MULTIPLE SYSTEM ATROPHY
Philip A. Hanna, M.D.
Parkinson's Disease and Movement Disorders Center
New Jersey Neuroscience Institute
Edison, New Jersey
Multiple system atrophy (MSA) is a sporadic, progressive disorder
encompassing three previously-described neurodegenerative disorders:
Shy-Drager syndrome (SDS), striatonigral degeneration (SND), and
olivopontocerebellar atrophy (OPCA). All three types of MSA are
characterized by parkinsonism, particularly rigidity, slowed and
decreased movement, and impaired balance, though rest tremor is not a
main feature. Other symptoms may include weakness, spasticity and
disturbance of the autonomic nervous system resulting in
lightheadedness or fainting spells upon standing (due to a marked fall
in blood pressure), incontinence of bowel and bladder, sleep apnea,
and sexual dysfunction (impotence) in males.
Shy-Drager Syndrome (SDS) has a predominance of autonomic
disturbances, as listed above including postural
lightheadedness/fainting, impotence and bowel and bladder dysfunction.
Patients may also have dry mouth and skin, abnormal sweating and
difficulty regulating body temperature, sleep apnea and problems with
chewing, swallowing and breathing. The treatment of SDS is difficult
as levodopa can further lower blood pressure. Means of reducing
postural blood pressure changes include increasing dietary salt, use
of Jobst stockings, and medications such as fludrocortisone or
midodrine. Since these methods can increase blood pressure when supine
(lying down), the head of the patient's bed should be elevated at
least 30 degrees.
Striatonigral degeneration (SND) is characterized by a predominance of
parkinsonism, and may have sleep apnea and irregular breathing (such
as sighing) while awake, slurred and soft speech and balance
difficulties with falls. Tremor is not a main feature. Though response
to levodopa is generally poor, some patients respond somewhat to high
dose of levodopa.
Olivopontocerebellar atrophy (OPCA) has a predominance of
incoordination (ataxia) and tremor during action. Though some forms of
OPCA run in families (genetic), MSA only includes non-inherited forms.
Patients may also develop difficulty with eye movements, slurred
speech and difficulty swallowing. Brain imaging such as MRI scans
typically demonstrate decreased size of the cerebellum and brainstem
structures. Again, response to levodopa is usually poor.
Hanna PA, Jankovic J, Kirkpatrick JB. Multiple system atrophy: the
putative causative role of environmental toxins. Arch Neurol
1999;56:90-94.
Gilman S. Multiple System Atrophy. In: Jankovic J, Tolosa E, eds.
Parkinson's Disease and Movement Disorders, 3rd edition. Williams and
Wilkins, Baltimore, 1998: 245-262.
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