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Pediatric Moyamoya Disease in Nepal and Challenges in a Resource-Limited Setting: A Case Report
Kapil Khanal1, Sunil Dhungana2, Bindu Gyawali2
1Maharajgunj Medical Campus (MMC), Institute of Medicine (IOM) Tribhuvan University (TU) Kathmandu Nepal.
Abstract:
Moyamoya disease (MMD) is a rare progressive cerebrovascular disorder characterized by stenosis of the terminal internal carotid arteries with fragile collateral vessel formation. It is common in East Asia but underreported in Nepal. Pediatric cases typically present with ischemic symptoms such as transient ischemic attacks, stroke, or seizures. We report a 7-year-old Nepali boy with 11 days of fever followed by recurrent tonic-clonic seizures, progressive right-sided weakness, and impaired fine motor function. Examination showed right hemiparesis with an upgoing plantar reflex. MRI and MR angiography revealed multiple acute infarcts with supraclinoid internal carotid artery stenosis and extensive collateral vessels, confirming MMD. Cerebrospinal fluid and autoimmune workup were unremarkable. The child was managed conservatively with aspirin and levetiracetam, leading to partial recovery. Surgery was deferred due to financial limitations and initial improvement. This case underscores the importance of considering MMD in pediatric strokes in non-endemic regions. In resource-limited settings like Nepal, conservative treatment offers symptomatic relief, but long-term follow-up is essential to monitor progression and surgical needs.
Insights
Moyamoya disease (MMD), a rare cerebrovascular disorder, was diagnosed in a Nepali child presenting with stroke-like symptoms. Conservative management provided partial recovery, highlighting MMD
Area of Science:
- Neurology
- Pediatric Neurology
- Vascular Neurology
Background:
- Moyamoya disease (MMD) is a rare, progressive cerebrovascular disorder.
- Characterized by internal carotid artery stenosis and fragile collateral vessels.
- Underreported in Nepal, though common in East Asia; pediatric cases present with ischemic symptoms.
Purpose of the Study:
- To report a pediatric case of Moyamoya disease in Nepal.
- To highlight diagnostic and management considerations in a resource-limited setting.
Main Methods:
- Case report of a 7-year-old boy with fever, seizures, and hemiparesis.
- Diagnostic workup included MRI and MR angiography.
- Conservative management with aspirin and levetiracetam was initiated.
Main Results:
- MRI/MRA confirmed Moyamoya disease with multiple infarcts and carotid stenosis.
- Cerebrospinal fluid and autoimmune markers were unremarkable.
- The patient showed partial recovery with conservative treatment; surgery was deferred.
Conclusions:
- Moyamoya disease should be considered in pediatric stroke cases, even in non-endemic regions.
- Conservative management can offer symptomatic relief in resource-limited settings like Nepal.
- Long-term follow-up is crucial for monitoring disease progression and surgical needs.
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