What's multiple system atrophy?
Multiple system atrophy (MSA) is a rare, sporadic, adult-onset neurodegenerative disease in the synucleinopathy family. It combines failure of the autonomic nervous system (drops in blood pressure on standing, bladder and sexual dysfunction) with either parkinsonism that responds poorly to levodopa (MSA-P) or cerebellar ataxia (MSA-C), and often breathing problems such as stridor. Its pathological hallmark is the build-up of alpha-synuclein as glial cytoplasmic inclusions in oligodendrocytes. MSA is sporadic and not inherited; no causal gene is confirmed here (COQ2 is reported only as a susceptibility factor, not a cause). There is no disease-modifying treatment; care is symptomatic.
| Also indexed as | ORPHA:102, MONDO:0007803 |
|---|---|
| Features mapped | 15 |
| Treatments mapped | 2 |
| Published sources | 7 |
| Last reviewed | 2026-08-04 |
Signs and symptoms
Progressive cerebellar ataxia
In the cerebellar form, movements become uncoordinated (ataxia): unsteady walking, clumsy hands, and slurred speech.
Abnormal rapid eye movement sleep
REM sleep behaviour disorder, in which people physically act out dreams, often appears years before other symptoms.
Postural instability
by the time multiple system atrophy is usually diagnosed it is often already at a late stage, which contributes to balance problems and a shortened survival.
Gait ataxia
multiple system atrophy can cause cerebellar ataxia, which affects balance and coordination and can make walking unsteady.
Parkinsonism
Many people develop parkinsonism (slowness, stiffness, sometimes tremor). Unlike Parkinson disease, it responds poorly and only briefly to levodopa.
Abnormal autonomic nervous system physiology
multiple system atrophy involves autonomic failure, meaning the body systems that run automatically (such as blood pressure and bladder control) stop working normally, alongside parkinsonism, cerebellar ataxia, or both.
Autonomic bladder dysfunction
Bladder problems are among the earliest signs of multiple system atrophy and can appear years before the movement symptoms. People may have urgency, frequency, trouble emptying the bladder, or incontinence, because the disease damages the nerves that control the bladder.
Central sleep apnea
Pauses in breathing during sleep (sleep apnea) are common and contribute to disrupted, unrefreshing sleep.
Autonomic erectile dysfunction
In men, erectile dysfunction is often one of the first symptoms of multiple system atrophy, sometimes appearing months or years before the movement or balance problems. It reflects the early damage the disease does to the autonomic nervous system.
Orthostatic syncope
a sudden drop in blood pressure on standing (orthostatic hypotension) is a key feature of multiple system atrophy and can cause light-headedness or fainting.
Orthostatic hypotension due to autonomic dysfunction
A key feature is orthostatic hypotension: blood pressure falls sharply on standing because the autonomic nervous system can no longer regulate it, causing lightheadedness, blurring, or fainting.
Stridor
A harsh, high-pitched breathing sound (stridor), often at night, reflects vocal-cord involvement and is an important sign to recognize because it can affect breathing safety.
Rigidity
in multiple system atrophy, parkinsonism such as stiffness can occur but is typically poorly responsive to levodopa.
Frequent falls
severe symptomatic autonomic failure at diagnosis, such as orthostatic hypotension or urinary incontinence, is associated with a worse outlook in multiple system atrophy.
Constipation
multiple system atrophy can slow the gut (gastrointestinal dysmotility), which may cause constipation; this is managed with a stepwise combination of lifestyle measures, medicines, and device-aided approaches.
How it is diagnosed
Multiple system atrophy
Diagnosed using: autopsy with demonstration of oligodendroglial cytoplasmic inclusions.
“Mean survival from time of diagnosis ranges between 6 to 10 years, and definitive diagnosis is made on autopsy with demonstration of oligodendroglial cytoplasmic inclusions consisting of fibrillar α-synuclein.”
Multiple system atrophy
Diagnosed using: Magnetic resonance imaging.
“Magnetic resonance imaging (MRI) may be positive for cruciform T2 hyperintensity within the pons (the 'hot cross bun sign'), volume loss in the pons and cerebellum, and T2 signal loss in the dorsolateral putamen with hyperintense rim on fluid attenuated inversion recovery (FLAIR) sequencing.”
Treatment and management
What the research describes, not a recommendation. Treatment decisions belong with your clinician.
This covers treatments that appear in the published research mapped here. Investigational and experimental therapies are not included, so their absence is a boundary of this map, not a sign they do not exist.
levodopa
levodopa is the main medicine tried for the movement symptoms of multiple system atrophy, but the parkinsonism is typically only poorly responsive to it.
Used to help with: Parkinsonism.
“Multiple system atrophy (MSA) is a rare adult-onset synucleinopathy associated with dysautonomia and the variable presence of poorly levodopa-responsive parkinsonism and/or cerebellar ataxia.”
non-motor symptomatic management
neurogenic orthostatic hypotension in multiple system atrophy is managed as part of non-motor care using a stepwise combination of lifestyle measures, medicines, and device-aided interventions.
Used to help with: Orthostatic hypotension due to autonomic dysfunction.
“Non-motor symptoms management target autonomic failure (neurogenic orthostatic hypotension, urinary/sexual dysfunction, etc.), gastrointestinal dysmotility and sleep disorders (REM sleep Behavior Disorder, stridor, etc.), using a stepwise combination of lifestyle measures, pharmacological agents and device-aided interventions.”
How to read the evidence labels
Where this comes from
This guide is built from 7 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.
Take it further
Printed, source-linked documents built from this condition's graph — ready to bring to an appointment or attach to a coverage request. Every claim carries its published source, the same as this guide.