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Titolo:
ACTH therapy of West syndrome: Finnish views
Autore:
Riikonen, R;
Indirizzi:
Kuopio Univ Hosp, Dept Child Neurol, Kuopio 70211, Finland Kuopio Univ Hosp Kuopio Finland 70211 hild Neurol, Kuopio 70211, Finland
Titolo Testata:
BRAIN & DEVELOPMENT
fascicolo: 7, volume: 23, anno: 2001,
pagine: 642 - 646
SICI:
0387-7604(200111)23:7<642:ATOWSF>2.0.ZU;2-V
Fonte:
ISI
Lingua:
ENG
Soggetto:
FIRST-LINE TREATMENT; INFANTILE SPASMS; CORTICOTROPIN THERAPY; CEREBROSPINAL-FLUID; TUBEROUS SCLEROSIS; PROGNOSTIC FACTORS; VIGABATRIN; CHILDREN; DRUG; PREDNISONE;
Keywords:
West syndrome; adrenocorticotropic hormone; vigabatrin;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
37
Recensione:
Indirizzi per estratti:
Indirizzo: Riikonen, R Kuopio Univ Hosp, Dept Child Neurol, POB 1777, Kuopio 70211, Finland Kuopio Univ Hosp POB 1777 Kuopio Finland 70211 70211, Finland
Citazione:
R. Riikonen, "ACTH therapy of West syndrome: Finnish views", BRAIN DEVEL, 23(7), 2001, pp. 642-646

Abstract

To provide up-to-date information on adrenocorticotropic hormone (ACTH) therapy in the treatment of West syndrome, a review of the Finnish studies was made in answer to the questions: what are (1) its efficacy: doses and comparison with vigabatrin (VGB), (2) its tolerability, (3) its mechanism of action? Why do some patients respond, but others do not? No other drugs havebeen shown to be more effective than ACTH. High doses were not more effective than low doses. Synthetic derivatives were associated with more frequent side effects, Individualized therapy was developed on the basis of etiology and response. With therapy consisting of ACTH 3-6 IU/k-g/day, all the cryptogenic and half of the symptomatic spasms could be controlled within over 2-3 weeks therapy and with minimal risk of side effects. In a Finnish study, 26% of the patients responded to VGB as the first-line drug. Some of the non-responders responded to ACTH In tuberous sclerosis, the initial response rate to ACTH was high (73%) and did not differ from the response rate to VGB in other series. Both drugs have severe side effects. The visual field defects caused by VGB occur even in children (in 18/91 Finnish children). The patients with cryptogenic spasms, who responded well to ACTH, differedin their biochemical parameters from the patients with symptomatic spasms. The therapeutic action of ACTH may be mediated by potentiation of nerve growth promoting activity. Neurodegeneration may be due to imbalance between nerve growth factors and nitrate/nitrite in the brain. ACTH should be used as the first choice for treatment of West syndrome (at the minimal effective dose and for shortest effective time). The side effects of steroids, unlike VGB, are well known, treatable, and reversible. (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 28/09/20 alle ore 01:43:18