The clinical spectrum and incidence of anti-MOG-associated acquired demyelinating syndromes in children and adults. de Mol CL, Wong Y, van Pelt ED, Wokke B, Siepman T, Neuteboom RF, Hamann D, Hintzen RQ. Mult Scler. 2019 May 16:1352458519845112. doi: 10.1177/1352458519845112. [Epub ahead of print]
OBJECTIVES: The aim of this study was to assess the Dutch nationwide incidence of myelin oligodendrocyte glycoprotein (MOG)-IgG-associated acquired demyelinating syndromes (ADS) and to describe the clinical and serological characteristics of these patients. METHODS: All serum samples for routine diagnostics from February 2014 to December 2017 were sent to the single central reference laboratory for the full-length MOG-IgG cell-based assay (CBA) in the Netherlands. Clinical data from patients known in our National ADS centre were available.RESULTS: A total of 1414 samples of 1277 patients were received; of these, 92 patients (7%) were MOG-IgG-seropositive. The mean incidence was 0.16/100,000 people, with higher seropositivity in children (0.31/100,000) than in adults (0.13/100,000). In MOG-IgG-positive patients at the National ADS centre (61/92, 66%), the most common presenting phenotype is acute disseminated encephalomyelitis (ADEM, 56%) in children and optic neuritis (ON, 44%) in adults. Relapsing disease occurred in 9/34 (26%) children and 11/27 (41%) adults during median follow-up of 27.5 months. Patients were tested MOG-IgG-positive >200 months after the initial attack, suggesting an extended time to first relapse (TTFR). Longitudinal analysis of MOG-IgG (25/61, 41%) showed that 67% of the monophasic patients remain seropositive and 60% in relapsing patients. Majority of seronegative patients had no relapses (89%). CONCLUSION: This nationwide study shows that the overall incidence of MOG-IgG-seropositive disorders is 0.16 per 100,000 people. The distribution over the clinical phenotypes differs between adults and children. Seropositivity can be maintained over years even without clinical activity, while seronegative patients generally had no relapses.
Although the contents of this post talk about the occurence of a myelin antibody-mediated demyelinating disease, this is not relevent.
It reminds me to send our condolences to the the family and friends of Dr. Rogier Hintzen (Eramus University, Rotterdam, The Netherlands) who sadly passed away this week, after a short battle with ill-health.
Rogier Hintzen was a Professor in MS and CNS neuroimmunology at Erasmus University and head of the MS Center ErasMS, Erasmus MC Rotterdam, The Netherlands.
His research interests focus around biological determinants of the cause and the course of MS, including prediction of MS after a first attack, genes and environmental factors involved in MS and NMO, and cellular immunology of CNS inflammation. He supervised ongoing local and international studies on biomarkers in cerebrospinal fluid using several proteomic techniques.
He was chair of the scientific committee of the Dutch Society for Neurology and (board) member of the European School for Neuroimmunology (ESNI), the International Pediatric MS study Group (IPMSSG), the International Society for Neuroimmunology (ISNI) and the International MS Genetics Consortium (IMSGC) .
A sad loss of a colleague, collaborator and friend