Clinical and cerebral angiographic evolutions of idiopathic progressive occlusive disease of the circle of Willis ("moyamoya" disease) in children

Abstract

In 33 pediatric cases of idiopathic progressive occlusive disease of the circle of Willis (so-called moyamoya disease), clinical features and CAG findings were studied with emphasis on their evolutionary aspects. The subjects were clinically classified as follows; 23 cases of pure transient ischemic attacks (TIA), one presenting purely with infarct, one mixed TIA and epileptic, two mixed TIA + infarct, and 6 mixed epileptic and infarct. The core symptom of the TIA type is a recurrent unilateral or alternating unilateral paresis persisting for minutes or hours. Frequently hyperpnea provoked a TIA. Follow-up observation of the TIA type patients for an average of 6 yrs 11 mos shows that about half the subjects became completely free of symptoms and the other half mostly improved considerably. In contrast, all patients not presenting purely with TIA ("non-TIA" type) still had persistent neurological deficits after a mean interval of 6 yrs 4 mos from the onset. The CAG findings in most cases belonged to stage III according to Suzuki's classification. Therefore the authors subdivided stage III into 3 substages, IIIa, IIIb and IIIc. The longer the duration of the disease up to the CAG examination, the more advanced was the CAG stage. Repeat CAGs examined after a mean interval of 2 yrs 11 mos demonstrated the same stage in 32%, and progress by one or two stages in 55% and 14% of the sides examined, respectively.