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Published on: September 25, 2009
Aplastic or twig-like middle cerebral artery and cardiogenic cerebral embolism mimicking moyamoya disease with RNF213
Hiroyasu Inoue1, Masahiro Oomura1, Yusuke Nishikawa2
1Department of Neurology, 38386Nagoya City University Graduate School of Medical Sciences, Aichi, Japan.
Abstract:
Progressive stenosis and occlusion of the bilateral internal carotid artery terminals and circle of Willis are typical features of Moyamoya disease. However, aplastic or twig-like middle cerebral artery (Ap/T-MCA)-wherein the unilateral main trunk of the middle cerebral artery (MCA) is not depicted, and a plexiform arterial network is formed-is similar to the findings of Moyamoya disease. Here, we describe a 78-year-old man who presented with mild right paralysis and aphasia. Magnetic resonance angiography (MRA) at admission did not show the bilateral MCAs. The findings were similar to those of Moyamoya disease, and his symptoms worsened after hospitalization. Endovascular treatment was performed, and the left MCA was completely recanalized. Later, paroxysmal atrial fibrillation was detected, and we finally determined that left MCA occlusion had occurred due to embolism. The right MCA was completely occluded at its origin, indicating an Ap/T-MCA. Embolic occlusion of the unilateral MCA and contralateral Ap/T-MCA made this case resemble Moyamoya disease in the acute stage. Even when chronic occlusion is suspected on MRA in acute cerebral infarction, endovascular treatment should be considered. Additionally, a heterotypic R4810K polymorphism was later found in the RNF213 gene. To our knowledge, this is the second report of Ap/T-MCA with the RNF213 gene polymorphism; however, their association remains unclear and requires further analyses.
Insights
A rare case of unilateral middle cerebral artery occlusion mimicking Moyamoya disease was treated with endovascular intervention. This case highlights the importance of considering endovascular treatment for acute cerebral infarction, even with suspected chronic occlusion.
Area of Science:
- Neurology
- Vascular Neurology
- Radiology
Background:
- Moyamoya disease is characterized by progressive stenosis of the internal carotid artery terminals and circle of Willis.
- Aplastic/twig-like middle cerebral artery (Ap/T-MCA) presents with an absent unilateral MCA trunk and a compensatory network of small vessels, mimicking Moyamoya disease findings.
Observation:
- A 78-year-old male presented with acute neurological deficits including right hemiparesis and aphasia.
- Magnetic resonance angiography revealed absent bilateral middle cerebral arteries, initially suggesting Moyamoya disease.
- Symptoms progressed during hospitalization, prompting endovascular intervention.
Findings:
- Endovascular treatment successfully recanalized the left middle cerebral artery, which was later identified as embolic occlusion secondary to paroxysmal atrial fibrillation.
- The right middle cerebral artery was occluded at its origin, consistent with Ap/T-MCA.
- The combination of unilateral embolic MCA occlusion and contralateral Ap/T-MCA created an acute presentation resembling Moyamoya disease.
Implications:
- This case underscores the critical role of endovascular treatment in acute cerebral infarction, even when chronic occlusion is suspected based on initial imaging.
- The presence of a heterotypic R4810K polymorphism in the RNF213 gene in a patient with Ap/T-MCA warrants further investigation into its potential association with cerebrovascular diseases.
- Early consideration of endovascular therapy can be crucial for improving outcomes in complex cerebrovascular presentations.
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