dysarthria and dysphagia 7

5. REM Sleep Behavior Disorder

• Clonazepam 0.5-2 mg at bedtime or melatonin 3-12 mg at bedtime 6, 7
• Ensure safe sleep environment (remove sharp objects, pad floor) 7

Multidisciplinary Care Coordination

Establish care team immediately upon diagnosis: 1

• Neurology (primary coordinator)
• Urology (for neurogenic bladder management)
• Physical/occupational therapy
• Speech therapy
• Palliative care (initiate at diagnosis, not end-stage)

Critical Palliative Care Discussion

Begin advance care planning at diagnosis, not when patient deteriorates: 1

• Median survival is only 6 years from diagnosis 1, 5
• Discuss goals of care, feeding tube decisions, and end-of-life preferences early while patient has decision-making capacity 1
• Palliative care involvement should occur at diagnosis for symptom management and prognostic discussions 1

Common Pitfalls to Avoid

• Do not delay treatment of orthostatic hypotension—it causes falls, syncope, and significantly impairs quality of life 5
• Do not aggressively pursue levodopa dose escalation beyond 200/2000 mg daily if no response after 2-3 months, as MSA-C patients rarely respond 3, 4, 6
• Do not use medications that worsen orthostatic hypotension (diuretics, alpha-blockers, nitrates) unless absolutely necessary 3, 4
• Do not wait until end-stage disease to involve palliative care—early involvement improves quality of life and allows for advance care planning 1