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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with tuberculous meningitis were treated with isoniazid and rifampicin for 12 months. To evaluate the result of treatment, we studied the outcome of patients treated from January 1979 to December 1985. Of the 51 patients, 27 were female, and 5, 25, and 21 patients were in the first, second, and third stages of the disease, respectively. Increased intracranial pressure of greater than 200 mm H2O was observed in 42 patients. Three patients required ventriculostomy, and one of them needed ventriculoperitoneal shunting. Three patients died within the first week of admission, and four patients were lost to follow-up. Forty-four patients were followed for 1 1/2 to 7 years; 31 of them recovered completely. Thirteen patients recovered with neurologic sequelae, which included mental retardation, motor weakness, seizures, and hydrocephalus. No serious side effect of the drugs were observed except for transient elevation of liver enzyme activities in four patients. The combination of isoniazid and rifampicin for 1 year, with appropriate management of increased intracranial pressure, seemed to be safe and effective enough to be used as a routine treatment of tuberculous meningitis in areas where resistance to these drugs is uncommon.
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PMID:Tuberculous meningitis in children: treatment with isoniazid and rifampicin for twelve months. 271 2

The prevalence of cerebral palsy (CP) in Swedish infants born in the four-year period 1979-82 is reported and related to the prevalence in infants born during the twenty-year period 1959-78. In 1979-82 it was 2.17 per 1,000 livebirths, 1.23 for children born at term and 0.94 for preterms, which means that the rising trend since the beginning of the 1970s persisted. The most pronounced rise, from 0.18 per 1,000 livebirths in the period 1967-70 to 0.67 in 1979-82 was found in the subgroup of preterms with spastic/ataxic diplegia. The severity of motor disability and the relative frequency of mental retardation, infantile hydrocephalus and epilepsy among preterm CP children successively increased over the same period of time. The livebirth prevalence of CP in term infants increased slightly but non-significantly during the period 1967-82. The birthweight-specific prevalence of CP per 1,000 newborns surviving the first week of life increased in all birthweight groups during the period 1967-82, significantly for birthweights below 1,500 g and over 2,500 g. The rising prevalence of CP was concomitant with a parallel fall in perinatal mortality, especially in very preterm infants. In the 1980s, severely multi-handicapped, very preterm children, only exceptionally seen among CP children born in the 1960s and 1970s, has become a matter of concern.
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PMID:The changing panorama of cerebral palsy in Sweden. V. The birth year period 1979-82. 278 17

A 1-year birth cohort from northern Finland comprised 12,058 children, 96% of all live-born infants born in the region in 1966. The development and morbidity of these children were followed up to the age of 14 years. Altogether, 40 children (25 boys and 15 girls) 3.32 per 1,000 suffered from congenital malformations of the central nervous system (CNS). Fourteen (1.16) had spina bifida cystica, 17 (1.41) hydrocephalus, and 9 (0.75) miscellaneous other malformations. Altogether, 14 children died during the follow-up period, giving a prevalence of 2.21 per 1,000 at 14 years. Additional neurological handicaps, mental retardation, cerebral palsy or epilepsy were present in 23 children, 13 of whom had multiple handicaps. Of these children 26% were able to attend an ordinary school in the class appropriate for their age. It is concluded that the incidences for CNS malformations obtained in this study are very much higher than those reported in the Finnish Register of Congenital Malformations, but correspond very well to the figures obtained in the British 1958 birth cohort, which was studied in an analogous way.
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PMID:Congenital malformations of the central nervous system in a 1-year birth cohort followed to the age of 14 years. 294 47

The case of a patient with concomitant regional odontodysplasia, hydrocephalus, and mental retardation is presented. Tooth eruption was retarded, probably because of the presence of a cementum-like substance on the enamel surface as well as the presence of discontinuous odontogenic epithelium that surrounded the affected teeth. Neural damage during intrauterine life is suggested as a likely cause of the dental abnormalities. This hypothesis is supported by the fact that both primary and permanent teeth in the area were affected. It is further supported by the finding of dysplastic dentin.
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PMID:Concomitant regional odontodysplasia and hydrocephalus. 303 55

We report the first Japanese female patient with Walker-Warburg syndrome. She had generalized muscle hypotonia with hydrocephalus due to Dandy-Walker malformation and bilateral microphthalmia with opaque corneas. She had severe motor and mental retardation. Muscle histology reflected advanced changes of muscular dystrophy. We discuss the relationship between Fukuyama congenital muscular dystrophy and Walker-Warburg syndrome, both of which fall within a spectrum of developmental abnormalities with a common cause. In Fukuyama congenital muscular dystrophy, ocular abnormalities are less severe.
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PMID:Walker-Warburg syndrome in a Japanese patient. 307 8

A case of quadrigeminal cistern arachnoid cyst associated with hydrocephalus is reported. A 1-year-old girl was admitted to our service on July 31, 1984, because of mental retardation and an enlarged head. She was born of a full-term pregnancy and normal vaginal delivery without prenatal complications. Progressive increase in her head circumference was noticed at the age of 6 months by her family physician. On admission she was found to be a well-nourished infant with a head circumference of 56 cm, bulging anterior fontanelle and mental retardation. Marked dilatation of the lateral ventricles and a large cyst in the quadrigeminal cistern were demonstrated on plain CT. There were no findings of communication between the ventricular system and the cyst on metrizamide CT ventriculography. The extension of the cyst from the quadrigeminal cistern to the right cerebello-pontine angle was demonstrated on reconstructed coronal CT. Reconstructed sagittal section revealed huge hydrocephalus caused by aqueductal stenosis. A vertebral angiography demonstrated opening of the para-mesencephalic segments of the bilateral posterior cerebral arteries and downward displacement of the right superior cerebellar artery. Accordingly, a large quadrigeminal cistern arachnoid cyst with hydrocephalus caused by aqueductal stenosis was suspected. Following V-P shunt operation for hydrocephalus, right temporo-parietal craniotomy was performed. The inner wall of the lateral ventricle was thin and an expanding cyst was observed through it. A partial resection of the cyst wall with the ventricular wall was performed to obtain communication between the cyst and lateral ventricle. The content of the cyst was watery clear fluid like CSF.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of quadrigeminal cistern arachnoid cyst associated with hydrocephalus]. 322 70

The outcome in a population-based series of 61 Swedish preterm infants born in 1967-82 with infantile hydrocephalus (IH) was investigated. Sixteen (26%) died before the age of two years. The available information was updated when the 45 surviving children were at least four years and six months old. A structured follow-up examination was performed in the 13 children who had passed the age of six years. Among the 45 survivors, 47% had cerebral palsy, 51% mental retardation and 33% epilepsy. The overall outcome for preterm infants with IH was found to be poorer than that for fullterm ones. Prognostic factors correlating to a poor outcome were an obvious origin of IH (pre- or perinatal) and a gestational age of less than 28 weeks. It is concluded that handicapped IH children born very or extremely prematurely constitute a new, and to a large extent severely brain-damaged group that has entered the Swedish IH panorama since the end of the 1970s.
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PMID:Epidemiology of infantile hydrocephalus in Sweden. Current aspects of the outcome in preterm infants. 322 86

Seven hundred five cases of agenesis of the corpus callosum (ACC) are reviewed from the literature (n = 660) and from our own observations (n = 45). The diagnosis was made or confirmed using neuroradiological techniques (n = 519) and necropsy or surgery (n = 231). Association with abnormalities often of chromosomes 8, 11, 13-15 and 18 suggests their involvement in abnormal corpus callosum (CC) morphogenesis. Four syndromes (e.g. Aicardi, acrocallosal, Andermann and Shapiro) are characterized by ACC, while others are only sporadically associated (e.g. fetal alcohol syndrome, Dandy-Walker syndrome, Leigh disease, Arnold-Chiari II syndrome). In non-Aicardi patients, the male-to-female ratio was 3:2 and X-linked recessive inheritance is postulated to play a role in some cases. Common abnormalities in acallosal patients included: mental retardation (MR), 73% [corrected]; seizures, 42%; ocular anomalies, 42%; gyral abnormalities, 32%; hydrocephalus, 23%; other central nervous system (CNS) lesions, 29%; costovertebral defects, 24%. Developmental disabilities are not attributable to absence of the CC per se, but due to other CNS malformation or dysfunction, which may be genetic or non-genetic. Future research using recombinant DNA techniques will enable isolation and identification of specific chromosomal defects in those cases with a genetic abnormality.
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PMID:Clinicopathological findings associated with agenesis of the corpus callosum. 331 Jul 13

We report on a family with X-linked hydrocephalus: progressive increase in head circumference (OFC) led to the diagnosis in 3 patients; however, in 5 with normal OFC, the initial diagnosis had been "nonspecific" mental retardation, until identification of relatedness between 3 macrocephalic boys suggested segregation of a major Mendelian gene. Moderate to severe hydrocephaly was present in all macrocephalic patients and in 3 of the 5 with normal OFC, but CT-scan of the brain did not show aqueductal stenosis in any of them. We stress the importance of a brain CT-scan in every male with "nonspecific" mental retardation, especially in cases with X-linked inheritance, because confirmation of X-linkage has important genetic counseling consequences.
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PMID:X-linked hydrocephalus. 342 2

Six new cases of the amniotic band sequence with a wide spectrum of defects were studied with respect to neurocutaneous manifestations, including histopathologic characteristics. Fetuses 1 and 2 were stillborn. Despite severe craniofacial involvement, infants 3 and 4 survived 1 yr and 22 months, respectively. Patients 5 and 6 had only limb defects. Craniofacial and neurologic manifestations of our longer-survival patients with severe craniofacial anomalies were microcephaly, encephalocele, micro- or anophthalmia, mental retardation, hydrocephaly, seizures, EEG abnormalities, impaired body temperature control, impaired vision, hypertonia, and other psychomotor deficits. Characteristic cutaneous manifestations seen in our patients were clefting, schisis, fibrous strings, ring constriction, lymphedema. pseudosyndactyly, and intrauterine amputation. Histologic examination of the amniotic bands attaching to the skin showed autolyzed amnion in patient 2, erosion of the band into upper dermis in patient 1, and fibrosis underlying the constricted areas in patient 5. Prenatal diagnosis by ultrasonography is possible in patients with severe craniofacial defects (patient 2). A brief review of the literature is offered.
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PMID:Amniotic band sequence and its neurocutaneous manifestations. 342 34


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