Case Report: Anti-NMDAR Encephalitis With Anti-MOG CNS Demyelination After Recurrent CNS Demyelination

Abstract

Introduction: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis, a serious neurological autoimmune disorder caused by autoantibodies with diverse clinical manifestations, may simultaneously onset with antimyelin oligodendrocyte glycoprotein (MOG) demyelination after recurrent central nervous system (CNS) demyelination.Case Report: We present a case of anti-NMDAR encephalitis combining with anti-MOG CNS demyelination following recurrent CNS demyelination. A 38-year-old man admitted to hospital developed epileptic seizures following recurrent episodes of cross-sensory disturbance and dizziness. Magnetic resonance imaging (MRI) showed a demyelinating lesion in the right brainstem initially. Despite a good response to methylprednisolone pulse therapy at the beginning, the patient still had relapses and progression after corticosteroid reduction or withdrawal. Then brain MRI discovered new serpentine lesions involving extensive cerebral cortex on his second relapse. Repeat autoantibodies test indicated cerebrospinal fluid (CSF) NMDAR antibodies coexisted with MOG-Abs simultaneously, suggesting the diagnosis of anti-NMDAR encephalitis with anti-MOG CNS demyelination.Results: After a definite diagnosis, the patient was treated with mycophenolate mofetil (MMF) and corticosteroid. He was discharged after his symptoms ameliorated. No neurological sequels remained, and there were no effects on his activities of daily living after 6 months of immunoregulatory therapy of MMF and corticosteroid.Conclusion: For individuals with recurrent CNS demyelination, especially combining with cortical encephalitis, repeated detection of autoantibodies against AE, and demyelination in CSF/serum can be helpful to enable a definite early diagnosis. For patients who suffer from anti-NMDAR encephalitis combining with anti-MOG CNS demyelination, second-line immunotherapy is recommended when first-line treatment such as steroids, intravenous immunoglobulin G (IVIG) and plasma exchange has been proven ineffective to prevent the relapse of disease.

Clinico‐radiological characteristics of anti‐myelin oligodendrocyte glycoprotein antibody‐associated autoimmune encephalitis in children

“…The coexistence of anti-MOG and anti-NMDAR antibodies has also been increasingly reported in both adult and paediatric patients. [22][23][24][25][26] In a recent study, Hou et al 22 reported seven patients with anti-NMDAR encephalitis combined with anti-MOG antibodies. Two of their patients did not show any clinical features of anti-MOG-associated demyelination during the follow-up period, which is similar to our single case (i.e.…”

Coexistence of Myelin Oligodendrocyte Glycoprotein Immunoglobulin G and Neuronal or Glial Antibodies in the Central Nervous System: A Systematic Review

“…Fifty-three relevant studies were identified after title and abstract screening. After reading the full texts and reviewing the references of the retrieved articles, 35 studies were finally included in the qualitative synthesis, of which 14 were retrospective studies [ 11 , 15 , 16 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 ] and 21 were case reports [ 12 , 13 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 ]. A total of 113 patients (46 males and 67 females) were reported to show the coexistence of MOG-IgG and neuronal or glial antibodies in these 35 studies.…”

Autoimmune diseases and cancers overlapping with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD): A systematic review

“… 3 This finding implies a difference in the immunopathogenic mechanisms involved in the development of each entity, such that AQP4-IgG + NMOSD is more attributable to a defect in immune tolerance, while MOGAD is more likely to be an aberrant autoimmune response to certain triggers, such as infection, vaccination, or malignancy. 3 …”

“…The major finding of our study was that the most frequent autoimmune disorder that has been reported to co-exist with MOGAD is anti-NMDAR-EN, which is a similar finding to previous studies. 53 Some studies have referred to this co-occurrence as “overlapping syndrome of MOGAD and anti-NMDAR-EN, (MNOS).” 19 , 51 In our literature review, we captured a total of 200 patients who either met the diagnostic criteria for autoimmune encephalitis and were simultaneously seropositive for both anti-NMDAR-IgG, and MOG-IgG or who met the diagnostic criteria for each of MOG-associated encephalitis (MOGAD-EN) or anti-NMDAR-EN and was seropositive for the antibody associated with the other one, at the same time. 10 Given the rarity of both MOGAD and anti-NMDAR-EN, the co-occurrence of these two entities may happen in the context of an association or could be just a coincidence.…”