PT  - JOURNAL ARTICLE
AU  - Adam, J
AU  - Crow, T J
AU  - Duchen, L W
AU  - Scaravilli, F
AU  - Spokes, E
TI  - Familial cerebral amyloidosis and spongiform encephalopathy.
AID  - 10.1136/jnnp.45.1.37
DP  - 1982 Jan 01
TA  - Journal of Neurology, Neurosurgery & Psychiatry
PG  - 37--45
VI  - 45
IP  - 1
4099  - http://jnnp.bmj.com/content/45/1/37.short
4100  - http://jnnp.bmj.com/content/45/1/37.full
SO  - J Neurol Neurosurg Psychiatry1982 Jan 01; 45
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.