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Original Contribution
October 2005

Small-Fiber Neuropathy/Neuronopathy Associated With Celiac Disease: Skin Biopsy Findings

Author Affiliations

Author Affiliations: Department of Neurology, Weill Medical College of Cornell University (Drs Brannagan, Sander, R. L. Chin, Magda, and Latov); Division of Neuropathology, Department of Pathology (Drs Hays and S. S. Chin), and Division of Gastroenterology, Department of Internal Medicine, (Dr Green), College of Physicians and Surgeons, Columbia University, New York, NY.

Arch Neurol. 2005;62(10):1574-1578. doi:10.1001/archneur.62.10.1574

Background  Celiac disease (CD) is increasingly recognized in North America and is associated with a peripheral neuropathy.

Objective  To report the clinical characteristics and skin biopsy results in patients with CD and small-fiber neuropathy symptoms.

Design  Case series.

Setting  Academic peripheral neuropathy clinic.

Patients  Eight patients with CD and neuropathy symptoms.

Intervention  Three-millimeter punch biopsy using the panaxonal marker protein gene product 9.5 to assess epidermal nerve fiber (ENF) density and a gluten-free diet.

Main Outcome Measure  Clinical data and ENF density.

Results  All patients had asymmetric numbness and paresthesias. Three had more prominent involvement of hands than feet, and 3 had facial numbness. Celiac disease was diagnosed in 5 after their neuropathy began. The following serum antibody levels were elevated: tissue transglutaminase (n = 6), IgA gliadin (n = 4), and IgG gliadin (n = 7). Results of nerve conduction studies were normal in 7 patients. One patient had mildly reduced sural amplitudes. The ENF density was reduced in 5 patients. The ENF density was at the low limit of the normal range in 3 additional patients, 2 of whom had morphologic changes in axons. Three patients had decreased ENF density at the thigh or forearm, which was more severe than at the distal leg, compatible with a non–length-dependent process. Four reported improvement with a gluten-free diet. One had no improvement after 4 months. Symptoms developed in 2 while receiving a gluten-free diet.

Conclusions  Patients with CD may have a neuropathy involving small fibers, demonstrated by results of skin biopsy. The pattern of symptoms, with frequent facial involvement and a non–length-dependent pattern on skin biopsy findings, suggests a sensory ganglionopathy or an immune-mediated neuropathy. Improvement of symptoms in some patients after initiating a gluten-free diet warrants further study.