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)

Early-onset benign childhood occipital seizures (EBOS) described by Panayiotopoulos constitute the commoner after the rolandic phenotype of a childhood seizure susceptibility syndrome. EBOS are the clinical representative of occipital spikes. Their cardinal features are infrequent (often single) partial seizures manifested with deviation of the eyes and vomiting, frequently evolving to hemi- or generalized convulsions. Ictal behavioral changes, irritability, pallor, and rarely cyanosis, and eyes wide open are frequent. Retching, coughing, aphemia, oropharyngolaryngeal movements, and incontinence may occur. Consciousness is usually impaired or lost, either from the onset or the course of the fits, but in a few children, it may be preserved. Duration varies from a few minutes to hours (partial status epilepticus). Seizures are usually nocturnal, but semiology is similar in nocturnal or diurnal fits. Onset is between 1 and 12 years with a peak at 5 years. One third of children have a single seizure, the median total number of fits is two to three, and the prognosis is invariably excellent, with remission usually occurring within 1 year from onset. A few children may later develop rolandic or other benign partial seizures. The likelihood to have seizures after age 12 years is exceptional and rarer than that of febrile convulsions. EEG shows occipital paroxysms demonstrating fixation-off sensitivity, but random occipital spikes, occipital spikes in sleep EEG alone, or normal EEG may occur. Centrotemporal and other spike foci may appear in the same or more frequently in subsequent EEGs. The EEG does not reflect clinical course and severity.
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PMID:Early-onset benign childhood occipital seizure susceptibility syndrome: a syndrome to recognize. 1038 32

Centrotemporal spikes are the EEG marker of Rolandic epilepsy, while ictus emeticus is one of the main seizure manifestations of Panayiotopoulos syndrome. Ictus emeticus has not been reported in Rolandic epilepsy. Out of a population of 1340 children with focal afebrile seizures we studied 24 children who had emetic manifestations in at least one seizure and centrotemporal spikes in at least one EEG. They were of normal neurological status and had a follow-up of at least two years after the last seizure. All children had sleep EEG following sleep deprivation. Two groups of patients were identified. Group A (12 patients) with EEG centrotemporal spikes only and group B (12 patients) with centrotemporal spikes and spikes in other locations. In 21 patients, ictal emetic manifestations culminated in vomiting and in three only nausea or retching occurred. The commonest presentation was ictus emeticus at onset followed by deviation of the eyes or staring, loss of contact and floppiness. In 79%, seizures occurred during sleep. Autonomic status epilepticus occurred in 37.5%. The mean age at onset was 5.3 years. Overall analysis of the clinical and EEG data points out that the vast majority of these patients primarily suffer from Panayiotopoulos syndrome. Twenty patients (83%) had ictal semiology typical of Panayiotopoulos syndrome, but five also had concurrent Rolandic symptoms and four later developed pure Rolandic seizures. The other four patients (17%) had typical Rolandic seizures with concurrent ictus emeticus. These findings suggest a link between Rolandic epilepsy and Panayiotopoulos syndrome, the two most important phenotypes of the benign childhood seizure susceptibility syndrome.
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PMID:Children with Rolandic spikes and ictal vomiting: Rolandic epilepsy or Panayiotopoulos syndrome? 1468 48