Amyotrophic lateral sclerosis with dementia: case report

Arquivos de Neuro-Psiquiatria

Paulo Roberto De Brito-marques¹  Roberto Vieira De Mello¹ 

[1] ,University of Pernambuco Faculty of Medical Sciences Department of Neurology,Brazil

关键词: dementia;    amyotrophic lateral sclerosis;    hypothyroidism;    brain SPECT;    demência;    esclerose lateral amiotrófica;    hipotireoidismo;    SPECT cerebral;   

DOI  :  10.1590/S0004-282X1999000200018

来源: SciELO

【 摘 要 】

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.

【 授权许可】

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