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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1998 Oct;65(4):600–603. doi: 10.1136/jnnp.65.4.600

Familial dementia lacking specific pathological features presenting with clinical features of corticobasal degeneration

J Brown 1, P Lantos 1, M Rossor 1
PMCID: PMC2170306  PMID: 9771798

Abstract

A family is described in which one member presented with symptoms and signs suggestive of corticobasal degeneration and a sibling presented with features of a frontal lobe dementia. Their mother developed a presenile dementia and movement disorder. At postmortem examination the member with clinical corticobasal degeneration had non-specific pathological features. Therefore, the clinical features of corticobasal degeneration can occur with non-specific pathological changes. Within a pedigree, different members can present with different clinical syndromes, which may reflect variation in the distribution and severity of the pathological process. 



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