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Titolo:
FAMILIAL PROGRESSIVE SUBCORTICAL GLIOSIS
Autore:
LANSKA DJ; CURRIER RD; COHEN M; GAMBETTI P; SMITH EE; BEBIN J; JACKSON JF; WHITEHOUSE PJ; MARKESBERY WR;
Indirizzi:
UNIV KENTUCKY,MED CTR,DEPT NEUROL,MS-129,800 ROSE ST LEXINGTON KY 40536 UNIV KENTUCKY,MED CTR,DEPT PATHOL LEXINGTON KY 40536 UNIV KENTUCKY,MED CTR,DEPT PREVENT MED & ENVIRONM HLTH LEXINGTON KY 40536 UNIV KENTUCKY,MED CTR,SANDERS BROWN CTR AGING LEXINGTON KY 40536 UNIV KENTUCKY,MED CTR,ALZHEIMERS DIS RES CTR LEXINGTON KY 40536 VET ADM MED CTR,DEPT NEUROL LEXINGTON KY 40511 UNIV MISSISSIPPI,MED CTR,DEPT NEUROL JACKSON MS 39216 UNIV MISSISSIPPI,MED CTR,DEPT PATHOL JACKSON MS 39216 UNIV MISSISSIPPI,MED CTR,DEPT GENET JACKSON MS 00000 UNIV CLEVELAND HOSP,DEPT PATHOL CLEVELAND OH 44106 UNIV CLEVELAND HOSP,DEPT NEUROL CLEVELAND OH 44106 UNIV CLEVELAND HOSP,ALZHEIMER CTR CLEVELAND OH 44106 CASE WESTERN RESERVE UNIV,SCH MED CLEVELAND OH 00000
Titolo Testata:
Neurology
fascicolo: 9, volume: 44, anno: 1994,
pagine: 1633 - 1643
SICI:
0028-3878(1994)44:9<1633:FPSG>2.0.ZU;2-E
Fonte:
ISI
Lingua:
ENG
Soggetto:
CREUTZFELDT-JAKOB DISEASE; FRONTAL-LOBE DEGENERATION; NON-ALZHEIMER TYPE; KLUVER-BUCY SYNDROME; PICKS DISEASE; NEURODEGENERATIVE DISEASES; DIFFERENTIAL-DIAGNOSIS; PRESENILE-DEMENTIA; FEATURES; INSOMNIA;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
84
Recensione:
Indirizzi per estratti:
Citazione:
D.J. Lanska et al., "FAMILIAL PROGRESSIVE SUBCORTICAL GLIOSIS", Neurology, 44(9), 1994, pp. 1633-1643

Abstract

We report clinical and pathologic findings from two kindreds afflicted with a familial form of progressive subcortical gliosis. The disorder segregated as an autosomal dominant trait. Onset was in the presenium and the course was slowly progressive. Affected individuals initially manifested personality change, degeneration of social ability, disinhibition, psychotic symptoms, memory impairment, or depression. Later,all developed progressive dementia, frequently associated with verbalstereotypy, decreased speech output, echolalia, or manifestations of the human Kluver-Bucy syndrome. Terminal clinical manifestations included profound dementia, frequently with mutism, dysphagia, and extrapyramidal signs. Autopsy of seven end-stage patients revealed generalizedcerebral atrophy, predominantly involving the white matter of the frontal and temporal lobes. Microscopically, prominent fibrillary astrocytosis was present in the subcortical white matter and in the subpial and deep layers of the overlying cerebral cortex. These changes were most pronounced in the frontal and temporal lobes, especially in the cingulate gyri and insulae. Mild cortical neuronal loss accompanied the gliosis, but no myelin loss was evident. The claustra and substantia nigra also showed severe astrocytosis and degenerative changes. Amyloid deposits and neuronal cytoskeletal inclusions were absent.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 23/09/20 alle ore 05:37:53