What is the initial management for patients with multiple system atrophy (MSA)?

Initial Management of Multiple System Atrophy

The initial management of multiple system atrophy (MSA) should focus on symptomatic treatment of autonomic dysfunction, particularly orthostatic hypotension, as this is the most disabling and life-threatening manifestation of the disease. 1, 2

Autonomic Dysfunction Management

Orthostatic Hypotension (OH)

1. Non-pharmacological interventions (first-line approach):

   • Acute water ingestion (240-480 mL) for temporary relief, with peak effect occurring 30 minutes after ingestion 1
   • Physical counter-pressure maneuvers such as leg crossing, lower body muscle tensing, and maximal force handgrip 1
   • Compression garments (at least thigh-high, preferably including abdomen) 1
   • Increased salt and fluid intake (6-9g salt per day) unless contraindicated by hypertension, renal disease, or heart failure 1
   • Avoid aggravating factors: large meals, alcohol, warm environments, and prolonged standing 3, 4

2. Pharmacological interventions (when non-pharmacological measures are insufficient):

   • Midodrine (first-line pharmacological therapy) - dose-dependent effect that increases standing blood pressure 1
   • Droxidopa - particularly effective for neurogenic OH in MSA 1
   • Fludrocortisone - increases plasma volume (use with caution if supine hypertension is present) 1
   • Pyridostigmine - for patients refractory to other treatments 1
   • Octreotide - may benefit patients with refractory recurrent postprandial or neurogenic OH 1

Urinary Symptoms

• Evaluate for urinary retention, incontinence, or both 3, 4
• Consider urodynamic studies to characterize bladder dysfunction 2
• Management should address specific urinary symptoms identified during evaluation 5

Motor Symptom Management

Parkinsonian Features (MSA-P)

• Trial of levodopa therapy - approximately 40-60% of MSA patients with predominant parkinsonian features show some response 3, 4
• Start with low doses and gradually increase while monitoring for orthostatic hypotension 5
• Be aware that response is often temporary, with only about 13% maintaining good response long-term 6
• Consider dopamine agonists or amantadine as alternatives, though they are generally not more effective than levodopa 3, 4

Cerebellar Features (MSA-C)

• Limited effective pharmacological options for cerebellar ataxia 5
• Physical therapy and assistive devices for gait and balance issues 5

Diagnostic Confirmation

• MRI Brain - preferably 3T for better resolution 2
  • Look for specific findings:
    • MSA-P: putaminal atrophy, hypointensity on T2-weighted images
    • MSA-C: cerebellar and pontine atrophy, "hot cross bun" sign, middle cerebellar peduncle hyperintensity 2
• Cardiovascular autonomic testing - to document orthostatic hypotension 2
• Ioflupane SPECT/CT (DaTscan) - demonstrates decreased radiotracer uptake in the striatum (cannot differentiate MSA from other parkinsonian syndromes) 2

Monitoring and Follow-up

• Regular monitoring of blood pressure (lying and standing) 1
• Watch for disease progression - >40% of patients become markedly disabled or wheelchair-bound within 5 years of motor symptom onset 6
• Adjust treatment as symptoms evolve, particularly as autonomic dysfunction often worsens over time 5

Important Considerations

• Supine hypertension is a common side effect of medications used to treat orthostatic hypotension and requires careful monitoring 1
• Treatment of one symptom may exacerbate another (e.g., medications for parkinsonism may worsen orthostatic hypotension) 5
• The disease typically progresses faster than Parkinson's disease, with median survival of approximately 9.5 years from symptom onset 6

Remember that while there is currently no disease-modifying treatment for MSA, appropriate symptomatic management can significantly improve quality of life and reduce morbidity 5, 7.