Abstract
Within generalized epilepsy, the syndrome of epilepsy with myoclonic absences is considered as intermediate between idiopathic and symptomatic forms. This syndrome is characterized by developing in childhood with a male predominance. Critical EEG shows paroxysms of PO at 3 Hz, and in the polygraphic recording rhythmic 3 Hz myoclonus is observed with a strict correspondence between EEG spike and myoclonus. The response to therapy is generally poor, and 18% develop generalized symptomatic epilepsy. We report 3 patients with epilepsy with myoclonic absences and good outcome, to emphasize the importance of a precise diagnosis by means of the polygraphic recording of the attack, the fact the association of sodium valproate and ethosuximide is the most useful therapy and, finally, the possibility that some patients with epilepsy with myoclonic absence may develop Janz's juvenile myoclonic epilepsy.
Highlights
Resistance to therapy and learning disabilities are stressed as frequent complications of the syndrome of "epilepsy with myoclonic absences" reported in 8 children, ages 6 to 16 years, seen at the University Hospital of Wales in a 10 year period
Febrile seizures had occurred in siblings of 3 patients. They could be precipitated by hyperventilation
The response to lamotrigine (LTG) reported by Wallace in this and a previous report is in contrast to the experience of Schlumberger E et al at the Hopital Saint Vincent de Paul, Paris, France. (Epilepsia March/April 1994;35:359-367)
Summary
Resistance to therapy and learning disabilities are stressed as frequent complications of the syndrome of "epilepsy with myoclonic absences" reported in 8 children , ages 6 to 16 years, seen at the University Hospital of Wales in a 10 year period. The mean age at onset was 4.9 years. Febrile seizures had occurred in siblings of 3 patients. Loss of awareness was associated with bilateral jerking of the head and upper limbs, and the EEG showed rhythmic 3 c/s spike-wave discharges.
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