It may also show the presence of abnormal collateral vessels in the basal ganglia that enhance after the injection of an intravenous contrast. However, these changes do not provide conclusive evidence of Moyamoya disease. Although, MRA is more specific than MRI in delineating the course and affection of intracranial vessels, its availaility is limited. Arteriography has traditionally been regarded as essential for definitive diagnosis of moyamoya disease. But MRI and MRA may soon substitute it as a diagnostic test.
Surgical intervention remains the mainstay of the treatment.
It is, therefore, important to be aware of typical as well as unusual presentations of this disease so that surgical intervention can be undertaken early, before irreversible damage occurs. RP collected the data, assisted in literature search and drafted the paper. SBB edited the draft. He will act as the guarantor for the paper. MNM performed the literature search, drafted the paper and assisted in editing the paper.