Background: Neurologic manifestations of primary Sjögren's syndrome (PSS) have been reported to vary from sensory polyneuropathy to encephalopathy or psychiatric problems. However, marked cerebellar degeneration associated with PSS has rarely been reported.
Case report: We describe a patient with Sjögren's syndrome who exhibited rapidly progressive cerebellar ataxia, nystagmus, cognitive decline, and psychiatric problems. Brain magnetic resonance imaging revealed marked atrophy of the cerebellum, and (18)F-fluorodeoxyglucose positron-emission tomography demonstrated glucose hypometabolism of the cerebellum.
Conclusions: Our PSS patient exhibited a progressive course of cerebellar syndrome, as evidenced by cerebellar atrophy on serial brain images.
Keywords: Sjögren's syndrome; ataxia; cerebellar degeneration; cognitive impairment.