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Titolo:
Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum)
Autore:
Nowak, DA; Trost, HA; Porr, A; Stolzle, A; Lumenta, CB;
Indirizzi:
Tech Univ Munich, Acad Hosp Munchen Bogenhausen, Dept Neurosurg, D-81925 Munich, Germany Tech Univ Munich Munich Germany D-81925 urosurg, D-81925 Munich, Germany Tech Univ Munich, Acad Hosp Munchen Bogenhausen, Dept Pathol, D-81925 Munich, Germany Tech Univ Munich Munich Germany D-81925 Pathol, D-81925 Munich, Germany
Titolo Testata:
CLINICAL NEUROLOGY AND NEUROSURGERY
fascicolo: 2, volume: 103, anno: 2001,
pagine: 105 - 110
SICI:
0303-8467(200107)103:2<105:LD(GOT>2.0.ZU;2-T
Fonte:
ISI
Lingua:
ENG
Soggetto:
COWDEN-DISEASE; PHAKOMATOSIS; ASSOCIATION; DIAGNOSIS;
Keywords:
dysplastic gangliocytoma; Lhermitte-Duclos disease; posterior fossa mass;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
36
Recensione:
Indirizzi per estratti:
Indirizzo: Trost, HA Tech Univ Munich, Acad Hosp Munchen Bogenhausen, Dept Neurosurg,Englschalkingerstr 77, D-81925 Munich, Germany Tech Univ Munich Englschalkingerstr 77 Munich Germany D-81925 y
Citazione:
D.A. Nowak et al., "Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum)", CLIN NEUROL, 103(2), 2001, pp. 105-110

Abstract

Objective: Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is a rare hamartomatous lesion of, the cerebellar cortex. The pathogenesis of the disease is still poorly understood. Lhermitte-Duclos disease was recently considered to be part of a multiple hamartoma-neoplasia syndrome (Cowden disease). We add two further cases to this rare entity. Patients: A 24-year old woman presented with occipital headaches, blurred vision, diplopia and ataxia of gait. Physical examination revealed turricephaly. Thesecond patient was a 37-year old woman, who presented with progressive occipital headache with nausea and vomiting. Physical examination revealed congenital facial asymmetry. Computed tomography and NMR-imaging, respectivelydemonstrated a space occupying mass of a cerebellar hemisphere in both cases. Results: Suboccipital craniotomy and complete removal of the infratentorial tumour were performed in both patients. Histopathological findings clinched the diagnosis of Lhermitte-Duclos disease. Postoperative course was uneventful in the first and complicated by progressive occlusive hydrocephalus in the second patient, necessitating permanent surgical shunt drainage. Both patients were discharged free of complaints. Conclusions: Dysplastic cerebellar gangliocytoma is commonly associated with progressive mass effects in the posterior fossa and typically presents with headaches, cerebellar dysfunction, occlusive hydrocephalus and cranial nerve palsies. The diseaseusually manifests in young adults, but the age at presentation ranges frombirth to the sixth decade. There is no sex predilection. NMR-imaging became a useful clue to the diagnosis within the last decade. Therapy consists of decompression of the posterior fossa by total surgical removal of the tumour mass. (C) 2001 Elsevier Science B.V. All rights reserved.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 28/11/20 alle ore 22:02:30