Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Mogadon as a parenteral solution was employed for treating status epilepticus (15 cases), intermitent epileptic seizures (10 cases), Lennox syndrome (14 cases), myoclonic seizures (5 cases) and absences (6 cases). The dosage layed between 3,3 mg and 10 mg according to each individual case. Status epilepticus and intermitent seizures disappeared following the injection and so did the irritative EEG alterations. Remission was observed in 10 Lennox cases while the remaining 4 improved. Mogadon abolished myoclonic seizures, in some cases beyond 6 hours. In simple absence improvement was also evident, although more shortlived than in myoclonic seizures. Sleepiness was side-effect, occurring in some cases.
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PMID:[Intravenous mogadon in epileptic patients. Clinico-electroencephalographic study]. 11 52

Neuropathological considerations were performed on a case, who went into Lennox syndrome after an acute encephalopathy at the infantile period, and moreover who fell into an akinetic-mute state derived from brain damage by herniation caused by a head injury and subsequent status epilepticus. Neuropahtological background in the present case of Lennox syndrome is thought to be based on the widespread unilateral cerebral lesions and the basal ganglional, especially thalamic, degenerations derived secondarily from the diffuse cerebral damage. The patient revealed akinetic mutism with the disappearance of the epileptic seizures and the desynchronization of the EEG's, when the brain lesions formed at the adult period spread over the opposite hemispheric limbic system and the brain stem tectum.
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PMID:Neuropathology of secondary generalized epilepsy--(Lennox-Gastaut syndrome)--a case report. 65 45

Convulsions or status epilepticus in 11 infants after pertussis vaccination are reported. In 3 cases grand mal epilepsy persisted and 2 children developed infantile epileptic encephalopathy (Lennox syndrome). On the basis of our own experience, the incidence of seizures approximates 1:4800 infants vaccinated or 1:12 800 vaccinations. According to a recent prospective study from the USA, the incidence of seizures may be closer to 1:600 infants. Since there is a significant difference between the incidence of spontaneous fits in children of the same age group and the incidence after vaccination, a causal relationship between the seizures and vaccination appears to be confirmed. The following conclusions are drawn from these observations: 1. In view of the usually benign course of whooping cough today, current vaccination against pertussis is hardly satisfactory. Improvement of the available vaccines is an urgent necessity. The protection should include the population most at risk, i.e. infants during the first few months of life. 2. Parents should be better informed about the risks involved in pertussis vaccination. 3. Booster inoculations should be abandoned. 4. Health authorities should decide whether the current pertussis vaccination program should be continued. 5. Complications following vaccination should be registered at a national centre.
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PMID:[Convulsions after whooping-cough vaccination]. 679 99

The purpose of this report is to describe the full clinical and electroencephalograpic spectrum of atypical benign partial epilepsy (ABPE) or pseudo-Lennox syndrome (PLS). We retrospectively analyzed the clinical and EEG data of 43 children with ABPE/PLS seen in our department during the last 25 years. Criteria for diagnosis of ABPE/PLS were the occurrence of generalized minor seizures as previously described for ABPE/PLS and the detection of focal sharp waves indistinguishable from those of rolandic epilepsy (RE) with generalization during slow sleep. Mental development prior to onset of epilepsy was retarded in 26% of patients. In 74%, age at onset of seizures ranged from 2-5 years of age. Atonic-astatic seizures, atypical absences, and more rarely, myoclonic seizures were the predominating seizure types in 67% of patients. Status of minor seizures occurred in 40%. One or several episodes of partial and secondarily generalized seizures were observed. Rolandic seizures occurred in 28%. The EEG was characterized by a normal or slightly slowed background activity and predominantly multi-focal sharp waves (88%) which were activated to a bioelectrical status in 56% during sleep. No tonic seizures and no fast > 10 Hz spike discharges were observed in any of the 1.291 EEG of the patients. Despite an often temporarily therapy-resistant course which led to use of steroids in 51% of patients, seizures had ceased in 84% and epileptic discharges had disappeared in 72% of patients at last follow-up. All patients older than age 15 were seizure-free. However, the rate of mentally handicapped patients had more than doubled from 26 to 56%. EEG which could be performed in 41 out of 56 siblings demonstrated shw in 40% of siblings in whom EEG were done at the age of maximum penetrance of sharp waves (3-10 years). ABPE/PLS overlaps broadly with RE, but also with electrical status epilepticus during sleep and Landau-Kleffner syndrome. Therefore, ABPE/PLS can be ranked alongside RE and other idiopathic partial epilepsies. The high incidence of sharp waves in siblings suggests that ABPE/PLS and RE have a common underlying genetic etiology.
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PMID:Atypical benign partial epilepsy/pseudo-Lennox syndrome. 1123 Dec 18

Seizure disorders of the rolandic region comprise a spectrum of different epilepsy syndromes ranging from benign rolandic epilepsy to more severe seizure disorders including atypical benign partial epilepsy/pseudo-Lennox syndrome,electrical status epilepticus during sleep, and Landau-Kleffner syndrome. Centrotemporal spikes are the unifying electroencephalographic hallmark of these benign focal epilepsies, indicating a pathophysiologic relationship between the various epilepsies arising from the rolandic region. The etiology of these epilepsies is elusive, but a genetic component is assumed given the heritability of the characteristic electrographic trait. Herein we report on three patients with intellectual disability, various dysmorphic features, and epilepsies involving the rolandic region, carrying previously undescribed deletions in 16p13. The only gene located in the critical region shared by all three patients is GRIN2A coding for the alpha-2 subunit of the neuronal N-methyl-D-aspartate(NMDA) receptor.
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PMID:Deletions in 16p13 including GRIN2A in patients with intellectual disability, various dysmorphic features, and seizure disorders of the rolandic region. 2038 27