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Myoclonic absence seizures with complex gestural automatisms
Kenneth A Myers1, Ingrid E Scheffer2
1Epilepsy Research Centre, Department of Medicine, The University of Melbourne, Austin Health, Heidelberg, Victoria, Australia.
Summary
Epilepsy with myoclonic absences, a rare generalized epilepsy, presents with unique seizures. Complex automatisms observed in these seizures may be misdiagnosed as focal epilepsy.
Area of Science:
- Neurology
- Epileptology
- Pediatric Neurology
Background:
- Epilepsy with myoclonic absences is a rare generalized epilepsy syndrome.
- Characterized by specific seizure types, it typically presents in childhood.
Observation:
- Two unrelated children with mild developmental impairment experienced onset of myoclonic absences at ages 3 and 8.
- Seizures involved bilateral 3 Hz myoclonic jerks with tonic upper limb abduction, lasting 10-60 seconds.
- Complex gestural automatisms, such as undressing or attempting to exit a vehicle, were observed during seizures.
Findings:
- Ictal electroencephalograms (EEGs) demonstrated 3 Hz generalized spike-wave discharges.
- The occurrence of complex automatisms in myoclonic absence seizures is a novel finding.
- Post-ictal recovery was immediate with no recollection of events.
Implications:
- The presence of complex automatisms can lead to misdiagnosis of epilepsy with myoclonic absences as focal seizures.
- This finding expands the clinical and EEG spectrum of generalized epilepsy syndromes.
- Highlights the importance of detailed seizure description and EEG analysis for accurate epilepsy diagnosis.
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