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

Cerebellar Degeneration Associated with Sjogren's Syndrome

• Kim MJ
• Lee MC
• Lee JH
• Chung SJ

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

Figure

• 

  Fig. 1 Brain MRI and 18F-FDG PET results for the patient. A-D: Initial brain MRI was unremarkable. E-H: Follow-up brain MRI, performed 6 months later, revealed marked cerebellar atrophy and an enlarged fourth ventricle and cisterna magna without cerebral cortical atrophy. I: 18F-FDG PET, performed at the same time as the follow-up MRI, revealed decreased glucose metabolism in the bilateral cerebellum. 18F-FDG PET: 18F-fluorodeoxyglucose positron-emission tomography.

• 

  Fig. 2 Salivary scan and biopsy of the salivary gland. A: Salivary scan showing no uptake of the parotid and submandibular glands. B: Biopsy of a minor salivary gland showing focal lymphocytic infiltration. ANT: anterior, RAO: right anterior oblique, Rt. LAT: right lateral.

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