Panayiotopoulos syndrome with convulsive status epilepticus at the onset: a long-term study

Area of Science:

• Pediatric Neurology
• Epilepsy Research
• Childhood Neurological Disorders

Background:

• Panayiotopoulos syndrome (PS) is a common, benign childhood epilepsy syndrome.
• Convulsive status epilepticus (CSE) is a neurological emergency.
• The relationship between CSE and PS, particularly at onset, requires further definition.

Purpose of the Study:

• To define convulsive status epilepticus (CSE) as a potential manifestation of Panayiotopoulos syndrome (PS).
• To evaluate the prognostic significance of CSE in children with PS.
• To clarify the clinical presentation and outcomes of PS with initial CSE.

Main Methods:

• Retrospective cohort study of children diagnosed with PS between 1993 and 2012.
• Identification of patients with CSE at PS onset and comparison with PS patients without CSE.
• Clinical follow-up and EEG analysis to assess outcomes and prognostic factors.

Main Results:

• 37 children with CSE at PS onset were identified, compared to 72 without CSE.
• The mean age for the first CSE episode was 6.5 years; generalized clonic seizures were most common.
• Excellent overall prognosis with all patients seizure-free at follow-up, despite initial CSE severity in some.

Conclusions:

• Convulsive status epilepticus is an infrequent but recognized presentation of Panayiotopoulos syndrome.
• CSE at the onset of PS does not indicate a poor prognosis.
• PS with initial CSE is a benign condition with a favorable long-term outcome.