The authors describe the clinical and radiological features in 3 @entity1 with a diffuse hemispheric @entity10053 ( @entity10053 ) presenting with severe @entity302 and a previously unreported and characteristic MR imaging appearance. The @entity10053 is a well-recognized cause of @entity1625 , usually with a very good response to resection. These @entity5 are usually intracortical, and most commonly arise in the @entity1315 . Radiologically they are usually sharply demarcated, and show little contrast enhancement. Three @entity1 (2 @entity1 and 1 @entity1 ) presented at 14, 17, and 22 months of age with @entity302 . The @entity410 were focal motor or complex focal. One @entity1 had @entity302 . The response to antiepileptic drug therapy was poor. Motor and cognitive development was delayed in all @entity1 . One @entity1 developed a severe @entity302 , with regression of @entity73 . Her electroencephalogram obtained at that time showed hypsarhythmia. Admission MR imaging showed a diffuse unilateral abnormality involving frontal, temporal, and parietal lobes with little or no mass effect. There was involvement of both gray and white matter, with a striking sparing of the internal capsule in spite of apparent @entity5 throughout the basal ganglia and thalamus. In 2 @entity1 there was prominent expansion of cortical gyri by @entity5 . In 1 @entity1 the initial radiological diagnosis was a @entity2500 . On subsequent review the radiological diagnosis was thought to be @entity11 in all @entity1 . The first @entity1 underwent 2 limited resections involving the temporal lobe. He has continued to have poorly controlled @entity410 and severe @entity73 . The other @entity1 had subtotal resection to the level of the internal capsule. One @entity1 is currently @entity410 free 24 months postsurgery, but remains @entity73 . The @entity1 in Case 3 is having some @entity410 3.5 years postsurgery and remains hemiplegic, but the regression has reversed and she is making steady developmental progress. The pathological specimens showed the typical features of a @entity10053 in all cases. This striking radiological pattern has not previously been described as a feature of a @entity10053 . Recognition of this radiological pattern in young @entity1 with @entity302 will allow early consideration for resection, which may lead to improved long-term cognitive outcome.

Diffuse hemispheric @entity10053 : a new radiological variant associated with early-onset severe XXXX .

@entity1 :: ('9606', 'Species') :: ['patient', 'child', 'children', 'girl', 'patients', 'boys'] @entity410 :: ('MESH:D012640', 'Disease') :: ['seizure', 'seizures'] @entity1625 :: ('MESH:D004828', 'Disease') :: ['focal epilepsy'] @entity5 :: ('MESH:D009369', 'Disease') :: ['tumor', 'tumors'] @entity73 :: ('MESH:D003072', 'Disease') :: ['behavioral and cognitive problems', 'cognitively impaired', 'motor and cognitive skills'] @entity2500 :: ('MESH:D020244', 'Disease') :: ['middle cerebral artery infarct'] @entity11 :: ('MESH:D005910', 'Disease') :: ['low-grade glioma'] @entity1315 :: ('MESH:D004833', 'Disease') :: ['temporal lobe or frontal lobes'] @entity302 :: ('MESH:D004827', 'Disease') :: ['epileptic encephalopathy', 'epileptic spasms', 'epileptic seizures', 'epilepsy'] @entity10053 :: ('MESH:D018302', 'Disease') :: ['DNET', 'dysembryoplastic neuroepithelial tumor']
@entity302 :: (MESH:D004827,Disease) :: ['epilepsy']
