Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

1) Of 267 patients with epilepsy who were examined in our clinic during the period between 1964 and 1966, 97 were investigated in a 10-year follow-up study. The patients were selected according to the following criteria: the age of onset of seizures was 10 years or older and the follow-up period was 10 years after the onset of seizures. Of the 97 patients, 36 were followed up by mail. 2) In the adult 10-year follow-up study, seizures disappeared in 43.3%, decreased in 34.0%, remained unchanged in 16.5%, and were aggravated in 6.2% of the 97 patients. Comparing the five-year follow-up study with the present study, the rate of cases which were free of seizures for three years was 22.7% in the five-year study and 43.3% in this study. The seizures were controlled for at least three years in 22.9% of the cases with partial seizure and in 63.3% of the cases with generalized seizure. The worst prognoses were found in cases with versive seizure in which the seizure was unchanged and/or aggravated in 4 (44.4%) of 9 cases. Of 16 cases with psychomotor seizure, 25% were unchanged or aggravated. 3) Comparing the impaired consciousness seizure with the psychomotor seizure, there was a tendency for the psychomotor seizures to remain as a single type of partial seizure, while the impaired consciousness seizure tended to develop easily into a secondarily generalized seizure. 4) Of the 97 patients, three were personality disorders in 19 cases (19.6%), episodic psychotic state in 2 cases (2.1%), and mental retardation in 6 cases (6.2%). Disorders of mood, irritability and explosiveness associated with personality disorders were improved in three of six cases whose seizures disappeared. Personality disorders were found in half of those cases with partial complex seizures. No correlation was found between mental symptoms and the prognosis of seizures. 5) The EEG was improved in 15 (27.8%) of a total of 54 cases, unchanged in 35 (64.8%) and aggravated in 4 (7.4%). In comparing the prognosis of clinical seizure with changes in the EEG, the seizures improved in 40 (74.1%) of the cases, whereas the EEG improved in only 15 (27.8%) of 54 cases. Most of the clinical seizures were controlled or decreased, whether the EEG improved or not.
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PMID:A 10-year follow-up study of 97 epileptics. 47 91

A multisource medical register review identified persons with active epilepsy in northern Sweden. Seven hundred thirteen persons aged greater than or equal to 17 years with epilepsy were determined on the prevalence day, December 31, 1985. The overall prevalence was 553 in 100,000 (566 in 100,000 if adjusted to the 1980 U.S. population). The ratio of males to females was 1.1, with a male prevalence of 575 and a female prevalence of 530 in 100,000. Age-specific prevalences varied between 530-644 in 100,000 except in persons aged greater than or equal to 70 years, for whom the prevalence was 321 in 100,000. Partial seizures were most common, 333 in 100,000, of whom the majority (250 in 100,000) had seizures that occasionally were secondarily generalized. Mental retardation was the foremost coexistent disorder, noted in 23%. The mean yearly seizure frequency was higher in persons with mental retardation than in nonretarded persons. Seventeen percent had seizures during the last week, 57% during the last year, whereas 16% had greater than or equal to 5 years' freedom from seizures. Most had onset of epilepsy before age 20 years. A presumed etiology was noted in 35%, more often in men than in women. Cerebrovascular disease was the most commonly identified presumed cause. Other nonepileptic diseases/disabilities were noted in 47%.
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PMID:Prevalence of epilepsy in adults in northern Sweden. 159 18

Epilepsy associated with Marfan's syndrome is rare. Although previous observations have shown that Marfan's patients with epilepsy had angioid streaks in the retina or coloboma of the iris, such ocular manifestations were absent in two patients reported here. One patient with mental retardation and lumbosacral meningocele had generalized seizure. Another patient with sinus bradyarrhythmia and prolapsed mitral valve had partial seizure with secondary generalization. This report and review of the literature suggest that epilepsy in Marfan's syndrome can be due to primary CNS disorders or secondary to disorders of connective tissue.
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PMID:Marfan's syndrome and epilepsy: report of two cases and review of the literature. 682 33

Thirty children (20 males, 10 females) with intractable epilepsy received vigabatrin (VGB) as an open label basis to preexisting antiepileptic drugs. The seizure types consisted of generalized tonic clonic seizure [10], complex partial seizure [8], myoclonic seizure [7], and mixed type with simple partial seizure, complex partial seizure and/or generalized seizure [5]. The cause of the epilepsy was cryptogenic in 16 and symptomatic in 12. The current dosage regime of anticonvulsants were maintained during the trial period. VGB at 40-80 mg/kg/day were titrated according to the clinical response for a period of 2-24 months. The result of treatment was categorized as 'responders' with 13 (43%) having 50-75% reduction of seizure frequency; and 'non-responders' which consisted of 17 children. There was no relationship between outcome of VBG add-on therapy and the sex, age of onset, type of seizure, type of epileptic syndrome, etiology, associated neurological abnormality, mental retardation or abnormal brain CT/MRI findings.
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PMID:Open label trial with vigabatrin in children with intractable epilepsy. 750 85

With reported prevalence rates of 4-9 cases per 1,000 children, childhood epilepsy is a major public health concern. Reported prevalence rates vary, mainly because researchers often use different epilepsy definitions. In addition, total prevalence may be underestimated if incomplete case-ascertainment methods are used. We used a multiple-source case-ascertainment method that included obtaining information from electroencephalogram laboratories to estimate the prevalence of epilepsy and to classify seizure types among 10-year-old children. In the metropolitan Atlanta (GA, U.S.A.) area, we found a lifetime prevalence of childhood epilepsy of 6 per 1,000 (95% confidence interval, 5.5-6.5) 10-year-old children. However, using capture-recapture analysis, this prevalence may be as high as 7.7 per 1,000. Proportionately more boys than girls had epilepsy. The prevalence did not vary appreciably by race. Partial seizures, including secondarily generalized seizures, were the most common seizure type (58%). Of the children with epilepsy, 35% had another developmental disability (mental retardation, cerebral palsy, visual impairment, or hearing impairment). An accurate estimate of the public health burden of childhood epilepsy and determination of possible risk factors for idiopathic epilepsy both depend on conducting complete community-based case ascertainment and obtaining detailed clinical data.
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PMID:Prevalence of epilepsy and epileptic seizures in 10-year-old children: results from the Metropolitan Atlanta Developmental Disabilities Study. 754 79

Four patients suffering for severe drug-resistant epilepsy from bihemispheric cortical dysplasias underwent anterior callosotomy. One of these patients also presented mental retardation of mild degree associated with the epileptic syndrome. There were no operative complications in this series. Clinical signs of interhemispheric disconnection were not detectable postoperatively. Twenty-eight to 53 months after surgery, the generalized seizures were completely suppressed in 2 cases, and were reduced by 89-97% in frequency in the other 2 cases. Partial seizures were less affected by callosotomy being reduced by 14-87%. In an additional fifth case of intractable epilepsy from bihemispheric cortical dysplasias with associated severe mental retardation operated upon elsewhere for callosotomy and followed at our institution, the outcome for seizures was completely unsatisfactory. Neurophysiological studies revealed that the interhemispheric transfer (IHT) of visuo-motor responses was functionally impaired after callosotomy only in one patient who harboured bilateral cortical dysplasias in the occipital lobes. This malformation might affect the pattern of axonal projection to the posterior portion of the corpus callosum which is considered of crucial importance for the integration of crossed visuo-motor responses. From this paper the following conclusions can be drawn: a) epileptic patients with severe drug-resistant epilepsy due to bihemispheric cortical dysplasias are good candidates for callosotomy, b) one-stage extensive anterior callosotomy sparing the splenium is the procedure of choice, c) associated severe mental retardation seems to contra-indicate callosotomy, d) the neurophysiological study of the IHT can yield information on the functional status of the corpus callosum.
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PMID:Callosotomy for intractable epilepsy from bihemispheric cortical dysplasias. 775 63

Active epilepsy was assessed in all children aged 0-16 years in an area of northern Sweden with about 250,000 inhabitants and around 50,000 children in the age group. One hundred and fifty-five children fulfilled the criteria of active epilepsy giving a prevalence rate of 4.2/1000. The proportion of boys to girls was 1:1.1. This dominance of girls had also been seen in a prospective study of incidence of epilepsy in the same area. Partial seizures were more common than generalized. Among the syndromes benign childhood epilepsy with centrotemporal spikes occurred in 17.4%, absence epilepsy in 6.5% and Lennox-Gastaut syndrome in 5.8%. Children with neurodeficit (ND) more often had generalized epileptic syndromes while the non-ND groups more often had localisation related syndromes. The aetiology was regarded to be remote symptomatic in 42%, idiopathic in 28% and cryptogenic in 30% of the children. Almost all children were on antiepileptic treatment, three quarters of them on monotherapy. Neurodeficit was noted in 42%. The prevalence of epilepsy and mental retardation was 1.7/1000. Relatively few children with neurodeficit had been seizure free during the last year, 43% had more than one seizures per month compared to 19% in the non-ND group.
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PMID:Prevalence and characteristics of epilepsy in children in northern Sweden. 879 30

For the treatment of intractable generalized epilepsies, two-staged total callosotomy was performed in five patients. In all patients, preceded anterior callosotomy failed to obtain satisfactory seizure control. All patients showed mental retardation with various degrees. Mean age at the first operation was 10.2 years and 4 patients were operated in their childhood. All patients showed various types of seizures; drop attack (DA) in 3 patients, tonic seizure (TS) in 2, myoclonus (MY) in 2, complex partial seizure (CPS) in 2, atypical absence (AA) in 1, and head drop (HD) in 2. After anterior callosotomy, complete cessation of CPS and 50-80% reduction of DA was obtained in one, respectively. However, only less than 50% reduction of seizures was obtained in other types of seizures. Two years after anterior callosotomy, posterior portion of the corpus callosum was divided. After staged total callosotomy, complete cessation of DA was obtained in all patients and 80-100% reduction of AA was obtained in one patient. One adult patient showed the disconnection syndrome which did not affect activities of his daily life. Our study revealed the efficacy of posterior callosotomy in DA patients with unsatisfactory results after anterior callosotomy. This strategy should be considered especially in childhood cases, since obvious complication was not observed in such cases.
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PMID:[Staged total callosotomy for medically intractable seizures]. 1119 41

A 20 year-old woman began to have epileptic attacks of focal inhibitory seizure with paralysis and hypesthesia of her left or right upper limb followed by complex partial seizure several times a week since age 19. She was born by breech presentation and umbilical cord was coiling around her neck at birth. EEG showed spike foci on P 3, O1 and T5. Cerebral MRI with 4 mm-section inversion recovery image revealed bilaterally symmetrical polymicrogyria involving the posterior portion of sylvian fissure extending posteriorly to the inner cortex of the postcentral gyrus and the supramarginal gyrus, and she was diagnosed as bilateral perisylvian polymicrogyria. 99mTc-ECD SPECT showed increased cerebral blood flow over the bilateral polymicrogyric lesion. On cerebral MRA, bilateral middle cerebral arteries were narrow all way through. Epileptic attacks were controlled with zonisamide and carbamazepin. This is a rare case of bilateral perisylvian polymicrogyria because epileptic attacks were the only manifestation and the patient showed neither mental retardation nor neurological abnormality.
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PMID:[A case of bilateral perisylvian polymicrogyria with epileptic attacks of focal inhibitory seizure followed by complex partial seizure]. 1273 83

Kabuki make-up syndrome is a mental retardation-malformation syndrome affecting multiple organ systems, with a broad spectrum of neuromuscular dysfunction and mental ability. The incidence of seizures associated with this syndrome ranges from 10 to 40%. However, details of the seizures in this syndrome have not been adequately reported or thoroughly evaluated. In this study, we analyzed seizure characteristics and clinical outcomes in nine patients with Kabuki make-up syndrome. Four patients had generalized seizures and two patients had complex partial seizures, extended to secondary generalized seizures. West's syndrome, complex partial seizure, and atonic seizure were seen in one case each, respectively. Electroencephalograms showed focal spikes in seven cases, diffuse spike and wave burst in one case, and hypsarrhythmia in one case. Seizures were well controlled in eight cases and incompletely controlled in only one case. Together with mental retardation, epilepsy can be a primary feature of Kabuki make-up syndrome. Epilepsy associated with Kabuki make-up syndrome is mainly localization-related epilepsy with a favorable seizure outcome.
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PMID:Favorable seizure outcome in Kabuki make-up syndrome associated with epilepsy. 1367 81


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