Familial cerebral amyloidosis and spongiform encephalopathy. Issue 1 (January 1982)

• Record Type: Journal Article
• Authors: Adam, J; Crow, T J; Duchen, L W; Scaravilli, F; Spokes, E
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 45:Issue 1(1982)
• Page Start: 37
• Page End: 45
• Publication Date: 1982-01
• Subjects: Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp.45.1.37
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : 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.