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