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Multiple system atrophy (MSA)
Multiple system atrophy (MSA) is an adult-onset, sporadic, rapidly progressive, multisystem, neurodegenerative fatal disease of undetermined etiology, characterized by parkinsonian features; cerebellar, autonomic, and urogenital dysfunction; and corticospinal disorders
Prevalence
1-9 / 100 000
Age of Onset
ICD-10
G23.2
G23.3
Inheritance
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
5 Facts you should know
FACT
Many people affected by MSA experience dysfunction of the autonomic nervous system, which commonly manifests as orthostatic hypotension, impotence, loss of sweating, dry mouth and urinary retention and incontinence
FACT
MSA often presents with some of the same symptoms as Parkinson's disease
FACT
However, those with MSA generally show little response to the dopamine medications used to treat Parkinson's disease
FACT
Multiple system atrophy can be explained as cell loss and gliosis or a proliferation of astrocytes in damaged areas of the central nervous system
FACT
The prognosis for patients diagnosed with multiple system atrophy is poor, with an average life expectancy of 6 to 10 years after initial diagnosis
Interest over time
Common signs & symptoms
Abnormal brain FDG positron emission tomography
Abnormal pyramidal sign
Abnormal rapid eye movement sleep
Autonomic bladder dysfunction
Autonomic erectile dysfunction
Axial dystonia
Bradykinesia
Current treatments
Pharmacologic therapy
Is directed mainly toward alleviation of symptoms of the movement disorder and orthostatic hypotension
Movement disorders
- levodopa
- dopaminergic agonists
- anticholinergic agents
- amantadine
Orthostatic hypotension
- corticosteroids
- droxidopa
- midodrine