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. 2023 Nov;81(11):980-988.
doi: 10.1055/s-0043-1777002. Epub 2023 Nov 30.

Clinical spectrum of myelin oligodendrocyte glycoprotein antibody-associated disease in Brazil: a single-center experience

Katharina Messias  1   2 , Renata Moreto  2 , Camila Aquino Cruz  1 , Nathalia Rossoni Ronchi  1 , Antonio Carlos Dos Santos  3 , André Messias  2 , Vanessa Daccach Marques  1
Affiliations

Clinical spectrum of myelin oligodendrocyte glycoprotein antibody-associated disease in Brazil: a single-center experience

Katharina Messias et al. Arq Neuropsiquiatr. 2023 Nov.

Abstract
in English, Portuguese

Background: Anti-myelin oligodendrocyte glycoprotein (anti-MOG) antibody-associated disease (MOGAD) is an immune-mediated neurological disorder with a broad spectrum of clinical presentation that is often difficult to distinguish from other demyelinating diseases, such as multiple sclerosis and neuromyelitis optica spectrum disorder.

Objective: To describe the clinical and paraclinical characteristics of MOGAD in a Brazilian tertiary center.

Methods: We retrospectively reviewed the records of adult and pediatric patients who tested positive for anti-MOG antibodies and presented with clinical and radiological diseases compatible with MOGAD.

Results: Forty-one patients (10 children) were included: 56% female, 58% Caucasian, mean age at onset 31 years (range 6-64), with a mean disease duration of 59.6 months (range 1-264 months). The most frequent onset presentation was optic neuritis (68%), acute disseminated encephalomyelitis (ADEM, 12%), and myelitis (10%). A monophasic disease course was observed in 49%. EDSS median was 2.1 at the last visit. Most patients (83%) were under continuous immunosuppressive treatment. Azathioprine was the first-line treatment in 59%. In all ADEM cases, conus, and root involvement was radiologically observed on MRI.

Conclusion: Brazilian MOGAD patients presented with a similar spectrum of previously reported MOGAD phenotypes. Conus and spinal root involvement seems to be frequently present in MOGAD-ADEM and could serve as radiologic characteristics of this clinical entity.

Antecedentes: A doença associada ao anticorpo da glicoproteína da mielina de oligodendrócitos (anti-MOG; MOGAD) é uma doença neurológica imunomediada com um amplo espectro de apresentações clínicas que muitas vezes é difícil de distinguir de outras doenças desmielinizantes, como a esclerose múltipla e o distúrbio do espectro da neuromielite óptica.

Objetivo: Descrever as características clínicas e paraclínicas da MOGAD em um centro terciário brasileiro. MéTODOS: Revisamos retrospectivamente os prontuários dos pacientes adultos e pediátricos que testaram positivos para anticorpos anti-MOG e apresentaram um quadro clínico e radiológico compatível com MOGAD.

Resultados: Quarenta e um pacientes (10 crianças) foram incluídos: 56% do sexo feminino, 58% caucasianos, idade média de início da doença foi 31 anos (intervalo de 6-64), com duração média da doença de 59,6 meses (intervalo de 1-264 meses). A apresentação inicial mais frequente foi neurite óptica (68%), seguida pela encefalomielite disseminada aguda (ADEM, 12%) e mielite (10%). Um curso monofásico da doença foi observado em 49%. EDSS foi de 2,1 na última visita. A maioria dos pacientes (83%) estava sob tratamento imunossupressor contínuo. Azatioprina foi o tratamento de primeira linha em 59%. Em todos os casos de ADEM, o envolvimento do cone medular e das raízes espinhais foi observado radiologicamente na ressonância magnética. CONCLUSãO: Os pacientes brasileiros com MOGAD apresentam um espectro clínico e radiológico semelhante aos fenótipos de MOGAD relatados anteriormente. O envolvimento do cone e das raízes espinhais parece estar frequentemente presente no MOGAD-ADEM e poderia servir como característica radiológica nesta entidade.

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Conflict of interest statement

There is no conflict of interest to declare.

Figures

Figure 1
Figure 1
The red line represents the Kaplan-Meier survival analysis calculated for the risk of a relapsing disease course for the total cohort over the follow-up period. Dashed lines indicate the 95% confidence limits. All 41 patients initially showed only one disease manifestation (monophasic), and the number of patients at risk for relapse is provided for various time points of interest (within parenthesis). In addition figure highlights that 50% of patients with disease duration longer than 61 months showed a relapsing course.
Figure 2
Figure 2
MRI findings of 5-year-old female patient with optic neuritis, combined central and peripheral demyelination, and peripheral enhancement of the ventral and dorsal nerve roots of the cauda equina. All the imagens are from the same date. Axial FLAIR with fat suppression (A,B, and F), coronal FLAIR (E), sagittal TSE T2 and T1 after gadolinium administration (C), axial T1 (D), and axial T2-weight image (G). The arrow points at contrast enhancement of the cauda equina (D), presence of hippocampal, corticospinal tract and mesencephalic lesions (E), and bilateral and symmetrical optic nerve head swelling (F).
Figure 3
Figure 3
MRI findings of patient diagnosed with FLAMES (FLAIR-hyperintense lesions in anti-MOG-associated encephalitis with seizure). A 23-year-old previously healthy man developed severe headache, left sided hemiparesis, bilateral blurry vision, mental confusion and first epileptic seizure 3 weeks after receiving the Pfizer-BioNTech COVID-19 mRNA vaccine. Brain MRI images before steroid pulse therapy are shown: 3D-FLAIR with fat suppression (A-D); 3DT1 with fat suppression and after gadolinium administration (E-H). The arrows indicate lesions in the corpus callosum, brainstem, and cortex. Lesions are observed with perivascular CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in image A, and cortical lesions with contrast enhancement in images E and F. There is peripheral enhancement of the chiasm (G) and bilateral optic nerves (H).
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