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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A large cirsoid aneurysm of the scalp that was present since birth in a 1 1/2 year old baby is reported. For six months the patient had been suffering from generalized seizures and mental retardation. After excision of the aneurysm, the patient became seizure free.
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PMID:A large cirsoid aneurysm of the scalp associated with epilepsy. 99 4

Acute hemiplegia of obscure cause occurred in 28 children: 13 had had prolonged seizures and a high temperature (considered to have been the direct cause of the brain damage); 5 had had brief seizures, a lower temperature and a depressed level of consciousness; and 10 had a nonfebrile onset of hemiplegia and were found to have vascular abnormalities. Most of the first group were retarded and epileptic at long-term follow-up, as were about half of the second group, whereas children in the third group were of normal intelligence and epilepsy was uncommon among them. Hemiplegia persisted at follow-up in most of the children in each group, the proportion being at least in the third group; if cerebral angiography had demonstrated carotid stenosis or occlusion there was usually poor recovery from the hemiplegia. Bilateral changes on plain skull films or pneumoencephalograms were associated with mental retardation. Failure to control prolonged seizures accompanied by a high temperature predisposes to brain damage; therefore, early and vigorous management is essential.
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PMID:Acute hemiplegia of childhood. 114 81

The hypothesis that specific defects in synaptic and dendritic development of cerebral cortex may form the anatomical basis in some cases of mental defect has been examined by electron microscopy and by use of the Golgi-Cos method. Two types of abnormality have been identified to date. One is a specific lesion of presynaptic terminals, first reported by Gonatas and Goldensohn (14) in a child with mental retardation and myoclonic seizures. This lesion, consisting of massive proliferation of membranous structures in terminal axons, appears to be rare and may be the anatomical substrate of one or more genetically determined dementing illnesses in infancy. More commonly, cerebral cortex from the severely retarded shows defects in number, length, and spatial arrangement of dendrites and synapses, best demonstrated by the Golgi method. Such abnormalities have been found in six out of eleven brains from severely retarded individuals examined by us. The etiology of the retardation was unknown in the majority; two had other recognizable developmental malformations of brain. It is suggested that a number of different etiological factors, if active during the period of rapid synaptic and dendritic growth in cerebral cortex (i. e., from the last trimester of pregnancy to the end of the first postnatal year) may result in stunted development of these structures.
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PMID:Snyaptic and dendritic development and mental defect. 119 9

The premise of this review is that chronobiology, the science of biologic time structure and rhythms, is important in investigations concerning the etiology, mechanisms and effects of deficient mental adaptive development. Chronobiology is also shown to have potential importance in therapeutics and rehabilitation. Most of the information available now and supporting this wide-spread relevance of chronobiology relates to circadian rhythms, but physiological and behavioral rhythms having other cycle lengths also contribute. Recent findings in seven topic areas of chronobiology are reviewed with emphasis on facts and relationships actually or potentially important for consideration in mental retardation research. These are: 1) development of sleep and EEG patterns; 2) rhythmic susceptibility to seizures; 3) adrenocortical and dependent rhythms; 4) circadian rhythms in amino acids and biogenic amines; 5) rhythmic behaviors; 6) circadian rhythms in susceptibility and responses to drugs; and 7) circadian rhythms in human perception and performance.
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PMID:Chronobiology in mental retardation research: progress and prospects. 121 6

The authors describe a 25-year-old woman with giant axonal neuropathy (GAN) and severe CNS involvement. She had been admitted to hospital with generalized seizures, and had gait disturbances followed by progressive mental deterioration since childhood. Neurological examination revealed mental retardation, scanning speech, cerebellar dysfunction, pyramidal signs, mainly in the lower extremities, and peripheral sensory neuropathy. Sensory nerve conduction velocity was decreased; brain CT and MRI showed diffuse demyelination. Sural nerve biopsy revealed characteristic signs of GAN. The patient's older sister had died at the age of 23, after having had similar neurological disturbances since childhood. This case illustrates an unfamiliar presentation of GAN, characterized by mild sensory neuropathy and serve CNS involvement, including seizures.
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PMID:Giant axonal neuropathy with predominant central nervous system manifestations. 131 Feb 92

The studies included 64 children with newly diagnosed epilepsy, aged from 6 to 15 years of life. In 25 children with partial and secondary generalized seizures monotherapy with carbamazepine was introduced; in 19 children with primary generalized seizures--with phenobarbital, and in patients with both types of seizures--with primidone. Monotherapy was controlled by means of blood serum drug concentration level monitoring; the therapy was successful in all the children. The group did not include patients with mental retardation, and epilepsy was idiopathic. Prior to the institution of treatment, a single determination of blood serum triiodothyronine, thyroxine, TSH, prolactin, cortisol, LH and testosterone was made. Psychological test were carried out employing Wechsler's scale, Bender-Santucci test, rhythmic structures developed by Mira Stambak and test of manual dexterity (card display). In order to evaluate short-term effects of the employed drugs upon the blood serum concentration values of the studied hormones, a repeated determination was made one month after the initiation of therapy. The third determination was made one year after the onset of treatment in order to assess the long-term effects. The effect of drugs upon their cognitive functions was assessed in a follow-up psychological testing performed after one year of therapy. The studies combined with statistical analysis led to a conclusion that after one month of monotherapy there occurred a significant drop in thyroxine concentration levels, still augmented after one year. Patients treated with carbamazepine showed a significant decrease of T3 levels after one month and one year, whereas treatment with phenobarbital and primidone did not result in significant changes of T3 concentration. Yet, T3 and T4 concentration values did not exceed normal limits. No type of monotherapy resulted in significant long-term changes of TSH concentration levels. No clinical signs of hypothyroidism nor goiter were observed in the studied children. After one month of monotherapy with carbamazepine and phenobarbital there was observed a significant increase of prolactin and cortisol levels, which was absent after one year. The values observed did lie within normal limits. No significant changes were observed with respect to the effect of the studied drugs upon blood serum LH and testosterone levels. After a one-year monotherapy with primidone the children revealed a significant improvement of results measured on performance scale and by means of a full Wechsler scale. Carbamazepine and phenobarbital did not affect the intelligence quotient of the studied children.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[The effect of monotherapy on concentration of selected blood serum hormones and upon cognitive function of children with epilepsy]. 134 59

This study investigated the actions of proline on CA1 hippocampal pyramidal cells with use of slice preparations. Bath-applied L-proline first induced these cells to fire multiple orthodromic population spikes in response to a single stimulus and then blocked their response to both orthodromic and antidromic stimulation. These effects could be explained by postsynaptic depolarization followed by depolarization block. Grease-gap studies confirmed that L-proline depolarizes CA1 pyramidal cells. D-Proline was inactive in these tests. Excitatory amino acid antagonists reduced depolarizing responses to proline and N-methyl-D-aspartate (NMDA) in parallel. Mn2+ failed to attenuate proline-evoked depolarizations at concentrations that substantially inhibited synaptic transmission, but at a higher concentration it reduced responses to both proline and NMDA. These results suggest that proline depolarized CA1 pyramidal cells mainly by activating postsynaptic NMDA receptors. The neuroexcitatory and neurotoxic actions of proline in the hippocampus may contribute to the seizures and mental retardation associated with hyperprolinemia.
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PMID:NMDA receptor-mediated depolarizing action of proline on CA1 pyramidal cells. 135 8

A new type of non-specific X linked mental retardation is described in a three generation family. The three affected males had severe mental retardation (IQ 20 to 30), mutism, growth failure, frequent infections, seizures, and the following minor anomalies: brachycephaly, frontal hair whorl, square face, large mouth, thick lips, and prognathism. There was not a characteristic facies. Normal laboratory studies on the proband included a karyotype with fragile X screening, skeletal survey, blood amino acid, urine organic acid, and HGPRT levels. Linkage analysis was performed with 10 X chromosome DNA probes of which probe DXS255 at chromosomal region Xp11.22 gave a maximal two point lod score of 2.10 if phase was inferred and 1.20 if it was not. Crossovers were shown with probes mapping to regions Xp22, Xp21, and Xq28. Comparison of these patients with 80 X linked causes of mental retardation, including 41 which might be classified as 'non-specific', showed no other disorders compatible with the phenotypic and linkage data.
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PMID:Non-specific X linked mental retardation with aphasia exhibiting genetic linkage to chromosomal region Xp11. 847 12

Psychogenic seizures can mimic convulsive epilepsy and with repetitive attacks, iatrogenic complications from aggressive treatment of status epilepticus can occur. We studied neuropsychiatric features of 20 patients in whom psychogenic seizures were intractable and at times continuous. Nineteen of 20 patients seen were female, and all but one were under 40 years of age. All had convulsive attacks resistant to various medications, normal neurological examinations, and negative imaging studies and electroencephalograms (EEGs). Sixteen had previous evidence of epilepsy and the other four had epileptic relatives. Seizures were atypically prolonged, included back arching and pelvic thrusting, and persisted despite intravenous diazepam and therapeutic phenytoin and phenobarbital levels. Seizures terminated spontaneously in five, were stopped by suggestion in four, and persisted until respiratory arrest or elective intubation in 11. Ten patients had conversion disorder, six borderline or mixed personality disorder and four mental retardation. Fifteen had had some precipitating stressor and the remainder had histories of exhibiting attention-seeking behaviour. Nine of 10 patients with conversion disorder had 'conversion V' Minnesota Multiphasic Personality Inventory (MMPI) profiles, while personality disorder patients had elevation of several psychopathological scales. Patients with conversion disorder gradually improved with anticonvulsant discontinuation, while retarded individuals were helped by behaviour modification, situational change or neuroleptics. Personality disorder patients continued to have attacks and eventually discontinued follow-up. Clinical evidence of non-epileptic seizures includes clinical atypicality and long duration, exacerbation by medications and frequent attacks despite normal examination and studies.(ABSTRACT TRUNCATED AT 250 WORDS)
Seizure 1992 Mar
PMID:Neuropsychological and psychiatric correlates of intractable pseudoseizures. 136 48

Authors discuss some specifics in treating epilepsies in children with mental retardation that have to be considered. First is the problem of precise diagnosis, because of certain phenomena in mentally retarded children, and a possibility of the so called pseudoretardation that can be caused by epileptic seizures or inadequate medication. A specific problem is vulnerability of these patients regarding antiepileptic drugs, especially phenytoin and phenobarbiton. Usage of psychopharmacs should be minimized and drugs interaction has to be considered permanently. A general trend in treatment should go towards increase in usage of carbamazepine and valproic acid, preferring monotherapy.
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PMID:[Specifics in the treatment of epilepsy in children with mental retardation]. 136 26


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