Familial cerebral amyloidosis and spongiform encephalopathy.

Author:

Adam J,Crow T J,Duchen L W,Scaravilli F,Spokes E

Publisher

BMJ

Subject

Psychiatry and Mental health,Clinical Neurology,Surgery

Reference24 articles.

1. In: Greenfield's Neuropathology;McMenemey, W.H.,1963

2. Ageing and the Dementias;Corsellis, J.A.N.

3. ' *: 40 while status spongiosus was prominent. No mention of spongiform change appears in the published case descriptions of amyloid angiopathy although astrocytic glial hyperplasia has been described around neuritic plaques and in the molecular layer of the cerebellum where there was also cell loss and torpedoes.15 16 The familial cases reported by Gerstmann, Straussler and Scheinker2l and Boellaard and Schlote22 were very similar to the "W" family with amyloid, spongiform changes and astrocytic hyperplasia in association with progressive cerebellar ataxia and dementia. As in the "W" family inheritance was by a dominant mode of transmission. Although mental deficiency is known in association with familial ataxia or spastic paraplegia beginning in childhood, the family reported by Akelaitis'9 was an example showing severe dementia with ataxia. However the pathology in this family is uncertain;v

4. pathology,1976

5. Jakob-Creutzfeldt Disease;Kirschbaum, W.R.,1968

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