Cerebellar degeneration associated with Sjögren's syndrome.

Background Neurologic manifestations of primary Sjogren'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 Sjogren'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 F-18-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. J Clin Neurol 2012;8:155-159