Fronto-temporal dementia: Nosology, neuropsychology, andneuropathology


D. Neary
Bok Engelsk 1996
Utgitt
1996
Omfang
Side 176- 187
Opplysninger
Fronto-temporal dementia is the commonest clinical manifestation offrontotemporal lobar atrophy. It is associated with three types ofunderlying histology: microvacuolation or spongiosus (frontal lobedegeneration type), gliosis with or without inclusion bodies andswollen neurones (Pick-type). And motor neurone disease type, Theclinical disorder presents in the presenium, is highly familial andis characterized by profound personality change, social incompetenceand stereotypic ritualistic behavior, Language becomes attenuated,culminating in total mutism. Visuo-spatial skills are strikinglypreserved, The electroencephalogram is normal and structural andFunctional imaging reflect fronto-temporal lobar atrophy. In aminority of cases the amyotrophic form of motor neurone diseasecoexists with fronto-temporal dementia and has a rapid and briefduration of illness. Phenotypic characterization of patients at eachlevel of description: clinical, anatomical, and histological,provides a Foundation for the molecular genetic studies which oughtto shed light on the etiology ok this form of focal cerebraldegeneration. (C) 1996 Academic Press, Inc.
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