Gerstmann-Sträussler-Scheinker syndrome


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Gerstmann-Sträussler-Scheinker syndrome

 [gerst´mahn-shtrois´ler-shīn´ker]
a group of rare prion diseases, inherited as an autosomal dominant trait but linked to different mutations of the prion protein gene. All forms of the syndrome have the common characteristics of cognitive and motor disturbances and the presence of numerous amyloid plaques in the brain. Three forms have been recognized: the ataxic form, which is accompanied by progressive cerebellar ataxia and dementia; the telencephalic form, which is accompanied by dysarthria, dementia, rigidity, tremor, and hyperreflexia; and Gerstmann-Strässler-Scheinker syndrome with neurofibrillary tangles, in which there are progressive short-term memory loss and clumsiness. Death occurs in 1 to 5 years.

Gerst·mann-Sträuss·ler-Schein·ker syn·drome

(gerst'mahn stris'lĕr shīn'kĕr), [MIM*137440]
a chronic cerebellar form of spongiform encephalopathy.

Gerstmann-Sträussler-Scheinker syndrome

[gerst′män shtrois′lershīn′ker]
a group of rare prion diseases, inherited as an autosomal-dominant trait but linked to different mutations of the prion protein gene. All forms of the syndrome have the common characteristics of cognitive and motor disturbances and the presence of numerous amyloid plaques in the brain. Three forms have been recognized: the ataxic form, which is accompanied by progressive cerebellar ataxia and dementia; the telencephalic form, which is accompanied by dysarthria, dementia, rigidity, tremor, and hyperreflexia; and Gerstmann-Sträussler-Scheinker syndrome with neurofibrillary tangles, in which there is progressive short-term memory loss and clumsiness. Death occurs in 1 to 5 years.

Gerstmann,

Josef, Austrian neurologist, 1887-1969.
Gerstmann syndrome - finger agnosia, agraphia, confusion of laterality of body, and acalculia caused by lesions between the occipital area and the angular gyrus.
Gerstmann-Sträussler syndrome - a more chronic cerebellar form of spongiform encephalopathy.
Gerstmann-Sträussler-Scheinker syndrome

Sträussler,

E., Austrian physician.
Gerstmann-Sträussler syndrome - see under Gerstmann
Gerstmann-Sträussler-Scheinker syndrome

Gerst·mann-Sträuss·ler-Schein·ker syn·drome

(gerstmahn-strislĕr-shīnkĕr sindrōm) [MIM*137440]
Chronic cerebellar form of spongiform encephalopathy.
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