A two-year-old male child with Doose syndrome: An unusual entity

Abstract

Introduction: Myoclonic-astatic epilepsy of early childhood (MAE)/Doose syndrome (DS) is an infrequent entity causing myoclonic-astatic seizures. Case Report: A 2-year-old male child presenting with history of seizures from one year, inability to speak and choreiform movements. He had isolated speech delay with other quadrants being normal (speaks monosyllables at two years). Parents noticed brief jerky movements of both upper and lower limbs with increasing intensity over 2–3 months which became a daily occurrence. Also, the child had episodes of abrupt loss of contact with surroundings and prolonged staring (3–5 minutes) and few eye blinks. Subsequently, by 1.5 years, he started having episodes of sudden forward bending of head followed by frequent falls. Jerks either preceded or followed falls. Decreased attention and hyperactivity had started by one year of the illness. Electroencephalography depicted polysharp waves with maximum voltage of 180 μV and 3–4 Hz@ an interval of 20–30 seconds bilaterally. He responded to oral sodium valproate, levetiracetam, and ketogenic diet. Conclusion: We report a 2-year-old male child with Doose syndrome, a distinct yet rare entity.