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)

Atonic seizures, atypical absence seizures, myoclonic seizures, tonic seizures, and infantile spasms are among the most difficult to control in children. Certain 1,4-benzodiazepines may be effective against these seizure types. Thus, clonazepam has been shown to reduce the frequency of absence, atypical absence, myoclonic, atonic, and complex partial seizures in children. Furthermore, both clonazepam and nitrazepam have been shown to be effective in the treatment of infantile spasms. However, their usefulness is limited by a deleterious effect on neurologic function, particularly on cognition. Controlled studies in animals and in adult patients and volunteers have demonstrated that clobazam has less neurotoxicity than 1,4-benzodiazepines and, in addition, may possess a psychotropic effect. Uncontrolled studies of clobazam in children suggest that this drug may be effective in the treatment of partial seizures, startle-induced seizures, infantile spasms, and Lennox-Gastaut syndrome. We studied the effect of clobazam in children whose seizures were resistant to most other antiepileptic drugs. Mental retardation was present in 80% of the children, and 62.5% had Lennox-Gastaut syndrome. Tolerance to clobazam developed in approximately one-third of patients, but this was frequently only partial and often responded to an increase in dosage. Of 50 children studied for a minimum of 3 months, seizures were controlled completely in 10 and frequency of seizures was reduced by more than 50% in a further 17. In most cases, parents observed a striking improvement in neurologic function, particularly in alertness, concentration, and balance. Thus, clobazam may be of value in the treatment of those seizures which are most difficult to control in children.
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PMID:Benzodiazepines in the treatment of children with epilepsy. 374 24

We report the results of 34 patients who underwent corpus callosotomy between 1986 and 1989 with 28-65 months of postoperative follow-up (mean 42 months). Thirty-two patients had mental retardation and 26 had significant behavioral problems. Thirteen patients had total section, 8 had subtotal section with preservation of the posterior half of the splenium, and 13 had section of the anterior two thirds of the callosum. Satisfactory seizure control was achieved in 25 patients (73.5%) Atonic seizures, followed by tonic seizures, generalized tonic-clonic seizures (GTCs), and atypical absence seizures were most improved. Myoclonic and complex partial seizures (CPS) did not improve significantly. No deterioration in seizure status was observed postoperatively. Two patients developed previously unobserved simple seizures and CPS postoperatively, but they were not as disabling as the preoperative seizures. Among the patients with behavioral problems, 81% had significant decrease in aggressiveness, hyperactivity, and/or attention deficit. Patients who underwent total section had interhemispheric disconnection symptoms that improved progressively and did not interfere with daily life. Decreased speech output, dysarthria, and gait dyspraxia occurred after total callosal section and persisted in 5 of the 13 patients. Patients who underwent anterior two thirds or subtotal sections did not have such symptoms. Early postoperative complications consisted of aseptic ventriculitis (5), subdural hematoma (1), and wound infection (4) and resolved without sequelae.
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PMID:Corpus callosotomy in treatment of medically resistant epilepsy: preliminary results in a pediatric population. 840 46

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

The aim of this study is to identify early predictors of refractory epilepsy. From 600 epileptic children followed for at least 2 years in the department of neurology of Charles Nicolle hospital of Tunis, were identified children with refractory epilepsy. Controls were children who responded well to antiepileptic drugs and who were seizure free for at least 2years. We collected anamnestic, clinical, neuropsychological and radiological data for all children. We identified 67 children with refractory epilepsy, representing 11.6% of the initial population. At diagnosis, the average age was 9.16 years. Some factors have been identified as predictors of drug resistance epilepsy: age of onset less than one year, partial and atonic seizure, combination of several types of attacks, presence of mental retardation and pyramidal syndrome, abnormal electroencephalogram especially focal abnormalities, spike, amplitude abnormalities, interhemispheric asymmetry; and resistance to first antiepileptic drug. Symptomatic epilepsy, especially if associated with radiological lesions such as hippocampal sclerosis and structural brain malformations, was highly correlated with drug resistance. Our study suggests that the initial presentation of epilepsy could predict long-term outcome to drug resistance epilepsy if a detailed analysis of anamnestic, clinical and complementary data is established.
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PMID:[Predictors factors of refractory epilepsy in childhood]. 2631 97