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Myoclonic absence seizure of unknown (idiopathic) cause (video-EEG photos) (Photo 1a)

An 11-year-old boy suffered from epilepsy with myoclonic absences of unknown (idiopathic) cause since the age of 12-months-old. These episodes were recorded during video-EEG recordings at the age of 11-years-old because he had 3 generalized tonic-clonic seizures. At the age of 12 months, he was noticed to have jerks in upper part of his body (head, upper limbs) mostly induced by noise at a frequency a few per week. These episodes were described as earthquake-like. At the age of 6-years-old he was reported to have about 10 episodes of vague looks concomitant with rhythmic jerking of head and upper limbs daily. These episodes had not responded to high doses of valproate, lamotrigine, clobazam, or levetiracetam. When ethosuximide was introduced to his treatment instead of lamotrigine absence episodes responded but he had for the first time 3 generalized tonic-clonic seizures during which his head and eyes turned left, twisted mouth, clonic-myoclonic movements of upper limbs for few minutes and subsequently did not respond for about 45 minutes. Neurologic assessment revealed no lateralizing signs and the MRI was negative. The episodes were controlled 80% combining valproate and ehtossuximide at optimal daily doses. The family and past history were noncontributory. (Contributed by Dr Athanasios Covanis.)

Associated Disorders

  • Epilepsy with myoclonic absences
  • Facial (perioral and or eyebrow) myoclonia with absences
  • Perioral myoclonia with absences