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Progressive hearing loss and cerebellar ataxia in anti-Ma2-associated autoimmune encephalitis

Perda auditiva progressiva e ataxia cerebelar na encefalite autoimune associada ao anti-Ma2

A 38-year-old Brazilian man presented with a two-year history of progressive bilateral hearing loss, cerebellar ataxia, emotional lability and hypersexuality. A limbic-brainstem syndrome was suspected and neuroimaging studies per-formed, disclosing marked brainstem signal changes (Figure). Cerebrospinal fluid analysis disclosed a mild protein increase. During paraneoplastic screening, testicular ultrasonography disclosed a heterogeneous complex mass in the right testicle (Figure) and serum anti-Ma2 antibodies were detected.

Figure
Brain MR imaging studies disclosing cortical atrophy, bilateral hyperintensity in the mesial temporal lobes and marked hyperintense signal change in the pons and superior cerebellar peduncles in axial FLAIR sequence (A,B) and axial (C) and coronal T2-weighted images (D). (E) Ultrasonography of the testis disclosing a heterogeneous complex mass in the right testicle with the presence of flow inside the lesion in the Doppler study.
  • Ethical statement: Full consent was obtained from the patient's family for the case report. This study was approved by our Ethics Institution.

References

  • 1
    Dalmau J, Graus F, Villarejo A, Posner JB, Blumenthal D, Thessen B et al. Clinical analysis of anti-Ma2-associated encephalitis. Brain. 2004;12(8)7:1831-44. doi: 10.1093/brain/awh203
    » https://doi.org/10.1093/brain/awh203
  • 2
    Mrabet S, Ben Achour N, Kraoua I, Benrhouma H, Klaaa H, Rouissi A et al. Anti-Ma2-encephalitis in a 2 year-old child: a newly diagnosed case and literature review. Eur J Paediatr Neurol. 2015;19(6):737-42. doi: 10.1016/j.ejpn.2015.06.005
    » https://doi.org/10.1016/j.ejpn.2015.06.005

Publication Dates

  • Publication in this collection
    Jan 2017

History

  • Received
    11 July 2016
  • Reviewed
    03 Sept 2016
  • Accepted
    09 Sept 2016
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