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BACKGROUND: Copper deficiency myelopathy (CDM) is a rare disease that can present with spastic quadriparesis and sensory ataxia. As a result, it can precisely mimic cervical spondylitic myelopathy (CSM). Copper deficiency may be seen following gastric bypass surgery, malabsorption syndromes such as celiac disease, and with excessive exogenous zinc intake. We present a systematic review of the literature for CDM and an illustrative case.
OBJECTIVE: Provide a systematic review of CDM to highlight the importance of recognizing the consideration of CDM in patients presenting to a spine surgeon with myelopathy that progress despite adequate surgical decompression, or myelopathy concomitant with cytopenia, thus requiring further workup.
METHODS: Retrospective medical record review and systematic review of the literature PATIENT SAMPLE: PubMed and Ovid-Embase database search was conducted in July 2022 OUTCOME MEASURES: Self-reported measures include PRISMA flow diagram for retrospective review; Physiological measures include retrospective review of MRI imaging of cervical spine; alternate demographic and laboratory value data extracted via literature review METHODS: A PubMed and Ovid-Embase database search was conducted in July 2022 searching for \"copper deficiency myelopathy (MeSH)\" from 2000 to 2022 via PRISMA guidelines. Following title and abstract review, the following data was extracted from full text: age, sex, etiology, hematological values upon presentation (mean corpuscular volume, white blood count, platelet count, and hemoglobin level), metal serum studies (serum copper, ceruloplasmin, and zinc), 24-hour collection of copper and zinc, and distinct radiographic findings on MRI.
RESULTS: A total of 116 studies were included in this review which contained 198 cases of copper deficiency myelopathy. The mean age was 53.57 ± 14.14 years, with the majority being females (63.8%). The most common etiology was prior gastric surgery (n=55, 36.2 %) followed by excessive zinc consumption from the use of zinc denture cream (n=39, 19.9%). The mean serum copper was 15.67 ± 17.84 (normal=80.0-155.0) mcg/dL and mean ceruloplasmin was 6.43 ± 5.25 (normal=16-45) mg/dL. In spite of appropriate treatment with copper supplementation, only 47 cases (24%) reported improvement in neurological status, and only 10 (5.1%) recovered to baseline. A hyperintense T2 signal abnormality resembling an inverted \"v\" in the dorsal columns was the most common radiographic abnormality.
CONCLUSIONS: Pertinent risk factors for copper deficiency myelopathy include prior upper gastrointestinal surgery, zinc excess, and malabsorption. Characteristic laboratory and imaging findings include cytopenia, low serum copper and ceruloplasmin, and distinct inverted \"v\" T2 signal hyperintensity in the dorsal columns. The neurologic deterioration with copper deficiency will progress in spite of decompressive surgery, and can be devastating and irreversible even with copper supplementation, reinforcing the importance of early detection. We thus recommend patients with myelopathy presenting with a history of gastric bypass, malabsorption syndromes, excessive zinc exposure, cytopenia, or imaging resembling an inverted \"v\" shaped hyperintense T2 MRI signal in the dorsal columns, should first undergo blood tests for copper, ceruloplasmin, and B12 levels prior to surgical consideration.

The symptoms of transverse myelitis, an acute demyelinating inflammatory condition of the spinal cord, include motor, sensory, and bowel-bladder dysfunction that can develop suddenly or gradually. Several etiologies, such as bacterial, fungal, or viral infections, cancer, autoimmune diseases, vascular problems, and environmental variables, can cause it. The identification of copper deficiency myelopathy (CDM) as a curable cause of non-compressive inflammatory myelopathy has only occurred recently. Patients frequently present with sensory complaints and a spastic gait. The neurological disease may exist independently of the hematologic signs. Only a few cases of copper myelopathy in peripartum women have been documented. Given that hypocupric myelopathy is a treatable cause of debilitating paraplegia, maintaining clinical vigilance will be crucial in minimizing neurological sequelae, as demonstrated in this case report.

The objective is to investigate the presentation, complications, management, and outcomes of copper deficiency-induced neurological pathologies due to Wilson disease (WD) overtreatment. We examined the case of a WD patient who developed a low thoracic dorsal myelopathy due to chronic hypocupremia from excessive zinc therapy. A comprehensive literature review was conducted to identify similar cases. Ten additional cases of neurological pathology resulting from copper deficiency in the context of WD over-treatment were identified, all occurring during therapy with zinc salts. Myelopathy and peripheral neuropathy were the most common complications, while two additional groups reported leukoencephalopathy. Early cytopenia was often associated with copper deficiency-related neurological pathology appearing early in the context of copper deficiency. WD patients undergoing treatment, especially with zinc salts, should be closely monitored to prevent over-treatment and the consequent copper deficiency. Regular complete blood counts could provide early detection of copper deficiency, avoiding irreversible neurological damage. Swift recognition of new neurological signs not consistent with WD and timely discontinuation of the decoppering therapy are critical for improving outcomes. The optimal management, including the potential benefit of copper supplementation in patients with WD and subsequent therapy adjustments, remains unclear and necessitates further investigation. Despite the general poor functional neurological outcomes, there were some exceptions that warrant further exploration.

We present the case of a 68-year-old man with progressive brachial and crural hypoaesthesia with gait ataxia suggesting subacute myelopathy of the posterior cords, demonstrated by MRI. After blood tests, a diagnosis of copper deficiency was made following zinc intoxication, secondary to the use of denture glue containing zinc. Treatment was started with copper and the dental glue was removed. Rehabilitation treatment was started with physiotherapy, hydrotherapy and occupational therapy. Functional improvement was achieved, going from an ASIAD level C4 to an ASIAD level C7 spinal cord injury. Copper levels should be studied in all non-compressive myelopathies of subacute onset if there is clear involvement of the posterior cords. Copper deficiency in analysis would establish the diagnosis. Rehabilitative treatment, supplementary copper supplementation and zinc withdrawal are essential to prevent irreversible neurological damage.

We are presenting six cases of patients with peripheral polyneuropathy due to malnutrition in settings of prior history of gastric bypass surgery, zinc-based dentures usage, or long-standing alcohol abuse. The clinical presentation in all six patients included sensory, motor, or combined peripheral polyneuropathy and gait instability due to imbalance. All patients included in this case series were found to have low copper levels. Electromyography (EMG) with nerve conduction study (NCS) showed predominantly axonal and length dependent sensory or sensory-motor polyneuropathies. Patients were treated with copper supplements with reportable improvement in their presenting symptoms.

Copper deficiency is an acquired condition that can lead to neurologic dysfunctions, such as myelopathy, motor neuron impairment, polyneuropathy, cognitive impairment, and optic nerve neuropathy. Associated biological findings are low serum copper and ceruloplasmin levels with low copper urinary excretion. We report the case of a previously healthy 59-year-old man who presented a complex neurological picture starting with symptoms and radiological signs consistent with degenerative myelopathy in the presence of persisting low serum copper and ceruloplasmin despite oral and intravenous copper supplementation. Over time, his symptoms evolved into a motor neuron disease evocating an amyotrophic lateral sclerosis (ALS) phenotype. The potential role of copper deficiency is discussed, together with the difficulties in biomonitoring copper supplementation.

Although copper plays a pivotal role in numerous physiological processes, its deficiency is virtually indistinguishable from subacute combined degeneration due to cobalamin deficiency. Moreover, the co-occurrence of deficiencies in other micronutrients and vitamins is common, making the diagnosis even more challenging. Here, we describe a case of copper deficiency in a 50-year-old woman who presented with altered mental status and bilateral upper and lower extremity weakness, numbness, and paresthesia. She was treated for cirrhosis and hepatic encephalopathy secondary to hepatic injury. While her mental symptoms improved, her physical symptoms continued to worsen, and she was transferred for further evaluation. The neurologic examination was positive for sensory neuropathy including decreased vibration/proprioception and ataxia in arms and legs; complete blood count showed pancytopenia; but infectious workup, cerebrospinal fluid analysis, autoimmune studies, and brain/spine magnetic resonance imaging were normal. A nerve conduction study showed generalized, axonal sensorimotor polyneuropathy. Micronutrient/trace element deficiency was suspected in the setting of gastric bypass surgery, and supplementation was successfully initiated. Though uncommon, clinical copper deficiency is increasingly frequently recognized in the inpatient setting, and permanent neurological damage can occur prior to diagnosis and treatment. Physicians should have an elevated clinical suspicion of copper deficiency in cases of polyneuropathy and pancytopenia in patients with a history of bariatric surgery.

Zinc deficiency is one cause of anemia. However, it has been reported that some patients who were treated with zinc supplementation to resolve this anemia subsequently experienced copper deficiency, which lead to continued anemia, as well as leukocytopenia and other symptoms. However, only two patients with copper deficiency induced by zinc supplementation undergoing peritoneal dialysis have been reported. Here, we report the case of a 59 year-old man with copper deficiency after zinc supplementation undergoing peritoneal dialysis (PD). He took meals only once a day and drank about 750 mL/day of wine every day. He had been receiving zinc supplementation for 4 months. He was diagnosed with severe leukocytopenia and worsening anemia at a planned outpatient visit; in addition, his copper levels had markedly decreased. Thus, zinc supplementation was discontinued, and the patient was instructed to take cocoa for copper supplementation. Because of severe leukocytopenia, he was admitted to our hospital, and granulocyte colony-stimulating factor was administered. Red blood cell transfusions were performed for anemia. After discontinuing zinc supplementation, his white blood cell count and hemoglobin levels improved.To avoid Cu deficiency, patients\' dietary history should be checked in detail and Cu should be monitored carefully when Zn is supplemented in patients undergoing PD.

Neuropathy may arise from many different etiologies - from diabetes and nerve compression to viral infections and chemotherapy side effects; many patients suffer from neuropathic symptoms. While some etiologies produce irreversible neuropathy, others, such as vitamin and mineral deficiencies, lead to a possibly reversible disease process once treated. General clinicians should strive for early and prompt diagnosis of copper deficiency neuropathy whenever possible, especially in patients with normal vitamin B12 levels who present with a subacute gait disorder or prominent sensory ataxia. We present a case of a 73-year-old female with a surgical history of Roux-en-Y gastric bypass (RYGB) 20 years prior, who presented with difficulty with ambulation due to sensory ataxia and bilateral, ascending, sensory neuropathy, who was diagnosed with acquired copper deficiency-induced myeloneuropathy.