Introduction: Celiac disease (CD) is an immune-mediated disease triggered by the ingestion of gluten in genetically susceptible individuals. Neurological manifestations are rare and severe and must be sought systematically.
Clinical cases: Two non related patients each from a consanguineous marriage developed progressive spastic paraplegia 2 and 8 years respectively after onset of CD. The radiological and biological findings were normal except for the presence of abnormalities related to CD.
Conclusion: The relationship between spastic paraplegia and CD is not well established. Autoimmune, metabolic and genetic mechanisms could be considered but the probability of a fortuitous association should not be ruled out.