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Levetiracetam/valproic acid/zonisamide Lack of efficacy: case report

An approximately 12-month-old Indian boy exhibited lack of efficacy while being treated with levetiracetam, valproic acid and zonisamide for infantile onset epileptic encephalopathy (nonstructural West’s syndrome). The boy presented with a 2-week history of flexor spasms (infantile spasms) at the age of 7 months. He had a normal developmental milestones until 7 months of age; however, a loss of previously attained milestones (neuroregression) was noted in the last 2 weeks. He was hypotonic. Based on investigations, he was diagnosed with infantile onset epileptic encephalopathy (nonstructural West’s syndrome). Thereafter, exome sequencing of a multigene epilepsy panel indicated a ATP6V1A mutation c.416A>G (p.Lys139Arg). He received adrenocorticotropic hormone with cessation of spasms. However, the infantile spasms reappeared 5 months later. Therefore, he was treated with levetiracetam, zonisamide and valproic acid [dosages and routes not stated]. Although, his seizure frequency decreased by >50%, he continued to experience flexor spasms (infantile spasms) in clusters. The disease was thus considered to be refractory to these medications (lack of efficacy). Later, an EEG showed resolution of symptoms with slow background and multifocal epileptiform discharges. His development quotient was found to be