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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2020; 78(11): 737
DOI: 10.1590/0004-282X20200091
Images in Neurology

Anti-Ri autoimmune encephalitis associated with breast cancer

Encefalite autoimune Anti-Ri associada a câncer de mama

Authors

  • Euripedes Gomes de CARVALHO  NETO

    1   Irmandade da Santa Casa de Misericórdia de Porto Alegre, Departamento de Neurologia, Porto Alegre RS, Brazil.

  • Matheus Ferreira GOMES 

    1   Irmandade da Santa Casa de Misericórdia de Porto Alegre, Departamento de Neurologia, Porto Alegre RS, Brazil.

  • Ronnie Peterson Marcondes ALVES 

    2   Irmandade da Santa Casa de Misericórdia de Porto Alegre, Departamento de Radiologia, Porto Alegre RS, Brazil.

  • Alexei Peter dos SANTOS 

    3   Irmandade da Santa Casa de Misericórdia de Porto Alegre, Departamento de Oncologia Clínica, Porto Alegre RS, Brazil.

  • Cristiano BRUM 

    4   Irmandade da Santa Casa de Misericórdia de Porto Alegre, Departamento de Psiquiatria, Porto Alegre RS, Brazil.

  • Ricardo SANTIN 

    1   Irmandade da Santa Casa de Misericórdia de Porto Alegre, Departamento de Neurologia, Porto Alegre RS, Brazil.
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A 73-year-old woman with breast cancer presented with asthenia, lethargy and imbalance, followed by bilateral ptosis and ophthalmoparesis. There was progression to tetraparesis. Magnetic resonance imaging (MRI) of the brain was performed ([Figure 1]). Cerebrospinal fluid (CSF) showed six leukocytes (with predominance of lymphocytes). The Anti-Ri neuronal antibody was positive. The patient presented respiratory distress, requiring orotracheal intubation. Despite pulse therapy and intravenous immunoglobulin, she presented systemic worsening and died.

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Figure 1 Magnetic resonance imaging of the brain. Axial fluid-attenuated inversion recovery (A, B, and C) and T2 weighted (a, b, and c) images showing areas of signal hyperintensity involving formations of the pontine tegmentum in the bulbar region (A, a, B, and b), periaqueductal gray region, and mesial temporal lobes, including the hippocampus (B) and the subcortical region of the superior frontal gyrus extending to the paracentral lobe (C and c).

Anti-Ri antibodies can produce heterogeneous paraneoplastic neurological syndrome. Involvement of the brainstem can lead to ophthalmoplegia, truncal ataxia, opsoclonus-myoclonus syndrome, and decreased central respiratory drive. Anti-Ri encephalitis requires extensive and urgent investigation of the underlying tumor whose treatment is imperative[1],[2],[3],[4].


Conflict of interest:

There is no conflict of interest to declare.

Authors’ contributions:

EGCN, MFG, RPMA, APS and CB: data acquisition and literature review. RS: critical revision of the manuscript for intellectual content and study supervision.


  • References

  • 1 Tay JK, Miller J, Joshi A, Athey RJ. Anti-Ri-associated paraneoplastic cerebellar and brainstem degenerative syndrome. J R Coll Physicians Edinb. 2012;42(3):221-4. https://doi.org/10.4997/JRCPE.2012.307
  • 2 Kim H, Lim Y, Kim K. Anti-Ri-antibody-associated paraneoplastic syndrome in a man with breast cancer showing a reversible pontine lesion on MRI. J Clin Neurol. 2009 Sep;5(3):151-2. https://doi.org/10.3988/jcn.2009.5.3.151
  • 3 Murphy BL, Zalewski NL, Degnim AC, McKeon A, Flanagan EP, Pittock SJ, et al. Breast cancer-related paraneoplastic neurologic disease. Breast Cancer Res Treat. 2018 Feb;167(3):771-8. https://doi.org/10.1007/s10549-017-4566-0
  • 4 Butt R, Vatandoust S, Kleinig T, Adams J, Basu S, Otto G, et al. Anti-Ri antibody-associated paraneoplastic brainstem encephalitis successfully treated after treating the underlying malignancy with letrozole. Intern Med J. 2013 May;43(5):605-6. https://doi.org/10.1111/imj.12123

Address for correspondence

Euripedes Gomes de Carvalho Neto

Publication History

Received: 10 June 2020

Accepted: 15 June 2020

Article published online:
07 June 2023

© 2020. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Tay JK, Miller J, Joshi A, Athey RJ. Anti-Ri-associated paraneoplastic cerebellar and brainstem degenerative syndrome. J R Coll Physicians Edinb. 2012;42(3):221-4. https://doi.org/10.4997/JRCPE.2012.307
  • 2 Kim H, Lim Y, Kim K. Anti-Ri-antibody-associated paraneoplastic syndrome in a man with breast cancer showing a reversible pontine lesion on MRI. J Clin Neurol. 2009 Sep;5(3):151-2. https://doi.org/10.3988/jcn.2009.5.3.151
  • 3 Murphy BL, Zalewski NL, Degnim AC, McKeon A, Flanagan EP, Pittock SJ, et al. Breast cancer-related paraneoplastic neurologic disease. Breast Cancer Res Treat. 2018 Feb;167(3):771-8. https://doi.org/10.1007/s10549-017-4566-0
  • 4 Butt R, Vatandoust S, Kleinig T, Adams J, Basu S, Otto G, et al. Anti-Ri antibody-associated paraneoplastic brainstem encephalitis successfully treated after treating the underlying malignancy with letrozole. Intern Med J. 2013 May;43(5):605-6. https://doi.org/10.1111/imj.12123

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Zoom
Figure 1 Magnetic resonance imaging of the brain. Axial fluid-attenuated inversion recovery (A, B, and C) and T2 weighted (a, b, and c) images showing areas of signal hyperintensity involving formations of the pontine tegmentum in the bulbar region (A, a, B, and b), periaqueductal gray region, and mesial temporal lobes, including the hippocampus (B) and the subcortical region of the superior frontal gyrus extending to the paracentral lobe (C and c).