Acrodystrophic axonal polyneuropathy with celiac disease: a case report

S N Bardakov,S V Lapin,Minh Duc Tran,A N Moshnikova,E U Kalinina,E G Bogdanova,A V Bolekhan,B L Gavriluk

doi:10.1186/s13256-021-03171-z

Abstract

BackgroundPatients with celiac disease present with not only gastrointestinal symptoms but also extraintestinal manifestations such as anemia, osteopathy, dermatitis herpetiformis, and celiac neuropathy. Despite a fairly wide range of celiac neuropathies, we report a case of the acrodystrophic variant of celiac polyneuropathy, which has not been previously described.Case presentationA 41-year-old Ukrainian male suffered from symmetric, sensorimotor axonal polyneuropathy and encephalopathy associated with celiac disease, which is characterized by severe trophic disorders in the lower extremities (trophic ulcers, hyperkeratosis, and anhidrosis). Acrodystrophic changes in the lower extremities were due to both neurogenic and direct immunoinflammatory damaging effects. Clinical–electrophysiological dissociation was also noted, which was represented by a gross axonal lesion with the preservation of muscle strength. The absence of enteropathic manifestations was accompanied by the pronounced histological changes in the duodenal mucosa by IIIb stage of Marsh. A gluten-free diet in combination with membrane plasma exchange and intravenous pulse methylprednisolone was prescribed to reduce the severity of sensory disorders and regression of encephalopathy within 7 months.ConclusionCeliac disease may be a potential cause of neuropathy and encephalopathy in adult patients. Further immunosuppressive treatment protocols for both intestinal and extraintestinal manifestations of celiac disease are required.

Highlights

Patients with celiac disease present with gastrointestinal symptoms and extraintestinal manifestations such as anemia, osteopathy, dermatitis herpetiformis, and celiac neuropathy
The disease is based on the synthesis of immunoglobulin (Ig)A- and IgG-class antigliadin antibodies (AGAs) and deamidated gliadin peptides, anti-endomysial
Genetic predispositions related to the presence of the Human leukocyte antigen (HLA)-DQ2 and HLA-DQ8 alleles characterize 95% of patients with celiac disease [2]

Summary

Introduction

Patients with celiac disease present with gastrointestinal symptoms and extraintestinal manifestations such as anemia, osteopathy, dermatitis herpetiformis, and celiac neuropathy. Difficulties occur in cases of celiac neuropathy in the absence of intestinal manifestations, which is characteristic in one-third of adult patients [21], and leads to an average of a 9-year delay in diagnosis [3, 22]. Morphological analysis of the duodenal mucosa biopsy (Table 2, Fig. 2) identified changes (subatrophy of villi in combination with crypt hyperplasia) that correspond to celiac disease according to the Marsh IIIB classification.

Results

Conclusion