Elsevier

Journal of Neuroimmunology

Volume 358, 15 September 2021, 577663
Journal of Neuroimmunology

Short Communication
Autoimmune neurology: Co-occurrence of anti-NMDAR encephalitis and anti-MOG associated disease, report of a case

https://doi.org/10.1016/j.jneuroim.2021.577663Get rights and content

Highlights

  • Clinical features of pathogenic anti-MOG and anti-NMDAR at the same time

  • Hyperphagia as a new clinical feature

  • Increasing awareness of both diseases when atypical symptoms are present.

Abstract

We report the case of a patient with symptoms of anti-NMDAR encephalitis and anti-MOG associated disease simultaneously, in whom the identification of antibodies guided to a more aggressive treatment strategy, resulting in a good clinical outcome. MRI is an important tool to diagnose this kind of patients. The co-occurrence of both diseases in infrequent, but atypical symptoms should increase our awareness of the possibility of an overlap syndrome.

Introduction

Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is associated with IgG antibodies in Cerebrospinal Fluid (CSF) against GluN1 subunit of NMDAR, which usually affects young adults with female predominance.(Graus et al., 2016) The clinical features frequently reported are psychiatric and behavioral symptoms, seizures, motor dysfunction, memory impairment, speech disorders, decrease in level of consciousness, autonomic dysfunction, and central hypoventilation.(Liu et al., 2017) Anti-Myelin Oligodendrocyte Glycoprotein (MOG) associated disease (MOGAD) is an oligodendrocytopathy that presents with a broad spectrum of clinical phenotypes associated to MOG-IgG antibodies that has evolved into a new inflammatory CNS disease.(Hegen and Reindl, 2020) The clinical phenotype includes monophasic or recurrent optic neuritis, myelitis, brainstem encephalitis, or acute disseminated encephalomyelitis.(Hegen and Reindl, 2020; Jarius et al., 2018).

The frequency of sequential or coexisting anti-NMDAR encephalitis and demyelinating disorders (particularly MOGAD) is unknown. The coexistence may result from autoimmune disorders targeting oligodendrocytes, a secondary immune reaction, or a bystander phenomenon.(Ren et al., 2019; Titulaer et al., 2014) This report aims to add knowledge on the clinical spectrum of anti-NMDAR encephalitis co-occurrence with MOGAD.

Section snippets

Case report

A 27-year-old male without past medical history was admitted to our institution in August 2020. He started his disease 3 months before with an unspecific headache. Two weeks later he had speech disorder, irritability, insomnia, aggressive behavior, and disorientation. After one month, he developed emotional lability, unmotivated crying, confusion, severe insomnia, visual hallucinations, episodic memory impairments, and psychomotor agitation. In August 2020, he developed absence seizures,

Discussion

Our case met the diagnostic criteria for “definite anti-NMDAR encephalitis”(Graus et al., 2016), since he presented with rapid onset of abnormal behavior, cognitive dysfunction, speech dysfunction, seizures, and decreased level of consciousness and has IgG anti-NMDAR. Likewise, it met the diagnostic criteria for “MOG-encephalomyelitis”(Jarius et al., 2018), since he developed brainstem dysfunction, had an MRI compatible with CNS demyelination and positivity for IgG anti-MOG. Giving those

Conclusion

We present here the first South American patient with co-occurrence of anti-NMDAR encephalitis and MOGAD. Our case report adds knowledge to the clinical spectrum of patients with this overlap syndrome.

Funding

This research did not receive any specific grant for funding agencies in the public, commercial, or not-for-profit sectors.

Conflicts of interest

None.

Aknowledgments

The authors thank Prof. Francesc Graus and Prof. Josep Dalmau (IDIBAPS, Hospital Clinic, University of Barcelona) for examining antibodies against cell-surface antigens and critical comments on this study.

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