Cerebellar involvement in patients withprimary Sjögren’s syndrome: diagnosis and treatment

The aim of this study is to describe the clinical features of cerebellar involvement in patients with primary Sjögren’s syndrome (pSS). We retrospectively analyzed the manifestations, treatments, and outcomes in patients with pSS-cerebellar complication in Peking Union Medical College Hospital and cases reported in literature. Altogether 13 patients were identified. They were 2 males and 11 females with a mean age at disease onset of 45.2 ± 14.6 years. Nine (69.2%) patients went to the clinic because of ataxia, and pSS was not suspected until accidental screening for autoantibodies. Dysarthria (7, 59.8%), limb tremor (4, 30.8%), and nystagmus (2, 15.4%) were the rest symptoms related to cerebellum. Of the patients, 81.8% (9/11) had abnormal cerebrospinal fluid findings, and 11 patients (84.6%) had cerebellar atrophy in the brain MRI. Dry eyes and dry mouth were detected in 9 (69.2%) and 7 (59.8%) patients, while positive objective xerostomia and ocular test in 82.5% (7/8) and 100% (10/10) of the patients, respectively. Anti-Ro/SSA antibody was positive in 12 (92.3%) and anti-La/SSB in 6 (46.2%) patients. Glucocorticoids were applied in 12 patients (92.3%). Cyclophosphamide (3, 20.1%), mycophenolatemofetil (1, 7.7%), and hydroxychloroquine (4, 30.8%) were chosen as immunosuppressants or anti-inflammatory drug. During a median follow-up of 9 months (range, 1–18 months), 8 (61.5%) patients remained stable, 3 (20.1%) patients were in remission, and 2 (15.4%) patients were in progression. Clinical cerebellar complication secondary to pSS was rare, and sometimes pSS was not suspected until accidental screening for autoantibodies. Because the onset of cerebellar manifestation is often insidious and rapid deteriorates, early diagnosis and empirical aggressive glucocorticoid treatment is warranted.