Proximal Limb Weakness in a Patient with Celiac Disease: Copper Deficiency, Gluten Sensitivity, or Both as the Underlying Cause?

Abstract

Celiac disease has been associated with several neurologic disorders which may result from micronutrient deficiencies, coexisting autoimmune conditions, or gluten sensitivity. Copper deficiency can produce multiple neurologic manifestations. Myeloneuropathy is the most common neurologic syndrome and it is often irreversible, despite copper replacement. We report the case of a 55-year-old man who presented with progressive proximal limb weakness and weight loss in the setting of untreated celiac disease without gastrointestinal symptoms. He had anemia, neutropenia, and severe hypocupremia. The pattern of weakness raised the suspicion that there was an underlying myopathy, although this was not confirmed by electrodiagnostic studies. Weakness and hematologic abnormalities resolved completely within 1 month of total parenteral nutrition with copper supplementation and a gluten-free diet. Myopathy can rarely occur in patients with celiac disease, but the mechanism is unclear. Pure proximal limb weakness has not been previously reported in copper deficiency. We propose that this may represent a novel manifestation of hypocupremia and recommend considering copper deficiency and gluten sensitivity in patients presenting with proximal limb weakness.

Nutritional Deficiencies in Children with Celiac Disease Resulting from a Gluten-Free Diet: A Systematic Review

“…This hematological disorder may be fully resolved by administering copper supplements together with a GFD [66]. Hypocupremia should be investigated in patients with proximal limb weakness, even in the absence of clinically overt enteropathy [67]. Copper replacement combined with gluten restriction is likely to lead to the complete resolution of symptoms in such patients.…”

Enfermedad celíaca y desarrollo de patologías secundarias

“…La literatura científica y la práctica clínica respaldan ampliamente la deficiencia de vitaminas (D, E y complejo B) y minerales (cobre y zinc) como causa de trastornos nerviosos centrales y periféricos, incluyendo neuropatías, parestesias, ataxias, delirio, demencia y depresión, entre otros [29]. De hecho, se han reportado casos de neuropatías en pacientes con EC cuya causa se atribuye a la deficiencia de micronutrientes como el cobre y la vitamina E, producto de la malabsorción [30,31].…”