Catalogo Articoli (Spogli Riviste)

OPAC HELP

Titolo:
Lhermitte-Duclos disease is a clinical manifestation of Cowden's syndrome
Autore:
Vantomme, N; Van Calenbergh, F; Goffin, J; Sciot, R; Demaerel, P; Plets, C;
Indirizzi:
Univ Hosp Gasthuisberg, Dept Neurosurg, B-3000 Louvain, Belgium Univ Hosp Gasthuisberg Louvain Belgium B-3000 g, B-3000 Louvain, Belgium Univ Hosp Gasthuisberg, Dept Neuropathol, B-3000 Louvain, Belgium Univ Hosp Gasthuisberg Louvain Belgium B-3000 l, B-3000 Louvain, Belgium Univ Hosp Gasthuisberg, Dept Neuroradiol, B-3000 Louvain, Belgium Univ Hosp Gasthuisberg Louvain Belgium B-3000 l, B-3000 Louvain, Belgium
Titolo Testata:
SURGICAL NEUROLOGY
fascicolo: 3, volume: 56, anno: 2001,
pagine: 201 - 204
SICI:
0090-3019(200109)56:3<201:LDIACM>2.0.ZU;2-C
Fonte:
ISI
Lingua:
ENG
Soggetto:
DYSPLASTIC GANGLIOCYTOMA; GERMLINE MUTATIONS; MULTIPLE HAMARTOMA; GENE; CANCER; PHAKOMATOSIS; ASSOCIATION; FAMILY; BREAST; PTEN;
Keywords:
Lhermitte-Duclos disease; dysplastic gangliocytoma; Cowden's syndrome;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
32
Recensione:
Indirizzi per estratti:
Indirizzo: Van Calenbergh, F Univ Hosp Gasthuisberg, Dept Neurosurg, Herestr 49, B-3000 Louvain, Belgium Univ Hosp Gasthuisberg Herestr 49 Louvain Belgium B-3000
Citazione:
N. Vantomme et al., "Lhermitte-Duclos disease is a clinical manifestation of Cowden's syndrome", SURG NEUROL, 56(3), 2001, pp. 201-204

Abstract

BACKGROUND Lhermitte-Duclos disease (LDD) is a hamartomatous overgrowth ofcerebellar ganglion cells, which replace granular cells and Purkinje cells. In recent years several cases involving the association between LDD and Cowden's syndrome (CS), an autosomal dominant condition characterized by multiple hamartomas and neoplastic lesions in skin and internal organs, have been reported. METHODS We reviewed the medical records and imaging studies of six patients with LDD who were treated at our institution, and we looked at other possible symptoms of CS. RESULTS Other clinical findings suggestive of CS were apparent in five patients: These included mucocutaneous lesions, acral keratosis, thyroid adenoma, fibrocystic disease ovarian cyst, intestinal polyposis, and arteriovenous malformation. Only in the youngest patient, a 5-year-old boy, were no cutaneous or other signs found, despite extensive clinical and ultrasound examination. CONCLUSION Our observations strengthen the hypothesis that LDD is a neurological manifestion of CS. Patients with LDD should receive a thorough dermatological and systemic screening, because some of the lesions (breast, etc...) can develop into malignant tumors. (C) 2001 by Elsevier Science Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 11/07/20 alle ore 20:03:08