| Autor: |
Adam, J, Crow, T J, Duchen, L W, Scaravilli, F, Spokes, E |
| Předmět: |
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| Zdroj: |
Journal of Neurology, Neurosurgery & Psychiatry; Jan1982, Vol. 45 Issue 1, p37-45, 9p, 8 Black and White Photographs, 1 Diagram, 3 Charts |
| Abstrakt: |
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. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
| Externí odkaz: |
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