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Published on: September 17, 2014
A Case of Moyamoya Vasculopathy Presenting as Simultaneous Ischemic and Hemorrhagic Strokes
Wayne Zhong1, Amit Mehta1,2, Nicholas Haberli1
1Department of Neurology, Yale University School of Medicine, New Haven, CT, USA.
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.
Insights
Moyamoya disease (MMD) is a rare condition causing stroke due to narrowed arteries. This case highlights a young woman with MMD, emphasizing the need for better diagnostic and treatment strategies for this complex cerebrovascular disorder.
Area of Science:
- Neurology
- Vascular Neurology
- Neuroscience
Background:
- Moyamoya disease (MMD) is a rare, progressive condition causing stenosis in the internal carotid arteries.
- MMD leads to ischemic and hemorrhagic strokes, with no definitive curative treatment available.
- Early diagnosis and intervention are crucial for managing MMD, especially given its bimodal age distribution.
Observation:
- A 23-year-old female presented with stroke symptoms including left arm weakness and thunderclap headache.
- The patient had recently started estrogen-containing birth control prior to symptom onset.
- Neuroimaging revealed simultaneous bilateral ischemic and hemorrhagic strokes, indicative of moyamoya vasculopathy.
Findings:
- The patient was diagnosed with idiopathic moyamoya disease after extensive workup ruled out secondary causes.
- No obvious precipitating factors were identified, complicating secondary prevention strategies.
- The case underscores the lack of clear guidelines for managing MMD, which presents with both ischemic and hemorrhagic strokes.
Implications:
- Further research is needed to understand MMD pathophysiology and develop targeted treatment modalities.
- Improved diagnostic and therapeutic strategies are essential for managing MMD patients.
- This case emphasizes the complexity of MMD and the need for enhanced clinical guidance.
