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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2-3
pubmed:dateCreated
2009-4-20
pubmed:abstractText
In order to find an effective treatment option for status epilepticus in progressive myoclonus epilepsy (PME), we reviewed the clinical course of 9 patients with PME. Initially, epilepsy was successfully treated with antiepileptics. However, it gradually became refractory to medication, and status epilepticus emerged 3-19 years after the onset of epilepsy. In these patients, status epilepticus in PME was classified into (1) myoclonic status epilepticus (MSE), (2) myoclonic-generalized status epilepticus (MGSE), and (3) generalized status epilepticus (GSE). MSE was common in patients with neuronal ceroid lipofuscinosis, and GSE was common in those with dentatorubral-pallidoluysian atrophy. MGSE was characterized by the mixture of escalating myoclonus and generalized seizures, and was observed in patients with Gaucher disease or unspecified PME. All patients were often refractory to infusion of benzodiazepines and barbiturates but phenytoin was able to terminate status epilepticus in 7 patients. Oral phenytoin administration as preventive therapy was effective in 6 patients. Aggravation of myoclonus was not provoked by these treatments. We propose that phenytoin should be considered as a treatment choice for PME patients at late stages to prevent the detrimental effects of prolonged or repeated status epilepticus on the brain tissues.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Apr
pubmed:issn
1872-6844
pubmed:author
pubmed:issnType
Electronic
pubmed:volume
84
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
201-9
pubmed:meshHeading
pubmed:year
2009
pubmed:articleTitle
Reassessment of phenytoin for treatment of late stage progressive myoclonus epilepsy complicated with status epilepticus.
pubmed:affiliation
Department of Child Neurology, National Center Hospital of Neurology and Psychiatry, National Center of Neurology and Psychiatry, 4-1-1 Ogawahigashi-cho, Kodaira, Tokyo 187-8551, Japan.
pubmed:publicationType
Journal Article, Evaluation Studies