IS UNILATERAL MOYAMOYA DISEASE DIFFERENT FROM MOYAMOYA DISEASE

Whether a diagnosis of moyamoya disease is justified in patients with typical angiographic evidence of moyamoya disease unilaterally and nor mal angiographic findings contralaterally remains controversial. In th is study the authors analyzed longitudinal angiographic change, famili al occurrence, and basic fibroblast growth factor (bFGF) concentration in the cerebrospinal fluid (CSF) of patients with ''unilateral'' moya moya disease. Over a 10-year period, 10 cases of unilateral moyamoya d isease were followed using conventional angiography or magnetic resona nce angiography. Basic FGF in CSF, obtained from the subarachnoid spac e of the cerebral cortex during revascularization surgery, was measure d in five cases. Among the 10 cases of unilateral moyamoya disease, on ly one pediatric case showed obvious signs of progression to typical b ilateral disease. The other nine cases (including six adults and three children) remained stable throughout follow-up radiological examinati ons (magnetic resonance angiography) with a mean observation period of 3.5 years. There was no familial occurrence in these cases of unilate ral moyamoya disease. Levels of bFGF, which are high in typical moyamo ya disease, were low in these patients. The progression from unilatera l moyamoya disease to the typical bilateral form of the disease appear s to be infrequent. The low levels of bFGF in the CSF of these patient s and the lack of familial occurrence strongly suggest the most cases of unilateral moyamoya disease, especially those found in adults, are distinct from typical bilateral moyamoya disease.