title: Subacute copper-deficiency myelopathy in a patient with occult celiac disease.
abstract: Acquired copper deficiency represents a rare cause of progressive myelopathy presenting with sensory ataxia and spastic gait. The time interval from neurological symptoms onset to diagnosis of myelopathy ranges from 2 months to several years in almost all cases, mimicking the clinical course of subacute combined degeneration due to vitamin B12 deficiency.
  
  
  
  A 60-year-old man, without any gastrointestinal symptoms, developed over the course of one week rapidly progressive gait imbalance, tingling and numbness in his feet and ascending lower limb weakness. Spine magnetic resonance imaging revealed hyperintensity involving cervical and dorsal posterior columns of spinal cord. Blood analysis revealed undetectable serum copper levels, low serum ceruloplasmin and positive serum Immunoglobulin A anti-tissue transglutaminase. Upper gastrointestinal endoscopy was performed revealing duodenal villous atrophy consistent with a malabsorption pattern. A gluten-free diet in association with intravenous then oral copper supplementation prompted sustained normalization of serum copper levels and progressive clinical improvement.
  
  
  
  We report a rare case of myelopathy induced by copper deficiency secondary to undiagnosed celiac disease, peculiarly presenting with a subacute onset. This case expands the neurological presentation and clinical course of myelopathy due to acquired copper deficiency. We suggest investigation of copper deficiency in patients presenting with subacute or even acute sensory ataxia and spastic gait. Detection of hypocupremia in patients without a previous history of gastric surgery should lead to diagnostic testing for celiac disease even in the absence of any obvious gastrointestinal symptoms.
authors.affiliation: a Department of Neuroscience , S. Agostino-Estense Hospital and University of Modena and Reggio Emilia , Via Pietro Giardini 1355 IT-41126 Modena , Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Cavallieri
authors.affiliation: a Department of Neuroscience , S. Agostino-Estense Hospital and University of Modena and Reggio Emilia , Via Pietro Giardini 1355 IT-41126 Modena , Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Fini
authors.affiliation: a Department of Neuroscience , S. Agostino-Estense Hospital and University of Modena and Reggio Emilia , Via Pietro Giardini 1355 IT-41126 Modena , Italy.
authors.forename: Sara
authors.initials: S
authors.lastname: Contardi
authors.affiliation: b Division of Internal Medicine 2, University Hospital of Modena; Department of Medical and Surgical Sciences for Children and Adults , University of Modena and Reggio Emilia , Italy.
authors.forename: Massimo
authors.identifier: 0000-0002-9165-539X
authors.initials: M
authors.lastname: Fiorini
authors.affiliation: b Division of Internal Medicine 2, University Hospital of Modena; Department of Medical and Surgical Sciences for Children and Adults , University of Modena and Reggio Emilia , Italy.
authors.forename: Elena
authors.identifier: 0000-0001-9477-2164
authors.initials: E
authors.lastname: Corradini
authors.affiliation: a Department of Neuroscience , S. Agostino-Estense Hospital and University of Modena and Reggio Emilia , Via Pietro Giardini 1355 IT-41126 Modena , Italy.
authors.forename: Franco
authors.initials: F
authors.lastname: Valzania
mesh_terms: D002446:Celiac Disease / Q000150:complications* / Q000175:diagnosis; D003300:Copper / Q000172:deficiency*; D006801:Humans; D008297:Male; D008875:Middle Aged; D013118:Spinal Cord Diseases / Q000150:complications / Q000175:diagnosis / Q000209:etiology*
chemical_list: D003300:Copper
keywords: Celiac disease; Copper; Neurophysiology; Spinal cord diseases