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

A combined preventive scheme was conducted in four hospitals in Guangzhou to lower the rate of kernicterus and mental retardation caused by related neonatal jaundice due to G6PD deficiency. Observation was focused on 330 G6PD deficient infants, and the effects were measured according to the incidence of hyperbilirubinemia and kernicterus. The results, as compared to those of a retrospective study, showed that the incidence of hyperbilirubinemia was significantly decreased (51.4% to 21.2%), and neither kernicterus nor mental retardation infant was found in this series (12.5% in the control group). The authors conclude that this combined scheme is extremely effective and can be used in any large population area in which there is a high gene frequency of G6PD deficiency.
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PMID:[Clinical study on the prevention of kernicterus caused by hereditary glucose-6-phosphate dehydrogenase deficiency]. 133 14

Alagille's syndrome or arteriohepatic dysplasia has been described in Cuba in nine patients between nine months and 12 years of age (8 males and one female). Among the clinical features we found five major abnormalities: chronic cholestasis with neonatal jaundice (9/9), peculiar facies (9/9), peripheral pulmonary artery hypoplasia associated with cardiac murmur (6/9), butter-fly-like arch defects (4/9), and posterior embryotoxon (6/7). Two children had a severe xanthomatosis. Laparoscopy showed green hepatomegaly depending on the degree of cholestasis, and only one patient had incipient signs of micronodular cirrhosis. Liver histology showed a paucity of interlobular bile ducts. Survival was of 60%. One patient survived more than 30 years. Four patients died of liver carcinoma (unique report in infants), broncho-pneumonia, acute renal failure, and sudden death respectively. Among the minor features were mental retardation (5/9), a peculiar voice (3/9), growth retardation observed in some of our patients. This is the first report on Alagille's syndrome in Latin America, because so far reports have come only from Europe and North America.
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PMID:[Alagille's syndrome in Cuba. A report of 9 cases]. 134 Aug 42

A pediatric neurologist analyzed the case histories of 30 4-16 year old children diagnosed with cerebellar ataxia in the pediatric neurology unit at the Royal Hospital for Sick Children in Edinburgh, Scotland to examine its clinical features, investigative findings, and etiology. Previous unfavorable events happened to 14 children (46.6%). Yet only 6 (42.8%) of these 14 children had unfavorable events of etiological significance. These previous unfavorable events occurred during the perinatal period (48%). These events in order of significance were asphyxia, prematurity, neonatal jaundice, and trauma. 66.6% of all children had an unsteady gait. The 2nd and 3rd most common signs of cerebellar ataxia were truncal ataxia (53.3%) and hypotonia (36.6%). The next most common symptom was considerable delay in reaching gross motor milestones (50%) such as not sitting until 2 years old. 23 (76.6%) of the children had dysfunctions in 1 of the cerebellar divisions. Clinical examination found dysfunctions most often in the paleocerebellum (86.6) followed by the neocerebellum (70%) and archicerebellum (56.6%). The paleocerebellum and the archicerebellum were the only divisions involved in 6 and 1 of the remaining children, respectively. The most common cause of ataxia was hydrocephalus (23.3%) followed by perinatal problems (20%). 70% of the patients also experienced other central nervous system conditions such as macrocephaly and mental retardation. 5 children had normal investigative findings, 3 of whom had cerebellar ataxia syndrome, 1 had congenital ataxic cerebral palsy, and 1 had familiar ataxia.
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PMID:Cerebellar ataxia in childhood: a review of clinical features, investigative findings and aetiology in 30 cases. 150 89

The significance of cerebrospinal fluid (CSF) bilirubin levels on the outcome of neonatal jaundice has been investigated by correlations between kernicterus or brain damage and CSF bilirubin levels (Torii 1974, 1981, Meisels 1984), This study is designed to clarify the significance of CSF bilirubin levels in the long-term prognosis of neonatal hyperbilirubinemia. Sixteen full-term newborn babies with hyperbilirubinemia (serum bilirubin level 20.0-30.0 mg/dl) who did not receive phototherapy because of low bilirubin in their spinal fluid (usually under 0.5 mg/dl in normal neonates) were followed in this study of growth, intelligence and neurobehavior. The subjects' neurobehavior was compared with that of a matched control group. The physical growth of the children with a history of hyperbilirubinemia was within normal limits except for one girl whose weight was less than-2 SD from the average. Verbal IQ's were lower than performance IQ's in 10 out of 14 children with a history of hyperbilirubinemia. However, no mental retardation was noted. No significant difference was found between the two groups in mean positive scores on the Garfield test. In the Prechtl test, mean score of the finger-touching test was significantly higher in the children with a history of hyperbilirubinemia than in the control group. The soft sign coefficient and the occurrence of attention deficit hyperactivity disorder were not significantly different in the two groups. In summary, a low bilirubin level (less than 0.5 mg/dl) in the spinal fluid seems to be a good prognostic sign in infants with neonatal bilirubinemia.
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PMID:[Long-term follow-up of neonatal hyperbilirubinemia when phototherapy was withheld because of low bilirubin levels in the spinal fluid--growth, intelligence and soft neurological signs in school children]. 176 Jan 98

A review of 965 children with neurological disorders, seen at the Paediatric Neurology Clinic of the University of Nigeria Teaching Hospital (UNTH), Enugu, over a 3-year period (1985-1987), revealed that epilepsy was the most common neurological problem affecting 60% of the children, followed by cerebral palsy (16%), speech disorders (8.3%), mental retardation (7.2%), behaviour disorders (2.2%), paralytic poliomyelitis (1.55%), premature craniosynostosis (1.0%), visual and auditory impairment (1.0%) and muscle disorders (0.72%). Perinatal problems such as birth asphyxia, severe neonatal jaundice and infections were the most common aetiological factors identified. Facilities for rehabilitation of the children were inadequate and this, together with the people's ignorance of the natural history of some of the neurological disorders, may account for the high rate of default from follow-up observed in this study. The need for improved maternal and perinatal health services and vigorous health education strategies is emphasized by this review. The positive effect of the Expanded Programme on Immunization (EPI) is reflected in the sharp decline in the proportion of children with neurological disorders owing to paralytic poliomyelitis, from 9.2% in the period 1978-1980, to 1.55% in the present study.
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PMID:A review of neurological disorders seen at the Paediatric Neurology Clinic of the University of Nigeria Teaching Hospital, Enugu. 248 97

This study identifies the pattern of mental retardation in 291 Nigerian children in Enugu. In 33.99% the etiology of mental retardation was congenital (present at birth), in 43.99% the cause was acquired and in the rest 23.02% no definite cause could be identified. The results, problems of diagnosis and management in relation to Nigeria are discussed. Massive support from the Government for improving the maternal and child welfare services as well as care for the mentally retarded children is solicited. The high % of cases due to birth trauma (23.38%) and severe neonatal jaundice (8.59%) reflects the quality of maternal and child welfare services as well as the obstetric care in the area. Full use of the available services and intensive health education in towns and villages could be most valuable. The increasing association of mental retardation with epilepsy in Nigerian children has been noted in other studies in this hospital. In the present study, epilepsy was found to cause mental retardation in 12.5% of cases in the acquired group and about 5% of the total number of cases. Malnutrition as a cause of defective mental development has been noted by several authors. However, in the present study, malnutrition could not be identified as a primary cause of mental retardation. In the congenital group, the high representation of Down's syundrome is striking. Children with this syndrome make up about 42.71% of the congenital group and 14% of the overall number of mentally retarded. The majority of mothers were over 35 and multiparous. The care of the mentally retarded child in Nigeria leaves much to be desired. Except for a few states, there are no homes or centers for the furthering of these handicapped children. Since the inception of the Pediatric Neurology Clinic in this hospital, efforts have focused on diagnosis and assessment. Some children aged 5-12 years have been referred to a privately-owned Therapeutic Day Center in Enugu for early furthering and play therapy.
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PMID:Aetiology of mental retardation in Nigerian children around Enugu. 398 90

Several complications and diseases of pregnancy, delivery, and infancy have been found to be more prevalent among the mentally retarded than among normal children. In the course of reviewing 904 families of retarded children examined at the Tel Hashomer Assessment Center for the retarded (Israel), a group of 87 children with a nonspecific phenotype were isolated. Genetic analysis showed this to be of 75% autosomal recessive etiology. These mainly genetically retarded patients were used as a control group to assess a number of pathogenic factors in mental retardation. 3 groups of retarded patients with complete and reliable medical histories were compared: 87 retarded children with unidifferentiated phenotype, whose parents had normal intelligence and were either uncle-niece pairs or 1st cousins, and intelligence quotients (IQs) were less than 50 in 61 of these cases and between 50 and 69 in the remaining 26; 161 cases with idiopathic retardation with unrelated parents of normal intelligence, without retarded siblings; and 75 idiopathic retarded cases similar to the group of 161 cases but with only mild retardation. Complications of pregnancy, labor, and infancy were seen in 21 of the 61 control retarded groups with IQs under 50 and in 9 of the 26 with IQs 50 to 69. They were observed in 100 of the 161 cases of severe idiopathic retardation and in 53 of the 75 cases of mild idiopathic retardation. 11 factors were common enough to permit individual analysis: maternal age; history of multiple or recent spontaneous abortions; bleeding during pregnancy; toxemia; prematurity; low birthweight at term; signs of fetal or neonatal distress; neonatal anoxia; significant neonatal jaundice; convulsions during the 1st year of life; and evidence of early maternal neglect or chronic infantile illness. Maternal age over 35 at the time of birth was of no etiological importance. Abortions, either multiple or in the immediately preceding or subsequent pregnancy, were more than twice as common in the cases of mild idiopathic retardation as in the control group. The group with severe idiopathic retardation showed only a slight, insignificant increase in maternal abortions relative to the control group. Bleeding during early or middle pregnancy was more than 3 times as common in both the mild and severe idiopathic retarded groups as in the control group of homozygotes. Toxemia of pregnancy was twice as common among those with mild idiopathic retardation and 2-1/2 times as common in severe idiopathic retardation compared with the homozygote control group. Prematurity and low birthweight showed only a slight and nonsignificant rise in the 2 idiopathic groups relative to the controls. Signs of perinatal stress were practically nonexistent in the controls and were significantly more common in both the mild and severe idiopathic groups. Neonatal anoxia requiring some degree of medical attention was about 3 times as common in the 2 test groups. Events of neglect were as common in the control as in the other groups. Seizure prevalence in the 1st year of life showed a nonsignificant increase in the mild idiopathic group and an increase of marked significance in the severe idiopathic group relative to the controls.
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PMID:Pathogenic factors in idiopathic mental retardation. 719 53

Single photon emission computed tomography (SPECT) using N-isopropyl-p-[123I]-iodoamphetamine(123I-IMP) was performed in twelve patients with severe athetotic cerebral palsy (Ath; 5 males and 7 females) who had both motor delay (unable to move) and mental retardation (I.Q, or D.Q, below 30). The neuroimaging findings of those patients were compared with those of patients mental and motor disorders. In five cases suffering from neonatal asphyxia, SPECT demonstrated a decreased regional cerebral blood flow (rCBF) in corpus striatum, thalamus, orbitofrontal areas, pericentral gyrus areas, prefrontal areas and medial temporal areas. In seven cases suffering from neonatal jaundice, SPECT demonstrated a decreased rCBF in orbito-frontal areas, prefrontal areas and medial temporal areas. SPECT showed hypoperfusion of peri-central gyrus areas in cases with complications of spastic palsy. The decreased rCBF in medial temporal areas mostly corresponded to an alteration in hippocampal formation as assessed by magnetic resonance imaging (MRI). Cases with hypoperfusion of bilateral medial temporal areas showed a lower score of language understanding than those with the unilateral damage. In cases with hypofusion of bilateral prefrontal areas and bilateral medial temporal areas, the grade of understanding of language was almost below 12 months. In cases with hypoperfusion of orbitofrontal areas, psychomotor hypersensitivity had been observed. Those results suggest that IMP-SPECT and MRI of the brain is useful tool for neurological assessment in handicapped patients with athetotic cerebral palsy.
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PMID:[Brain MRI and single photon emission computed tomography in severe athetotic cerebral palsy: a comparative study with mental and motor disorders]. 761 86

The magnetic resonance examination was performed in 38 patients with severe cerebral palsy (CP; 15 males and 23 females) who had both motor delay (unable to move anywhere) and mental retardation (I. Q or D. Q below 30). Neuroimaging findings were compared with the CP type, etiology, and grade of understanding of language. Cranial magnetic resonance imagings (MRI) in CP were divided into five types. Type 1 : nine predominantly showed cyst-liked ventricles and periventricular hyperintensity on T2-weighted imaging (PVH) and only scarred basal ganglia and thalamus were visible. All suffered from neonatal asphyxia and the clinical type was rigospastic tetraplegia (RST). Type 2: eleven predominantly showed PVH and hyperintensity on T2-weighted (HT2) in basal ganglia and thalamus. All suffered from neonatal asphyxia and the clinical type was RST or rigospastic diplegia. Type 3: five showed PVH and three had cortical atrophy. All suffered from neonatal asphyxia and the clinical type was spastic diplegia. Type 4: four predominantly showed HT 2 in putamen and thalamus. Three had cortical atrophy. All suffered from neonatal asphyxia. The clinical type was athetotic CP (ATH). Type 5: nine predominantly showed HT 2 in globus pallidus. Four had cortical atrophy and two had hippocampal atrophy. All suffered from neonatal jaundice and the clinical type was ATH. All patients who suffered from neonatal asphyxia and spastic CP had MRI in PVH. All patients who suffered from neonatal asphyxia and ATH showed HT 2 in putamen and thalamus. Almost patients who suffered neonatal jaundice and ATH showed HT 2 in globus pallidus. With athetotic CP, cases with atrophy of the cerebral cortex or/and hippocampus were lower grade of understanding of language than no atrophy of both. The result of studies of MRI are in agreement with neuropathological findings.
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PMID:[The cranial MRI in severe cerebral palsy: a comparative study with clinical data]. 839 33

We examined the clinical picture of eight patients with severe intellectual and motor disabilities, who had experienced prolonged and severe neonatal jaundice, and showed localized lesions in the globus pallidus, subthalamic nuclei and hippocampus on MRI. All patients had athetoid tetraplegia, and five patients showed disturbed ocular movements and seven showed dysphagia. Five patients could communicate with others or utter words, and all showed mental retardation. Auditory brainstem responses were abnormal in seven, and the percentage of REM sleep on all-night polysomnography was reduced in three. Neither CT nor T1-weighted MR images could detect any changes in the pallidum or subthalamic nuclei, while T2-weighted MR images disclosed bilateral high signals in the pallidum, especially in the internal segment, in all patients. Five of the 7 patients, in whom coronal T2-weighted MR imagings were obtained, showed high signals in the subthalamic nuclei. The hippocampus showed atrophy and/or T2-prolongation in seven patients. In one autopsy case, these MRI changes were concordant with pathological lesions. In patients with athetoid cerebral palsy, brainstem dysfunctions, and abnormal ABR, localization of MRI lesions to the pallidum and subthalamic nuclei is evidence for neonatal bilirubin encephalopathy.
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PMID:[Localized lesions on MRI in the globus pallidus, subthalamic nuclei and hippocampus in patients with severe intellectual and motor disabilities]. 939 98


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