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

In 50 cases of primary non-obstructive non-progressive hydrocephalus in infancy, possibilities of predicting later intelligence were investigated. The mean IQ was 80 +/- 24; in 50% of the cases IQ was normal, in 22% between 85 and 55 and in 28% below 55. The occipitofrontal circumference and the degree of ventricular enlargement had no correlation with the intellectual outcome in the children. Most prognostic value on the IQ had the age when reaching the developmental milestones, the degree of motor disability and the number of minor malformations. As there was no relation between the degree of hydrocephalus and the intellectual impairment, the conclusion can be made that associated anomalies of the CNS and defects in the cyto-architecture of the neocortex are more important factors in explaining mental retardation in cases of hydrocephalus.
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PMID:Intellectual sequelae of primary non-obstructive hydrocephalus in infancy: analysis of 50 cases. 409 5

The results of neuroradiologic studies of 71 children with infantile spasms treated with ACTH were correlated with the developmental outcome at follow-up (mean 62 months, range 14 to 207 months). Fifty-two (73%) patients had an abnormal NRS on initial evaluation; 49% had cerebral atrophy, 18% had congenital anomalies, and 6% had hydrocephalus. Twenty patients were normal on initial clinical evaluation. At follow-up only the eight (40%) with normal NRS were normal. Twelve (60%) who had unexpected abnormalities on NRS were retarded at follow-up. CAT scanning is necessary to predict the developmental outcome in developmentally normal children with infantile spasm. Eight of nine patients with normal NRS in the early treatment group were developmentally normal at presentation and follow-up. Ten patients who were developmentally normal before spasms began, and had normal NRS but were in the late treatment group, were retarded at initial evaluation and follow-up. This finding suggests that early treatment of children who have a normal NRS and normal development at onset of spasm prevents mental retardation.
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PMID:The value of neuroradiology in infantile spasms. 627 22

An apparently balanced de novo constitutional translocation (7;13) (q36;q13) was detected on peripheral lymphocytes and fibroblasts of a 14-month-old boy. The patient presented a facial dysmorphism with hydrocephaly and mental retardation associated with a Wilms' tumor. A pure coincidence of random association cannot be ruled out but one can equally assert the plausibility of a minimal unnoticed deletion or a position effect.
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PMID:Wilms' tumor, malformative syndrome, mental retardation and de novo constitutional translocation, t(7;13)(q36;q13). 632 Nov 91

Among 341 childhood brain tumors treated at Northwestern University--Children's Memorial Hospital during the years 1967-1980, there were 39 children (11%) who presented during the first year of life. Half of the total number of childhood choroid plexus papillomas, meningeal sarcomas and teratomas we treated occurred in this particular age group. Supratentorial tumors were more common than infratentorial, a rate of 1.8:1. Medulloblastoma and benign astrocytoma were the most common histological types. Hydrocephalus was present in 82% of the children and papilledema in 28%, so that progressive enlargement of head circumference was the most common reason for referral. For the 37 patients who underwent surgical removal or biopsy of the tumor, the 1-month mortality rate was 19% and the 1-, 3- and 5-year survival rates were 46, 30 and 22%, respectively. Whenever tolerated, roentgen therapy was given. Most of the 24 deaths occurred within 6 months of the time of diagnosis. 5 patients (1 each with malignant astrocytoma, medulloblastoma, meningeal sarcoma, and 2 with choroid plexus papilloma) are still alive 5 years later, without neurological or mental deficit, and with no sign of recurrence. There were three exceptions to Collin's rule. Among the 15 survivors, 5 suffer mental retardation.
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PMID:Brain tumors during the first year of life. 660 38

We report a boy aged 12 years 7 months with mental retardation, hydrocephalus, dysmorphic facial features, congenital heart disease, and skeletal and renal anomalies. The karyotype showed a mosaic tetrasomy 9p involving the secondary constriction. This result was confirmed by tetraplex gene dosage effect for galactose-1-P-uridyltransferase (GALT). Comparing the clinical features of our case with those of previously reported patients, tetrasomy 9p appears to be a distinctive and clinically recognisable malformation syndrome.
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PMID:Tetrasomy 9p confirmed by GALT. 664 73

Cerebral computerized tomography (CT) was obtained in 37 children with congenital hemiparesis to document the type of cerebral anomalies found in this group, correlate them with various features of the neurological handicap and clinical history and determine the prognostic and practical value of this examination in a newly diagnosed case. The CT-findings were: 1. normal CT: 4 children (11%); 2. unilateral ventricular enlargement: 9 children (24%); 3. others (cortical and subcortical lesions): 24 children (63%). In this latter group, special patterns were found: a) focal ischemic lesions: 7 cases; b) cystic porencephaly: 2 cases; c) ventricular deformities with hydrocephalus: 7 cases; d) varia: 8 cases. The children with normal CT or unilateral ventricular enlargement had mild or moderate hemiparesis without epilepsy or mental retardation. The third group comprised the more severe cases but the prognosis was very variable. The different types of CT-lesions, their possible origin and neuropathological correlates are described and compared with two previous studies. The variety of lesions found in this series and the relative prognostic value of the CT-Scan justify this examination in a newly diagnosed case.
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PMID:Congenital hemiparesis: the spectrum of lesions. A clinical and computerized tomographic study of 37 cases. 666 95

Neurological prospective follow-up study of premature newborns must be carried out to determine the usefulness and efficacy of modern perinatal intensive care. Over the last 30 years an improvement in perinatal care has led to a dramatic reduction from 70% to 20%-40% in mortality rate among VLBW infants (less than or equal to 1500 g); while among those weighing less than or equal to 1000 g the mortality rate has dropped below 60% in some intensive care units. The concentration of high-risk pregnancies and deliveries in the same hospital in which the premature is cared for has led to a further reduction in the neonatal mortality rate. Most, though not all, foreign research reports a lower handicap rate than prior to the era of newborn intensive care. Studies from newborn intensive care units show considerable variation in the rates of handicaps among surviving LBW infants. Studies carried out at the same time in intensive care units and in routine care units reveal a higher or equal handicap percentage among those from intensive care units. It should be however remembered that the sometimes higher handicap rate is linked to a considerably increased survival rate and the gain in healthy lives saved is considerable. A recent comparison made in various european, american and australian centres reveals an average of 7% of cerebral palsy and 15% of mental retardation among VLBW (less than or equal to 1500 g) infants. In recent years there has been a dual tendency towards a stabilisation in the frequency of handicaps and a reduction in the gravity of the worst handicaps. Over the last 10 years there has been a renewed increase in retrolental fibroplasia: as much as 5-11% in VLBW infants less than or equal to 1000 g. Squint is the other most frequent visual defect. Hearing defects vary from 1,8% to 15%. Posthemorrhagic Hydrocephalus which takes place either early or later in the neonatal period is the most frequent complication of intraventricular haemorrhages and the prognosis depends on the gravity of the haemorrhage. Epilepsy is rare; spastic diplegia, now almost always accompanied by normal intellectual development, though considerably less frequent than in the past, is still the most frequent form of cerebral palsy. Serious mental retardation is present only among one third-one quarter of major handicaps.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Neuropsychic follow-up of the premature infant: mortality, severe sequelae, early diagnosis and prognosis]. 676 28

In 20 patients with congenital brain disorders, the influence of the CNS maldevelopment on the neuroendocrine system was investigated by assessment of the hypothalamic-pituitary function through measurements of the secretory reserve of pituitary hormones (GH, PRL, TSH, LH and FSH) in response to injections of insulin, TRH and LH-RH, and of the secretion of sleep-dependent pituitary hormones with polygraphic recording. The subjects consisted of 9 patients with midline anomalies of the brain and face, 3 patients with hydrocephalus, hydroencephalodysplasia or microcephalus, and 8 patients with the malformation syndrome associated with mental retardation. Ten of the 18 patients examined showed normal responses of GH secretion in the loading test (secretory peaks: greater than 10 ng/ml). But only 4 of these patients were found normal in respect of GH secretory peaks of more than 10 ng/ml during sleep. Of these 17 children, 5 showed abnormal values for basal secretion of PRL, and/or the secretory peak of PRL on injection of TRH. Two children showed hypersecretion of PRL during sleep. One patient out of the 19 examined was unresponsive in gonadotropin secretion to injection of LH-RH and 2 patients displayed excessive responses. During sleep, 4 of the 13 patients studied were found to be hypersecretory and 2 hyposecretory of gonadotropin. These results suggest that abnormalities in pituitary hormone secretion are frequently present in patients with CNS maldevelopment, and growth disturbances and abnormal sexual development may in some instances be due to endocrine abnormalities.
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PMID:Hypothalamic-pituitary function in patients with congenital malformations accompanied by central nervous system disorders. 681 79

Nonspecific mental retardation (i.e., mental retardation with no known etiology) at all levels of functioning was investigated in the population of British Columbia, with ascertainment through the British Columbia Health Surveillance Registry. The age-specific prevalence of nonspecific retardation for the 15- to 29-year age group, where ascertainment was best, was 4.4 per 1,000 population at year-end 1978. A minimum incidence of 5.2 per 1,000 male live births and 4.0 per 1,000 female live births was calculated for the 1952-1965 birth cohort. Data on the number of cases associated with either microcephalus, hydrocephalus, epilepsy, or cerebral palsy were presented. Seventy-three percent of the cases with mild mental retardation had no associated disability, whereas only 26.9 percent of profoundly retarded cases were without any of these conditions. Survival rates varied with respect to the level of mental retardation.
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PMID:Nonspecific mental retardation in British Columbia as ascertained through a registry. 683 41

In an attempt to minimize the selection bias inherent in reporting the outcome of premature infants from a particular neonatal intensive care unit, this study presents data on all 294 live births 501 to 1,500 gm birth weight born to residents in the Hamilton-Wentworth region during 1973-78. The survival rate was 31.9% in infants less than or equal to 1,000 gm and 82.6% in infants between 1,001 to 1,500 gm. In all, 184 infants (62.6%) were discharged alive and 37 of these had weight less than or equal to 1,000 gm. The mean BW of the survivors was 1,216 +/- 214 gm and the mean gestation was 30.0 +/- 2.9 weeks, with 18.0% being small-for-gestational age. Neurologic handicaps including cerebral palsy, hydrocephalus, microcephaly, blindness, deafness, and mental retardation occurred in 30/179 (16.8%) survivors. The incidence of neurologic handicaps was 30% among babies who received IPPV versus 10% in those who did not. Within the IPPV and non-IPPV groups, there were no significant differences in handicap rates by 500 gm BW class.
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PMID:Follow-up of infants 501 to 1,500 gm birth weight delivered to residents of a geographically defined region with perinatal intensive care facilities. 703 80


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