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Titolo:
Late onset X-linked hydrocephalus with normal cerebrospinal fluid pressure
Autore:
Katsuragi, S; Teraoka, K; Ikegami, K; Amano, K; Yamashita, K; Ishizuka, K; Miyakawa, T;
Indirizzi:
Natl Kikuchi Hosp, Div Clin Res, Kumamoto 8611116, Japan Natl Kikuchi Hosp Kumamoto Japan 8611116 in Res, Kumamoto 8611116, Japan Kumamoto Univ, Sch Med, Dept Neuropsychiat, Kumamoto 860, Japan Kumamoto Univ Kumamoto Japan 860 Dept Neuropsychiat, Kumamoto 860, Japan
Titolo Testata:
PSYCHIATRY AND CLINICAL NEUROSCIENCES
fascicolo: 4, volume: 54, anno: 2000,
pagine: 487 - 492
SICI:
1323-1316(200008)54:4<487:LOXHWN>2.0.ZU;2-0
Fonte:
ISI
Lingua:
ENG
Soggetto:
COMPLICATED SPASTIC PARAPLEGIA; MASA-SYNDROME; CONGENITAL HYDROCEPHALUS; CLINICAL SPECTRUM; MUTATIONS; L1; RETARDATION; STENOSIS; FAMILIES; GENE;
Keywords:
HSAS; normal pressure hydrocephalus; X-linked hydrocephalus;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
16
Recensione:
Indirizzi per estratti:
Indirizzo: Katsuragi, S Natl Kikuchi Hosp, Div Clin Res, Koshi Machi, Kumamoto 8611116, Japan Natl Kikuchi Hosp Koshi Machi Kumamoto Japan 8611116 , Japan
Citazione:
S. Katsuragi et al., "Late onset X-linked hydrocephalus with normal cerebrospinal fluid pressure", PSY CLIN N, 54(4), 2000, pp. 487-492

Abstract

A family with X-linked hydrocephalus with normal cerebrospinal fluid (CSF)pressure and in which three brothers and a grandson of case 11 a proband, were affected is reported. The symptoms at onset were epileptic attacks that started in adulthood in the three brothers and at the age of 6 years in the grandson. In the three brothers, from 10 to 27 years after the onset of epileptic episodes, disorganization of intelligence and psychiatric deterioration were gradually noticed by their families. At the same time, they showed occasional urinary incontinence. Brain computed tomography (CT) scans revealed dilatation of the ventricular systems. Based on the results of the measurement of CSF pressure and radioactive-iodinated human serum albumin (RISA)-cysternography, two of the brothers were diagnosed as having normal pressure hydrocephalus (NPH), and they were treated neurosurgically. However, no obvious improvement in clinical symptoms was observed. Although the grandson had shown normal psychomotor development during his early childhood,temporal epilepsy and temper tantrums started at the age of 6 years. Computed tomography-scanning revealed dilatation of the ventricular system similar to the other three cases at the age of 8 years. With the diagnosis of NPH, the patient underwent a shunt operation, which resulted in no obvious effects. As it is reasonable to surmise that the pathological gene would havebeen transferred via the daughter of the proband to the grandson, it is suggested that the inheritance manner might be X-linked recessive. The cases presented here are different from the cases of hydrocephalus due to stenosis of the aqueduct Sylvius (HSAS) and other types of X-linked hydrocephalus reported previously in terms of the age of onset, course? symptoms, and CT findings. Thus, it is suggested that the present cases might be a new type of X-linked hydrocephalus.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 28/11/20 alle ore 21:23:33