Myelopathy secondary to copper deficiency as a complication of treatment of Wilson's disease

Herrero, Jesús Lozano; Bertrán, Eduardo Muñoz; González, Isabel Ortega; Espín, Rosa Gómez; Espín, María Isabel López

doi:10.1016/j.gastrohep.2012.03.008

Cited by 18 publications

(21 citation statements)

References 18 publications

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“…Early diagnosis can be obtained in approximately 50% of cases with simultaneous measurement of copper and zinc plasma levels . When low urinary copper is detected during routine outpatient follow up, a MRI of the cervical spine may be warranted to investigate for a subclinical manifestation of subacute combined degeneration of the spinal cord as seen in this case . Spinal MRI has been shown to be abnormal in 47% of patients with copper‐deficiency myelopathy and usually shows high T2 signal in the posterior cervical and thoracic cord …”

Section: Discussionmentioning

confidence: 95%

“…Zinc blocks copper absorption from the intestinal tract and promotes copper excretion through the gut . However, overtreatment with zinc may lead to copper deficiency, which may cause neurological and haematological complications …”

Section: Discussionmentioning

confidence: 99%

“…Zinc toxicity can cause copper deficiency through entrapment of copper in the enterocytes, which can subsequently cause sideroblastic anaemia and subacute combined degeneration of the spinal cord . Copper deficiency may occur at differing times after the commencement of zinc therapy, and hence regular neurological examination, copper metabolism and haematological monitoring is necessary.…”

Section: Discussionmentioning

confidence: 99%

“…Physical examination revealed distal weakness of both legs, brisk knee reflexes, absent ankle reflexes bilaterally, absent vibration sense at the level of the big toe, ankle and knee, as well as diminished pain and touch sensation below the knees. These examination findings are considered classical signs of subacute combined degeneration of the spinal cord . Subsequent nerve conduction study for tibial, peroneal and sural nerve demonstrated decreased sensory nerve action potential and compound motor action potential, mildly reduced distal latencies and normal conduction velocity, F ‐wave and H‐reflex latencies.…”

mentioning

confidence: 95%

“…The patient was diagnosed with subacute combined degeneration of the spinal cord secondary to iatrogenic copper deficiency, as a result of zinc therapy for the treatment of Wilson disease . She was prescribed 10 mg copper daily for 6 months and, with allied health follow up for rehabilitation, made a complete recovery with no residual neurological deficits.…”

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Wilson disease: copper deficiency and iatrogenic neurological complications with zinc therapy

Ekladious

Wheeler

et al. 2020

Internal Medicine Journal

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A 17-year-old female was diagnosed with Wilson disease and commenced on oral zinc therapy. She re-presented 6 months later with a fall and had classical signs of subacute combined degeneration of the spinal cord confirmed on nerve conduction studies, as a result of zinc-induced copper deficiency. After 6 months of copper therapy, she made a complete recovery with no residual neurological deficits. Early detection of zincinduced copper deficiency and stringent follow-up mechanisms are crucial. Early initiation of copper replacement may both limit and completely reverse neurological deficits. AbstractClinical experience suggests higher occurrence of carbapenem-associated seizures in the elderly than what is reported in the available literature (range between 0.2% and 0.7%). An audit of 1345 patients with age 60 years or older, who received imipenem, ertapenem or meropenem during their acute hospitalisation found 32 (2.4%) subjects developed seizures. Subjects with more than one central nervous system disorders were 11.6 times more likely to develop seizures (odds ratio 11.61, P < 0.001) and subjects with prior history of seizures is associated with four times greater risks (odds ratio 4.02, P = 0.005). Physicians should exercise caution when prescribing carbapenems in elderly, especially those with known epilepsy and a high number of intracranial pathologies.

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 95%

mentioning

confidence: 99%

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Wilson disease: copper deficiency and iatrogenic neurological complications with zinc therapy

Ekladious

Wheeler

et al. 2020

Internal Medicine Journal

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Regional distribution of cytochrome c oxidase activity and copper in sclerotic hippocampi of epilepsy patients

Opačić

Ristić²,

Sokić³

et al. 2020

Brain and Behavior

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Introduction Disruption of copper homeostasis and dysfunction of mitochondria have been documented in sclerotic hippocampi (HS) of patients with mesial temporal lobe epilepsy (mTLE). However, a potential link between these pathological changes has not been tackled so far. Herein, we analyzed regional distribution of neuron somata density, copper concentration, and the activity of cytochrome c oxidase (CCO), a component of mitochondrial electron transport chain and copper‐containing metalloprotein, in HS. Methods Histochemical staining and laser ablation inductively coupled plasma mass spectrometry were carried out to construct comparable maps of these parameters in coronal sections of hippocampi of 3 mTLE‐HS patients and 3 control subjects. Results Copper levels were decreased in all regions of HS with pyramidal neuron somata. CCO activity was significantly reduced in stratum pyramidale (PY) 1 and cornu Ammonis field 4, the two regions with significant reduction in neuron somata density. CCO activity was also lower in layers that contain apical dendrites of pyramidal neurons and mossy fibers. It appears that copper deficiency in PY2 and PY3 comes before CCO activity reduction and neuronal loss. A strong positive correlation was found between neuron density, Cu concentration, and CCO activity. Conclusions Presented results imply that pathological alterations in Cu and energy metabolism could be involved in the development of HS. A limitation of this study was the relatively small number of patients. However, presented results underline copper deficiency as a component of pathological mechanisms of epilepsy and warrant further investigation of cuproproteins and members of copper transport machinery.

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Can Patients with Wilson's Disease Develop Copper Deficiency?

Chevalier

Obadia

Djebrani‐Oussedik

et al. 2023

Movement Disord Clin Pract

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BackgroundWilson's disease (WD) is a rare genetic condition characterized by a copper overload in organs secondary to mutation in ATP7B gene. Lifelong decoppering treatments are the keystone of the treatment but must be regularly adapted to obtain a correct copper balance and could lead to copper deficiency (CD).ObjectivesStudy the characteristics of CD in WD patients.MethodsCD cases from our cohort of 338 WD patients have been investigated. CD was defined by the association of serum copper, exchangeable copper and urinary copper excretion assays less than two standard deviations from the mean with cytopenia and/or neurological damage of spinal cord origin. A systematic review of literature about cases of CD in WD patient was performed in PubMed database according to PRISMA guidelines.ResultsThree WD patients were diagnosed with CD in our cohort. Review of the literature found 17 other patients. Most of the patients had anemia and neutropenia associated with neurological symptoms (especially progressive posterior cord syndrome). All the patients were treated with Zinc salts and the symptoms occurred more than a decade after the initiation of treatment. The adaptation of the treatment allowed a correction of the cytopenia but only a partial improvement of the neurological symptoms.ConclusionsWD patients can develop CD after many years of zinc therapy. Anemia and neutropenia are red flags that should evoke CD.

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Myelopathy secondary to copper deficiency as a complication of treatment of Wilson's disease

Cited by 18 publications

References 18 publications

Wilson disease: copper deficiency and iatrogenic neurological complications with zinc therapy

Wilson disease: copper deficiency and iatrogenic neurological complications with zinc therapy

Regional distribution of cytochrome c oxidase activity and copper in sclerotic hippocampi of epilepsy patients

Can Patients with Wilson's Disease Develop Copper Deficiency?

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