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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2017; 75(01): 74-75
DOI: 10.1590/0004-282X20160169
Images in Neurology

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
Paulo Victor Sgobbi de Souza
1   Universidade Federal de São Paulo, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brasil
,
Thiago Bortholin
1   Universidade Federal de São Paulo, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brasil
,
Wladimir Bocca Vieira de Rezende Pinto
1   Universidade Federal de São Paulo, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brasil
,
Adrialdo José Santos
1   Universidade Federal de São Paulo, Departamento de Neurologia e Neurocirurgia, São Paulo SP, Brasil
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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.

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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.Anti-Ma2-associated encephalitis classically emerges in the context of seminomatous and nonseminomatous testicular tumors and presents with a complex spectrum of neurological manifestations[1], including limbic encephalitis, atypical parkinsonism, cerebellar ataxia, brainstem dysfunction, myelopathy, radiculoplexopathy, REM sleep behavior disorder and narcolepsy[1],[2].

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Conflict of interest:

There is no conflict of interest to declare.

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
  • 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

Address for correspondence

Wladimir Bocca Vieira de Rezende Pinto
Departamento de Neurologia e Neurocirurgia da UNIFESP; Rua Pedro de Toledo, 650; 04039-002 São Paulo SP
Brasil   

Publication History

Received: 11 July 2016

Accepted: 09 September 2016

Article published online:
06 September 2023

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