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  • Gerstmann-Strauissler-Scheinker disease(GSS)
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  • Gerstmann's s.
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DGS decompression sickness; developmental Gerstmann syndrome; diabetic glomerulosclerosis; Di George seq...
GSD genetically significant dose; Gerstmann-Straussler disease; glutathione synthetase deficiency; glyco...
GSS gamete-shedding substance; General Social Survey; Gerstmann-Straussler-Scheinker [disease]
GSSD Gerstmann-Straussler-Scheinker disease
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GSS Gerstmann-Straussler-Scheinker Syndrome
GSS Gerstmann-Strausler syndrome
GSS Gerstmann-Straussler-Scheinker
GSS Gerstmann-Straussler-Scheinker disease
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Gerstmann syndrome <syndrome> Tetrad of finger agnosia, dysgraphia or agraphia, dyscalculia or acalculia, and right-left disorientation producing confusion of laterality of the body.
The syndrome can occur in brain-damaged and apparently normal children as well as in adults who have had vascular accidents, and is caused by lesions between the occipital area and the angular gyrus.
(12 Jul 2000)
Gerstmann, Josef <person> Austrian neurologist, 1887-1969.
See: Gerstmann syndrome, Gerstmann-Straussler-Scheinker syndrome.
(05 Mar 2000)
Gerstmann-Straussler-Scheinker syndrome <syndrome> A familial spongiform encephalopathy. Transgenic mice with a mutant form of the PrP gene from patients with this syndrome develop degenerative brain disease that is similar, but not identical, to that caused by scrapie.
It is a more chronic cerebellar form of spongiform encephalopathy, producing a neurodegenerative condition that has morphological similarities to Creutzfeldt-Jakob syndrome. However, in this syndrome there is slower progression, signs of spinocerebellar ataxia, and the spongiosis is less pronounced.
Prion proteins and amyloid plaques are found in the brain of patients with the syndrome. A germline mutation of the prion protein has been demonstrated.
(12 Jul 2000)
MeSH(Medical Subject Headings) ¸ÂÃã °Ë»ö (http://www.nlm.nih.gov) °á°ú : 2 ÆäÀÌÁö: 1
  • Gerstmann Syndrome - »õâ A disorder of cognition characterized by the tetrad of finger agnosia, dysgraphia, dyscalculia, and right-left disorientation. The syndrome may be developmental or acquired. Acquired Gerstmann syndrome is associated with lesions in the dominant (usually left) PARIETAL LOBE which involve the angular gyrus or subjacent white matter. (From Adams et al., Principles of Neurology, 6th ed, p457)
    Synonyms : Acquired Gerstmann Syndrome, Acquired Gerstmann's Syndrome, Developmental Gerstmann's Syndrome, Gerstmann's Syndrome, Developmental Gerstmanns Syndrome, Finger-Agnosia-Left-Right-Confusion-Acalculia-Agraphias, Gerstmann Syndrome, Acquired, Syndrome, Gerstmann
  • Gerstmann-Straussler-Scheinker Disease - »õâ An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA, spastic paraparesis, extrapyramidal signs, and DEMENTIA. Clinical onset is in the third to sixth decade of life and the mean duration of illness prior to death is five years. Several kindreds with variable clinical and pathologic features have been described. Pathologic features include cerebral prion protein amyloidosis, and spongiform or neurofibrillary degeneration. (From Brain Pathol 1998 Jul;8(3):499-513; Brain Pathol 1995 Jan;5(1):61-75)
    Synonyms : Gerstmann-Straussler Inherited Spongiform Encephalopathy, Inherited Spongiform Encephalopathy, Gerstmann-Straussler, Gerstmann Straussler Inherited Spongiform Encephalopathy, Gerstmann Straussler Scheinker Disease, Gerstmann Straussler Syndrome
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Gerstmann syndrome The inability to recognize and use the fingers, differentiate left from right, problems writing and problems calculating, due to angular gyrus damage.
Ãâó: www.lieberson.com/en/neurgosurgery_glossary/g.htm
Gerstmann's s. a combination of finger agnosia, right-left disorientation, agraphia, acalculia, and often constructional apraxia; it was formerly attributed to a lesion in the angular gyrus of the dominant hemisphere, but now that etiology is in doubt.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
Gerstmann's syndrome see under syndrome.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
Gerstmann-Sträussler s. a group of rare prion diseases, of autosomal dominant inheritance but linked to different mutations of the prion protein gene, having the common characteristics of cognitive and motor disturbances and the presence of multicentric amyloid plaques in the brain. In the ataxic form, there are progressive cerebellar ataxia and dementia; in the telencephalic form, there are dysarthria, dementia, rigidity, tremor, and hyperreflexia; in GSS with neurofibrillary tangles, there are progressive short-term memory loss and clumsiness. Death occurs in 1 to 5 years.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
Gerstmann-Sträussler syndrome Gerstmann-Strässler-Scheinker syndrome.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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