PURPOSE: To delineate a catastrophic @entity302 of unknown cause presenting with persistent intractable multifocal status. METHODS: Case note review. RESULTS: Six @entity1 aged 5 months to 6 years presented with focal @entity410 that progressed within days to intractable multifocal @entity410 with or without secondary generalisation, which recurred every few minutes and persisted for weeks. One developed @entity410 started. The two younger @entity1 showed mild @entity245 before onset but the others were normal. The @entity410 were unresponsive to all conventional anticonvulsants, @entity230 or @entity2764 and could only be controlled with doses of @entity4490 sufficient to cause electrical suppression. MRI scans were initially normal but later showed focal cortical @entity4 followed by generalised @entity194 . Two developed @entity382 , with a normal liver biopsy in one and @entity1560 in the other. No cause has been found even after neuropathological investigation. Three have died, two within 3 months of onset, while the three survivors have very severe @entity16 and continued @entity410 . CONCLUSION: The similarity of the clinical features suggests that this is a consistent clinical syndrome.

Idiopathic XXXX presenting with acute onset intractable status.

@entity1 :: ('9606', 'Species') :: ['children'] @entity410 :: ('MESH:D012640', 'Disease') :: ['seizures', 'impaired consciousness shortly before seizures'] @entity2764 :: ('MESH:D011736', 'Chemical') :: ['pyridoxine'] @entity1560 :: ('MESH:D005234', 'Disease') :: ['steatosis'] @entity4 :: ('MESH:D004487', 'Disease') :: ['swelling'] @entity16 :: ('MESH:D009422', 'Disease') :: ['neurological impairment'] @entity245 :: ('MESH:D002658', 'Disease') :: ['developmental delay'] @entity4490 :: ('MESH:D013874', 'Chemical') :: ['thiopentone'] @entity194 :: ('MESH:D001284', 'Disease') :: ['atrophy'] @entity302 :: ('MESH:D004827', 'Disease') :: ['childhood epileptic syndrome'] @entity382 :: ('MESH:D006529', 'Disease') :: ['hepatomegaly'] @entity230 :: ('MESH:D013256', 'Chemical') :: ['steroids']
@entity302 :: (MESH:D004827,Disease) :: ['catastrophic epileptic encephalopathy']
