2014•NeuropediatricsRequires access
MOG- and AQP-4-IgG Antibodies in Children with Neuromyelitis Optica Spectrum Disorders and NMO-Related Symptoms
Christian J. Lechner, M. Baumann, Kathrin Schanda, Astrid Blaschek, T. Lücke, Andrea Klein, Steffen Leiz, Ursula Gruber‐Sedlmayr, Michaela Brunner‐Krainz, Martin Pritsch, J Koch, Mareike Schimmel, MG Häusler, Michael Karenfort, Markus Reindl, K. Rostásy
Open publisher page 0 citations
Abstract
Background: Neuromyelitis optica (NMO) is characterized by episodes of recurrent or bilateral optic neuritis (ON) and longitudinally extensive transverse myelitis (LETM). Particularly in adults, aquaporin-4-antibodies (AQP-4-Abs) are found in the serum in up to 80% of all the cases. Some patients do not fulfill all criteria initially and present only with a recurrent ON, bilateral ON, or LETM with AQP-4-Abs. These cases belong to the NMO Spectrum Disorders (NMOSD). Recently, it was shown that children with AQP-4-Ab negative NMOSD can have myelin oligodendrocyte glycoprotein antibodies (MOG-Abs). Objectives: Frequency of MOG- and AQP-4-IgG antibodies in children with NMO, recurrent ON, bilateral ON, or LETM. Methods: Children with NMO, recurrent ON, bilateral ON, or LETM were included in the study. The following parameters were studied: age, sex, CSF cell count, presence of oligoclonal bands, serum MOG-, and AQP-4-Ab status. Results: A total of 35 children were included. Of the 35 children, 13 fulfilled the criteria of NMO (four males and nine females), 11 of 35 had an LETM (six males and five females), 7 of 35 recurrent ON (one male and six females), and 4 of 35 bilateral ON (three males and one female). In 6 of 35 children (five NMO and one LETM) AQP-4-Abs were detected (median, 1:320; range, 1:160 to 1:1,280). Of the 35 patients, 16 (five NMO, four LETM, four recurrent ON, and three bilateral ON; nine males and seven females) had MOG-Abs (median, 1:1,280; range, 1:160-1:5,120). Neither AQP-4- nor MOG-Abs were found in 13 of 35 patients (37%). In none of the children both antibodies were detected at the same time. Children with AQP-4-Abs were older (median, 12 years; range, 8-14 years) than children with MOG-Abs (median, 6 years; range, 4-15 years). However, no differences were found in the frequency of intrathecal IgG synthesis-present only in three children-or in the male-to-female ratio. Conclusion: Of the 35 children with NMOSD and NMO-related symptoms, only 6 children had AQP-4-Abs. On the contrary, MOG-Abs were found in 16 of 35 children. We therefore recommend to evaluate the MOG-Abs status at initial manifestation, because of the diagnostic and possible therapeutic implications.