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Titolo:
Rasmussen's syndrome - Aetiology, clinical features and treatment options
Autore:
Tran, TX; Day, JA; Eskin, TA; Carney, PR; Maria, BL;
Indirizzi:
Univ Florida, Dept Paediat, Gainesville, FL USA Univ Florida Gainesville FL USA orida, Dept Paediat, Gainesville, FL USA Univ Florida, Dept Pathol Immunol & Lab Med, Gainesville, FL USA Univ Florida Gainesville FL USA l Immunol & Lab Med, Gainesville, FL USA
Titolo Testata:
CNS DRUGS
fascicolo: 5, volume: 14, anno: 2000,
pagine: 343 - 354
SICI:
1172-7047(200011)14:5<343:RS-ACF>2.0.ZU;2-Y
Fonte:
ISI
Lingua:
ENG
Soggetto:
EPILEPSIA PARTIALIS CONTINUA; LOCALIZED ENCEPHALITIS RASMUSSENS; GLUTAMATE-RECEPTOR GLUR3; INTERFERON-ALPHA THERAPY; IPSILATERAL UVEITIS; DOUBLE PATHOLOGY; CHILDHOOD; HEMISPHERECTOMY; AUTOANTIBODIES; REAPPRAISAL;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
73
Recensione:
Indirizzi per estratti:
Indirizzo: Maria, BL Div Paediat Neurol, POB 100296, Gainesville, FL 32610 USA Div Paediat Neurol POB 100296 Gainesville FL USA 32610 32610 USA
Citazione:
T.X. Tran et al., "Rasmussen's syndrome - Aetiology, clinical features and treatment options", CNS DRUGS, 14(5), 2000, pp. 343-354

Abstract

Rasmussen's syndrome (chronic encephalitis with epilepsy) is a rare neurological disorder characterised by progressive dysfunction of one cerebral hemisphere. Clinical and radiological features of the syndrome include intractable epilepsy, cognitive and motor decline, progressive unilateral cortical atrophy and the occurrence of epilepsia partialis continua (EPC) in many cases. Histopathological findings include the presence of perivascular cuffing, microglial nodules, gliosis and spongiosis. The aetiology of Rasmussen's encephalitis is unknown but viral, autoimmune and genetic mechanisms have been postulated. Proposed therapies have included anticonvulsants, corticosteroids, antiviral agents, interferon-alpha, immunogobulin and plasmapheresis. The mainstay of therapy is hemispherectomy. To date, no medical therapies have permanently halted neurological deterioration. A better understanding of pathogenesis is required to develop safe and effective medical therapies.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 05/04/20 alle ore 21:03:09