Moyamoya Disease: A Rare Presentation and Literature Review

Moyamoya disease is a rare vasculopathy involving the vessels of the central nervous system, predominantly the internal carotid arteries. A 21-year-old female patient from the Murshidabad district of West Bengal presented to us with an altered sensorium. She was referred to us from another hospital in Kolkata, where she was diagnosed and being treated as a case of ischemic stroke 2 weeks prior to the presentation. There was a significant past history of excruciating headaches, which would be more at night, and did not have any other obvious aggravating or relieving factors. On examination, the plantar was bilaterally extensor, and the patient was afebrile with no signs of meningeal irritation. Neuroimaging with noncontrast computed tomography (CT) head revealed a large right-sided middle cerebral artery (MCA) territory infarct along with acute hemorrhage in the left basal ganglia region. Upon a thorough perusal of the course of treatment at the previous hospital, it was ascertained that there was no usage of antiplatelet or anticoagulant agents there. A magnetic resonance (MR) angiogram of the intracranial blood vessels was therefore ordered, which was suggestive of moyamoya disease. Therefore, it was concluded that the spontaneous and near-simultaneous occurrence of infarct and hemorrhage was due to the vascular anomalies induced by moyamoya disease, making this case an uncommon presentation of a very rare disease.