[Primary Gougerot-Sjogren's syndrome presenting as a cerebellar syndrome]

Rev Neurol (Paris). 2007 Dec;163(12):1242-5. doi: 10.1016/S0035-3787(07)78411-4.
[Article in French]

Abstract

Primary Sjogren's syndrome is an autoimmune exocrinopathy with various extraglandular complications. Central neurological manifestations were described in 20 percent of cases. Cerebellar syndrome is uncommon in Sjogren's syndrome, but can occur after diagnosis, or rarely as an inaugural sign of the disease. We report the case of a 54-year-old man who presented a cerebellar syndrome with xerostomy and xerophthalmos. The diagnosis of primary Sjorgren's syndrome was confirmed on the accessory salivary gland biopsy. The patient received oral and intravenous corticosteroid therapy associated with cyclophosphamide monthly. Outcome was good.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Anti-Inflammatory Agents / therapeutic use
  • Biopsy
  • Cerebellar Diseases / diagnosis
  • Cerebellar Diseases / pathology*
  • Cyclophosphamide / therapeutic use
  • Humans
  • Immunoelectrophoresis
  • Immunoglobulin G / immunology
  • Immunosuppressive Agents / therapeutic use
  • L-Lactate Dehydrogenase / blood
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Salivary Glands / pathology
  • Sjogren's Syndrome / diagnosis
  • Sjogren's Syndrome / pathology*

Substances

  • Adrenal Cortex Hormones
  • Anti-Inflammatory Agents
  • Immunoglobulin G
  • Immunosuppressive Agents
  • Cyclophosphamide
  • L-Lactate Dehydrogenase