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Published on: April 21, 2017
Pediatric Moyamoya disease
Mohamed Nagiub1, Iqbal Allarakhia
1Department of Pediatrics, St. John Providence Children's Hospital, Detroit, Michigan, U.S.A.
Insights
Moyamoya disease (MMD) is a rare cerebrovascular condition affecting children, particularly in East Asia. Early diagnosis through clinical evaluation and imaging, confirmed by conventional angiography, is crucial for effective management and surgical planning.
Area of Science:
- Neurology
- Vascular Medicine
- Pediatric Neurology
Background:
- Moyamoya disease (MMD) is a progressive cerebrovascular occlusive disease affecting bilateral internal carotid arteries.
- It causes a compensatory abnormal vascular network at the base of the brain.
- MMD is the most common pediatric cerebrovascular disease in East Asia, with lower incidence in the USA.
Purpose of the Study:
- To present a case of Moyamoya disease in a pediatric patient.
- To highlight diagnostic considerations for MMD.
Main Methods:
- Case report of a 7-year-old female presenting with transient altered mental status.
- Diagnostic evaluation included history, physical examination, and brain imaging.
- Conventional angiography was utilized for definitive diagnosis.
Main Results:
- The patient was diagnosed with Moyamoya disease.
- Conventional angiography confirmed the diagnosis and provided information for surgical planning.
Conclusions:
- A high index of suspicion based on clinical presentation and imaging is key for diagnosing Moyamoya disease.
- Conventional angiography is the gold standard for diagnosis and surgical planning in MMD patients.
Background:
Moyamoya disease (MMD) is a progressive cerebrovascular occlusive disease of the bilateral internal carotid arteries that leads to a compensatory abnormal vascular network at the base of the brain. Its average annual incidence 0.54 per 100,000 population but it is the most common pediatric cerebrovascular disease in East Asia. The reported incidence in USA is approximately 0.086 per 100,000 patients.
Case Report:
We present a case of Moyamoya disease that was to detected in a 7-year-old female who presented with transient altered mental status.
Conclusions:
Moyamoya disease can be diagnosed if history, physical exam and brain imaging is highly suspicious. Conventional angiography remains the gold standard for diagnosis and aids in surgical planning for patients with suspected Moyamoya disease.
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