Small-Fiber Neuropathy/Neuronopathy Associated With Celiac Disease

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Vol. 62 No. 10, October 2005 Arch Neurol. 2005;62:1574-1578.

Small-Fiber Neuropathy/Neuronopathy Associated With Celiac Disease

Skin Biopsy Findings

H. Brannagan III, MD; Arthur P. Hays, MD; S. Chin, MD,

PhD; W. Sander, MD; L. Chin, MD; Magda, DO;

H. R. Green, MD; Norman Latov, MD, PhD

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.

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.