The long-term outcomes of 25 patients with childhood moyamoya disease
(18 with the transient ischemic attack [TIA] type and seven with the n
on-TIA type), who were monitored to adulthood (older than 20 years of
age), were evaluated in terms of residual clinical symptoms, intellect
ual development, and activities of daily living. Surgical treatment wa
s performed in ten patients, encephaloduroarteriosynangiosis in nine,
and superficial temporal artery to middle cerebral artery anastomosis
plus encephalomyosynangiosis in one. only seven with the TIA type (thr
ee surgically and four medically treated) demonstrated good activities
of daily living without TIA or headache and normal IQ, Two patients w
ith the TIA type and three with the non-TIA type demonstrated poor out
comes. Three of these patients with poor outcomes had renal artery ste
nosis, Surgery was effective in nine. Since the longterm outcomes of p
atients with childhood moyamoya disease are generally poor, surgical t
reatment is believed to be an effective procedure for preventing the p
rogression of clinical symptoms. (C) 1998 by Elsevier Science Inc. All
rights reserved.