Table 1 Diagnostic criteria of Moyamoya disease

RCMJ criteria: all of the following findings:

  Steno-occlusive lesions around the terminal portions of the internal carotid arteries (including the proximal portions of the anterior cerebral arteries and middle cerebral arteries)

  Moyamoya vessels at the base of the brain illustrated by abnormal vascular networks on conventional angiography or more than two flow voids in the basal ganglia on MRI

  Findings 1 and 2 are present bilaterally

  Known diseases with similar angiographic findings (i.e., arteriosclerosis, autoimmune disease, meningitis, brain neoplasm, Down syndrome, neurofibromatosis type 1, head trauma, irradiation to the head, protein C deficiency, protein S deficiency, and other diseases) should be ruled out

Broad classification: any steno-occlusive lesions that fulfill the following findings:

  Steno-occlusive lesions around the terminal portions of the internal carotid arteries

  Findings of moyamoya vessels may be absent

  Bilateral involvement is not essential

  Known diseases with similar angiographic findings should be ruled out