Familial cerebral amyloidosis and spongiform encephalopathy.
AUTOR(ES)
Adam, J
RESUMO
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.
ACESSO AO ARTIGO
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=491262Documentos Relacionados
- Rapidly progressive dementia caused by spongiform encephalopathy.
- Cerebral palsy and neonatal encephalopathy.
- Retrovirus antigens in brains of mice with scrapie- and murine leukemia virus-induced spongiform encephalopathy.
- Infectious amyloid precursor gene sequences in primates used for experimental transmission of human spongiform encephalopathy.
- Vidarabine encephalopathy.