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From the Department of Neurology and Neuroscience (Drs. Chin, Sander, Brannagan, Alaedini, and Latov), Weill Medical College of Cornell University, New York, NY; Department of Neurology (Dr. Sander), New York University School of Medicine, New York, NY; Department of Neurology (Dr. Sander), New York Medical College, Valhalla, NY; and Departments of Medicine and Celiac Disease Center (Dr. Green) and Pathology (Dr. Hays), College of Physicians and Surgeons, Columbia University, New York, NY.
Address correspondence and reprint requests to Dr. Russell L. Chin, Peripheral Neuropathy Center, Weill Medical College of Cornell University, Department of Neurology, 635 Madison Avenue, Suite 400, New York, NY 10022; e-mail: RUC9002{at}med.cornell.edu
Background: Celiac disease (CD) is a chronic inflammatory enteropathy resulting from sensitivity to ingested gluten. Neurologic complications are estimated to occur in 10% of affected patients, with ataxia and peripheral neuropathy being the most common problems. The incidence and clinical presentation of patients with CD-associated peripheral neuropathy have not previously been investigated.
Objective: To determine the incidence of CD in patients with neuropathy and to characterize the clinical presentation.
Methods: The records of 20 patients with neuropathy and biopsy-confirmed CD were reviewed.
Results: Six of the 20 patients had neuropathic symptoms alone without gastrointestinal involvement, and neuropathic symptoms preceded other CD symptoms in another 3 patients. All patients had burning, tingling, and numbness in their hands and feet, with distal sensory loss, and nine had diffuse paresthesias involving the face, trunk, or lumbosacral region. Only two had weakness. Results of electrophysiologic studies were normal or mildly abnormal in 18 (90%) of the patients. Sural nerve biopsies, obtained from three patients, revealed mild to severe axonopathy. Using the agglutination assay, 13 (65%) of the patients were positive for ganglioside antibodies. Excluding patients who were referred with the diagnosis of celiac neuropathy, CD was seen in approximately 2.5% of all neuropathy patients and in 8% of patients with neuropathy and normal electrophysiologic studies seen at our center.
Conclusion: CD is commonly associated with sensory neuropathy and should be considered even in the absence of gastrointestinal symptoms.
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