Veerappan Neurology

[Efraine Ton]

A64-year-old Black male with a reported history of seizure disorder was brought to the ED by emergency medical services (EMS) for altered mental status. Prior to arrival to the ED the patient was found by paramedics with seizure activity, and midazolam 5 milligrams was administered intravenously, causing cessation of the tonic-clonic movements. The patient remained unresponsive during transport and was not intubated prior to arrival. He was altered on arrival to the ED. There was no family present with patient, no family contact information available, and no prior documentation in the electronic health record; therefore, no additional history was available.

The patient arrived nonverbal with minimal spontaneous movements of all extremities and was not consistently following commands. He was protecting his airway and maintained adequate oxygen saturations. Given these findings on arrival and that the patient was likely in a postictal period without sign of ongoing seizure and improvement in his Glasgow Coma Scale compared to the EMS report, the physicians decided to not intubate the patient on arrival and closely monitor his neurologic status. Initial vitals also revealed that he was afebrile but with an initial blood pressure of 84/52 millimeters of mercury (mmHg). A point-of-care ultrasound was performed to evaluate the hypotension and revealed no sites of bleeding and normal cardiac output. Intravenous fluids were administered and blood pressure quickly normalized. Electrocardiogram was unremarkable. On repeat neurological examinations, the patient continued to exhibit altered mental status, clinically inconsistent with a postictal state.

A computed tomography (CT) of the brain without contrast showed loss of gray/white matter differentiation on the right side, evidence of right middle cerebral artery (MCA) acute infarction. The on-call neurologist was emergently consulted. The patient was not eligible for tissue plasminogen activator. CT angiography with perfusion imaging was then emergently pursued and showed bilateral MCA infarction at the first segment (M1) with core to penumbra mismatches and no significant core seen on the left. Based on this imaging, the neurology team activated the large cerebral vessel occlusion protocol. The patient was subsequently taken for cerebral angiography, which revealed bilateral internal carotid artery stenosis. Additionally, it found evidence of stenosis of the right and left M1 sections but no thrombus. Extensive perforator collateral circulation was found, consistent with moyamoya disease (Image). No additional intervention was indicated, and the patient was admitted to the intensive care unit. He was eventually discharged to a rehabilitation facility with residual left-sided hemiparesis and dysphagia.

By maintaining clinical suspicion for alternate diagnoses as well as pursing continued diagnostic testing and patient reassessment, the rare finding of moyamoya disease, was identified. Moyamoya could be considered in adult patients presenting with acute seizures or strokes in addition to patients with altered mental status.

Conflicts of Interest: By the CPC-EM article submission agreement, all authors are required to disclose all affiliations, funding sources and financial or management relationships that could be perceived as potential sources of bias. The authors disclosed none.

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