J Clin Neurol.  2012 Jun;8(2):155-159.

Cerebellar Degeneration Associated with Sjogren's Syndrome

Affiliations
  • 1Department of Health Screening and Promotion Center, Asan Medical Center, Seoul, Korea.
  • 2Department of Neurology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. sjchung@amc.seoul.kr
  • 3Department of Neurology, Cheongshim International Medical Center, Gapyeong, Korea.

Abstract

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 18F-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.

Keyword

Sjogren's syndrome; cerebellar degeneration; ataxia; cognitive impairment

MeSH Terms

Ataxia
Atrophy
Brain
Cerebellar Ataxia
Cerebellar Diseases
Cerebellum
Glucose
Humans
Magnetic Resonance Imaging
Neurologic Manifestations
Polyneuropathies
Positron-Emission Tomography
Sjogren's Syndrome
Glucose
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