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A case of perioral myoclonia with absences and its evolution in adulthood? Volume 20, numéro 3, June 2018

TEST YOURSELF

(1) What are the features of typical perioral myoclonia with absences (POMA)?

(2) What features make this case atypical of POMA?

(3) Describe the ictal EEG patterns of this case with regards to the clinical manifestations?

(4) Discuss the localizing value of the “chapeau de gendarme” sign with regards to seizures.

 

 

 

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Answers

(1) The rare syndrome of perioral myoclonia with absences (POMA) was first described by Panayiotopoulos et al. as a specific type of idiopathic generalized epilepsy in which absence seizures are accompanied by prominent perioral myoclonus as a consistent symptom.

(2) Firstly, consciousness was preserved during seizures, with no manifestation of absences. Secondly, regarding the EEG features, in some of the seizures, the perioral motor symptoms were tonic rather than myoclonic.

(3) Pattern 1: Fronto-centrally prominent, generalized, 3-4-Hz spikes/multiple spikes-and-slow-wave paroxysms, lasting 2-7 seconds. Pattern 2: trains of generalized, 10-12-Hz sharp-wave activity, lasting 1-3 seconds.

(4) The “chapeau de gendarme” sign confers a high localization value to the frontal lobe. However, here, it was observed in a patient with a generalized epilepsy syndrome.

 

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