Moyamoya Syndrome Presenting as Refractory Status Epilepticus in a 32-Year-Old Female

Abstract

Moyamoya disease (MMD) is a rare cerebrovascular occlusion condition characterized by ‎progressive stenosis in the terminal portion of the internal carotid arteries on both sides. The measured incidence of MMD is 0.086 per 100,000 people. MMD has variable ‎neurological manifestations, however, seizure is a significant manifestation of MMD with ‎few reported studies. The combination of sickle cell disease (SCD) and MMD confers a much ‎higher risk of ischemic stroke. In this paper, we describe a 32-year-old female with a known ‎case of SCD taking tramadol for a vaso-occlusive crisis, who was presented to the emergency ‎department by a family member due to a low level of consciousness. Despite ongoing ‎management, the patient developed multiple seizure attacks and intubation was ‎performed. A computed tomography (CT) brain angiogram was performed, and the ‎diagnosis of MMD was made. The patient was shifted to the intensive care unit (ICU) and in spite of the ongoing management in the ICU, the patient died. In this case, we highlight the importance of considering MMD as a ‎differential diagnosis when dealing with an SCD patient who developed refractory status epilepticus.‎.