title: Early clinical characteristics according to developmental stage in children with definite moyamoya disease.
abstract: The objective is to clarify the early clinical characteristics in childhood moyamoya disease (MD). Epidemiologic characteristics, symptoms and diagnostic rates were assessed in 64 children (0-18 years) with definite MD according to developmental stage: infancy (5; 0-1 years); toddlerhood/preschool age (22; 2-5 years); school age (29; 6-10 years); and adolescence (8; 11-18 years). The median ages at onset was 6.25 years and the female to male ratio was 1.9 (~2.5 in toddlerhood/preschool age and in adolescence, P=0.71). Previous headache was observed in 23% (14/64): frequently in school age (38%, P=0.02) and within 6 months before main symptoms (6/11). As an initial symptom, weakness was observed in 78% (50/64) mainly as transient ischemic attack (TIA, 61%) in limbs (90%) and unilaterally (82%). TIA was less frequent in infancy (40%, P=0.04). Seizure was observed in 27% (17/64): frequently in infancy (100%, P<0.01), as the focal type (71%), and in the right extremity (3:1). Isolated seizures without other symptoms was frequent in children ~5 years (P<0.01). Severe headache associated with MD was observed in 14% (9/64). Provoking events were positive in 42% (27/64): in school age, frequently during eating (28%); and in toddlerhood/preschool age, during crying (27%). The diagnostic rates at 3 and 12 months from symptom-onset were 39% (80% during infancy vs. 28% in school age, P=0.14) and 67%, respectively. Symptomatic progression at diagnosis was observed in 38% (24/64). Initial clinical characteristics in childhood definite MD differed according to developmental stage and from at diagnosis.
authors.affiliation: Department of Pediatrics, School of Medicine, Chonnam National University, Gwangju, Republic of Korea. ik052@chonnam.ac.kr
authors.forename: Young Ok
authors.initials: YO
authors.lastname: Kim
authors.forename: Sung-Pil
authors.initials: SP
authors.lastname: Joo
authors.forename: Bo-Ra
authors.initials: BR
authors.lastname: Seo
authors.forename: Young Il
authors.initials: YI
authors.lastname: Rho
authors.forename: Woong
authors.initials: W
authors.lastname: Yoon
authors.forename: Young Jong
authors.initials: YJ
authors.lastname: Woo
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000175:diagnosis* / Q000453:epidemiology*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009072:Moyamoya Disease / Q000175:diagnosis* / Q000453:epidemiology*; D012189:Retrospective Studies