AQP4-IgG NMOSD, MOGAD, and double-seronegative NMOSD: is it possible to depict the antibody subtype using magnetic resonance imaging?

Fragoso, Diego Cardoso; Salles, Luana Michelli Oliveira de Paula; Pereira, Samira Luisa Apóstolos; Callegaro, Dagoberto; Sato, Douglas Kazutoshi; Rimkus, Carolina de Medeiros

doi:10.1055/s-0043-1768669

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Original Article • Arq. Neuro-Psiquiatr. 81 (6) • June 2023 • https://doi.org/10.1055/s-0043-1768669 copy

AQP4-IgG NMOSD, MOGAD, and double-seronegative NMOSD: is it possible to depict the antibody subtype using magnetic resonance imaging?

NMOSD AQP4-IgG, MOGAD e NMOSD duplo soronegativo: é possível caracterizar o tipo de anticorpo pela ressonância magnética?

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Background

There is clinical and radiological overlap among demyelinating diseases. However, their pathophysiological mechanisms are different and carry distinct prognoses and treatment demands.

Objective

To investigate magnetic resonance imaging (MRI) features of patients with myelin-oligodendrocyte glycoprotein associated disease (MOGAD), antibody against aquaporin-4(AQP-4)-immunoglobulin G-positive neuromyelitis optica spectrum disorder (AQP4-IgG NMOSD), and double-seronegative patients.

Methods

A cross-sectional retrospective study was performed to analyze the topography and morphology of central nervous system (CNS) lesions. Two neuroradiologists consensually analyzed the brain, orbit, and spinal cord images.

Results

In total, 68 patients were enrolled in the study (25 with AQP4-IgG-positive NMOSD, 28 with MOGAD, and 15 double-seronegative patients). There were differences in clinical presentation among the groups. The MOGAD group had less brain involvement (39.2%) than the NMOSD group (p = 0.002), mostly in the subcortical/juxtacortical, the midbrain, the middle cerebellar peduncle, and the cerebellum. Double-seronegative patients had more brain involvement (80%) with larger and tumefactive lesion morphology. In addition, double-seronegative patients showed the longest optic neuritis (p = 0.006), which was more prevalent in the intracranial optic nerve compartment. AQP4-IgG-positive NMOSD optic neuritis had a predominant optic-chiasm location, and brain lesions mainly affected hypothalamic regions and the postrema area (MOGAD versus AQP4-IgG-positive NMOSD, p= 0 .013). Furthermore, this group had more spinal cord lesions (78.3%), and bright spotty lesions were a paramount finding to differentiate it from MOGAD (p = 0.003).

Conclusion

The pooled analysis of lesion topography, morphology, and signal intensity provides critical information to help clinicians form a timely differential diagnosis.

Keywords
Neuromyelitis Optica; Myelin-Oligodendrocyte Glycoprotein; Magnetic Resonance Imaging

Variable		Demyelinating disease			Test
Variable		MOGAD (28)	AQP4-IgG NMOSD (25)	Double seronegative (15)	Test
Sex	Male	11	11	5	Chi-squared 0.557
Sex	Female	17	14	10	Chi-squared 0.557
Age (mean ± SD)		32.3 ± 2.1	38.4 ± 4.1	28.2 ± 3.0	ANOVA 0.218
Disease status	Active	18	14	7	Chi-squared 0.846
Disease status	Inactive	10	11	8	Chi-squared 0.846
Active attack to MRI^* Notes: *days; (mean ± SD)		7.8 ± 2.6	17.2 ± 2.3	19.1 ± 3.3	ANOVA 0.01
Time elapse from last attack to MRI^τ τ months. (mean ± SD)		9.5 ± 2.4	46 ± 18.3	16.3 ± 11.2	ANOVA 0.172

Variable			Demyelinating disease Affected variable No./total No. (%)			p-value	Multiple comparison	p-value
Variable			MOGAD (28)	AQP4-IgG NMOSD (25)	Double seronegative (15)	p-value	Multiple comparison	p-value
Topography	Supratentorial	Supratentorial lesions	11/28 (39.2)	18/25 (72)	12/15 (80)	0.002	M vs. NMO+	0.004
		Supratentorial lesions	11/28 (39.2)	18/25 (72)	12/15 (80)	0.002	M vs. DN	0.016
		Juxtacortical	4/11 (36.4)	3/18 (16.7)	3/12 (25)	0.507
		Subcortical	6/11 (54.5)	5/18 (27.8)	6/12 (50)	0.299
		Subependymal^†	4/11 (36.4)	7/18 (38.8)	9/12 (75)	0.256
		1. Extensive	1/11 (9.1)	3/18 (16.7)	7/12 (58.3)	0.010	M vs. DN	0.017
		1. Extensive	1/11 (9.1)	3/18 (16.7)	7/12 (58.3)	0.010	NMO+ vs. DN	0.025
		2. Dawson finger	2/11 (18.2)	1/18 (5.5)	2/12 (16.7)	0.534
		3. Nonperpendicular focal lesion	1/11 (9.1)	3/18 (16.7)	2/12 (25)	0.842
		Third ventricle	1/11 (9.1)	3/18 (16.7)	3/12 (25)	0.617
		Temporal lobe	2/11 (18.2)	1/18 (5.5)	3/12 (25)	0.329
		Diencephalic	1/11 (9.1)	3/18 (16.7)	3/12 (25)	0.617
		Thalamus	2/11 (18.2)	1/18 (5.5)	4/12 (33.3)	0.147
		Basal ganglia	0	0	2/12 (16.7)	0.081
		Deep gray matter^á á Thalamus + Basal Ganglia.	2/11 (18.2)	1/18 (5.5)	4/12 (33.3)	0.147
		Corpus callosum^‡ ‡ Extensive, Focal oval, round, or band;	3/11 (27.3)	2/18 (11.1)	6/12 (50)	0.082
		1. Extensive	0	0	3/12 (25)	0.018	M vs. DN	0.048
		1. Extensive	0	0	3/12 (25)	0.018	NMO+ vs. DN	0.024
		2. Oval	2/11 (18.2)	0	1/12 (8.3)	0.198
		3. Round	1/11 (9.1)	2/18 (11.1)	1/12 (8.3)	0.968
		4. Band	0	0	1/12 (8.3)	0.306
		Corticospinal tract	2/11 (18.2)	2/18 (11.1)	6/12 (50)	0.004	NMO+ vs. DN	0.003
		1. Focal	1/11 (9.1)	1/18 (5.5)	4/12 (33.3)	0.093
		2. Extensive	1/11 (9.1)	1/18 (5.5)	2/12 (16.7)	0.620
	Infratentorial	Infratentorial	8/28 (28.6)	13/25 (52)	7/15 (46.6)	0.094
		Area postrema	1/11 (9.1)	12/18 (67)	4/12 (33.3)	0.016	M vs. NMO+	0.013
		Brainstem	8/11 (72.7)	10/18 (55)	7/12 (58.3)	0.657
		Periaqueductal	1/11 (9.1)	1/18 (5.5)	2/12 (16.7)	0.620
		Fourth ventricle	5/11 (45.4)	3/18 (16.7)	2/12 (16.7)	0.173
		Cerebellum	3/11 (27.3)	1/18 (5.5)	2/12 (16.7)	0.283
		Cerebellar peduncles	3/11 (27.3)	2/18 (11.1)	1/12 (8.3)	0.393
Morphology	Oval well-circumscribed		2/11 (18.2)	1/18 (5.5)	2/12 (16.7)	0.534
	Infiltrative		5/11 (45.4)	13/18 (72)	10/12 (83.3)	0.140
	Non-specific		4/11 (36.4)	7/18 (39)	4/12 (33.3)	0.956
	Cavitated		2/11 (18.2)	1/18 (5.5)	4/12 (33.3)	0.147
	Tumefactive^§ § Defined as lesions > 2 cm;		0	0	3/12 (25)	0.018	M vs. DN	0.048
	Tumefactive^§ § Defined as lesions > 2 cm;		0	0	3/12 (25)	0.018	NMO+ vs. DN	0.024
	Round well-circumscribed		1/11 (9.1)	0	1/12 (8.3)	0.458

Disease status	Variable	p-value	Multiple comparison	p-value
Active	Tumefactive lesion	0.043	DN	0.076^* * Tendency to statically significant differences.
	Tumefactive lesion	0.043	DN > NMO+	0.053
	Area postrema	0.024	NMO+ > M	0.019
	Subependymal	0.046	DN > M	0.037
	Deep gray matter	0.046	DN > M	0.050
	Corticospinal tract	0.035	DN > NMO+	0.029
Inactive	Subependymal	0.049	NMO+ > M	0.056^* * Tendency to statically significant differences.
	Subependymal extensive	0.000	DN > M	0.000
	Subependymal extensive	0.000	DN > NMO+	0.000
	Infiltrative lesion	0.042	DN > M	0.035

Variable N		Demyelinating Disease Affected variablen / totaln (%)			p-value	Multiple comparison	p-value
Variable N		MOGAD (27)	AQP4-IgG NMOSD (23)	Double seronegative (14)	p-value	Multiple comparison	p-value
Spinal lesion		10/27 (37)	18/23 (78.3)	7/14 (50)	0.012	M vs. NMO+	0.009
Number of segments^† Note:†Mean deviation within brackets;		6 [ ± 5.3]	7.6 [ ± 4.7]	12.3 [ ± 5.5]	0.049	M vs. DN	0.044
LETM		6/10 (60)	17/18 (94.4)	6/7 (85.7)	0.068	M vs. NMO+	0.055^* * Tendency to statically significant differences.
Column segment	Cervical	6/10 (60)	17/18 (94.4)	5/7 (71.4)	0.124
	Thoracic	7/10 (70)	11/18 (61.1)	7/7 (100)	0.840
	Lumbar	4/10 (40)	1/18 (5.5)	3/7 (42.8)	0.857
Enhancement		5/10 (50)	7/18 (38.9)	4/7 (57.1)	0.791
Bright spotty		0	11/18 (61.1)	3/7 (42.8)	0.004	M vs. NMO+	0.003
H medullar sign		4/10 (40)	1/18 (5.5)	3/7 (42.8)	0.621
Longitudinal line		4/10 (40)	2/18 (11.1)	4/7 (57.1)	0.684

Variable N		Demyelinating disease Affected variablen / totaln (%)			p-value	Multiple comparison	p-value
Variable N		MOGAD (23)	AQP4-IgG NMOSD (20)	Double seronegative (12)	p-value	Multiple comparison	p-value
Neuritis		16/23 (69.5)	14/20 (70)	10/12 (83.3)	0.660
	Unilateral	10/16 (62.5)	7/14 (50)	6/10 (60)	0.087
	Bilateral	6/16 (37.5)	7/14 (50)	4/10 (40)	0.087
Number of segments		1.7 [ ± 1.4]	3.7 [ ± 2.8]	3.6 [ ± 2.3]	0.006	M vs. NMO+	0.010
Number of segments		1.7 [ ± 1.4]	3.7 [ ± 2.8]	3.6 [ ± 2.3]	0.006	M vs. DN	0.040
Segment	Intraorbital	14/16 (87.5)	12/14 (85.7)	9/10 (90)	0.134
	Canalicular/prechiasmic	13/16 (81.2)	13/14 (92.8)	10/10 (100)	0.093
	Optic-chiasm	2/16 (12.5)	8/14 (57.1)	6/10 (60)	0.053^* Notes: *Tendency to statically significant differences.	M vs. NMO+	0.048
	Optic-tract	1/16 (6.2)	7/14 (50)	3/10 (30)	0.063^* Notes: *Tendency to statically significant differences.	M vs. NMO+	0.052^* Notes: *Tendency to statically significant differences.
Extension	< 50%	10/16 (62.5)	5/14 (35.7)	4/10 (40)	0.472
Extension	> 50%	6/16 (37.5)	9/14 (64.3)	7610 (60)	0.720
Perineuritis		4/16 (25)	1/17 (5.9)	2/10 (20)	0.789

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[1] Address for correspondence Diego Cardoso Fragoso (email: diego.fragoso@hc.fm.usp.br).