CC BY-NC-ND 4.0 · International Journal of Epilepsy 2017; 04(01): 090-093
DOI: 10.1016/j.ijep.2016.07.001
Case report
Thieme Medical and Scientific Publishers Private Ltd.

A case series of reflex myoclonic absence epilepsy of infancy – an age-dependent idiopathic generalized epileptic (IGE) syndrome – with good prognosis

Umesh Kalane
1  Department of Pediatrics, Deenanath Mangeshkar Hospital, Pune, India
,
Shilpa Kalane
1  Department of Pediatrics, Deenanath Mangeshkar Hospital, Pune, India
› Author Affiliations
Further Information

Publication History

Received: 29 November 2015

Accepted: 11 July 2016

Publication Date:
06 May 2018 (online)

  

Abstract

Background Myoclonic epilepsies in the infantile age have varied presentations including benign myoclonic epilepsy of infancy (MEI) on one side and Doose, west, Dravet syndrome as well as recognized syndromes on the severe spectrum on the other side. MEI is an idiopathic disorder characterized by spontaneous myoclonic attacks with onset in the first 2 years of life. Reflex myoclonic epilepsy of infancy (RMEI) has startle- and tectile-induced myoclonias and needs distinct consideration from MEI.

Results Hereby, a case series of 3 infants, started with reflex myoclonic absence seizures before 12 months of age, has been described. All the infants have unremarkable birth history, normal development, and examination. They started with myoclonic jerks involving torso and upper extremities and were associated with startle induced by unexpected sounds and tectile stimulation. All children were treated with valproate and were followed till date with minimum follow-up of 12 months and showed complete seizure control at low dose of valproate. At 6-month follow-up, all children were seizure-free with normal development.

Conclusion Reflex myoclonic absence epilepsy is an age-dependent idiopathic generalized epileptic (IGE) syndrome, with an apparently good prognosis. It needs to be recognized separately as new syndrome and should be differentiated from the other myoclonic epilepsies of same age.