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

Eighty-six asphyxiated full-term neonates with CNS complications such as hypoxic-ischemic encephalopathy, admitted during a 10-year period (1972-81), were studied. Sixty-three (73%) of the infants survived the neonatal period, and 55 of these, excluding one who died at 7 months, were followed for 3 to 13 years. Thirteen (24%) of these 55 children showed either major (n = 8) or minor (n = 5) abnormalities. The former had multiple significant handicaps such as cerebral palsy, epilepsy and mental retardation. The latter had mild sequelae that did not interfere with normal life. High risk factors of predictive value in infancy for the sequelae were the absence of the Moro reflex over 6 days and abnormal neurological signs on discharge (P less than 0.001, respectively). Although the neonatal mortality decreased slightly in the last four years (1978-81) compared to in the first and second three year periods (1972-74, 1975-77) (P less than 0.10), the unchanged pattern of the outcome over the 10 years might indicate the importance of more preventive and intensive care for perinatal asphyxia to reduce the incidence of handicapped children.
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PMID:Long-term prognosis of asphyxiated full-term neonates with CNS complications. 360 39

We report here two cases in a family with pleomorphic clinical features which include mitochondrial myopathy, encephalopathy, stroke-like episodes, episodic disturbances of consciousness and other multisystemic abnormalities. The other signs observed in multisystemic abnormalities were ophthalmoplegia, short stature, diabetes mellitus, diabetes insipidus, renal dysfunction, optic atrophy, retinal degeneration, impairment of hearing and mental retardation or deterioration. A symptomatological variation was observed in cases in the same family. It is suggested that these widely varying symptoms may be expressions caused by a common biochemical defect which involves different tissues in different individuals in the family. The syndromes observed in the present cases were compared with other possibly-related mitochondrial encephalomyopathies.
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PMID:Familial mitochondrial encephalomyopathy with stroke-like episodes and episodic disturbances of consciousness: a study of pedigree including three generations with multisystemic abnormalities. 362 95

Hypoxic ischaemic encephalopathy (HIE) is a major cause of motor and mental retardation. There ist no doubt that the haemorrhagic form of HIE can be detected by cerebral sonography, but it is of great interest to recognize non-haemorrhagic HIE as well. We demonstrate the follow-up of three patients with different types of non-haemorrhagic HIE: Periventricular leukomalacia in a critical ill premature infant 35 weeks gestational age, multicystic encephalopathy in a term newborn with severe perinatal asphyxia and circumscript ischaemic leukomalacia in a five month old infant with near miss event for sudden infant death. Correlation between ultrasound and computed tomography proved that non-haemorrhagic HIE produces global or circumscript high echogenicity in the first week after the hypoxic event whereas computed tomography shows pathologic hypodensity in the same areas. Cerebral sonography is a very helpful and harmless method for the difficult diagnosis HIE. In the case of periventricular leukomalacia, CT scan gives no further information and can be avoided. Concerning HIE of term newborns and small infants, CT scan remains necessary, to evaluate the extension of cerebral injury.
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PMID:Hypoxic ischaemic encephalopathy: correlation between ultrasound and computed tomography. 368 8

We report a 3-year-old Japanese girl who developed acute encephalopathy while receiving calcium hopantenate (Calcium D-(+)-4-(2, 4-dihydroxy-3,3-dimethylbutyramido) butyrate hemihydrate). She had hyperammonemia, elevated CPK, lactic acidemia and pyruvic acidemia, however, she did not show elevated SGOT or SGPT. Calcium hopantenate has been used in Japan for the treatment of mental retardation with behavior abnormalities. Recently there have been three reports on the occurrence of Reye-like syndrome in patients receiving this drug. Clinical signs and laboratory data of these patients are similar to those of Reye syndrome. Calcium hopantenate causes pantothenic acid deficiency in the young rat, which may reduce the content of coenzyme A. If this drug decreases coenzyme A biosynthesis, it may reduce beta-oxidation of fatty acids and levels of dicarboxylic acids would increase because of increasing omega-oxidation. We suspect that there is a possible relationship between the occurrence of acute encephalopathy and calcium hopantenate therapy.
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PMID:Acute encephalopathy with hyperammonemia and dicarboxylic aciduria during calcium hopantenate therapy: a patient report. 382 51

Determination of monoamine metabolites was accomplished in the spinal fluid of 22 psychotic children and in 22 sex- and almost-age-matched "normal" controls. Also, specimens from groups of mentally retarded children and children with progressive encephalopathy or meningitis were used for comparison. The psychotic children showed raised levels of homovanillic acid. Thirteen children diagnosed as autistic by Rutter's criteria showed isolated increase of this metabolite. In the group of 9 children with other psychoses, both the level of homovanillic acid and that of 5-hydroxy-indoleacetic acid was raised. The comparison with the group of "simply" mentally retarded children and results within the psychotic group revealed that the increased concentration of monoamines was not attributable to mental retardation per se.
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PMID:Childhood psychosis and monoamine metabolites in spinal fluid. 619 18

Research in mental retardation in the GDR has been influenced primarily by the brain damage--development theory, which assumes biological as well as social determinants in human development. The theory was based on the results of brain damage (encephalopathy) research, which distinguishes between primary and secondary symptoms of mental retardation. The importance of social factors such as family conditions for development was demonstrated in a variety of studies, including research with adopted children. Positive results have been achieved with early education programming for developmentally disabled children, as well as with medical treatment for some genetically induced metabolic disorders. Results from longitudinal studies suggest that intelligence continues to develop in educable mentally retarded adolescents. New diagnostic methods have been developed; test of motor skills, visuomotor coordination, and a developmental test for young infants. Further developments of traditional school entrance diagnosis for special education classes is reported. Several studies show that there has been some success in the vocational and social integration of mentally retarded persons in the GDR.
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PMID:Research in mental retardation in the German Democratic Republic (GDR). 639 72

From a group of 66 patients with the Lennox-Gastaut syndrome, 12 whose manifestations had started after the 6th year of life were selected for study. These patients were observed clinically and electroencephalographically for an average period of 2.5 years. We concluded that the late-onset syndrome can: occur after a long interval between diffuse encephalopathy and the first clinical manifestations, with or without previous alterations in psychomotor development; be associated from the onset with serious mental retardation; exhibit simple, complex and mixed seizures similar to those observed in the early form. These patients can also: suffer complex and mixed epileptic seizures previously unreported; paroxysmal interictal EEG abnormalities that overlap those of the early form; and spike-slow wave complexes in the EEG that can be actived by hyperpnea. Our results demonstrate that the incidence of LGS after 6 years of age does not necessarily imply a lower frequency of organic antecedents, or better neuropsychomotor development up to the onset of the syndrome or the presence of a higher rate of nonspecific seizures (generalized or partial seizures, and mainly those with elaborate symptomatology). The critical and encephalographic expression of the syndrome, which is secondary and starts after the 6th year of age, may depend at least in part on the age when diffuse encephalopathy started.
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PMID:Secondary late-onset Lennox-Gastaut syndrome: a critical view. 646 53

The clinical and cytogenetic features of 15 families with mental retardation linked to the fragile site on the X chromosome are presented. The 15 propositi were all prepubertal, and one was a girl. Although the clinical picture varied in severity, it was sufficiently constant to suggest the diagnosis from the facial features and the encephalopathy with language retardation and disturbed behavior. Macroorchidism was not seen before puberty. The fragile X chromosome was found in seven of the nine mothers studied and in two mildly retarded sisters and has also been demonstrated in fibroblasts in eleven subjects with the abnormality.
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PMID:X-linked mental retardation with the fragile X. A study of 15 families. 733 82

A 33-year-man with an encephalopathy of unknown aetiology, had an history of epilepsia for 30 years. Different types of seizures were seen, including grand mal and frontal attacks. Epilepsia was associated with mental retardation and behavioral disorders. At the age of 33, he was admitted for repetitive general convulsions. Epilepticus status lasted for two weeks and improved with vigabatrin et clonazepam. General seizures, frontal motor convulsions with arms and trunk antepulsion, and dacrystic attacks were seen. The latter seemed to be like normal crying because they were accompanied by lacrimation, contorted and mournful facies, and sobbing sounds. One year later, repetitive cardiac arrests occurred during a new epilepticus status. Cardiac arrests, observed on ECG holter lasted 10 to 24 seconds, without cardiac dysfunction. EEG patterns on ECG holter lasted 10 to 24 seconds, without cardiac dysfunction. EEG patterns included theta and delta activity with rhythmic slow wave epileptic activity, predominating on right side, in temporal areas. CT scan was normal. MRI showed right cerebral atrophy, prevailing in the temporo-mesial region, with right temporal horn enlargement. This case report of dacrystic seizures, the first one with MRI study, suggests that temporo-mesial structures of the non-dominant hemisphere may be involved in dacrystic and asystolic attacks.
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PMID:[Dacrystic and asystolic epileptic seizures]. 748 7

We review 160 cases of gliomatosis cerebri from the literature and report an additional three infants and young children who presented with intractable epilepsy, corticospinal tract deficits, and developmental delay in whom a pathologic diagnosis was made. The progressive nature of the encephalopathy in our cases was documented by serial clinical examination, electroencephalograms, magnetic resonance imaging, and positron emission tomographic scans. The natural history of gliomatosis cerebri was determined by a retrospective review of the literature of 160 cases in 85 reports. The most common neurologic symptoms and signs included corticospinal tract deficits (58%), dementia/mental retardation (44%), headache (39%), seizures (38%), cranioneuropathies (37%), increased intracranial pressure (34%), and spinocerebellar deficits (33%). The most commonly involved central nervous system structures were the centrum semiovale and cerebrum (76%), mesencephalon (52%), pons (52%), thalamus (43%), basal ganglia (34%), and the cerebellum (29%). Fifty-two percent of patients were dead within 12 months of onset. Different grades of glial neoplasm may also coexist within gliomatosis cerebri such as astrocytoma with anaplastic astrocytoma, atypical or anaplastic oligodendroglioma, and glioblastoma multiforme. Hypotheses regarding the pathogenesis of gliomatosis cerebri include blastomatous dysgenesis, diffuse infiltration, multicentric origin, in situ proliferation, and "field transformation." The biologic determinants of whether a transformed glial cell behaves as a relatively localized tumor mass or truly loses anchorage dependence to become migratory as well as proliferative are not understood.
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PMID:Gliomatosis cerebri presenting as intractable epilepsy during early childhood. 753 65


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