Sensory ataxic neuropathy and esophageal achalasia in a patient with Sjogren's syndrome

Muscle Nerve. 2007 Apr;35(4):532-5. doi: 10.1002/mus.20716.

Abstract

We describe a patient who developed an ataxic sensory syndrome associated with xerophthalmia and progressive dysphagia with regurgitation. Electrophysiological findings were consistent with an axonal sensory neuropathy, and superficial peroneal nerve biopsy showed a reduction in number of myelinated fibers with epineurial inflammation. Rheumatoid factor, anti-SSA/SSB and antinuclear antibodies were positive and a diagnosis of Sjogren's syndrome was made. An endoscopic investigation revealed esophageal achalasia. We suggest that there may be a common autoimmune mechanism directed to different targets on the basis of this rare association.

Publication types

  • Case Reports

MeSH terms

  • Afferent Pathways / pathology
  • Afferent Pathways / physiopathology
  • Ataxia / diagnosis
  • Ataxia / immunology
  • Ataxia / physiopathology
  • Autoantibodies / analysis
  • Autoantibodies / immunology
  • Biomarkers / analysis
  • Esophageal Achalasia / diagnosis*
  • Esophageal Achalasia / immunology
  • Esophageal Achalasia / physiopathology*
  • Esophagus / innervation
  • Esophagus / physiopathology
  • Female
  • Humans
  • Middle Aged
  • Nerve Fibers, Myelinated / pathology
  • Peripheral Nerves / pathology
  • Peripheral Nervous System Diseases / diagnosis*
  • Peripheral Nervous System Diseases / immunology
  • Peripheral Nervous System Diseases / physiopathology*
  • Peroneal Nerve / pathology
  • Peroneal Nerve / physiopathology
  • Sensation Disorders / diagnosis
  • Sensation Disorders / immunology
  • Sensation Disorders / physiopathology
  • Sjogren's Syndrome / complications*
  • Sjogren's Syndrome / diagnosis
  • Sjogren's Syndrome / physiopathology*
  • Spinal Cord / pathology
  • Spinal Cord / physiopathology

Substances

  • Autoantibodies
  • Biomarkers