Prolonged Postictal Asystole in a Child with Focal Epilepsy and Cerebellar Atrophy

Background: Cardiac arrhythmias can be observed in the context of seizures. Seizure-induced asystole has been associated with focal epilepsies originating from the temporal and frontal lobe. We present a case with video recording during sleep EEG with postictal asystole and discuss the risk factors for SUDEP (sudden unexplained death in epilepsy).

Case Presentation: A 12.5-year-old girl was diagnosed with a focal epilepsy at the age of 6 years. Initial neurocognitive testing as well as MRI of the brain were normal. Under antiepileptic treatment, a seizure frequency of one to two per year was reported and regular follow-up EEGs were normal. Marked neurocognitive decline initiated follow-up investigations. Brain MRI revealed progressive cerebellar atrophy. Sleep-EEG recorded a secondary generalized seizure with left fronto-temporal origin lasting for 85 seconds followed by postictal asystole for 7 seconds. After an isolated QRS complex, asystole continued for another 27 seconds with a low voltage EEG trace. Another three isolated QRS complexes were noted over 14 seconds before regular heart beat reoccurred. Flat EEG trace lasted for 130 seconds. An interdisciplinary decision was taken and the patient admitted for transvenous single chamber pacemaker implantation.

Conclusion: The coexistence of cerebellar atrophy and fronto-temporal epilepsy increases the risk for SUDEP. Repetitive postictal asystole with prolonged EEG recovery time, together with progressive cerebellar atrophy might be responsible for the neurocognitive decline in this patient. Careful monitoring is mandatory to identify patients at risk for SUDEP. Decision for preventive pacemaker implantation should be taken individually for each patient.