There have been few case reports of perioral myoclonia with absences (PUMA) because of the lack of video-polygraphic recordings clarifying the electroencephalogram (EEG)-electromyogram (EMG) correlations. We describe one of the first video-polygraphic studies of PUMA in a patient who underwent repeated and prolonged split-screen video-polygraphic recordings. The ictal EEG showed generalized and irregular discharges of spikes or multiple spikes and slow waves, while two concomitant EMG patterns appeared: (1) a rhythmic enhancement of the orbicularis oris and masseter muscles on both sides with minimal asymmetry corresponding to perioral movements, and (2) a progressive increment in muscular tone in the mylohyoideus muscle corresponding to oroalimentary automatisms. Myoclonic jerks were inconstantly time-locked to the spike component of the spike-wave complex. The evidence of a complex pattern of activation of the facial muscles suggests that the involvement of subcortical central pattern generators, related to masticatory activity, through the disinhibitory effect of the spike-wave discharge is a possible pathophysiological mechanism underlying POMA. (C) 2011 Elsevier Inc. All rights reserved.
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Hosp Pediat Prof Dr Juan P Garrahan, Dept Neurol, Buenos Aires, DF, ArgentinaSapienza Univ Rome, Dept Human Neurosci, Rome, Italy
Caraballo, Roberto H.
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Giuliano, Loretta
Yilmaz, Tulay
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Istanbul Univ, Istanbul Fac Med, Dept Neurol, Istanbul, Turkey
Istanbul Univ, Dept Clin Neurophysiol, Istanbul Fac Med, Istanbul, TurkeySapienza Univ Rome, Dept Human Neurosci, Rome, Italy
Yilmaz, Tulay
Morano, Alessandra
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Sapienza Univ Rome, Dept Human Neurosci, Rome, ItalySapienza Univ Rome, Dept Human Neurosci, Rome, Italy
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Univ Utrecht, Univ Med Ctr, Dept Neurosurg & Epilepsy, Utrecht, NetherlandsSapienza Univ Rome, Dept Human Neurosci, Rome, Italy
Trenite, Dorothee G. A. Kasteleijn-Nolst
Gambardella, Antonio
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Magna Graecia Univ Catanzaro, Inst Neurol, Catanzaro, ItalySapienza Univ Rome, Dept Human Neurosci, Rome, Italy
Gambardella, Antonio
Baykan, Betul
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Istanbul Univ, Istanbul Fac Med, Dept Neurol, Istanbul, Turkey
Istanbul Univ, Dept Clin Neurophysiol, Istanbul Fac Med, Istanbul, TurkeySapienza Univ Rome, Dept Human Neurosci, Rome, Italy