Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0238111 (Lennox-Gastaut syndrome)
 861 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 9-year-old male with congenital bilateral perisylvian syndrome is described. He had pseudobulbar palsy, mental retardation, and intractable epilepsy. Computed tomography and magnetic resonance images of the brain demonstrated bilateral perisylvian malformations and a diffuse pachygyric appearance. At 8 years of age, he had episodes of excessive drooling, fluctuating impairment of consciousness, unsteady sitting, and frequent head drop that lasted several days. The electroencephalogram demonstrated continuous diffuse slow spike and waves. These findings suggested atypical absence status epilepticus. Intravenous administration of diazepam resulted in transient improvement of clinical and electroencephalographic findings. Status epilepticus recurred within several minutes after diazepam administration. Although no patient has been reported to have a history of status epilepticus among those affected by this syndrome, it seems that atypical absence status can occur more frequently than expected, as seen in Lennox-Gastaut syndrome. After recognition and confirmation of nonconvulsive status epilepticus, immediate treatment must be attempted.
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PMID:Nonconvulsive status epilepticus in a child with congenital bilateral perisylvian syndrome. 1046 49

Lennox-Gastaut syndrome is a severe childhood epileptic syndrome with encephalopathy and multiple seizure types, which are often intractable to treatment. Most of these children will ultimately become mentally retarded and dependent on others for their daily care. Antiepileptic drugs are the mainstay of treatment, however, no particular drug is entirely effective. Apart from the use of antiepileptic drugs, nonpharmacologic treatments are also considered (i.e., callosotomy, ketogenic diet, and vagus nerve stimulation), which have proven to be partially effective. We prospectively studied 14 children (11 months-8 years of age) with medication-resistant Lennox-Gastaut syndrome, being treated with nitrazepam (open-label compassionate protocol). We compared the 1-month baseline seizure frequency with the median seizure rate reduction during the first 12 months of treatment with nitrazepam. The median seizure rate reduction during the first 12 months of treatment with nitrazepam was 41% (P = 0.001), with more than 50% seizure reduction in 60% of patients. Two patients became seizure free, five patients demonstrated at least 50% reduction in seizure rates, six patients had at least 25% seizure rate reduction, and one patient did not respond. No patient had any serious adverse effects. Side effects included sedation in six children (40%) and drooling in nine patients (60%).
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PMID:Nitrazepam for the treatment of Lennox-Gastaut syndrome. 1265 14

The long-term effects of vagus nerve stimulation (VNS) on seizure frequency were studied in 30 patients with Lennox-Gastaut syndrome. Median observation time was 52 months (17-123). The effect parameters investigated were total number of seizures and different seizure types. The median reduction in number of seizures was 60.6%. The effects of VNS varied considerably between seizure types. Best effects were observed with atonic seizures (80.8% median reduction, number of responders: 8/12), followed closely by tonic seizures (73.3% median reduction, number of responders: 8/13). Least effects were with generalized tonic-clonic seizures (median reduction 57.4%, number of responders: 11/20). Additional positive effects included milder or shorter ictal or postictal phase in 16 patients. Improved alertness was reported in 76.7%. Adverse effects, of which drooling and voice alteration were most frequent, were reported in 20 patients. There was a statistically significant reduction in the median number of antiepileptic drugs used. The discontinuation rate was 16.7%.
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PMID:Long-term vagus nerve stimulation in the treatment of Lennox-Gastaut syndrome. 1969 54