Abstract
BackgroundBased on clinical, immunological and histopathological evidence, MOG-IgG-associated encephalomyelitis (MOG-EM) has emerged as a distinct disease entity different from multiple sclerosis (MS) and aquaporin-4-antibody-positive neuromyelitis optica spectrum disorder (NMOSD). MOG-EM is associated with a broader clinical phenotype including optic neuritis, myelitis, brainstem lesions and acute disseminated encephalomyelitis with a substantial clinical and radiological overlap to other demyelinating CNS disorders.ObjectiveTo evaluate common clinical, MRI and CSF findings, as well as therapy responses in patients with longitudinal extensive transverse myelitis (LETM) as initial clinical presentation of MOG-EM.MethodsAfter excluding patients with a known diagnosis of MS, we identified 153 patients with myelitis of which 7 fulfilled the inclusion criteria and were investigated for MRI, CSF and clinical parameters.ResultsPatients with LETM as first clinical presentation of MOG-EM display similar characteristics, namely a lack of gadolinium-enhancement in spinal cord MRI, marked pleocytosis, negative oligoclonal bands, a previous history of infections/vaccinations and response to antibody-depleting treatments for acute attacks and long-term treatment.ConclusionsWe identify common pathological findings in patients with LETM as first clinical presentation of MOG-EM which distinguishes it from other forms of LETM and should lead to testing for MOG-IgG in these cases.
Highlights
Myelin oligodendrocyte glycoprotein (MOG) is an encephalitogenic protein on CNS oligodendrocytes that can initiate demyelinating autoimmune responses in experimental models of inflammatory demyelinating diseases [1]
In the past few years, growing clinical, immunological and histopathological evidence suggests that MOG-EM can be considered as a clearly distinct disease entity from both multiple sclerosis (MS) and aquaporin-4-positive neuromyelitis optica spectrum disorder (NMOSD) [2,3,4,5]
While anti-myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) were originally linked especially to acute disseminated encephalomyelitis (ADEM)-like presentation in pediatric patients [6, 7], more recent studies proposed a wider clinical spectrum including uni- and bilateral optic neuritis (ON), short and longitudinal extensive transverse myelitis (LETM), brainstem and cerebellar lesions or seizures [8,9,10,11,12,13,14,15]
Summary
Myelin oligodendrocyte glycoprotein (MOG) is an encephalitogenic protein on CNS oligodendrocytes that can initiate demyelinating autoimmune responses in experimental models of inflammatory demyelinating diseases [1]. While anti-myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) were originally linked especially to acute disseminated encephalomyelitis (ADEM)-like presentation in pediatric patients [6, 7], more recent studies proposed a wider clinical spectrum including uni- and bilateral optic neuritis (ON), short and longitudinal extensive transverse myelitis (LETM), brainstem and cerebellar lesions or seizures [8,9,10,11,12,13,14,15]. Isolated uni- or bilateral optic neuritis has been reported as the most common symptom at MOG-EM disease onset (64% and 73% of patients in a German and an Australasian/New Zealand cohort of patients with MOG-EM) [8, 16] and isolated LETM was the first clinical symptom in only 11% and 18% of these patients This lies in the range of 7.4–23.2% of previously reported prevalence of MOGIgG seropositivity in AQP4-IgG-seronegative LETMs [4]. Conclusions We identify common pathological findings in patients with LETM as first clinical presentation of MOG-EM which distinguishes it from other forms of LETM and should lead to testing for MOG-IgG in these cases
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
