Researchers at St Thomas’ Hospital, London, and Leeds General Infirmary, UK studied prospectively the clinical features of syncope-like epileptic seizures (SLES) and their frequency in children aged 1 – 15 years with Panayiotopoulos syndrome (PS) who were referred for an EEG. PS was defined by the occurrence of at least one autonomic seizure (AS) in a neurodevelopmentally normal child and one EEG with focal spikes. PS was diagnosed in 33 of 394 consecutive children with at least one AS (8.4%). SLES occurred at least once in 17 of 33 PS cases (51.5%); 12 had SLES in all AS. Overall, 53 of 74 AS presented with SLES (71.6%); 25 occurred during the course of an AS (AS + SLES) and 28 were pure SLES without other autonomic or convulsive manifestations. Concurrent autonomic symptoms during AS + SLES included emesis, incontinence, mydriasis, miosis, and cardiorespiratory abnormalities. Pure SLES presented with sudden collapse without premonitory symptoms, while standing, sitting, lying down, or asleep; flaccidity and unresponsiveness persisted for several minutes, up to 1 hour. Recovery was spontaneous, rapid and complete. All children with follow-up >2 years are seizure-free.

A typical case report of PS describes a 5-year-old boy who at age 13 months, woke, vomited profusely, became unresponsive and floppy, and later that night, woke again, vomited, and collapsed. He remained flaccid and unresponsive for 1 hour, and had dilated nonreactive pupils. At age 20 months, he collapsed and was unresponsive and flaccid for 10 min. His last seizure occurred at age 28 months, he fell and remained unresponsive and flaccid for 20 min; cardiology exam was normal. [1]

COMMENT. A consensus view of Panayiotopoulos syndrome (PS) determined by an international consortium concludes that PS is a common idiopathic, benign seizure disorder of childhood that should be classified as an autonomic epilepsy, rather than an occipital epilepsy [2]. Recognized by the ILAE as a distinct clinical entity within the spectrum of benign focal epilepsies of childhood, PS is manifested electrographically with multiple interictal spikes. [3]

Autonomic epilepsy is frequently misdiagnosed and treated as atypical migraine, syncope, or abdominal disorder, leading to unnecessary and sometimes invasive investigations. The present UK study of PS concludes that in atypical cases of suspected syncope in children having focal spikes in the EEG, a diagnosis of syncope-like epileptic seizures in PS should be considered.