Session Time: 12:00pm-1:30pm
Location: Exhibit Hall located in Hall B, Level 2
Objective: To report a case of acute ataxia with cerebellar atrophy as the presenting feature of Sjögren’s syndrome (SS).
Background: Cerebellar ataxia is rare in SS, and in previously reported cases it is associated with MRI findings suggestive of inflammation, such as FLAIR hyperintense lesions and gadolinium enhancement.
Methods: Case report.
Results: A previously healthy 36 year-old female noticed mild dysarthria in the last trimester when pregnant with her third child and failed to seek medical attention. Three days after delivery she suddenly became unsteady, her dysarthria worsened and she reported dysphagia and tremor. Neurological examination revealed generalised ataxia with scanning speech, bilateral dysmetria, dysdiadococinesia and intention tremor in the limbs. The rest of the neurological exam was unremarkable. Brain MRI revealed cerebellar atrophy, with no other abnormalities. Results of HIV and hepatitis serology, anti-glutamate decarboxylase, anti-endomysial and antigliadin antibodies were negative. Cerebrospinal fluid (CSF) analysis showed no alterations. Antinuclear antibodies were positive with a 1:320 titre, with a positive anti-SSA/Ro. Despite denying sicca symptoms, a tear breakup time test (TBUT) of 5 seconds in both eyes and a Sicca score of 3 in both eyes confirmed keratoconjunctivitis sicca. Labial salivary gland biopsy revealed focal lymphocytic sialidenitis, a finding consistent with the diagnosis of Sjögren syndrome. Treatment was started with monthly cycles of high-dosage methylprednisolone and cyclophosphamide.
Conclusions: We conclude that cerebellar degeneration, albeit rare, is a possible neurological manifestation of SS. We suggest that screening for SS should be included in the evaluation of patients with acute onset sporadic cerebellar degeneration, even without signs of inflammation on imaging or CSF analysis.
To cite this abstract in AMA style:R. Maciel, S. Camargos, F. Cardoso. Acute cerebellar degeneration as the first manifestation of Sjögren syndrome: A case report [abstract]. Mov Disord. 2016; 31 (suppl 2). https://www.mdsabstracts.org/abstract/acute-cerebellar-degeneration-as-the-first-manifestation-of-sjgren-syndrome-a-case-report/. Accessed September 25, 2023.
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