Abeer Yousaf Ali Matter, Aalia Akhtar Hayat


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ABSTRACT

The syndrome of malignant migrating partial seizures in infancy was first described by Coppola and colleagues in 1995. The International League Against Epilepsy defines this form of epilepsy as a seizure onset in the first 6 months of life, occurrence of almost continuous migrating polymorphous focal seizures, combined with multifocal ictal EEG discharges, and progressive deterioration of psychomotor development. Most cases are pharmacoresistant and have poor outcomes. A lot of publications described the trial of several medications such as Stiripentol, Rufinamide, Cannabidiol, and finally Ketogenic diet, to control the refractory devastating seizures. We describe a 13-month-old girl with malignant migrating partial seizures in infancy who was started on Quinine for the control of her refractory seizures after the trial of multiple antiepileptic medications that failed to control her seizures, including Clonazepam, Carbamazepine, Phenobarbitone, Phyntion, Midazolam, Valproate, Perampanel & Ketogenic diet, all were tried by different combination at different times. Finally, as malignant migrating partial seizures in infancy are sometimes linked to K channelopathy, a trial of Quinine was given in a dose of 30mg/kg/d. Patients showed an excellent response with control of clinical & electrographic seizures. Now she is seizure-free for five months and undergoing physiotherapy. She started rolling over but doesn't have much improvement in motor milestones, is not following or cooing, and is unable to say clear words.

Keywords: MMPSI – malignant migrating partial seizures in infancy- Quinine – Intractable epilepsy- CPLANE-1 gene defect



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