Espectro clínico de doença associada com anticorpos contra a glicoproteína de oligodendrócito de mielina no Brasil: uma experiencia de centro único

Messias, Katharina; Moreto, Renata; Cruz, Camila Aquino; Ronchi, Nathalia Rossoni; Santos, Antonio Carlos dos; Messias, André; Marques, Vanessa Daccach

Katharina Messias

conceptualization

writing – original draft

data curation

project administration

Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Departamento de Neurociências e Ciências do Comportamento, Ribeirão Preto SP, Brazil.

Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Departamento de Oftalmologia, Otorrinolaringologia e Cirurgia de Cabeça e Pescoço, São Paulo SP, Brazil.

https://orcid.org/0000-0002-5112-5722

Renata Moreto

writing – review & editing

Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Departamento de Oftalmologia, Otorrinolaringologia e Cirurgia de Cabeça e Pescoço, São Paulo SP, Brazil.

https://orcid.org/0000-0001-6374-9578

Camila Aquino Cruz

writing – review & editing

Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Departamento de Neurociências e Ciências do Comportamento, Ribeirão Preto SP, Brazil.

https://orcid.org/0009-0007-7496-5090

Nathalia Rossoni Ronchi

writing – review & editing

Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Departamento de Neurociências e Ciências do Comportamento, Ribeirão Preto SP, Brazil.

https://orcid.org/0000-0002-3367-1949

Antonio Carlos dos Santos

writing – review & editing

data curation

Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Departamento de Imagens Médicas, Hematologia e Oncologia Clínica, Ribeirão Preto SP, Brazil.

https://orcid.org/0000-0002-5502-4734

André Messias

writing – review & editing

software

formal analysis

Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Departamento de Oftalmologia, Otorrinolaringologia e Cirurgia de Cabeça e Pescoço, São Paulo SP, Brazil.

https://orcid.org/0000-0002-5328-9608

Vanessa Daccach Marques Address for correspondence Vanessa Daccach Marques (email: vdaccachm@gmail.com).

conceptualization

supervision

writing – review & editing

Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto, Departamento de Neurociências e Ciências do Comportamento, Ribeirão Preto SP, Brazil.

https://orcid.org/0000-0002-2131-9438

Address for correspondence Vanessa Daccach Marques (email: vdaccachm@gmail.com).

Conflict of interest

There is no conflict of interest to declare.

Figuras (3)
Tabelas (2)

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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.

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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).

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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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Table 1
Summary of the epidemiologic, clinical and laboratory characteristics of the MOGAD cohort

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Table 2
Magnetic resonance findings at disease onset

Variable	All patients (n = 41)	Pediatric onset (n = 10)	Adult-onset (n = 31)
Female, % (n)	56.1 (23)	80.0 (8)	45.0 (14)
Caucasian/white, % (n)	58.8 (24)	70.0 (7)	54.8 (17)
Mean age, years (range)	35.8 (8-71)	14.3 (8-36)	42.7 (23-71)
Age at disease onset, mean years (range)	31.0 (6-64)	10 (6-15)	37.8 (18-62)
Disease duration, mean months (range)	59.6 (1-264)	55 (1-256)	61.2 (2-264)
Symptoms at disease onset	% of patients (n)
Optic neuritis	68.3 (28)	30 (3)	80.1 (25)
Optic neuritis + concomitant myelitis	4.9 (2)	0	6.5 (2)
ADEM (+/− optic neuritis and/or myelitis)	12.2 (5)	50 (5)	0
Myelitis	9.8 (4)	10 (1)	9.7 (3)
Others: FLAMES; cortical encephalitis	4.9 (2)	10 (1)	2.4 (1)
Infectious prodrome/vaccination before disease onset, % (n)	34 (14)	60 (6)	25.8 (8)
EDSS during first attack at nadir, average (range)	4.4 (1-8.5)	6.1 (3-8.5)	2.5 (1-8.5)
EDSS at final visit, average (range)	1.9 (0-8)	1.0 (1-4)	2.25 (0-8)
Relapses	% of patients (n)
Relapsing disease course	51.2 (21)	60 (6)	48.4 (15)
Number of relapses per patient (range)	2.5 (1-13)	2 (1-3)	2.6 (1-13)
Number of relapses	n = 53	n = 8	n = 45
Optic neuritis	69.8 (37)	50 (4)	73.3 (33)
Optic neuritis + myelitis	13.2 (7)	25 (2)	11.1 (5)
Myelitis	13.2 (7)	0	15.6 (7)
ADEM	1.9 (1)	1 (12.5)	0
Optic neuritis at any time point, % (n)	82.9 (34)	60 (6)	90 (28)
Other Autoimmune disease	2.4 (1)	0	3.2 (1)
Laboratory
Positive antinuclear antibodies, n = 36	7.3 (3)	0	9.6 (3)
Mean CSF leucoyctes (cells/mm³), range	22.3 (0-200)	67.9 (2.7-200)	8.4 (0-80)
> 5 leucocytes/mm³, % (n)	43.9 (18)	70 (7)	32.3 (10)
Mean CSF protein (mg/dL), range	36.9 (12-70)	41.1 (30-70)	34.6 (12-69)
> 45 mg/dL protein % (n)	26.8 (11)	30 (3)	25.8 (8)
Positive oligoclonal bands, n = 29	17.2 (5)	12.5 (1 out of 8)	19.0 (4 out of 21)
Treatment	% of patients (n)
Acute treatment at onset	80.5 (33)	90 (9)	77.4 (24)
• IVMP	45.4 (15)	20 (2)	54.2 (13)
• IVMP + PLEX	36.4 (12)	30 (3)	37.5 (9)
• Oral or intramuscular steroids	9.1 (3)	10 (1)	8.3 (2)
• IVIG	9.1 (3)	30 (3)	0
• Oral steroid taper	46.3 (19)	60 (6)	41.9 (13)
Chronic treatment ever used	82.9 (34)	70 (7)	87.1 (27)
1^st line chronic treatment	% of patients (n)
• Azathioprine	58.8 (20)	57.1 (4)	59.3 (16)
• Rituximab	32.4 (11)	28.6 (2)	33.3 (9)
• Other	8.8 (3)	14.3 (1)	7.4 (2)
Treatment change	38.2 (13)	1 (14.3)	44.4 (12)

MR findings available at disease onset (n = 25/41), 61%
Optic neuritis	48% (n = 12)	Unilateral, % (n)	67 (8)
		Length > 50%	75 (9)
		Anterior involvement	92 (11)
		Chiasmal involvement	8 (1)
		Perineuritis	83 (10)
		Contrast enhancement	83 (10)
		Brain lesions	17 (2)
Myelitis	24% (n = 6)	LETM, % (n)	83 (5)
		Central GM involvement	83 (5)
		Conus involvement	33 (2)
		Contrast enhancement	67 (4)
ADEM	20% (n = 5)	Brain lesions only, % (n)	0 (0)
		Brain lesions + myelitis	60 (3)
		Brain lesions + ON	0 (0)
		Brain lesions + myelitis +ON	40 (2)
		Conus and root involvement	100 (5)
FLAMES/cortical encephalitis	8% (n = 2)	Brain lesions only, % (n)	50 (1)
		Brain lesions + myelitis	0 (0)
		Brain lesions + ON	50 (1)

[1] Address for correspondence Vanessa Daccach Marques (email: vdaccachm@gmail.com).

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Espectro clínico de doença associada com anticorpos contra a glicoproteína de oligodendrócito de mielina no Brasil: uma experiencia de centro único

Resumo

Antecedentes

Objetivo

Métodos

Resultados

Conclusão