Perioral myoclonia with absences (POMA)

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Perioral myoclonia with absences (POMA). This is a genetic generalized epilepsy (GGE). Fully referenced neurology checklist.

Definition

  • This is a genetic generalized epilepsy (GGE)
  • The onset is in late childhood or adolescence
  • Females are more frequently affected
  • It is characterized by Valproate-responsiveness

Clinical features

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Complications

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Electroencephalogram (EEG)

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Treatment

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References

  1. Rudrabhatla PK, Er S, Radhakrishnan A, Menon RN. Unmasking the entity of 'drug-resistant' perioral myoclonia with absences: the twitches, darts and domes! Epileptic Disord 2021; 23:313-324.
  2. Bourcy E, Leroy P, Dubru JM. Perioral myoclonia syndrome with absences: about 2 cases. Rev Med Liege 2013; 68:537-541.
  3. Gulati P, Jain P, Qashqari H, Bradbury L, Cooper P, Otsubo H, Whitney R. Peri-oral myoclonia with absences with multiple facial and upper body myoclonia: Overlap epilepsy syndrome. J Clin Neurosci 2018; 54:135-137. 
  4. Bilgiç B, Baykan B, Gürses C, Gökyiğit A. Perioral myoclonia with absence seizures: a rare epileptic syndrome. Epileptic Disord 2001; 3:23-27.
  5. Vrielynck P, Rostomashvili N, Degroote E, Ghariani S, van Rijckevorsel K. Perioral myoclonia with absences and myoclonic status aggravated by oxcarbazepine. Epileptic Disord 2011; 13:308-312. 
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