Amyotrophic lateral sclerosis with dementia: case report

A patient is described in whom a profound and rapidly progressive dementia occurred in association with clinical features of amyotrophic lateral sclerosis. A magnetic resonance imaging showed signs of frontal and especially left temporal atrophy. The pattern of dementia indicated impaired frontotemporal lobe functions, evidenced by reduced tracer uptake in the frontotemporal lobes on brain single photon emission computed tomography. Neuropathological examination in this patient revealed mild frontotemporal atrophy with spongiform changes and neuronal loss affecting mainly layers II and III of the frontotemporal cortices. There was atrophy of the hypoglossal nuclei. The spinal cord changes were consistent with motor neuron disease. The patient showed an irreversible and progressive course. A review of the relevant literature was made.

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Bibliographic Details
Main Authors: BRITO-MARQUES,PAULO ROBERTO DE, MELLO,ROBERTO VIEIRA DE
Format: Digital revista
Language:English
Published: Academia Brasileira de Neurologia - ABNEURO 1999
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X1999000200018
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