Methylmalonic acidemia
Mar. 15, 2024
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Myoclonic and myoclonic-atonic seizures in 6-year-old boy with epilepsy with myoclonic-atonic seizures (Video-EEG)
The background activity is normal, but there are frequent (at least every 10 seconds) 3 to 6 Hz generalized spike/polyspike-waves with anterior maximum; they are brief for 1 to 4 seconds. These are frequently associated with single jerks of mainly the shoulders but also, on other occasions, of the thumb or eyelids. The jerks occur simultaneously with the first or the second polyspike-wave complex of the discharges. Some jerks are followed by atonic attacks. The EEG also showed brief (shorter than 0.5 seconds) abortive 1.5 Hz generalized spike/polyspike-waves with anterior maximum and an alternating but not consistent side emphasis. There were no clinical manifestations. The paroxysmal discharges occurred with eyes opened and closed, spontaneously and during overbreathing. IPS did not evoke photoparoxysmal responses. (Courtesy of Dr. C P Panayiotopoulos 2010.)