Demyelinating disease associated with MOG-IgG antibodies and chronic brainstem encephalitis: When to suspect it
PDF (Español)
XML (Español)

Keywords

Brainstem encephalitis
Optic neuritis
Neuroimmunology
MOG antibodies
Myelin Sheath
Steroids
Antibodies

Abstract

Introduction: MOG antibody-associated disease (MOGAD) comprises a broad spectrum of clinical manifestations and imaging findings. Recent evidence indicates that brainstem encephalitis is a possible manifestation of MOGAD.

Case presentation: We present the case of a 32-year-old female patient with a 9-year history of a fluctuating disease course and good response to steroids, with almost exclusive brainstem involvement and subsequent optic nerve involvement. The patient did not meet the criteria for multiple sclerosis or neuromyelitis optica, and other differentials were excluded. The diagnosis of MOGAD was confirmed by IgG antibodies against the MOG protein.

Discussion: Brainstem lesions in MOGAD have been described in 30% of a Spanish cohort of MOG-IgG positive patients. The lesions tend to be bilateral, poorly defined, and large. Although low titers on the assay may raise doubts about the diagnosis, Banwell et al recently proposed new diagnostic criteria that include low MOG-IgG titers. In the face of low titers, the supporting clinical criteria support the diagnosis.

Conclusions: Patients with signs of brainstem encephalitis without a clear etiology should be evaluated for MOG-associated antibodies to confirm or rule out the diagnosis.

https://doi.org/10.22379/anc.v40i2.1837

PDF (Español)
XML (Español)

References

Jarius S, Paul F, Aktas O, Asgari N, Dale RC, de Seze J, et al. MOG encephalomyelitis: international recommendations on diagnosis and antibody testing. J Neuroinflammation. 2018;15(1):134. https://doi.org/10.1186/s12974-018-1144-2

Jarius S, Kleiter I, Ruprecht K, Asgari N, Pitarokoili K, Borisow N, et al. MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 3: Brainstem involvement - frequency, presentation and outcome. J Neuroinflammation. 2016;13(1):281. https://doi.org/10.1186/s12974-016-0719-z

Denève M, Biotti D, Patsoura S, Ferrier M, Meluchova Z, Mahieu L, et al. MRI features of demyelinating disease associated with anti-MOG antibodies in adults. J Neuroradiol. 2019;46(5):312-8. https://doi.org/10.1016/j.neurad.2019.06.001

Banwell B, Bennett JL, Marignier R, Kim HJ, Brilot F, Flanagan EP, et al. Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria. The Lancet Neurology. 2023;22(3):268-82. https://doi.org/10.1016/S1474-4422(22)00431-8

Whittam DH, Cobo-Calvo A, Lopez-Chiriboga AS, Pardo S, Gornall M, Cicconi S, et al. Treatment of MOG-IgG-associated disorder with rituximab: an international study of 121 patients. Mult Scler Relat Disord. 2020;44:102251. https://doi.org/10.1016/j.msard.2020.102251

Whittam DH, Karthikeayan V, Gibbons E, Kneen R, Chandratre S, Ciccarelli O, et al. Treatment of MOG antibody associated disorders: results of an international survey. J Neurol. 2020;267(12):3565-77. https://doi.org/10.1007/s00415-020-10026-y

Polman, CH, Reingold SC, Banwell B, Clanet M, Cohen JA, Filippi M, et al. Diagnostic criteria for multiple sclerosis: 2010 Revisions to the McDonald criteria. Ann. Neurol. 2011;69(2): 292–302. https://doi.org/10.1002/ana.22366

Thompson AJ, Reingold SC, Cohen JA. Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria. Lancet Neurol. 2018; 17(6): 162–73. https://doi.org/10.1016/S1474-4422(18)30168-6

Hamid SHM, Whittam D, Mutch K, Linaker S, Solomon T, Das K, et al. What proportion of AQP4-IgG-negative NMO spectrum disorder patients are MOG-IgG positive? A cross sectional study of 132 patients. J Neurol. 2017;264(10):2088-94. https://doi.org/10.1007/s00415-017-8596-7

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Downloads

Download data is not yet available.