Acrodystrophic axonal polyneuropathy with celiac disease: a case report

Area of Science:

• Neurology
• Gastroenterology
• Immunology

Background:

• Celiac disease is linked to various neurological issues beyond gastrointestinal symptoms.
• Extraintestinal manifestations include anemia, osteopathy, dermatitis herpetiformis, and celiac neuropathy.

Observation:

• A 41-year-old male presented with sensorimotor axonal polyneuropathy and encephalopathy associated with celiac disease.
• The patient exhibited severe trophic disorders in the lower extremities, including ulcers and anhidrosis.
• Clinical-electrophysiological dissociation was observed, with significant axonal damage despite preserved muscle strength.

Findings:

• The patient had Marsh IIIb histological changes in the duodenum without overt enteropathic symptoms.
• Acrodystrophic changes were attributed to neurogenic and immunoinflammatory effects.
• Treatment with a gluten-free diet, plasma exchange, and methylprednisolone led to symptom improvement.

Implications:

• Celiac disease should be considered in adult patients presenting with neuropathy and encephalopathy.
• Effective treatment protocols for both intestinal and extraintestinal celiac disease manifestations are crucial.
• Further research into immunosuppressive therapies for celiac-related neurological disorders is warranted.