Expanding the clinical spectrum of the rare epilepsy syndrome of Benign Infantile Focal Epilepsy with Midline Spikes (BIMSE)

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Objective: NA Background: BIMSE is a rare epilepsy syndrome first described in 2000. The cardinal electrographic feature is of distinctive sleep-activated low amplitude grouped spikes followed by a bell-shaped slow wave occurring in a restricted field limited to the midline vertex. The epilepsy presents between 13 to 30 months of age in normal children. Stereotypic seizures, typically occurring in the wake state, consisting of motionless arrest, perioral cyanosis and stiffening over 1 to 5 minutes are described. Seizures are sporadic and clustering is not noted. All children outgrow the epilepsy Design/Methods: Case report Case Description: A 6-year-old normal girl presented at 32 months of age with a history of a prolonged seizure. The seizure began in sleep with repeated gurgling vocalizations, drooling and eyelid flickering. She vomited twice within the first few minutes of seizure onset. After 10 minutes, bilateral body jerking set in. The seizure lasted 60 minutes. Interictal EEG showed the unexpected finding of frequent sleep activated narrow based and very low amplitude spike and slow wave discharges in a limited midline central field suggestive of those described in BIMSE. She was started on Levetiracetam 250 mg BID after the seizure. There have been no further seizures. Conclusions: The seizure in our patient with midline spikes showed mixed characteristics of Panayiotopoulos syndrome (early vomiting, autonomic features. prolonged seizure duration) and childhood epilepsy with centrotemporal spikes (gurgling vocalization, drooling). This suggests an overlap of BIMSE with Panayiotopoulos syndrome (PS) and childhood epilepsy with centrotemporal spikes (CECTS). This contrasts with the literature which suggests BIMSE is a novel syndrome and not an early presentation of PS or CECTS. No reported cases of BIMSE have presented with similar semiology.




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