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

1. Stereotyped behavior as a type of repetitive behavior was studied by observing four major groups of exceptional children by a natural observation method. The total 400 subjects are made up as follows: 46 with visual disturbance, 85 with auditory disturbance, 29 with infantile autism and 240 with mental retardation. 2. From analyses of our observational data, it was inductively proved that each group has its own characteristic sterotypy. There were marked differences in stereotypy between the auditory disturbance group and the visual disturbance group. Autistic children showed a perfect form of stereotypy and outstandingly bizarre characteristics. It was also found that blindism and autism have much in common. Stereotyped behavior shown by mentally retarded children differed with different intelligence levels. 3. There was no significant difference in the stereotypy appearance rate among the blind as well as the deaf-mute. Comparison between the blind with mental retardation and the blind with normal intelligence showed that there was significant difference of 0.5% or less. When taking the intelligence factor into account, significant differences aroused. As against the autism group, each group showed a significant difference of over 0.5%. In conclusion, it can be said that mental retardation factor has close bearing on the stereotypy appearance rate. 4. With respect to the cause of stereotyped behavior, the author based its argument on its own theory that blindism accrues from congenital visual disturbance. Weighing the fact that stereotypy differs between the blind and the deaf-mute, the author discussed with reference to the theory developed by Kaufman and Berkson that the inability of the child to monitor normal stimulation due to sensory disturbance produces frustration in him and this frustrated bodily need manifests itself in stereotyped movements. As for stereotypy of the mentally retarded children, the author pointed out that the arousal level theory is applicable only to the cases of severe-grade mental regardation, and that it fails to explain the parallelism between the differences of intelligence level and stereotypy. As regards stereotyped movements of the autistic children, due consideration was given to subtleties in action and bizarre nature of their behavior. Based on Orniz's theory that autistic children lack the constancy of central perceptual reception, the author maintained that the occurrence of stereotypy in autistic children suggests the underlying mechansims of perception are functioning pathologically as well as neurophysiologically. 5. As our observation as been conducted for a short period of time, the results might have been rather cross sectional. But the author thinks this shortcoming would have been made up for by Rutter's five-year follow-up study and the records available at the institutions which proved autism-stereotypy has continued to appear for a long period.
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PMID:A comparative study by the behavioral observation for sterotypy in the exceptional children. 124 57

We described a case of XYY syndrome associated with motor neuropathy. The case was a 27-year-old male, who had 47-XYY chromosome karyotype. His symptoms were distal dominant muscle weakness and muscle atrophy in his lower extremities without objective sensory disturbance. He had no mental retardation or family history of neurologic diseases. The electrophysiological and pathological examinations revealed that his symptoms were due to peripheral motor neuropathy (neuronal type). His motor neuropathy may be of sporadic type. There may be some relation in the pathogenesis between XYY syndrome and motor neuropathy, although in the literature, XYY syndrome with motor neuropathy had not been reported so far.
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PMID:[XYY syndrome associated with motor neuropathy]. 162 42

A case of adult Moyamoya disease, with formation of a transcranial external carotid-internal carotid (EC-IC) anastomosis through burr holes which had been made previously. A 43-year-old male suffered sudden headache and vomiting. Neurological examination revealed mild consciousness disturbance and dysarthria. The computed tomography (CT) scans showed intraventricular hemorrhage, which was drained through burr holes bifrontally. The diagnosis of Moyamoya disease was subsequently made by cerebral angiography. A month later he was discharged with mild gait disturbance and mental retardation. Seven years later he suddenly complained of gait disturbance, dysarthria and sensory disturbance involving the right upper extremity. A CT scan revealed a small hemorrhage in the left putamen. Carotid angiograms disclosed transcranial EC-IC anastomosis through the burr holes which had been made previously. It is suggested that revascularization can be expected after opening burr holes and incising the dura matter for Moyamoya disease in adults as well as, possibly, in children.
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PMID:[Adult moyamoya disease with a transcranial internal carotid-external carotid (EC-IC) anastomosis through burr holes]. 237 49

The clinical courses of four patients with occult spinal dysraphism who were also found to have anorectal malformations and tethered cord syndrome are evaluated in this report. These patients were among 27 patients with occult spinal dysraphism treated in the Division of Neurosurgery of Tohoku University School of Medicine during the last 3 years. All four patients experienced urinary disturbances early in life, and initially, these disturbances were considered to be caused by urogenital malformations. As a result, treatments were delayed. Only one patient with male Turner's syndrome exhibited mental retardation. Other symptoms, such as sensory disturbance and pain in the lower extremities, became apparent from complaints by the patients without atresia ani. When surgery was performed earlier in our patients with occult spinal dysraphism, the outcome was better. We believe in early surgery for tethered cord syndrome, although there is no consensus for the timing of this surgery. Unfortunately, the diagnosis of tethered cord syndrome was delayed in our four patients. However, because occult spinal dysraphism and anorectal malformations seem to be closely related, the presence of anorectal malformations may result in an earlier diagnosis of tethered cord syndrome.
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PMID:Evaluation of tethered cord syndrome associated with anorectal malformations. 832 77

We report here a family in which 4 out of 11 sibs of unrelated parents showed the typical clinical features of Dejerine-Sottas disease. Sensory disturbance was present in only one case and age at onset was delayed to 4 years in another. The others all conformed to the infantile form of the disease. This is the first time in which mental retardation has been reported in this disorder.
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PMID:The neuropathy of Dejerine and Sottas: report of an Indian family. 892 1

The somatosensory evoked potentials in two children with a unilateral migration disorder (pachygyria) of the cerebrum, which was detected by MRI, were examined in order to evaluate the function of the malformed sensory cortex. A 5-year-old girl had slight left hemiparesis, seizures, and mental retardation, and a 4-month-old boy had left hemiparesis. Neither patient showed distinct sensory disturbance. Short latency somatosensory evoked potentials and somatosensory evoked potentials recordings demonstrated that the early cortical component, N20, was absent and a positive wave appeared on paretic left-hand stimulation. On nonparetic right-hand stimulation, the primary evoked response (N20-P30) of the left hemisphere, which originates in Broadmann area 3b, was almost normal. Multichannel recordings on the scalp of one patient revealed that a positive wave without polarity inversion appeared posterior to the right central sulcus on median nerve stimulation on the paretic side. The radial dipole in the sensory cortex (area 1 or area 3a) or motor cortex (area 4) could have formed the positive/negative biphasic wave in the relatively wide centroparietal area in the present patients. In the case of unilateral cortical dysplasia, the malformed cortex with subnormal function of sensation might induce the change in the early component of somatosensory evoked potentials.
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PMID:Somatosensory evoked potentials with a unilateral migration disorder of the cerebrum. 962 11

We report a 48-year-old woman with familial spastic paraplegia (FSP) showing mental retardation, amyotrophy and sensory disturbance. Her parents were second cousins and there were two other affected siblings in the family. Autopsy revealed degenerative lesions characterized by neuronal loss and gliosis in the upper and lower motor neuron systems, thalamus, lateral geniculate body, dentate nucleus and posterior column of the spinal cord. The remaining neurons often contained ubiquitinated lipofuscin granules. Although the corpus callosum was severely attenuated, it exhibited well-preserved myelination and only minimal gliosis. In the substantia nigra, the number of pigmented neurons was apparently low, but there was slight gliosis and no extraneuronal free melanin pigment in the background. The neurons in this brain region contained much smaller amounts of melanin pigment than might be expected for the patient's age. These findings suggest that this is an example of a family with autosomal recessive FSP with thin corpus callosum, and that maldevelopment of the corpus callosum and substantia nigra is a characteristic feature of the disease.
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PMID:Autosomal recessive spastic paraplegia with hypoplastic corpus callosum, multisystem degeneration and ubiquitinated eosinophilic granules. 1119 44

Patients with cerebral palsy (CP) may have some problems other than this motor impairment: mental retardation, epilepsy and sensory disturbance. Healthy children and children with CP have an enhanced capacity for learning and memory compared to adults. There are few tools for brain plasticity investigations. The utility of the neurophysiologic and MRI techniques in the determination of brain reorganization and repair in patients with cerebral palsy is described. The authors discuss their results of quantitative EEG and spectroscopy MRI studies in children with CP. Quantitative EEG and spectroscopy MRI can be useful tools in the determination of these processes in children with CP.
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PMID:Neurophysiologic and neuroimaging studies of brain plasticity in children with spastic cerebral palsy. 1641 42