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Mother and daughter with eyelid myoclonia and absences

(A), (B), (C), (D), and (E) A 4.5-year-old girl was referred for a routine EEG by her pediatrician for immaturity and was discovered to have eyelid myoclonia. She was noted to have tonic eyelid contraction at eye closure each time she closed her eyes. At times, her eyelids would fling open and jerk, with a very transient absence. Her mother commented that she had been aware of this condition for the past 18 months, particularly when her daughter was watching television. The sleep-awake video-EEG showed irregular 3 to 3.5 Hz spike-and-wave and polyspike-and-wave discharges lasting 0.5 to 2 seconds on eye closure, with eyelid myoclonia and occasional jerks involving the head or upper part of her body. The photosensitivity range was 5 to 45 Hz, with concomitant clinical phenomena. Her mother, who was sitting next to her during monitoring, complained of dizziness and was taken to the next room where she had a generalized tonic-clonic seizure for the first time in her life. She was 28 years old. The mother’s video-EEG showed eyelid myoclonia on eye closure, with irregular spike-and-wave discharges. (F) She was photosensitive. (G) Sodium valproate was prescribed for both mother and daughter. The mother did not agree to start treatment, and her daughter’s compliance was reported as not good when she was last seen at the age of 15 years. She continued to have educational problems. (Contributed by Athanasios Covanis MD PhD.)

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Associated Disorders

  • (cryptogenic) and symptomatic generalized epilepsies
  • Jeavons syndrome
  • absence status epilepticus with eyelid myoclonia
  • eyelid myoclonia