ATYPICAL PARKINSONISM
(PARKINSONISM-PLUS)
Philip A. Hanna, M.D.
Parkinson's Disease and Movement Disorders Center
New Jersey Neuroscience Institute
Edison, New Jersey
What is "atypical" parkinsonism?
"Parkinsonism" describes symptoms usually seen in
Parkinson's disease (PD), but may also be due to other disorders.
Patients with atypical parkinsonism have some features of Parkinson's
disease (tremors at rest, slowness of movement, stiffness,
unsteadiness and freezing while walking), but their symptoms result
not only from loss of cells of the substantia nigra (the area of the
brain most affected in classic PD), but also from degeneration of
cells in other areas of the nervous system which have receptors for
dopamine, such as the "striatum". In addition to the above
symptoms, these patients may have unstable blood pressure which
decreases markedly upon standing, abnormal eye movements, impaired
coordination, and early impairment of bowel, bladder, and sexual
function. Since patients with "atypical parkinsonism" have
loss of areas of the brain which contain dopamine receptors, they do
not respond to levodopa as well as patients with typical Parkinson's
disease, because of loss of the target site for levodopa.
Although the cause of most forms of atypical parkinsonism is unknown,
usually only one member of a family is affected and so these disorders
are likely sporadic (not inherited). This is different than
Parkinson's disease where genetic factors appear to be important. The
atypical parkinsonian syndromes are classified based on the pattern of
damage they produce in the nervous system, the clinical symptoms, and
their natural course. Atypical parkinsonism includes cortical-basal
ganglionic degeneration (or "corticobasal degeneration" -
CBGD), dementia with Lewy bodies (diffuse Lewy body disease), multiple
system atrophy (Shy-Drager, olivopontocerebellar atrophy (OPCA), and
striatonigral degeneration), progressive supranuclear palsy (PSP),
drug-induced parkinsonism, and vascular parkinsonism (due to small
strokes).
Hanna PA, Cardoso F, Jankovic J. Basal Ganglia and Movement Disorders.
In: Rolak LA, ed. Neurology Secrets, 2nd ed. New York: Hanley and
Belfus, 1998:137-169.
Jankovic J. Treatment of parkinsonian syndromes. In: Kurlan R, ed.
Treatment of Movement Disorders. Philadelphia: J.B. Lippincott
Company, 1995:95-114.
National Parkinson Foundation, Inc. (NPF)
1501 N.W. 9th Ave./Bob Hope Rd.
Miami FL 33136-1494
1-800-327-4545 in U.S. except Florida & California
1-800-433-7022 in Florida, 1-800-400-8448 in California, 305-547-6666
in Miami
310-203-8448 in Los Angeles
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