Serum autoantibodies in childhood opsoclonus-myoclonus syndrome: An analysis of antigenic targets in neural tissues☆,☆☆
Section snippets
Postviral syndrome (patient 2)
A 10-month-old boy had an inner ear infection with subsequent development of irritability, opsoclonus, myoclonus, and mild ataxia. Examination showed random conjugate, dancing-like eye movements. Myoclonus was bilateral and involved the arms more than the legs. Ataxia was both appendicular and truncal. He was unable to stand unassisted. Sensation was intact to light touch and pain. Results of computed tomography of the brain and cerebrospinal fluid examination were normal. No clinical,
Patients and sera
Sera from nine children (3.9 ± 2.4 years at the time of testing) with OM syndrome were studied (Table I).
Patient No. Sex Age at onset of symptoms (yr) Age at testing (yr) Neuroblastoma Stage 28 IgM-positive autoantibodies to neural antigens 1 F 2.2 2.2 − NA + 2 M 0.8 0.8 − NA + 3 F 1.1 1.1 − NA + 4 F 2.1 2.1 − NA + 5 M 1.2 2.9 − NA + 6 M 0.8 5.2 − NA + 7 F 3.0 14.4 + I + 8 F 1.2 2.6 + I + 9 F 1.6 7.8 + I + 10 M NA 3.4 + IV − 11 M NA 1.3 + IV − 12 M NA 5.6 + IV − 13 M NA 4.6 + IV − 32 Neurologic controls NA 2.7 ± 1.4
IgM
All nine serum samples from the children with OM showed IgM binding to Purkinje cell cytoplasm and to axons in peripheral nerve (Fig. 1) and cerebellar white matter.
DISCUSSION
IgM autoantibodies from the nine OM sera all demonstrated a similar pattern of binding to neural antigens by both immunocytochemistry and Western blot analysis. Immunohistochemistry showed that IgM and IgG from all nine OM sera stained cerebellar Purkinje cell cytoplasm and axons. Western blot analysis of white matter, gray matter, and cerebellar subfractions showed IgM autoantibodies from all nine OM sera bound to proteins with six different molecular weights. The largest, 210 kd protein,
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2017, Seminars in Pediatric NeurologyCitation Excerpt :Cerebellar autoimmunity has been reported by different groups, but there was no definitive antigen identified. Meanwhile, several different childhood OMS-associated autoantigens have been described (Table 2), which can be divided in four groups: (1) DNA/RNA-binding proteins, (2) PSD, (3) other intracellular proteins, and (4) cerebellar surface autoantigens.25,55,68-73 These autoantigens are often described as onconeuronal antigens and occur frequently in adult paraneoplastic neurological disease.
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2014, Aminoff's Neurology and General Medicine: Fifth Edition
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Reprint requests: Anne Connolly, MD, Department of Neurology, Box 8111, Washington University School of Medicine, 660 S. Euclid Ave., St. Louis, MO 63110.