Clinical Reasoning: Refractory status epilepticus in a primigravida

Abstract

A 29-year-old woman, gravida 1 para 0, 14 weeks pregnant, presented with first seizure of life. Thirty minutes before the seizure, she reported a new mild bifrontal headache and visual changes described as difficulty seeing her right fingers when using her phone. She then had witnessed right arm extension, followed by shaking in bilateral arms and legs for 2 minutes, and bladder incontinence followed by 30 minutes of postictal confusion. Her medical history was notable for ataxia, myoclonus, and foot numbness diagnosed 4 years prior as sensory ataxic neuropathy, dysarthria, and ophthalmoplegia (SANDO) syndrome; whole exome sequencing had confirmed compound heterozygous polymerase gamma (POLG) mutations. Her home medications were amantadine 100 mg BID, coenzyme Q10 100 mg BID, and prenatal multivitamin daily.