Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 22-year-old woman with the Lennox-Gastaut syndrome developed serial apnoeic-tonic seizures with concomitant bursts of repetitive spikes at 16-20/sec, after receiving therapeutic doses of chlormethiazole, clonazepam, and diazepam for the treatment of serial tonic-clonic generalized seizures. There was a direct relationship between dose of chlormethiazole and frequency of apnoeic-tonic seizures. Since these tonic seizures have been noted during natural sleep and after sleep induced by various sedative drugs in patients with slow spike-wave in EEG, it is likely that the reduction in level of consciousness induced by sedative drugs is the causative factor.
 ...
PMID:Anticonvulsant-induced status epilepticus in Lennox-Gastaut syndrome. 678 86

Twenty-two cases with the diffuse slow spike-wave, whose onset of clinical symptoms was at the age of seven or later, were examined. Half of them presented symptoms pertinent to the Lennox-Gastaut syndrome. The other half were cases of absence, psychomotor seizure or grand mal. The former group was distinguished from the latter in that it had worse prognoses concerning the seizure and intelligence, and discussions were held to see if it corresponds to the "Lennox-Gastaut syndrome of late onset" (Lipinski). Atypical absence and atonic seizure were the commonest symptoms in this group, and they, preceded by other types of seizures such as grand mal, developed mostly in adolescence, and their appearance was closely followed by that of the slow spike-wave on the EEG.
 ...
PMID:Epileptic cases of late onset with the diffuse slow spike-wave. 679 73

Seven children with an unusual epileptic syndrome are reported. The main clinical features in each patient included onset between 2 1/2 and six years of age; the occurrence of several types of seizure, especially partial motor fits, atypical absences and myo-atonic seizures; and the persistence of normal neurological and mental function throughout the course. The EEG picture was characterized by a striking contrast between waking records, which usually displayed focal paroxysms, and sleep tracings which showed an almost continuous, diffuse, slow spike-wave activity. Although the electroclinical features suggested the diagnosis of Lennox-Gastaut syndrome or myoclonic epilepsy, the seizures remitted spontaneously in the five oldest patients and may well do so in the two youngest ones. The authors discuss the clinical and electroencephalographic features that permit these cases of atypical benign partial epilepsy to be distinguished from the more severe myo-atonic epileptic syndromes of childhood.
 ...
PMID:Atypical benign partial epilepsy of childhood. 680 33

Complex and mixed atypical absences and tonic epileptic seizures of 12 patients with the Lennox-Gastaut syndrome with onset after age 6 were studied. By complexes seizures we mean the associations of two elementary epileptics phenomena and by mixed that of three or more. Complex and mixed epileptic seizures already reported for patients with the early form of the syndrome were identified. The seizures reported, but not classified, in the literature were named by us as follows: gestural and mimic tonic-atonic automatic, tonic-gelastic, atypical absence with atonic cervical-cephalic seizures. New forms were described and classified. The following terminology was proposed for the convulsive seizures: procursive tonic-automatic, and procursive hemitonic-automatic. The non convulsive seizures--atypical absences--were described also in terms of critical EEG findings, and named as follows: automatic alimentary with palpebral myoclonic jerks; versive with palpebral myoclonic jerks; with palpebral myoclonic jerks and cervical and oculogyric atonic seizure; with intermittent cervical oculogyric atonic seizure and palpebral myoclonic jerks; with intermittent cervical atonic and oculogyric seizure; with palpebral myoclonic jerks and tonic seizure in cervical flexion. It was found that polymorphism of the complexes and mixed seizures is greatest for patients with the syndrome of early onset when compared with that with the syndrome of late onset.
 ...
PMID:[Tonic epileptic crisis and complex and mixed atypical absences in Lennox-Gastaut syndrome in patients over 6 years of age]. 682 Jun 30

Two patients with childhood epileptic encephalopathy were studied by positron emission tomography before and after corpus callosotomy. Preoperatively, both patients showed in the temporal lobe unilateral hypometabolism that is characteristic of interictal epileptic foci. Postoperatively, the first patient had no seizures by the time of scanning, and his temporal lobe metabolism was bilaterally symmetric. Seizure control in the second patient did not improve by the time of scanning, and unilateral temporal hypometabolism persisted. This finding suggests a temporal lobe focus in two patients with Lennox-Gastaut syndrome.
 ...
PMID:Positron emission tomography in two cases of childhood epileptic encephalopathy (Lennox-Gastaut syndrome). 698 84

Five patients with the Lennox-Gastaut syndrome who have shown generalized nonconvulsive seizures were presented. The seizure manifestations which occurred spontaneously were documented by simultaneous recording and analyzed in terms of clinical and electroencephalographic correlates. According to the diagnostic criteria of the International Classification, it was possible, on the one hand, to regard them as "atypical complex absences" in which the impairment of consciousness is accompanied by other symptoms, which tend to dominate the clinical picture. They were: hypotonic, hypertonic, myoclonic and akinetic components, respectively. On the other hand, if we give a special weight to the accompanying symptoms, it is entirely possible that they are at the same time diagnosed atonic, axial tonic, bilateral myoclonic and akinetic seizures. The initial impairment of consciousness is common to all the seizure manifestations, and the ictal and interictal EEG expressions are not of diagnostic significance. A question arises as to whether two different nomenclatures were arbitrarily given to a unique ictal manifestation or not as far as the generalized non-convulsive seizures were concerned.
 ...
PMID:A tautology in the classification of generalized non-convulsive epileptic seizures. 721 10

Some results of a follow-up study concerning 103 observations of chronic and transitory Lennox-Gastaut syndrome (SLG) are presented. The full-blown syndrome in the chronic SLG (mean duration 21 years and 2 months) is in 100% of the cases characterized by tonic seizures and 'complex absences', mental retardation and abnormal EEG with slow spike-waves and 10 c/sec recruiting bursts. Slow spike-waves are not the most specific EEG pattern of SLG with chronic outcome. The statement is discussed.
 ...
PMID:[Nosological limits of the Lennox-Gastaut syndrome (author's transl)]. 734 3

Immunological mechanisms have been implicated in the pathogenesis of epileptic seizures in some patients and in experimental animal models of epilepsy. A beneficial effect of high dose intravenous gammaglobulin (IVIG) has been demonstrated for some children with intractable epilepsy. In this study we treated 9 children ages 1.1-9.2 years (mean 5.0 years) with intractable epilepsy not responsive to conventional antiepileptic drugs (AEDs) and steroid therapy. Eight children had Lennox-Gastaut syndrome and 1 had complex partial seizures with secondary generalization. Each child received 3 doses of IVIG (200 mg/kg of polyvalent immunoglobulin) on Days 1, 15 and 36. Concomitant AEDs were not changed. Four children had complete remission, 3 had partial response with a more than 50% reduction in seizure frequency and 2 had no response. Onset of response varied from immediate to 7 months after the last injection. No toxicity was noted. Duration of remission was 9 months in 1 case. The other 3 cases have remained in remission to date with a follow up period of 22-26 months. We conclude that IVIG is a safe therapy which appears to be effective in some children with intractable seizures. Children with shorter duration of their seizure disorder (< 1 year) and relatively preserved cognitive function (IQ > 70) appear to have a more favorable response. Larger scale controlled trials are needed to determine the optimal timing and dosage, as well as to identify specific subgroups which may benefit most from IVIG treatment.
 ...
PMID:Intravenous high-dose gammaglobulins for intractable childhood epilepsy. 750 61

There are several new antiepileptic drugs undergoing extensive clinical investigation. Five new drugs--vigabatrin, lamotrigine, gabapentin, felbamate and oxcarbazepine--appear to be the most widely tested and promising agents. Vigabatrin is most effective in drug-resistant partial epilepsy. Vigabatrin is also effective in infantile spasms, but seems to have negative effects on myoclonic epilepsies and absence seizures. Lamotrigine and felbamate seem to be effective in partial epilepsy and in Lennox-Gastaut syndrome. In addition, lamotrigine and felbamate seem to have efficacy in idiopathic generalised epilepsies. Oxcarbazepine appears to be equally as effective as carbamazepine, but less toxic. Gabapentin has few adverse effects and has efficacy in some patients with drug-resistant partial epilepsy. Some of the new antiepileptic drugs modify excitatory or inhibitory amino acid transmission, but some of them may employ new, still unknown mechanisms of action. Depending on the mechanism of action, the therapeutic effectiveness of the antiepileptic drugs may differ in specific epileptic syndromes. Future antiepileptic drugs may thus give us the possibility to design rational polypharmacy for individual patients by combining agents with different spectra of effectiveness. Considering the goal of good tolerability in the development of the new antiepileptic drugs, polypharmacy with these agents is not expected to increase adverse effects significantly.
 ...
PMID:Place of newer antiepileptic drugs in the treatment of epilepsy. 751 Jun 9

Two sporadic cases of tuberous sclerosis presented with flexion spasms in a male and early intractable seizures evolving into a Lennox-Gastaut syndrome in a female. Early hypotonia and lack of substantial motor development are key features of the Rett syndrome, more easily overlooked than hand-wringing. Clumsy self-feeding and immature ambulation were the highest achievements in the second case now aged 36 years. Immaturity rather than degeneration, dementia, or assumed tissue destruction, is the capital feature of many disorders of early brain development leading to profound motor as well as mental retardation. Studying unusual clinical combinations is more likely to shed light on the underlying etiology than focusing on procrustean syndrome definitions.
 ...
PMID:Rett syndrome associated with tuberous sclerosis in a male and in a female: evidence for arrested motor and mental development. 751 Sep 33


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>