White matter T2 hyperintensity development and clinical deterioration after status epilepticus in a patient with dentatorubral-pallidoluysian atrophy.

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Serial T 2 -weighted magnetic resonance imaging in a 29 -year -old woman with juvenile type dentatorubral-pallidoluysian atrophy (DRPLA ) demonstrated that a cerebral white matter hyperintensity appeared within 2 months after status epilepticus and persisted for more than 20 months . The patient had rapidly progressive mental regression and became akinetic after status epilepticus . The chronological relationship between the signal changes and the clinical deterioration suggested that the epilepsy , at least in part , contributed to the progression of white matter degeneration , the hallmark of DRPLA .