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Paediatric brainstem encephalitis associated with glial and neuronal autoantibodies
Author(s) -
Hacohen Yael,
Nishimoto Yukihiro,
Fukami Yuki,
Lang Bethan,
Waters Patrick,
Lim Ming J,
Yuki Nobuhiro,
Vincent Angela
Publication year - 2016
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/dmcn.13090
Subject(s) - autoantibody , myelin oligodendrocyte glycoprotein , medicine , neuromyelitis optica , pathology , encephalitis , antibody , brainstem , grey matter , immunology , white matter , central nervous system , myelin , magnetic resonance imaging , virus , radiology
Aim Central nervous system ( CNS ) autoantibodies have been reported in a range of neuroimmune diseases, but there has not been a systematic evaluation of autoantibodies in paediatric patients with brainstem encephalitis. Method Serum samples from 57 children (40 male, 17 female, median age 12y, range 0.6–18y) with a diagnosis of brainstem encephalitis were tested retrospectively for antibodies to GQ 1b, aquaporin‐4 ( AQP 4), myelin oligodendrocyte glycoprotein ( MOG ), N ‐methyl‐D‐aspartate receptor, LGI 1, CASPR 2, glycine receptor (GlyR), DPPX , and the voltage gated potassium channel ( VGKC )‐complex. Results Disease localized to the brainstem was seen in 19 patients: Bickerstaff's brainstem encephalitis ( n =14) and clinically isolated syndrome ( n =5). Polyfocal presentation was seen in 38 children, with predominantly white matter disease in 18 patients and grey matter in 20 patients. CNS surface antibodies were found in 22/57 patients (two patients with double positivity): GQI b ( n =6), NMDAR ( n =7), GlyR ( n =5), MOG ( n =5), and one AQP 4. Three patients were positive for VGKC ‐complex antibodies. All patients were negative for antibodies to DPPX and the VGKC ‐complex antigens LGI 1, CASPR 2, and contactin‐2. Although there were some partial differences in the presentations, the clinical features and outcomes did not relate clearly to the presence or absence of specific antibodies. Interpretation As determined retrospectively, 39% of patients had cell surface antibodies. The results did not suggest any relationship with treatment or outcomes obtained but it is possible that specific antibody detection could be a helpful guide to more intensive immunotherapies in some cases.