Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The occurrence of behavioural problems in a population-based series of children with infantile hydrocephalus (non-spina bifida) was analysed, using parent questionnaires. Children with both infantile hydrocephalus and mental retardation had significantly more behavioural problems compared with those with no mental retardation and controls. Inattentiveness and hyperactivity were particularly typical. No differences were found between children with infantile hydrocephalus and no mental retardation and the control group.
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PMID:Behavioural problems in children with infantile hydrocephalus. 206 25

Neonatal intracranial hemorrhage secondary to immune thrombocytopenia has been uniformly associated with neurological sequelae in survivors. These sequelae are seizures, hydrocephalus, mental retardation, and developmental delay. We report 7 survivors of intracranial hemorrhage who were prospectively evaluated regarding their long-term outcome at a mean of approximately 5 years of age. Five children were completely normal. One was delayed in speech, and one had a ventriculoperitoneal (VP) shunt in place and a residual hemiparesis. Four children had had seizures including the two with sequelae (speech delay and hemiparesis); only the patient with the VP shunt was still taking anticonvulsant medication. This latter patient was also the only one who required special education classes in which she was maintaining her grade level. In summary, a good long-term outcome can be expected in at least some patients with neonatal intracranial hemorrhage in cases of severe neonatal thrombocytopenia caused by maternal antiplatelet antibodies. This good outcome may be a result of, and should encourage, early diagnosis and vigorous supportive care in the neonatal intensive care unit.
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PMID:Favorable neurological outcome in 7 cases of perinatal intracranial hemorrhage due to immune thrombocytopenia. 206 23

We examined the brains of 385 mentally retarded adults aged 23-90 years without Down's syndrome (DS), metabolic disorder, or hydrocephalus to extend our knowledge about the occurrence of Alzheimer-type neuropathology in this population. Relevant measures of neuropathology also were related to selected information available from clinical records. The presence of one or more neurofibrillary tangles (NFT) and/or neuritic plaques (NP) was observed in 63.4% of all cases and varied with age. The prevalence of positive cases was higher when mental retardation was due to head trauma, congenital malformation, or familial factors and when a history of seizures was reported. Comprehensive morphometric analyses of neocortical, hippocampal and parahippocampal areas indicated that recommended age-specific quantitative criteria for the diagnosis of Alzheimer disease [Khachaturian ZS (1985) Arch Neurol 42:1097-1105] were met in 9.5% of cases less than 50 years of age, 54.2% between 50 and 65, 70% between 66 and 75, and 87% of the cases greater than 75 years of age. However, a limited immunohistochemical study revealed that in most cases the NP did not have a neuritic component containing paired helical filaments and in this respect most of the plaques observed in this population may differ from those most strongly associated with Alzheimer disease. In addition, substantial numbers of NFT were seen in frontal cortex, contrasting with results reported in the literature for nonretarded populations. The number of NP per mm2 consistently increased with age for all areas examined, while the relationship between NFT density and age varied across areas, and was clearly not monotonic.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Alzheimer neuropathology in non-Down's syndrome mentally retarded adults. 223 48

Ten infants and children with choroid plexus papilloma of the III ventricle are presented. Hydrocephalus of various degrees was present in all patients, and seven patients had bilateral ventriculoperitoneal shunts prior to craniotomy. All patients were investigated with computed tomography and angiography. Tumor was resected through the transfrontal-transventricular approach in nine and through a transcallosal approach in one. One patient died intraoperatively due to an uncontrollable hemorrhage from a subependymal vein at its point of entry into the homolateral internal cerebral vein, and another died shortly after surgery due to hypothalamic trauma. The remaining eight patients are alive without recurrence over a minimum follow-up period of 3 years; three have mental retardation and seizure disorder. Despite this tumor's deep location and vascularity and occurrence in infancy, choroid plexus papillomas of the III ventricle can be successfully resected. Appropriate care for hydrocephalus and intra- and postoperative management are important.
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PMID:Choroid plexus papillomas of the III ventricle in childhood. Their diagnosis and surgical management. 225 46

We describe a two generation family in which two males have the X linked recessive MASA syndrome (mental retardation, aphasia, shuffling gait, and adducted thumbs). A third male in this family died at the age of 15 years from congenital hydrocephalus. In the present family cerebral abnormalities are reported for the first time. Linkage analysis confirms the chromosome localisation at Xq28. A crossover between the coagulation factor VIII locus (F8C) and MASA syndrome, but not with DXS52 and DXS305, locates the gene on the same side of F8C as DXS52 and DXS305. The possible relationship between MASA syndrome and X linked hydrocephalus is discussed.
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PMID:MASA syndrome: new clinical features and linkage analysis using DNA probes. 227 84

In an epidemiological study of a county cohort of 4,138 liveborn children, surveyed at age four, 45 had disability (1.1%). The following period prevalences from birth to age four in per mille were found: motor handicap 5.1, to include cerebral palsy 4.1, myelomeningocele and hydrocephalus 0.5, and metabolic disorders 0.5; severe mental retardation 2.9, mild 1.4, subnormality 2.7; epilepsy 4.6; severe visual defect 1.4; severe auditory defect 0.7. Perinatal damage alone was likely in only three children out of 45. Two thirds had a prenatal cause or a combination of prenatal and perinatal causes. Birth asphyxia seemed to be a rare cause of motor disability and mental retardation. Prematurity per se gave a risk of spastic diplegia, but not of mental retardation. Among the mentally retarded, half were light for gestational age, and in more than half, the damage occurred prenatally.
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PMID:An epidemiological study of disability in 4-year-old children from a birth cohort in Frederiksborg County, Denmark. 234 71

Methodological issues in research concerning intellectual sequelae of infantile hydrocephalus are reviewed, and a multivariate statistical approach to this problem is proposed and exemplified. The significance of a variety of medical history variables with regard to psychometric intelligence was assessed in a sample of 5- to 8-year-old children who had been shunted in the 1st year of life. Stepwise discriminant analyses revealed that many medical history variables were neutral with regard to intellectual outcome (as assessed by performance on psychometric tests). The presence of additional medical problems in infancy, as well as current ocular defects, were the most significant variables that were associated with a high likelihood of mental retardation. Implications for further research are discussed.
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PMID:Psychometric intelligence after infantile hydrocephalus. A critical review and reinterpretation. 235 12

The long-term outcome of infantile hydrocephalus (IH) in children born at term during a period of active shunt treatment was studied in a population-based survey. The series consisted of 68 children greater than or equal to 6 years old and born in 1967-78 in the south-western Swedish health care region. The clinical follow-up included neuro-paediatric assessment, Stott's test of motor impairment, the WISC test, CT and EEG analyses. Nineteen of the 68 children (28%) had cerebral palsy, 17 (25%) minor motor dysfunction and 32 (47%) no motor dysfunction; mental retardation was present in 26 (38%), 16 with an IQ 50-70 and 10 with IQ less than 50; 42 children (62%) had normal intelligence and epilepsy was found in 15 (22%). Compared with a non-shunted IH series from the 1950s, the survival of IH children had considerably increased. Of constituents characterizing the IH syndrome from the time prior to shunting, ataxia, divergent squint and the special "Cocktail-party behaviour" had significantly decreased, all of which conditions are highly related to chronic expansion of the ventricular system. The frequencies of other impairments such as mental retardation and epilepsy were fairly similar, reflecting the present increased survival of IH children with primarily non-IH-dependent brain damage. IH children with associated brain parenchymal defects had the poorest outcome, and those without had in general a much more favourable one. Thus the single most important factor for the outcome of IH was found to be the presence or absence of associated primary brain damage or maldevelopment.
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PMID:Epidemiology of infantile hydrocephalus in Sweden: a clinical follow-up study in children born at term. 246 73

We report a family with an 18p trisomic mother and two 18p tetrasomic daughters. The mother is phenotypically normal and healthy, but with an unusual type of trisomy 18p: 47,XX,del(18)(pter----p11.21),+i(18p) de novo. The older sister has microcephaly, mental retardation, an asymmetrical and peculiar face with low set ears, pinched up nose, high arched palate, small mouth, micrognathia, tapering fingers, asymmetrical length of legs, and an asthenic body. The younger sister was stillborn with extensive defects of the skull, congenital hydrocephalus, severe facial anomalies, and lumbosacral meningocele. Both daughters have inherited one normal chromosome 18 and an isochromosome 18p from their mother, and one normal chromosome 18 from their father. Although one quite similar family has been reported, to the best of our knowledge there have been no reports of families in which two daughters with tetrasomy 18p syndrome have been born to a mother with trisomy 18p with isochromosomes.
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PMID:Sibs with tetrasomy 18p born to a mother with trisomy 18p. 265 71

Toxoplasmic retinochoroiditis usually presents during the first three decades of life as a consequence of intra-uterine infection by Toxoplasma gondii. The ingestion of infected undercooked meat, or foodstuffs contaminated by infected cat faeces, constitute the primary sources of infection for the non-immune mother. It is thought that following congenital infection, Toxoplasma cysts remain dormant in otherwise normal retina and that acute retinochoroiditis is the result of reactivation of the parasite, perhaps by cyst rupture. Treatment is indicated for sight threatening disease and comprises anti-Toxoplasma agents. The addition of steroids may be required to diminish the inflammatory response. Photocoagulation of normal retina around focal lesions probably decreases the incidence of recurrent inflammation. Women should be advised not to eat undercooked meat and to avoid contact with cat excrement during pregnancy. These measures will decrease the incidence of both eye disease and the more severe manifestations of congenital toxoplasmosis, which include congenital abnormalities, mental retardation, hydrocephalus and blindness.
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PMID:Toxoplasmic retinochoroiditis--a historical review and current concepts. 266 25


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