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Unusual presentation of more common disease/injury
CASE REPORT
Cerebellar degeneration in primary Sjӧgren syndrome
  1. Mohammad Heidary1,
  2. Samira Alesaeidi2,3,
  3. Khashayar Afshari4
  1. 1 Department of Internal Medicine, Tehran University of Medical Sciences, Tehran, The Islamic Republic of Iran
  2. 2 Amir Alam Research Center, Tehran University of Medical Sciences, Tehran, Tehran, Iran
  3. 3 Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Tehran, Iran
  4. 4 School of Medicine, Tehran University of Medical Sciences, Tehran, The Islamic Republic of Iran
  1. Correspondence to Dr Samira Alesaeidi, s-alesaeidi{at}sina.tums.ac.ir

Summary

Neurological manifestations are reported as a consequence of primary Sjӧgren syndrome (PSS). Any part of the brain and peripheral nervous system can be involved in PSS. However, cerebellar degeneration and atrophy associated with PSS have been rarely reported. Our report describes a 22-year-old woman who presented with cerebellar ataxia, arthritis and arthralgia. Evaluation of her symptoms, autoantibodies and salivary gland pathology was in favour of the diagnosis of Sjögren syndrome. Also, her brain MRI revealed cerebellar degeneration. There are only four patients reported to be affected by cerebellar atrophy associated with PSS. Administration of high doses of methylprednisolone and cyclophosphamide leads to substantial improvement in the cerebellar symptoms of this case. In addition, after 2 months of follow-up, the patient’s ataxia recovered significantly. It could be concluded that in addition to neurological degenerative disorders, in some cases cerebellar atrophy could also be associated with autoimmune conditions such as PSS.

  • sjogren’s syndrome
  • brain stem / cerebellum
  • movement disorders (other than parkinsons)

https://doi.org/10.1136/bcr-2017-223952

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    Footnotes

    • Contributors MH contributed to taking the clinical history and examination of the patient while helping SA in the management and treatment of the patient. KA contributed to writing and final editing and submission of the manuscript. Also, both SA and MH contributed to the writing of the manuscript and its submission.

    • Funding The authors declare that there is no specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.