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