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An institutional experience of 26 patients with Moyamoya disease: A study from Northwest India
Trilochan Srivastava1, Raghavendra Bakki Sannegowda1, R S Mittal2
1Department of Neurology, Sawai Man Singh Medical College Hospital, Jaipur, Rajasthan, India.
Aim:
Moyamoya disease (MMD) is a slowly progressive bilateral stenocclusive process of the distal internal carotid and proximal portions of the anterior and middle cerebral arteries and the formation of an abnormal vascular network at the base of the brain. The purpose of this retrospective study was to identify clinical features, salient features, radiological features and yield of diagnostic cerebral angiography in MMD.
Materials And Methods:
We analyzed the records of 26 patients with MMD evaluated and treated at our institute from August 2010 until March 2013. Diagnosis of MMD was made on the basis of features of angiographic findings. Cerebral angiography showed typically fine network of vessels at the base of the brain with puff of smoke appearance suggestive of MMD. CT angiography (CTA) was done in 25 (96.15%) patients where as Digital substraction angiography (DSA) was done in 18 (69.23%) patients.
Results:
Out of the 26 patients 13 were in the pediatric age group and 13 were adults. At presentation 14 patients had infarcts and 10 patients had hemorrhages. Among the hemorrhagic group 20% had isolated intracerebral hemorrhage (ICH), 50% patients had ICH with intraventricular extension (IVE) and 30% patients had primary intraventricular hemorrhage (PIVH). 50 % of the patients had involvement of the posterior circulation.
Conclusion:
Posterior circulation involvement is frequent in MMD. Though parenchymal bleed with/without intraventricular extension is the usual presentation of hemorrhagic MMD, isolated intraventricular hemorrhage could also be the mode of presentation.
Insights
Moyamoya disease (MMD) frequently affects posterior circulation. Hemorrhagic MMD often presents with intracerebral hemorrhage, but isolated intraventricular hemorrhage is also a possible presentation.
Area of Science:
- Neurology
- Vascular Neurology
- Neuroimaging
Background:
- Moyamoya disease (MMD) is a progressive stenosis of major cerebral arteries.
- It leads to the formation of abnormal blood vessels at the brain's base.
- Diagnosis relies on characteristic angiographic findings.
Purpose of the Study:
- To identify clinical, radiological, and salient features of MMD.
- To evaluate the diagnostic yield of cerebral angiography in MMD.
- To analyze patient demographics and presentation patterns.
Main Methods:
- Retrospective analysis of 26 MMD patients (August 2010-March 2013).
- Diagnosis confirmed by angiographic features, including "puff of smoke" appearance.
- CT angiography (CTA) used in 96.15%, Digital Subtraction Angiography (DSA) in 69.23%.
Main Results:
- Equal distribution between pediatric (13) and adult (13) patients.
- 14 patients presented with infarcts, 10 with hemorrhages.
- Hemorrhagic presentations included ICH (20%), ICH with IVE (50%), and PIVH (30%).
- 50% of patients showed posterior circulation involvement.
Conclusions:
- Posterior circulation involvement is common in MMD.
- While parenchymal or intraventricular bleeds are typical, isolated intraventricular hemorrhage can occur.
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