TY - JOUR
T1 - Early-onset dementia and extrapyramidal disease
T2 - Clinicopathological variant of Gerstmann-Straussler-Scheinker or Alzheimer's disease?
AU - Hart, J.
AU - Gordon, B.
PY - 1990
Y1 - 1990
N2 - A case of progressive dementia and extrapyramidal signs beginning at age 29, with a ten year course until death, is presented. Necropsy examination showed an assortment of plaque types (including striatal plaques), neurofibrillary tangles, granulovacuolar degeneration, and depigmentation of the substantia nigra and locus ceruleus. This case had pathological features found in both Gerstmann-Straussler-Scheinker disease and in Alzheimer's disease. While somewhat similar to several other cases with features of both diseases, it differs in the presence of dystonia and striatal plaques. Although such cases may be difficult to categorise at present, they must be considered in the differential diagnosis of early onset dementia.
AB - A case of progressive dementia and extrapyramidal signs beginning at age 29, with a ten year course until death, is presented. Necropsy examination showed an assortment of plaque types (including striatal plaques), neurofibrillary tangles, granulovacuolar degeneration, and depigmentation of the substantia nigra and locus ceruleus. This case had pathological features found in both Gerstmann-Straussler-Scheinker disease and in Alzheimer's disease. While somewhat similar to several other cases with features of both diseases, it differs in the presence of dystonia and striatal plaques. Although such cases may be difficult to categorise at present, they must be considered in the differential diagnosis of early onset dementia.
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U2 - 10.1136/jnnp.53.11.932
DO - 10.1136/jnnp.53.11.932
M3 - Article
C2 - 2283522
AN - SCOPUS:0025001770
SN - 0022-3050
VL - 53
SP - 932
EP - 934
JO - Journal of Neurology, Neurosurgery and psychiatry
JF - Journal of Neurology, Neurosurgery and psychiatry
IS - 11
ER -