TY - JOUR T1 - Familial cerebral amyloidosis and spongiform encephalopathy. JF - Journal of Neurology, Neurosurgery & Psychiatry JO - J Neurol Neurosurg Psychiatry SP - 37 LP - 45 DO - 10.1136/jnnp.45.1.37 VL - 45 IS - 1 AU - J Adam AU - T J Crow AU - L W Duchen AU - F Scaravilli AU - E Spokes Y1 - 1982/01/01 UR - http://jnnp.bmj.com/content/45/1/37.abstract N2 - 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. ER -