A Case of Hemiconvulsion-hemiplegia Syndrome in a 2-year-old Girl with Exanthema Subitum Who Experienced Prolonged Seizures Followed by Right Hemiparesis Featuring Non-convulsive Status Epilepticus
Department of Pediatrics, Graduate School of Medicine, Nippon Medical School
A 2-year-old girl was admitted to our hospital because of prolonged seizures (right hemiconvulsions) and high-grade fever. A diazepam suppository was ineffective in treating the seizures, because an acetaminophen suppository had already been administered in an attempt to control the fever; thus, both suppositories were ineffective. An intravenous injection of flunitrazepam finally inhibited the hemiconvulsions, which had lasted 70 minutes. Thereafter, no jerking was observed, but the right hemiparesis and depressed consciousness persisted. The fever abated on the 5th day, and rashes developed over the entire body surface on the 6th day. Electroencephalograms (EEGs) recorded on the 5th day showed continuous irregular sharp waves in the left frontal area, suggesting non-convulsive status epilepticus. A further intravenous injection of flunitrazepam attenuated the paroxysmal EEG discharges and revived her consciousness, but the right hemiparesis remained. Human herpes virus 6 (HHV-6) DNA was detected in a blood sample, so exanthema subitum was diagnosed. Hemiconvulsion-hemiplegia syndrome was also diagnosed, because diffusion weighted magnetic resonance imaging on the 15th day showed high intensity areas in the cortices of the left-hemisphere and in the subcortical white matter of the left occipital lobe. Exanthema subitum is a common infectious disease in infants, and accompanying febrile seizures are not unusual in those affected. However, when febrile seizures are accompanied by such symptoms as prolonged seizures, hemiconvulsions followed by hemiparesis, and persistent depressed consciousness, early EEG recordings should be made.
日医大医会誌 2010; 6(4), 185-189
prolonged seizures, non-convulsive status epilepticus (NCSE), exanthema subitum, hemiconvulsions-hemiplegia syndrome (HH syndrome), human herpes virus-6 (HHV-6)
Kentaro Kuwabara, Department of Pediatrics, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku Tokyo 113-8603, Japan