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隐匿性乳糜泻所致铜缺乏性脊髓神经病

Copper deficiency myeloneuropathy due to occult celiac disease.

作者信息

Goodman Brent P, Mistry Deven H, Pasha Shabana F, Bosch Peter E

机构信息

Departments of Neurology and daggerGastroenterology, Mayo Clinic, Scottsdale, AZ.

出版信息

Neurologist. 2009 Nov;15(6):355-6. doi: 10.1097/NRL.0b013e31819428a8.

Abstract

INTRODUCTION

Copper deficiency is an increasingly recognized cause of gait unsteadiness. Recognized causes of copper deficiency include excess zinc ingestion, and malabsorption. Although hematologic abnormalities have been attributed to copper deficiency in patients with celiac disease, myeloneuropathy due to copper deficiency has not been well described in patients with celiac disease.

CASE REPORT

A 69-year-old woman was evaluated for a 5-year history of progressive gait unsteadiness and weight loss. She had no other gastrointestinal symptoms. Her neurologic examination revealed a sensory ataxia, and electrodiagnostic testing confirmed a myeloneuropathy. She had decreased serum copper levels and markedly elevated gliadin and tissue transglutaminase antibodies. Subsequent duodenal biopsy showed findings consistent with celiac disease. The patient was diagnosed with copper deficiency myeloneuropathy due to celiac disease. Adoption of a gluten-free diet along with copper supplementation resulted in significant clinical improvement, including improvement on electrodiagnostic testing.

CONCLUSIONS

Celiac disease should be considered in patients found to have copper deficiency, even in patients without gastrointestinal symptoms. Furthermore, the authors suggest that some cases of ataxia associated with celiac disease are likely due to copper deficiency myeloneuropathy.

摘要

引言

铜缺乏是一种越来越被认识到的步态不稳的病因。已确认的铜缺乏病因包括过量摄入锌和吸收不良。虽然乳糜泻患者的血液学异常已归因于铜缺乏,但铜缺乏所致的脊髓神经病在乳糜泻患者中尚未得到充分描述。

病例报告

一名69岁女性因进行性步态不稳和体重减轻5年接受评估。她没有其他胃肠道症状。神经系统检查显示感觉性共济失调,电诊断测试证实为脊髓神经病。她的血清铜水平降低,麦醇溶蛋白和组织转谷氨酰胺酶抗体明显升高。随后的十二指肠活检结果与乳糜泻一致。该患者被诊断为乳糜泻所致的铜缺乏性脊髓神经病。采用无麸质饮食并补充铜后,临床症状有显著改善,包括电诊断测试结果的改善。

结论

即使在没有胃肠道症状的患者中,发现有铜缺乏时也应考虑乳糜泻。此外,作者认为,一些与乳糜泻相关的共济失调病例可能是由于铜缺乏性脊髓神经病所致。

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