Evidence map›Paper›PMID 40087235›Full record

ArticleActa neurologica Belgica2025

Characterization of nerve biopsy in copper deficiency peripheral neuropathy due to over-treatment of Wilson's disease: A case report.

Cuilin Peng, Wei Li, Haishan Jiang, Zichang Yin, Zhihua Zhou

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Article in Acta neurologica Belgica, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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5 authors.

Cuilin PengDepartment of Neurology, the First Affiliated Hospital, School of Clinical Medicine of Guangdong Pharmaceutical University, Guangzhou, China.
Wei LiDepartment of Neurology, Nanfang Hospital, Southern Medical University, Guangzhou, China.
Haishan JiangDepartment of Neurology, Nanfang Hospital, Southern Medical University, Guangzhou, China.
Zichang YinDepartment of Pathology Guangzhou, Guangzhou Kingmed Laboratory Center, Guangzhou, China.
Zhihua ZhouDepartment of Neurology, the First Affiliated Hospital, School of Clinical Medicine of Guangdong Pharmaceutical University, Guangzhou, China. zhouzh20051103@163.com.

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6 · The paper itself

Abstract

backgroundCopper deficiency peripheral neuropathy caused by excessive treatment in Wilson's disease has been reported. But its pathological characteristics are rarely reported. Here, we report a case of copper deficiency peripheral neuropathy in Wilson's disease and present the characteristics of its nerve biopsy. CASE DESCRIPTION: A 44-year-old female patient diagnosed with Wilson's disease was on long-term oral administration of zinc gluconate and copper chelators. In June 2022, she developed symptoms of peripheral neuropathy, accompanied by anemia and neutropenia. The 24-hour urinary copper excretion was 53.85 µg. Serum copper was 66.6 µg/L, and ceruloplasmin was 0.01 g/L. Electromyography showed length-dependent axonal damage in peripheral nerves. Sural nerve biopsy showed mainly axonal damage and decreased density of nerve fibres of all classes (large and small myelinated and unmyelinated), accompanied by demyelination. Ultimately, the diagnosis of copper deficiency peripheral neuropathy caused by excessive treatment of Wilson's disease was established. After discontinuing copper chelation therapy, she experienced mild alleviation of symptoms.

conclusionThe pathological features of copper deficiency peripheral neuropathy in Wilson's disease include the formation of axonal vacuoles, the presence of myelin ovoids, and a reduced number of large and small myelinated and unmyelinated nerve fibers. This case emphasizes the importance of monitoring copper metabolism during the stable phase of treatment for patients with Wilson's disease.

Indexed as

Chelating AgentsCopperGluconatesHepatolenticular DegenerationPeripheral Nervous System DiseasesSural NerveAdultBiopsyFemaleHumansChelating AgentsCopperGluconatesAnti-GM4 antibodiesCopper deficiency peripheral neuropathySural nerve biopsyWilson’s disease

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