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Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nonconvulsive status epilepticus (NCSE) is much more common than is generally appreciated and is certainly underdiagnosed, but its long-term effects are largely undetermined and remain controversial. There is increasing experimental evidence that generalized convulsive status epilepticus produces lasting neuropathologic damage in the hippocampus, but experimental models often include provocation of status epilepticus (SE) by physical (e.g., electrical stimulation) and chemical (including excitotoxic) agents that may induce damage independent of the epileptiform discharges. Also, damage appears to be related to the intensity and duration of electrical stimulation. Such models usually include high-frequency discharges sustained over long periods, somewhat different from the electrical activity of typical human NCSE. Pathologic studies in humans pertain primarily to patients who have had generalized convulsive status epilepticus. Clinical studies of the effects of NCSE are mandatory, but conclusions are difficult to come by, in part because of diverse definitions of NCSE. An altered mental status is obligatory, but the pertinent EEG and medication response criteria are controversial. Response to medication can be delayed by many hours or even days. Absence SE appears to cause no lasting effects. Complex partial SE is less uniform. Most reported cases have returned to baseline neurologic function, but several well-described patients have had prolonged memory deficits. The significance of other deficits is difficult to interpret in light of concomitant vascular and other diseases causing neurologic dysfunction. Clinical series usually lack premorbid neurologic and neuropsychologic assessment. The few exceptions are complicated by preexisting mental retardation and other deficits, by the coexistence of progressive illness, by the later effects of recurrent seizures, and almost always by the confounding influence of anticonvulsant medications. Most morbidity appears attributable to the underlying illnesses rather than to the NCSE itself. It is possible that relatively infrequent cases of prolonged NCSE or those with the synergistic effect of concomitant systemic illness, focal lesions, or very rapid excitatory epileptiform discharges may suffer more long-lasting damage, but these observations are still preliminary. NCSE should be treated expeditiously because of the acute neurologic impairment of the patients, because of the attendant morbidity including physical injury, and because it may go on to generalized convulsions. There is reasonable concern about possible long-term effects, but permanent neurologic damage from NCSE has not yet been established as a mandate for urgent treatment.
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PMID:Evidence against permanent neurologic damage from nonconvulsive status epilepticus. 1047 5

Nonconvulsive status epilepticus (NCSE) is characterized by behavioral or cognitive change from baseline for at least 30 minutes with EEG evidence of seizures. Categorized into complex partial status epilepticus (with lateralized seizures), and generalized nonconvulsive status epilepticus (bilateral diffuse synchronous seizures), there is debate regarding the diagnosis and morbidity of NCSE. Because EEG is needed for diagnosis, only a high index of suspicion leads to a request for the study, whereas EEG is often unavailable after hours or on weekends. Furthermore, the cognitive changes during NCSE are often incorrectly ascribed to a postictal state, intoxication, psychogenic or psychotic states, and mental retardation. Regarding categorization, present classifications address EEG features but fail to distinguish among depths of coma. Deeply comatose patients (with coma etiologies that themselves carry poor prognoses) are mixed with lightly obtunded patients with no morbidity, confusing the prognosis. Thus, a classification that subsumes depth of coma, and possibly etiology, is sorely warranted. Regarding treatment, comatose NCSE patients treated with benzodiazepines may worsen, whereas generalized nonconvulsive status epilepticus patients may suffer iatrogenically from aggressive treatment (hypotension and respiratory depression) necessitating balancing the potential neurologic morbidity of NCSE against the possible morbidity of IV antiepileptic drugs. A high index of suspicion is needed to initiate EEG studies. Better stratification of level of consciousness will be needed to distinguish among morbidity due to underlying conditions, treatment, and the effects of status epilepticus, proper.
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PMID:Assessing the outcomes in patients with nonconvulsive status epilepticus: nonconvulsive status epilepticus is underdiagnosed, potentially overtreated, and confounded by comorbidity. 1047 7

The case of a 12-year-old girl with pyridoxine-dependent seizures is reported. She developed status epilepticus just after birth and ordinary antiepileptic drugs were administered without effect. Her seizures ceased only on the administration of pyridoxine. Status epilepticus associated with the withdrawal of pyridoxine occurred three times, and ceased only after the renewed administration of pyridoxine. She now has mental retardation and mild spastic diplegia. The reported cases of pyridoxine-dependent seizures usually have been neonates and infants. Older patients were not fully investigated, and so we have reviewed these cases of pyridoxine-dependent seizures. As known previously, pediatricians should not forget that pyridoxine should be continued for life.
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PMID:Pyridoxine-dependent seizures in an older child. 1051 45

Whether seizures are the direct cause of cognitive deterioration in epileptic children is undetermined. This retrospective study aimed to delineate a subgroup of pediatric patients with cognitive deterioration and refractory seizures in the absence of recognized causes for mental retardation. Of the 80 children identified as having mental retardation and refractory seizure disorder, seven (8.7%) had normal cognitive development until at least 1 year of age. Their metabolic status was normal. Five of them suffered repeated frequent partial seizures with onset in the first year of life and two had repeated episodes of status epilepticus. All seven had similar characteristics of early onset partial seizures, six of them had partial seizures secondarily generalized and one had complex partial seizures. The time of peak cognitive deterioration correlated with increases in seizure frequency during that period. Evaluation revealed a well-defined epileptic focus in the absence of neuroimaging abnormality except for hippocampal atrophy in the two children with complex partial seizures and a small vascular malformation in one child. Uncontrolled partial seizures in the first months of life may result in cognitive deterioration.
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PMID:Mental retardation subsequent to refractory partial seizures in infancy. 1076 31

Epileptic encephalopathy, a major neurologic disorder, leads to upper motor involvement and epilepsy. Its clinical expression varies according to cerebral maturity and etiology. Epilepsy manifests by spasms, partial seizures or myoclonia in the newborn, essentially by spasms in the infant, but also by myoclonia or sometimes by multifocal partial seizures. In the child, epileptic seizures have a more varied expression: atypical loss of contact, tonic or tonic-clonic seizures, myoclonia and sometimes status epilepticus. Inter-seizure electroencephalograms of patients of all ages show severe diffuse, constant anomalies. These epilepsies are associated with delayed development, mental retardation and behavioural disorders, requiring appropriate treatment as early as possible.
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PMID:[Epileptic encephalopathies]. 1088 99

Lennox-Gastaut syndrome (LGS) is one of the most severe types of childhood epilepsy. It is usually resistant to treatment and associated with mental retardation. To delineate the risk factors associated with the outcome of LGS, we evaluated, in a retrospective and multicentre study, the course of the disease, EEG tracings, and intellectual function in 101 patients. Inclusion criteria were the presence of tonic seizures as well as slow spike and wave complexes in the EEG. The average documented observation period was 16 years (range 4-31 years). Overall, the intellectual and neurological outcome was poor. At the last follow-up, 38% of the patients could not speak, 21% were unable to walk and only 4% were free of seizures. Four independent risk factors for severe mental retardation were identified by multivariate analysis. These were in a decreasing order of importance: nonconvulsive status epilepticus (NCSE), odds ratio (OR) 25.2, a previous diagnosis of West syndrome (OR 11.6), a symptomatic etiology of epilepsy (OR 9.5), and an early age at onset of epilepsy (OR 4.7). The results highlight the association between NCSE and the severity of mental retardation in patients with LGS; this association appears to be independent of symptomatic etiology. Our data provide an indirect evidence that, at least in some of the patients, NCSE is not only a concomitant feature, but also a cause of severe mental retardation.
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PMID:Nonconvulsive status epilepticus--a possible cause of mental retardation in patients with Lennox-Gastaut syndrome. 1107 Nov 39

The medical management of epilepsy in the multi-handicapped patient requires careful evaluation, classification, and pharmacologic treatment. It is estimated that 20-40% of patients with mental retardation and cerebral palsy have epilepsy. This review reports the clinical trial data and personal experience related to the use of newer AEDs in the chronic management of epilepsy syndromes in children and adults, as well as information available on the treatment of seizures in individuals with mental retardation and associated handicaps. Furthermore, clusters of seizures, prolonged seizures and status epilepticus are more commonly seen in the multiply handicapped and mentally retarded population and require special attention. The new antiepileptic drugs felbamate, gabapentin, lamotrigine, levetiracetam, oxcarbazepine, tiagabine, topiramate, vigabatrin and zonisamide show specific advantage in some multiply handicapped patients, be it for seizure control or medication tolerance. Furthermore, new modalities of treatment for prolonged seizures allow better efficacy both outside of hospital and within hospital facilities. The treatment of epilepsy in multi-handicapped and retarded adults and children has significantly advanced in the past few years, and much of this improvement can be attributed to improved knowledge and monitoring of new antiepileptic drugs. Conventional anticonvulsants remain first line therapy for most clinicians, but newer AEDs must broaden the therapeutic option and do allow improved therapy for some multiply handicapped patients.
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PMID:Treatment of epilepsy in the multiply handicapped. 1110 96

The classification of benign partial epilepsies and related conditions includes (besides rolandic epilepsy) atypical benign partial epilepsy, bioelectrical status epilepticus (ESES) and a variety of other syndromes. The broad overlap of the clinical and bioelectrical symptomatology might reflect a pathogenetic background common to these epilepsies. In order to understand the great phenotypic variability, the clinical symptomatology in 56 sibships with focal sharp waves of genetic origin was analyzed. A genetic determination was assumed if, in addition to the index case, at least one sibling or offspring revealed typical focal sharp waves. The 56 index-cases and their 61 sib/offspring/parents showed a broad spectrum of epileptic and non-epileptic conditions ranging from mild selective performance deficits to severe complex mental retardation, from neonatal seizures, febrile convulsions, and simple rolandic epilepsy to severe epilepsies with minor seizures or ESES. The different conditions are not disease entities but sets of variably weighted symptoms of a complex pathogenetic background, in which a genetic disposition to focal anomalies of brain function is of decisive importance. As can be demonstrated by the data, this genetic liability coincides with other widespread genetic traits, expressed in certain EEG patterns, as well as with lesional pathogenetic factors. The biological background of the genetic focal anomaly is currently unknown. The marked age dependence of the symptoms justifies the assumption of an hereditary impairment of brain maturation.
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PMID:The concept of hereditary impairment of brain maturation. 1123 Dec 24

L-2-Hydroxyglutaric aciduria (L-2-HGA) is a rare organic aciduria with a slowly progressive course regarding CNS involvement. We present a 13.5-year-old female patient who presented at the Emergency Department with a generalized status epilepticus, which promptly responded to intravenous phenytoin. CT and MRI demonstrated subcortical white matter alterations. The neurological examination revealed mild mental retardation, macrocephaly and ataxic gait with cerebellar signs. Repeated urinary organic acid analysis demonstrated increased excretion of 2-hydroxyglutaric acid which was of the L-configuration. The constellation of macrocephaly in a patient with mental retardation, cerebellar tract involvement and subcortical white matter signal alterations on MRI should alert the physician to the possibility of L-2-HGA. Although rare, epileptic seizures or even status epilepticus can be among the presenting symptoms in organic acidurias with a slow course, such as L-2-HGA.
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PMID:L-2-Hydroxyglutaric aciduria presenting as status epilepticus. 1137 7

Ring chromosome 20 mosaicism is associated with dysmorphic features, mental retardation, and intractable seizures, including recurrent episodes of nonconvulsive status epilepticus. The authors' findings in four children, all without dysmorphic features, indicate that mental deterioration and frequent subtle nocturnal frontal lobe seizures, associated with a characteristic EEG pattern, represent prominent additional clinical features not previously described in this syndrome. This emphasizes the importance of full-night video-EEG in children with frontal lobe seizures and cognitive deterioration.
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PMID:Ring chromosome 20 epilepsy syndrome in children: electroclinical features. 1191 29


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