Dementia lacking distinctive histologie features: A common non‐Alzheimer degenerative dementia

作者: D. S. Knopman , A. R. Mastri , W. H. Frey , J. H. Sung , T. Rustan

DOI: 10.1212/WNL.40.2.251

关键词: DysarthriaSenile plaquesAutopsyPathologyPsychologyDementiaPersonality changesDegenerative diseaseCharged multivesicular body protein 2BFrontal lobe

摘要: From a series of 460 dementia patients referred to regional brain bank, 14 (3%) had pathologic diagnosis primary degeneration the involving multiple sites (frontoparietal cortex, striatum, medial thalamus, substantia nigra, and hypoglossal nucleus), with cell loss astrocytosis. There were no neuronal inclusions essentially senile plaques. This entity, which we have termed “dementia lacking distinctive histology” (DLDH), presented memory personality changes, led death, usually within 2 7 years. Dysarthria dysphagia prominent in later phases illness most patients. The psychometric findings some consistent “frontal” lobe dementia. A few caudate atrophy on CT as well neuropathologically. Eight our positive family histories for neurologic disease, mainly DLDH, addition Pick9s is major member frontal-lobe group. In under age 70 years, frontal dementias represent an important diagnostic consideration.

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