Posterior circulation and high prevalence of ischemic stroke among young pediatric patients with Moyamoya disease: evidence of angiography-based differences by age at diagnosis.
OBJECTIVE
At diagnosis, the primary clinical manifestations of pediatric Moyamoya disease are TIA or CSs. CSs are reported to be more prevalent in younger than in older children. We sought to determine whether age-related differences in clinical manifestations are associated with age-related angiographic differences.


METHODS
We divided 78 patients diagnosed with Moyamoya disease before 16 years of age into four 4-year age groups and examined the relationships between age at diagnosis and clinical manifestations and angiographic and MR imaging findings.


RESULTS
Among the 4 diagnostic age groups, in those younger than 4 years of age, the prevalence of CSs and of infarctions on MR images was highest, and along with severity of steno-occlusive lesions of the PCA, the prevalence was significantly higher than that in the next diagnostic age group (4-7 years), though the severity of steno-occlusive lesions in the ICA and the degree of transdural collaterals did not differ significantly. The prevalence of CSs and infarctions did not differ significantly in the 3 oldest diagnostic age groups, whereas ICA and PCA lesions and transdural collaterals correlated positively with diagnostic age.


CONCLUSIONS
The high prevalence of CSs and infarctions in patients diagnosed before 4 years of age is associated with advanced steno-occlusive lesions of the PCA. In patients 4 years of age and older at diagnosis, transdural collaterals develop in parallel with advancement of ICA and PCA lesions, which may contribute to the nearly constant prevalence of CSs.
Mugikura
S
S
Department of Diagnostic Radiology, School of Medicine, Tohoku University, Sendai, Japan. mugi@rad.med.tohoku.ac.jp
Higano
S
S
Shirane
R
R
Fujimura
M
M
Shimanuki
Y
Y
Takahashi
S
S
D000293:Adolescent; D017677:Age Distribution; D020520:Brain Infarction / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D002533:Cerebral Angiography / Q000706:statistics & numerical data*; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D002546:Ischemic Attack, Transient / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D007564:Japan / Q000453:epidemiology; D008297:Male; D009072:Moyamoya Disease / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D015995:Prevalence; D018570:Risk Assessment; D012307:Risk Factors
Lancet Neurol. 2008 Nov;7(11):1056-66
18940695
Radiology. 1995 Oct;197(1):239-46
7568830
Stroke. 1983 Jan-Feb;14(1):104-9
6823678
Stroke. 1997 Jun;28(6):1170-3
9183345
Arch Neurol. 1969 Mar;20(3):288-99
5775283
J Neurosurg. 2004 Feb;100(2 Suppl Pediatrics):142-9
14758941
Childs Nerv Syst. 1990 Dec;6(8):440-3
2095302
Neurol Res. 1989 Sep;11(3):173-6
2573854
Surg Neurol. 1997 Feb;47(2):138-43
9040816
Stroke. 2002 Jun;33(6):1497-500
12052981
Brain Dev. 1999 Dec;21(8):535-9
10598054
Pediatr Neurol. 1998 Apr;18(4):321-5
9588527
Neurosurgery. 2004 Apr;54(4):840-4; discussion 844-6
15046649
J Neurosurg. 1984 Dec;61(6):1032-7
6502231
AJNR Am J Neuroradiol. 1999 May;20(5):917-22
10369366
AJNR Am J Neuroradiol. 1999 Feb;20(2):336-43
10094366