A Case of Moyamoya Vasculopathy Presenting as Simultaneous Ischemic and Hemorrhagic Strokes

Abstract

Background

Moyamoya disease (MMD) is a rare pathological state characterized by progressive stenosis of the terminal portion of the internal carotid arteries (ICA). Complications include both ischemic and hemorrhagic strokes, for which there is no curative treatment for MMD. Early diagnosis with surgical intervention is vital for there is no definitive treatment. Due to the bimodal age distribution, moyamoya should be considered for patients presenting with stroke and supraclinoid ICA vasculopathy.

Case

We present a case of a 23-year-old female who presented with left arm weakness and sudden onset thunderclap headache. Upon further questioning, it was revealed that the patient had started an estrogen-containing birth control two weeks prior to presentation. Neuroimaging at our tertiary care center demonstrated simultaneous ischemic and hemorrhagic strokes in the bilateral hemispheres associated with vasculopathy seen in both invasive and noninvasive cerebrovascular imaging. She was diagnosed with idiopathic moyamoya disease since her serum and cerebrospinal fluid studies did not reveal any obvious precipitators to suggest moyamoya syndrome (MMS).

Conclusion

There were no obvious precipitating factors identified in the extensive workup for this patient. Therefore, further secondary prevention is difficult for this otherwise young and healthy individual. While there is data to support the use of antiplatelet medications for the prevention of ischemic stroke secondary to intracranial atherosclerotic disease, there are no clear guidelines for the treatment of MMD that simultaneously causes ischemic and hemorrhagic stroke. Further research on the pathophysiology and treatment modalities for MMD are needed to guide clinicians in treating this complex disease.