[An autopsied case of dementia with lewy bodies presenting with hemispheric cerebral cortical atrophy with selective neuronal necrosis after status epilepticus].

We report on a 72-year-old-Japanese man with dementia with Lewy bodies (DLB) who presented with hemispheric cerebral cortical atrophy with selective neuronal necrosis after status epilepticus. His disease manifested with psychiatric symptoms, such as a "hot feeling" in the abdomen, at the age of 68 years. He was found to have hypochondriasis and anxiety disorder and was treated in the Department of Psychiatry. Parkinsonism gradually developed approximately 1 year later. Approximately 3 years after the onset of psychiatric symptoms, he suddenly experienced left hemiclonic seizures with consciousness disturbance, and he was admitted to our hospital. Status epilepticus occurred for several hours. His convulsive seizures were controlled by the administration of antiepileptic drugs (diazepam, phenytoin); however, he later showed prolonged disturbance of consciousness and left spastic hemiparesis. Gastrostomy was performed, but the patient died of aspiration pneumonia approximately 9 months after the onset of status epilepticus. Pathological examination showed widespread neuron loss with gliosis involving the entire right cerebral cortex, particularly in the frontotemporal lobe and hippocampus, and with patchy involvement of the contralateral cerebellum, especially in the cortex and dentate nucleus. These findings seemed to be consistent with a chronic stage of selective neuronal necrosis and secondary cerebellar degeneration due to diaschisis of the cerebral cortical involvement. Evidence of limbic-type DLB was also observed. We believe that selective neuronal necrosis associated with status epilepticus is an interesting neuro-pathological finding.