PANAYIOTOPOULOS SYNDROME

Overview

Panayiotopoulos syndrome is characterized by the onset in early childhood of focal autonomic seizures that are often prolonged. The EEG commonly shows high amplitude focal spikes and may be activated by sleep. Seizures are infrequent in most patients, with 25% only having a single seizure (which may be autonomic status epilepticus) and 50% having six seizures or less. Seizures are self-limiting with remission typically within a few years from onset.

NOTE Self-limiting refers to there being a high likelihood of seizures spontaneously remitting at a predictable age.

Clinical context

Panayiotopoulos syndrome is characterized by onset of seizures between 1 and 14 years of age (majority between 3 and 6 years). Seizures are infrequent in most patients, with 25% having a single seizure (which may be autonomic status epilepticus) and 50% having six seizures or less. Frequent seizures can occur in some patients. Seizures usually resolve by age 11-13 years. Both sexes are affected equally. Antecedent and birth history is normal. Head size and neurological examination are usually normal. Development and cognition are normal. However, during active seizure periods, subtle neuropsychological deficits in language and executive functioning have been reported. A history of febrile seizures is seen in 5-17% of patients.