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

Four cases with intractable epilepsy and mental retardation (Epi + MR), four cases of mental retardation (MR), one case of mental retardation without epileptic seizures for the last several years (MR + (Epi] and two normal children were studied on their sleep pattern. Besides these, two cases of epilepsy (Epi) were examined. Awake time increased in the Epi + MR group. Slow wave sleep decreased markedly in the Epi + MR group. REM sleep decreased in the MR + (Epi) and Epi + MR groups. REM density was lowered in the following order: normal----Epi----MR----Epi + MR groups. The difference of sleep pattern among the normal, Epi and MR groups was not exhibited clearly, but severe sleep disturbances were shown in the Epi + MR group, implicating the severe brain dysfunction in the cortex and the brain stem.
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PMID:Sleep pattern in children with intractable epilepsy and mental retardation. 654 13

Although the enzymatic etiology and the link between this and the accumulation of phenylalanine and its metabolites in phenylketonuria has been amply established, the pathogenesis of the brain dysfunction accompanying this inherited metabolic disease is still under research. Effects of phenylalanine and its metabolites, some leading to irreversible defective structural features in the nervous system, and others to reversible defective neurotransmission, are described as responsible of the mental retardation and other neurological and behavioural symptoms characteristic of phenylketonuria.
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PMID:[Pathogenesis of cerebral dysfunction in phenylketonuria]. 714 76

The spectrum of developmental disabilities encompasses a diverse group of interrelated disorders with multiple manifestations resulting from brain dysfunction. Motor disability has been highlighted, as it is the most frequent presenting sign of developmental disability. Such disability may vary from severe to minimal. Severe disability, or cerebral palsy, is well appreciated, as are the additional associated disabilities that affect habilitation. Mild to minimal motor disability (mild to minimal cerebral palsy), because it is usually nonhandicapping, is less frequently appreciated as a marker of brain dysfunction. Further delineation of the child's nonmotor abilities is necessary in order to define the scope of the disability. Cognitive limitation is not an important cause for the gross motor delay seen in children with mental retardation. It is our postulation that the motor deficit is on the basis of mild to minimal cerebral palsy. This diagnosis is frequently obscured by the mental retardation.
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PMID:Spectrum of developmental disabilities: continuum of motor dysfunction. 720 90

The evolutionary changes of evoked potentials (EPs) were studied from the neonatal period up to 1 year of age in 41 infants with various perinatal disorders. Abnormal EPs in the first week of life recovered quickly. In infants with normal outcome, abnormal EPs became normal within a month. In infants with cerebral palsy (CP) or mental retardation (MR), EPs recovered within 2-3 months of age. Infants with more severe neurological damage showed abnormal EPs even beyond 6 months of age. Abnormal EPs beyond 2 weeks of age indicated poor prognosis. As for the wave form of EPs, absent responses or abnormal wave form reflected more severe brain dysfunction. AEPs tended to show more profound abnormalities than VEP. However, some infants with absent AEP in the first week of life had a favorable prognosis. AEPs seemed to be more easily affected by brain dysfunction.
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PMID:The evolution of visual and auditory evoked potentials in infants with perinatal disorder. 731 93

Thirty-one individuals awaiting trial or sentencing for murder or undergoing an appeal process requested a neurologic examination through legal counsel. We attempted in each instance to obtain EEG, MRI or CT, and neuropsychological testing. Neurologic examination revealed evidence of "frontal" dysfunction in 20 (64.5%). There were symptoms or some other evidence of temporal lobe abnormality in nine (29%). We made a specific neurologic diagnosis in 20 individuals (64.5%), including borderline or full mental retardation (9) and cerebral palsy (2), among others. Neuropsychological testing revealed abnormalities in all subjects tested. There were EEG abnormalities in eight of the 20 subjects tested, consisting mainly of bilateral sharp waves with slowing. There were MRI or CT abnormalities in nine of the 19 subjects tested, consisting primarily of atrophy and white matter changes. Psychiatric diagnoses included paranoid schizophrenia (8), dissociative disorder (4), and depression (9). Virtually all subjects had paranoid ideas and misunderstood social situations. There was a documented history of profound, protracted physical abuse in 26 (83.8%) and of sexual abuse in 10 (32.3%). It is likely that prolonged, severe physical abuse, paranoia, and neurologic brain dysfunction interact to form the matrix of violent behavior.
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PMID:Neurologic abnormalities in murderers. 896 Jul 68

The neurologic outcomes of 61 children with asymmetric plantar grasp responses during infancy were evaluated during a follow-up period of 2.8-11.9 years (mean: 6.1 years). They all had perinatal risk factors and/or neurologic signs except for asymmetric plantar grasp responses recorded during infancy. The outcomes consisted of cerebral palsy in 38, delay in motor development in 6, mental retardation in 3, borderline intelligence in 9, and normal in 5. Most patients demonstrated concordance between the side of the abnormal response and the laterally in motor function and abnormal computed tomographic findings. The asymmetry in the plantar grasp response strongly suggests the existence of brain dysfunction. Infants with this asymmetry should be carefully monitored for the development of neurologic abnormalities.
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PMID:Asymmetry in plantar grasp response during infancy. 774 62

The relationship between the number of cortical tubers observed by magnetic resonance imaging (MRI) and the severity of cerebral dysfunction of tuberous sclerosis patients has been examined in a meta-analysis of the published literature. The literature review has identified five independent studies for examining the association. These studies consistently reveal that the cortical tuber count detected on MRI scans is increased among those with more severe cerebral disease. Severity of the cerebral dysfunction is measured by the seizure status and its control and by the developmental status and the level of mental retardation. Meta-analysis demonstrates that within a study population, the MRI-detected cortical tuber count is six times more likely to be above the median count for tuberous sclerosis patients with severe cerebral dysfunction (poor seizure control or moderate-severe retardation or both) than more mildly affected tuberous sclerosis patients. Similarly, across studies, moderately to severely affected patients are five times more likely to have greater than seven MRI-detected cortical tubers than those more mildly affected. These associations are both statistically significant and strong. The cortical tuber count is a biomarker that reasonably predicts the severity of cerebral dysfunction of tuberous sclerosis. Cortical tubers of tuberous sclerosis form in the early gestational period. The embryologic disruption determining the clinical severity of the cortical dysfunction of tuberous sclerosis is set in the early gestational period.
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PMID:Cortical tuber count: a biomarker indicating neurologic severity of tuberous sclerosis complex. 907 14

Recent studies have shown that the persistence of the cavum septi pellucidi beyond the neonatal period is a marker of cerebral dysgenesis. It has been suggested that the finding of a persistent cavum vergae is also a marker of disturbed brain development. In order to investigate this hypothesis we reviewed 161 brain magnetic resonance imaging scans from normal individuals for the presence of cavum septi pellucidi or cavum vergae, or both. In the 34 prospectively obtained normal adults, there were no individuals with either a cavum septi pellucidi or cavum vergae. In the "defined" normal subjects 3 of 127 individuals (2.4%) had a cavum septi pellucidi whereas a cavum vergae was noted in 26 of 127 (20.5%). We next reviewed the neuroimaging studies of 249 children and adults evaluated for mental retardation or developmental delay. A cavum septi pellucidi was found in 38 of 249 (15.3%) and a cavum vergae in 48 of 249 (19.3%) of these patients. A cavum septi pellucidi and cavum vergae were found together in 19 of 249 (7.6%). We interpret these data as showing that the cavum septi pellucidi is rarely seen in normal individuals although the cavum vergae is seen with the same frequency in normal and retarded populations. Thus we conclude that the cavum septi pellucidi serves as a significant marker of cerebral dysfunction manifested by neurodevelopmental abnormalities while the cavum vergae alone does not identify individuals at risk for cognitive delays.
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PMID:Cavum septi pellucidi and cavum vergae in normal and developmentally delayed populations. 953 37

We describe 11 patients affected by Landau-Kleffner syndrome (LKS) with a mean follow-up of 9 years and 8 months. EEG recordings during wakefulness, NREM and REM sleep showed a bitemporal electrical status epilepticus during sleep (BTESES) in all cases; four of them presented a shift from a BTESES towards an 'intercalated electrical status epilepticus during sleep' (IESES) accompanied by a global regression of cognitive and behavioural functions in 3/4 of cases. At the last observation, only 18.2% of cases presented a complete language recovery and mental retardation was evident in 63.6%. The prognosis of LKS in our cases may depend on the interaction of different negative factors such as onset of aphasia before 4 years, its duration for longer than 1 year, long-lasting duration and continuity without fluctuations of BTESES/IESES, probably preexisting mild speech delay. It is important for the prognosis to utilize antiepileptic treatment and possibly neurosurgical techniques to eliminate EEG paroxysmal abnormalities. At present, no similar cases with clinical-EEG evolution from LKS to electrical status epilepticus during sleep (ESES) have ever been described. Our observation demonstrates that LKS and ESES classified as different clinical-EEG syndromes represent two aspects of the same brain dysfunction and they may exist separately or pass one into the other with a change in the clinical-EEG picture. The common origin of the two syndromes is confirmed by recent functional brain imaging, neurophysiological and neurosurgical techniques.
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PMID:Landau-Kleffner syndrome (LKS): long-term follow-up and links with electrical status epilepticus during sleep (ESES). 1020 25

Mentally retarded children and adolescents not infrequently develop affective disorders, including a pattern of rapid cycling bipolar disorder. From work with nonretarded adults this pattern is known to have a tendency to chronicity and treatment resistance. Among persons with mental retardation, such a pattern is often marked by periods of severe overexcitation and underexcitation, often producing serious caretaking stress. Generally, it has been difficult to make this diagnosis in persons with severe to profound mental retardation and other severe disabilities, and it is probably often missed. However, knowing which questions to ask may facilitate diagnosis. It is our hypothesis that a positive family history of affective disorder in the presence of brain dysfunction increases the risk of rapid cycling affective disorder. Treatment results so far are limited to case reports and open trials. Much more research needs to be done to identify predictors of bipolar disorder and rapid cycling, the extent to which findings from studies of adults without mental retardation apply, and the most effective methods of treatment.
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PMID:Rapid Cycling Disorders in Mentally Retarded Children. 1032 Apr 12


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