Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
THE NEUROPATHOLOGY OF CHROMOSOME 17-LINKED DEMENTIA
Autore:
SIMA AAF; DEFENDINI R; KEOHANE C; DAMATO C; FOSTER NL; PARCHI P; GAMBETTI P; LYNCH T; WILHELMSEN KC;
Indirizzi:
UNIV MICHIGAN,MED CTR,DEPT PATHOL,1331 E ANN ST,BOX 0580 ANN ARBOR MI48109 UNIV MICHIGAN,MED CTR,DEPT INTERNAL MED ANN ARBOR MI 48109 UNIV MICHIGAN,MED CTR,DEPT NEUROL ANN ARBOR MI 48109 CORK UNIV HOSP,DEPT PATHOL CORK IRELAND CASE WESTERN RESERVE UNIV,INST PATHOL CLEVELAND OH 44106 COLUMBIA PRESBYTERIAN MED CTR,DEPT NEUROL NEW YORK NY 10032 COLUMBIA PRESBYTERIAN MED CTR,DIV NEUROPATHOL NEW YORK NY 10032
Titolo Testata:
Annals of neurology
fascicolo: 6, volume: 39, anno: 1996,
pagine: 734 - 743
SICI:
0364-5134(1996)39:6<734:TNOC1D>2.0.ZU;2-8
Fonte:
ISI
Lingua:
ENG
Soggetto:
PROGRESSIVE SUBCORTICAL GLIOSIS; AMYOTROPHIC-LATERAL-SCLEROSIS; KLUVER-BUCY SYNDROME; ALZHEIMERS-DISEASE; SUPRANUCLEAR PALSY; OLIVOPONTOCEREBELLAR ATROPHY; PARKINSONS-DISEASE; PICKS DISEASE; DEGENERATION; TANGLES;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
59
Recensione:
Indirizzi per estratti:
Citazione:
A.A.F. Sima et al., "THE NEUROPATHOLOGY OF CHROMOSOME 17-LINKED DEMENTIA", Annals of neurology, 39(6), 1996, pp. 734-743

Abstract

We recently described a family with chromosome 17-linked dementia, characterized clinically by disinhibition-dementia-parkinsonism-amyotrophy complex. We report now the neuropathology of 6 affected family members. This included semiquantitative scoring of neuronal loss, gliosis,and spongiosis and immunocytochemical and ultrastructural characterization of neuronal and glial inclusions. The changes consisted of circumscribed neuronal loss, gliosis, and spongiosis of limbic neocortical areas and frontal, temporal, and occipital association areas. Similar changes were present in subcortical nuclei, most severe in the substantia nigra, but also involved the ventral striatum and amygdala. The hippocampus was spared except for degeneration of the afferent perforanttract, secondary to entorhinal nerve cell loss, Argyrophilic neuronalinclusions, with a characteristic immunocytochemical profile, were found in brainstem nuclei, hypothalamus, and basal ganglia. Ultrastructurally, in 3 patients these inclusions showed hitherto undescribed abnormally assembled filaments. Glial cytoplasmic inclusions were widespread in white matter structures. Immunocytochemistry failed to demonstrate the protease-resistant prion protein. The pathology appears to be unique, involving various cortical and subcortical structures, and is consistent with the clinical findings of Kluver-Bucy-like syndrome, parkinsonism, and frontal lobe dementia. For this entity we suggest the term ''chromosome 17-linked dementia.''

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 05/07/20 alle ore 09:48:01