Gerstmann-Sträussler-Scheinker syndrome

(redirected from Gerstmann-straussler-scheinker disease)
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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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References in periodicals archive ?
Amyloid protein of Gerstmann-Straussler-Scheinker disease (Indiana kindred) is an 11 kd fragment of prion protein with an N-terminal glycine at codon 58.
Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P 102L Gerstmann-Straussler-Scheinker disease.
Except for 1 patient with Gerstmann-Straussler-Scheinker disease, all of these patients had sCJD.