From the Guidelines
Management of Multisystem Atrophy (MSA) symptoms, including parkinsonian symptoms, autonomic dysfunction, and orthostatic hypotension, requires a comprehensive approach prioritizing morbidity, mortality, and quality of life.
Overview of Management Strategies
The management of MSA involves a multifaceted approach targeting its various symptoms. For parkinsonian symptoms, levodopa therapy should be tried, though response is often limited and diminishes over time [no direct evidence provided].
Autonomic Dysfunction Management
Autonomic dysfunction management includes treating urinary symptoms with anticholinergics like oxybutynin for urgency, intermittent catheterization for retention, and potentially desmopressin for nocturia [no direct evidence provided].
Orthostatic Hypotension Management
For orthostatic hypotension, non-pharmacological measures are first-line: gradual position changes, compression stockings, increased salt and fluid intake, and elevating the head of the bed. According to the 2017 ACC/AHA/HRS guideline 1, acute water ingestion can temporarily restore orthostatic tolerance, and physical counter-pressure maneuvers, such as leg crossing or squatting, can increase blood pressure. Compression garments, at least thigh high and preferably including the abdomen, can improve orthostatic symptoms and blunt associated decreases in blood pressure 1.
Pharmacological Options
Pharmacological options for orthostatic hypotension include:
- Midodrine, which improves symptoms of OH in patients with neurogenic OH, with a dose-dependent effect 1
- Droxidopa, which improves symptoms of neurogenic OH due to Parkinson disease, pure autonomic failure, and multiple system atrophy 1
- Fludrocortisone, which increases plasma volume, with a resultant improvement in symptoms of OH 1
- Pyridostigmine, which may be beneficial in patients with syncope due to neurogenic OH who are refractory to other treatments 1
- Octreotide, which may be beneficial in patients with syncope and refractory recurrent postprandial or neurogenic OH 1
Additional Considerations
Additional symptoms require specific management, including physical therapy for motor symptoms, speech therapy for dysarthria, and swallowing evaluations for dysphagia [no direct evidence provided]. Sleep disorders may require CPAP for sleep apnea or clonazepam for REM sleep behavior disorder [no direct evidence provided]. Since MSA is progressive with no disease-modifying treatments available, management focuses on symptom control and maintaining quality of life through a coordinated multidisciplinary approach involving neurologists, urologists, cardiologists, and rehabilitation specialists [no direct evidence provided].
From the Research
Management Strategies for Multisystem Atrophy (MSA) Symptoms
The management of MSA symptoms, including parkinsonian symptoms, autonomic dysfunction, and orthostatic hypotension, is primarily focused on alleviating these symptoms, as there is currently no cure for the disease 2, 3.
- Parkinsonian Symptoms: Some patients with MSA may respond to levodopa, although the response is often limited and transient 4.
- Autonomic Dysfunction: Autonomic and urogenital features of MSA can be treated effectively in many instances, and early identification of these symptoms is crucial 4.
- Orthostatic Hypotension: Management of orthostatic hypotension in MSA patients is important to prevent falls and other complications.
Pathogenesis and Diagnosis
The pathogenesis of MSA is still not well understood, but it is characterized by the accumulation of aggregated α-synuclein in oligodendrocytes, forming glial cytoplasmic inclusions 2, 5.
- Glial Cytoplasmic Inclusions: The formation of glial cytoplasmic inclusions is a key feature of MSA, and understanding the molecular pathogenesis of these inclusions is crucial for the development of disease-modifying therapies 5.
- Diagnostic Criteria: The new Movement Disorders Society (MDS) diagnostic criteria for MSA have improved accuracy in early disease stages, and α-synuclein-based biomarkers are emerging as valuable diagnostic tools 6.
Treatment and Future Directions
While there are some symptomatic treatments available for MSA, neuroprotection remains an urgent unmet treatment need 2.
- Clinical Trials: Clinical trials are ongoing to investigate drugs targeting α-synuclein aggregation or preventing α-synuclein expression, along with stem cell and gene therapies to halt disease progression 6.
- Future Perspectives: Further research into the etiology, pathogenesis, early diagnosis, and therapeutic management of MSA is necessary to improve the outcome of this devastating disorder 4.