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11/13. epilepsia partialis continua of childhood involving bilateral brain hemispheres.

    OBJECTIVES: epilepsia partialis continua (Kojewnikow's syndrome) can be classified into 2 groups. The 1st group had stable neurological deficit, and the 2nd group had slowly progressive neurological deficit. The latter usually manifests not tetraplegia, but hemiplegia. We describe 3 patients with epilepsia partialis continua, rapid neurological and mental deterioration, resulting in tetraplegia and serious mental deficits within 2 to 3 years from the onset. RESULTS: Their interictal EEGs showed progressive findings of deterioration, which resulted in an inactive pattern or a pattern reminiscent of suppression-burst within several years. Their cranial CTs revealed rapid progressive atrophy of both hemispheres. Various screening tests failed to confirm a metabolic disease, a degenerative disease, or an infectious disease in the central nervous system. CONCLUSION: Frequent status epilepticus might contribute to the bilateral brain involvement and the serious neurological and mental outcomes in young patients with epileptia partialis continua.
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keywords = status epilepticus, epilepticus, status
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12/13. Progressive cerebral degeneration of childhood with liver disease (Alpers Huttenlocher disease) with cytochrome oxidase deficiency presenting with epilepsia partialis continua as the first clinical manifestation.

    A previously healthy and normally developed 17-year-old young female presented with a sudden onset of focal motor seizure status that proved to be refractory to anticonvulsive treatment. Severe encephalopathy with visual impairment leading to blindness, mental deterioration, and predominantly left spastic tetraparesis developed progressively. Hepatic disease evolved 4 months after onset of the first symptoms and led to death in hepatic failure 1 month later. Diagnostic studies revealed an elevated protein and lactate in the cerebrospinal fluid, slow-wave and intermittently continuous spike-wave activity in the EEG, and a complex i.v. (cytochrome-C oxidase) deficiency in the muscle biopsy. MRI scans revealed signal abnormalities in the occipital lobe, thalamus, and basal ganglia only after 3 months. Histopathological findings in liver biopsy and in postmortem brain examination displaying widespread predominantly right cortical spongiosis, neuronal loss and astrocytosis were consistent with the clinically suspected diagnosis of progressive neuronal degeneration of childhood with liver disease (PNDC) or Alpers Huttenlocher disease. This rare disorder of unknown origin is usually seen in infants and young children and is rarely reported in adolescence.
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ranking = 0.0050192067975447
keywords = status
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13/13. Clobazam in partial status epilepticus.

    Clobazam is the first and only 1,5-benzodiazepine to be used in the management of epilepsy. The use and effectiveness of oral clobazam in patients with status epilepticus has only been previously described in one study of 16 cases, seven of whom were in complex partial status. We have used clobazam in four patients with EEG-proven partial status epilepticus refractory to standard antiepileptic drugs. In all cases, except one, the seizure activity was controlled within 2 hr of administering clobazam. Our four patients responded promptly to an oral loading of clobazam which was approximately twice the usual daily maintenance dose. The drug was well tolerated and no adverse effects were seen. Clobazam's effectiveness as a first-line agent remains to be studied. Further controlled studies are recommended.
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ranking = 54.178854882769
keywords = partial status epilepticus, partial status, complex partial status, status epilepticus, epilepticus, status
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