NR AABB
AU Adam,J.; Crow,T.J.; Duchen,L.W.; Scaravilli,F.; Spokes,E.
TI Familial cerebral amyloidosis and spongiform encephalopathy
QU Journal of Neurology, Neurosurgery and Psychiatry 1982 Jan; 45(1): 37-45
PT journal article
AB Clinical and neuropathological investigations are presented of the "W" family in which there is a dominant inheritance of slowly progressive cerebellar ataxia and dementia. The disease is of insidious onset and its average duration more than 4 years. Pathological findings included amyloid deposition in cerebellar and cerebral tissue; vascular amyloid in one case; and spongiform encephalopathy and astrocytic hyperplasia typical of Creutzfeldt-Jakob disease. Neuritic plaques of the senile (Alzheimer) type were present to a lesser extent. This study confirms the familial association between cerebral amyloidosis and spongiform encephalopathy.
MH Adult; Aged; Amyloidosis/*genetics/pathology; Astrocytes/pathology; Brain/*pathology; Brain Diseases/*genetics/pathology; Case Report; Comparative Study; Creutzfeldt-Jakob Syndrome/pathology; Female; Genes, Dominant; Human; Hyperplasia; Male; Middle Age; Pedigree; Syndrome
SP englisch
PO England