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

A male infant with early infantile epileptic encephalopathy (EIEE) was reported. Tonic spasms in series appeared since 1 month after birth and EEG showed a typical suppression-burst pattern. The patient was treated with a high-dose pyridoxal phosphate and thyrotropin-releasing hormone (TRH), but seizures were not controlled. ACTH was administered and the seizures disappeared transiently. The seizures reappeared during tapering ACTH and apparent cerebral shrinkage followed the ACTH therapy. Then, the patient who evolved into West syndrome was treated with ketogenic diet. The seizures disappeared immediately and EEG findings were improved. It is suggested that the ketogenic diet should be tried early for the treatment of EIEE.
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PMID:[Effect of the ketogenic diet for West syndrome into which early infantile epileptic encephalopathy with suppression-burst was evolved]. 757 84

The records of twenty-six infants with both symptomatic infantile spasms and classic hypsarrhythmia were reviewed to determine the efficacy of various ACTH dosages and time of initiation of therapy. Mean age of infantile spasm onset was 6.4 months. Most patients (13) had sustained perinatal hypoxic-ischemic insults. Seventeen patients (65%) had complete cessation of spasms. Between these responders and the 9 nonresponders there was no difference in duration of spasms prior to treatment (2.6 and 2.0 months) or mean ACTH dose (87.4 and 84.5 U/m2, respectively). Infants treated with high-dose ACTH (> 100 U/m2) did not have an improved response rate. The most favorable outcomes were associated with spasm onset at > 8 months of age (all of whom were responders, regardless of dose) or when treatment was started within 1 month of onset of infantile spasms with > 80 U/m2 ACTH (88% responders). Infants treated more than 2 months after onset often did not respond (57%) regardless of dose. Nonresponders with spasm onset at < 4 months of age had the worst prognoses; all had poorly controlled seizures and regressed developmentally. Although all infants in the study were neurologically abnormal, development either improved or did not deteriorate in most responder infants following spasm resolution and one-half remained seizure free. Nonresponder infants continued to have infantile spasms or other seizure types. These data suggest that ACTH is valuable in the treatment of significantly impaired infants with symptomatic infantile spasms, but the most important determinants of outcome may be age of onset and rapidity of treatment rather than dosage.
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PMID:Therapeutic efficacy of ACTH in symptomatic infantile spasms with hypsarrhythmia. 760 53

In a retrospective study the results of therapy in 60 children with so-called benign partial epilepsies are reported. It has been shown that the assessment of the therapeutic effect has to include the EEG, especially in epilepsies with atypical course. Carbamazepine has no effect on the EEG, in epilepsies with atypical course (atypical benign partial epilepsy, Landau-Kleffner syndrome, epilepsy with continuous spikes and waves during slow sleep [CSWS]) carbamazepine usually has no effect either on the seizures or on the EEG, on the contrary, in some cases both may even get worse. In our experience, the drug of choice in all types of benign childhood epilepsy is sulthiame, if necessary in combination with clobazam. Other drugs previously administered, including carbamazepine, should be dropped quickly. If the treatment with sulthiame or sulthiame/clobazam in children with atypical course is not effective, ACTH-therapy should be considered as soon as possible. These results should be confirmed in a prospective randomized study.
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PMID:Treatment of "benign" partial epilepsies of childhood, including atypical forms. 779 51

In an add-on pilot study, a group of 15 children with cryptogenic and intractable West syndrome (3) and Lennox-Gastaut syndrome (12) received intravenous immunoglobulin (IVIg, 0.4 g/kg body weight per day for 5 consecutive days, followed by the same dose once every 2 weeks for 3 months). Five patients had been treated previously with ACTH without success. The reduction in clinical seizures averaged 70%. Electroencephalographic (EEG) recordings revealed a mean reduction in epileptic discharges of 40%. In all 15 patients, acceleration of EEG background activity occurred, and psychomotor development improved. Prior to IVIg administration, CSF examinations were normal. After IVIg administration, the serum total IgG concentration increased by an average of 76%, and the CSF IgG concentration by 44%. According to our data, IVIg crosses the blood-CSF barrier, and might be effective in the treatment of West syndrome and Lennox-Gastaut syndrome. We suggest it should be considered when other treatments, such as ACTH, have failed.
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PMID:High-dose intravenous immunoglobulin treatment in cryptogenic West and Lennox-Gastaut syndrome; an add-on study. 781 37

Although infantile spasms (IS) constitute a well-recognized epileptic syndrome, only recently did investigators propose that spasms be classified as a distinct seizure type, characterized by axial flexion/extension jerks in clusters. Five older children (aged 4.5-14.2 years) who underwent video-EEG monitoring in 1992 in our epilepsy program for intractable mixed seizure disorder (cryptogenic 1, symptomatic 4) demonstrated flexor and extensor spasms in clusters. Seizure onset was from birth to 1.33 years. All 5 had spasms during infancy that continued as the children aged. Ictal EEG during spasms showed a brief high-amplitude delta burst followed by diffuse background attenuation or diffuse background decrease with superimposed rhythmic beta or alpha activity. Multiple other seizure types were present. Interictal EEGs were markedly abnormal and demonstrated slowing, multifocal spikes, generalized slow spike-wave, and polyspike-wave. Two children with spasms were unsuccessfully treated with ACTH, and 3 underwent corpus callosotomy. We conclude that spasms occur in older children with intractable mixed seizure disorders and may persist beyond infancy.
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PMID:Epileptic spasms in older children: persistence beyond infancy. 782 Dec 72

ACTH has been used extensively for treatment of massive infantile spasms (MIS) and other intractable seizures. The mechanisms by which ACTH exerts anticonvulsant effects are unknown. ACTH is a neuropeptide with both endocrine and neuromodulatory properties; its efficacy against MIS could derive from intrinsic anticonvulsant properties or from hormonal effects, either directly or through glucocorticoids. We tested ACTH activity against exogenous corticotropin-releasing hormone (CRH)-induced seizures in the infant rat model. CRH was administered into the cerebral ventricles of 85 infant rats aged 5-13 days. ACTH was used either 20-60 min before CRH administration or "chronically" (pretreatment with four doses of ACTH every 6 h, before CRH administration). In a separate group of rat pups, we measured plasma corticosterone to ascertain ACTH availability. Administration of CRH, an age-specific endogenous convulsant, resulted in a prolonged series of seizures after 2- to 55-min latency. There was no difference in latency between controls (9.5 +/- 1.2 min) and ACTH-treated rats (12.4 +/- 2.8 min for combined acute and chronic groups). CRH-induced seizure duration (88.2 +/- 9 vs. 74.7 +/- 9.4 min) and severity of seizures was also unchanged by ACTH treatment. ACTH reached the circulation and caused significant increase in plasma glucocorticoids. ACTH does not block the convulsant action of exogenous CRH in infant rats. An alternative mechanism for the anticonvulsant effect of ACTH may be suppression of synthesis and secretion of an endogenous convulsant, i.e., CRH.
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PMID:ACTH does not control neonatal seizures induced by administration of exogenous corticotropin-releasing hormone. 782 Dec 75

After an uneventful birth and normal early milestones, Kate presented with infantile spasms at the age of seven months. Seizures terminated within two days of initiation of ACTH but her subsequent development was delayed. At two-and-a-half years of age she developed complex partial seizures that responded to carbamazepine monotherapy. The dosage was increased when she developed generalized tonic-clonic seizures and she is currently maintained on a maximal dose of a controlled release formulation. The EEG strongly supports the clinical diagnosis of complex partial seizures and an MRI shows a classical neuronal migration defect with gross band heterotopia throughout both cerebral hemispheres. She attends a school for children with moderate learning difficulties.
Seizure 1994 Dec
PMID:Case summary: Kate. 789 49

To elucidate the neurochemical mechanism of epilepsy, we investigated the role of neurotransmitter systems in the animal models of epilepsy, the mechanism of anticonvulsants and proconvulsants, the neurotransmitters in the CSF of children with epilepsy, and the new therapy for epilepsy. The main results are as follows. 1) In El mice, the increased activity of excitatory amino acids system in cortex and decreased activity of noradrenergic system in striatum and hippocampus were related to the increased seizure susceptibility. 2) GABA sensitivity was reduced in young DBA 2 J mice which are susceptible to audiogenic seizures. 3) Lower benzodiazepine receptor densities were found in hippocampus of 4 and 16 weeks-old tremor rats. GABA concentrations in the cerebral cortex and hippocampus of the tremor rats increased at 5 weeks-old and decreased at 15 weeks-old. These changes may be related to the absence-like seizures in tremor rats. 4) Anticonvulsant mechanism of ACTH may be due to antagonizing glutamate binding. Proconvulsant mechanism of thyroid hormone may be related to the decrease in number of cerebral cortical neuronal benzodiazepine receptors. Penicillin acts its proconvulsant effect through inhibiting GABA-gated chloride ion influx. 5) CSF GABA level in children with infantile spasms was lower than in controls. The combination of vitamin B6 and valproic acid is effective and safe therapy in the treatment of infantile spasms. Further investigations by the neurochemical approaches are necessary to understand the mechanisms of epilepsy and develop the new therapy.
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PMID:[Neurochemical approach to epilepsy]. 790 87

The authors systematically treated 94 patients with West syndrome using the same protocol of sodium valproate and steroids, starting with hydrocortisone (HC) orally for two weeks. If seizures stopped, HC was withdrawn; if they persisted, tetracosactrin (TA; synthetic ACTH) was administered for another two weeks then HC was slowly withdrawn. 90 per cent of the symptomatic cases were controlled by HC, the remainder by TA. 65 per cent of symptomatic cases were controlled by HC; this rose to 78 per cent if patients treated by HC then TA were included. At 31 months follow-up, the percentage of favourable results was 72 per cent for cryptogenic and 60 per cent for symptomatic cases. For the latter, best results were obtained in patients with periventricular leukomalacia, postnatal distress and porencephaly. Patients suffering from sequelae of full-term distress or encephalopathy of unknown aetiology were the most resistant.
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PMID:A simple, effective and well-tolerated treatment regime for West syndrome. 785 75

We report a patient who began to have clusters of seizures characterized by brief elevation of the right arm at 6 months of age. An interictal electroencephalogram (EEG) at 7 months revealed hypsarrhythmia without definite asymmetry. Simultaneous EEG and video recording disclosed that these focal spasms were associated with fast wave bursts superimposed on slow waves most markedly in the left centro-midtemporal region. The patient became seizure-free after synthetic ACTH therapy. The patient is developmentally normal at 3 years 5 months, but magnetic resonance imaging studies revealed findings suggestive of delayed myelination in the left frontal region. This patient is considered to have had an unusual variant of West syndrome associated with focal delayed myelination.
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PMID:Focal spasms in clusters, focal delayed myelination, and hypsarrhythmia: unusual variant of West syndrome. 798 93


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