A previously normal 12-year-old boy presented with 2 episodes of generalized tonic-clonic seizures in the past month. The parents also noticed brief episodes of twitching of the mouth for the last 2 years, which increased in frequency after treatment with oxcarbamazepine. Examination and neuroimaging were normal. The seizure semiology (see the video on the Neurology® Web site at www.neurology.org) and EEG (figure) were suggestive of perioral myoclonia with absences. Brief episodes of absences (2–9 seconds) with perioral myoclonia, infrequent generalized tonic-clonic seizures, and frequent occurrence of absence status epilepticus should suggest the diagnosis.1 The interictal EEG may reveal focal abnormalities that may lead to a misdiagnosis of a focal epilepsy. The seizures may be treatment-resistant and oxcarbamazepine must be avoided.2
(A) The interictal awake record shows bilateral frontal spike-wave discharges with generalization, with normal background. (B) The ictal record (semiology in the video) shows generalized 3-Hz spike-and-wave discharges lasting for 6 seconds with frequent irregularities in the number of spikes in the spike-wave complex and in the spike amplitude. The photic stimulation and hyperventilation did not reveal any additional abnormalities (not shown in the figure).
AUTHOR CONTRIBUTIONS
Drs. Sharma and Jain provided the clinical care to the patient under the supervision of Dr. Aneja. Drs. Jain and Sharma drafted the manuscript, which was read and approved by all the authors.
STUDY FUNDING
No targeted funding reported.
DISCLOSURE
The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.
Footnotes
Supplemental data at www.neurology.org
- © 2013 American Academy of Neurology
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