Screening for autoantibodies in children with opsoclonus-myoclonus-ataxia.
Various paraneoplastic autoantibodies have been linked to discrete neurologic syndromes and tumors in adults, but little is known about their incidence in children. We report a cross-sectional study of known paraneoplastic antibodies in 59 children with opsoclonus-myoclonus-ataxia, 86% of whom were moderately or severely symptomatic, and 68% of whom had relapsed at the time of testing. This total number of patients includes 18 children with low-stage neuroblastoma (tested after tumor resection), six of whom had never been treated with immunosuppressants. All were seronegative for anti-Hu, anti-Ri, and anti-Yo, the three paraneoplastic antibodies most associated with opsoclonus-myoclonus or ataxia in adults. These data contrast with reports of anti-Hu-positive sera in children with high-stage tumors and suggest that anti-Hu, anti-Ri, and anti-Yo do not explain relapses in pediatric opsoclonus-myoclonus-ataxia. They underscore the need to search for unique autoantibodies, as well as cellular mechanisms of pediatric paraneoplastic disease.
Pranzatelli
Michael R
MR
Department of Neurology, Southern Illinois University School of Medicine, Springfield, Illinois 62702, USA.
Tate
Elizabeth D
ED
Wheeler
Alisa
A
Bass
Nancy
N
Gold
Arnold P
AP
Griebel
May L
ML
Gumbinas
Maria
M
Heydemann
Peter T
PT
Holt
Philip J
PJ
Jacob
Pierre
P
Kotagal
Suresh
S
Minarcik
Chester J
CJ
Schub
Howard S
HS
D000008:Abdominal Neoplasms / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology; D017668:Age of Onset; D001259:Ataxia / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology; D001323:Autoantibodies / Q000097:blood*; D002675:Child, Preschool; D015897:Comorbidity; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007223:Infant; D008297:Male; D008403:Mass Screening / Q000706:statistics & numerical data*; D009447:Neuroblastoma / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology; D020361:Paraneoplastic Syndromes, Nervous System / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology; D012008:Recurrence; D012698:Serologic Tests; D017678:Sex Distribution; D013899:Thoracic Neoplasms / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology; D014481:United States / Q000453:epidemiology
D001323:Autoantibodies; D007074:Immunoglobulin G