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Neeraj Kumar - One of the best experts on this subject based on the ideXlab platform.
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clinical physiological and pathological characterisation of the sensory predominant peripheral neuropathy in Copper Deficiency
2017Co-Authors: Neeraj Kumar, Brent P. Goodman, Christopher J. Klein, Sean W Taylor, Ruple S Laughlin, Peter J Dyck, James P B DyckAbstract:Introduction Myelopathy is considered the most common neurological complication of Copper Deficiency. Concurrent peripheral neuropathy has been recognised in association with Copper Deficiency but has not been well characterised. Objectives To characterise the clinical, physiological and pathological features of Copper-deficient peripheral neuropathy. Methods Patients with simultaneous Copper Deficiency ( Results 34 patients were identified (median age 55 years, range 36–78) including 24 women and 10 men. Myelopathy was found in 21 patients. Median serum Copper level was 0.11 μg/mL (range 0–0.58). The most frequent clinical and electrophysiological pattern of neuropathy was a sensory predominant length-dependent peripheral neuropathy (71%). Somatosensory evoked potentials demonstrated central slowing supporting myelopathy (96%). Quantitative sensory testing demonstrated both small and large fibre involvement (100%). Autonomic reflex screens (77%) and thermoregulatory sweat test (67%) confirmed sudomotor dysfunction. 14 cutaneous nerve biopsies revealed loss of myelinated nerve fibres (86%), increased regenerative clusters (50%), increased rates of axonal degeneration (91%) and increased numbers of empty nerve strands (73%). 71% of biopsies demonstrated epineurial perivascular inflammation. Conclusions An axonal, length-dependent sensory predominant peripheral neuropathy causing sensory ataxia is characteristic of Copper Deficiency usually co-occurring with myelopathy. Neurophysiological testing confirms involvement of large, greater than small fibres. The pathological findings suggest axonal degeneration and repair. Inflammatory infiltrates are common but are small and of doubtful pathological significance.
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Copper Deficiency in celiac disease.
2009Co-Authors: Thorvardur R. Halfdanarson, Neeraj Kumar, William J. Hogan, Joseph A. MurrayAbstract:Copper Deficiency is an uncommonly reported complication of celiac disease that has not received much attention in recent years. Copper Deficiency may result in anemia and thrombocytopenia and also irreversible myeloneuropathy if it is not detected and treated appropriately. The prevalence of Copper Deficiency in patients with celiac disease is unknown. We describe 5 patients with celiac disease and associated Copper Deficiency diagnosed at our institution in recent years. All 5 patients had neurologic complications of Copper Deficiency and 3 patients also presented with hematologic abnormalities. We also review the literature regarding Copper Deficiency in celiac disease.
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hematological manifestations of Copper Deficiency a retrospective review
2008Co-Authors: Thorvardur R. Halfdanarson, Neeraj Kumar, Robert L Phyliky, William J. HoganAbstract:Copper Deficiency is an established cause of hematological abnormalities but is frequently misdiagnosed. Copper Deficiency can present as a combination of hematological and neurological abnormalities and it may masquerade as a myelodysplastic syndrome. We reviewed the records of patients with hypocupremia and hematologic abnormalities identified between 1970 and 2005. Patients with hypocupremia unrelated to Copper Deficiency (e.g. Wilson's disease) were excluded. Forty patients with Copper Deficiency and hematological abnormalities were identified. Ten patients (25%) had undergone bariatric (weight reduction) surgery and an additional 14 patients (35%) had undergone surgery on the gastrointestinal tract, most commonly gastric resection. In 12 cases, no cause for Copper Deficiency was identified. Anemia and neutropenia were the most common hematologic abnormalities identified and the majority of the patients also had neurologic findings, most commonly due to myeloneuropathy. Abnormalities observed on bone marrow examination including vacuoles in myeloid precursors, iron-containing plasma cells, a decrease in granulocyte precursors and ring sideroblasts may be valuable clues to the diagnosis. Copper Deficiency is an uncommon but very treatable cause of hematologic abnormalities.
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Copper Deficiency myelopathy human swayback
2006Co-Authors: Neeraj KumarAbstract:The hematologic manifestations of Copper Deficiency are well known and include anemia and neutropenia. In the past few years, the neurological manifestations of acquired Copper Deficiency in humans has been recognized, the most common being a myelopathy presenting with a spastic gait and prominent sensory ataxia. The known causes of acquired Copper Deficiency include prior gastric surgery, excessive zinc ingestion, and malabsorption; however, often the cause is unclear. Hyperzincemia may be present even in the absence of exogenous zinc ingestion. The clinical features and neuroimaging findings are similar to the subacute combined degeneration seen in patients with vitamin B12 Deficiency. Copper and vitamin B12 Deficiency may coexist. The neurological syndrome may be present without the hematologic manifestations. Copper supplementation resolves the anemia and neutropenia promptly and completely and may prevent the neurological deterioration. Improvement, when it occurs, is often subjective and preferentially involves sensory symptoms. This article describes patients with Copper Deficiency myelopathy seen at the Mayo Clinic in Rochester, Minn, and reviews the literature on neurological manifestations of acquired Copper Deficiency in humans.
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Imaging features of Copper Deficiency myelopathy: a study of 25 cases
2006Co-Authors: Neeraj Kumar, J. Eric Ahlskog, Christopher J. Klein, John D. PortAbstract:Acquired Copper Deficiency presents with a spastic gait and sensory ataxia. Spinal cord magnetic resonance imaging (MRI) in patients with Copper Deficiency myelopathy may show increased T2 signal, most commonly in the dorsal midline cervical and thoracic cord. These imaging findings may be reversible with normalization of serum Copper. The clinical and imaging picture is very similar to the subacute combined degeneration seen in patients with vitamin B12 Deficiency. Neuroradiologists should consider this possibility when a long segment of symmetric dorsal spinal cord T2-hyperintensity is identified.
Eric J Ahlskog - One of the best experts on this subject based on the ideXlab platform.
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myelodysplasia myeloneuropathy and Copper Deficiency
2005Co-Authors: Neeraj Kumar, Eric J Ahlskog, Michelle A Elliott, James D Hoyer, C M Harper, Robert L PhylikyAbstract:We describe a patient with a suspected myelodysplastic syndrome that developed in association with a neurologic disorder resembling subacute combined degeneration but without vitamin B12 Deficiency. Ultimately, the hematologic manifestations and the neurologic syndrome were linked to severe Copper Deficiency. Prompt and complete reversal of the hematologic abnormalities occurred with Copper replacement. Serum Copper determination should be included in the work-up of patients with anemia and leukopenia of unclear etiology who have associated myeloneuropathy. The hematologic picture can resemble sideroblastic anemia or myelodysplastic syndrome. Hyperzincemia can be an accompanying abnormality even without exogenous zinc ingestion. The reason for the Copper Deficiency may not be evident.
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Copper Deficiency myelopathy produces a clinical picture like subacute combined degeneration
2004Co-Authors: Neeraj Kumar, John B Gross, Eric J AhlskogAbstract:Background: Copper Deficiency in ruminants is known to cause an ataxic myelopathy. Copper Deficiency as a cause of progressive myelopathy in adults is underrecognized. Objective: To describe the clinical, biochemical, electrophysiologic, and imaging characteristics in 13 patients with myelopathy associated with Copper Deficiency. Methods: The records of patients with a Copper Deficiency–associated myelopathy were reviewed. Clinical characteristics, laboratory investigations, and responses to therapeutic intervention were summarized. Results: Thirteen such patients were found, 11 of them in a 15-month period. All patients presented with prominent gait difficulty, reflecting a sensory ataxia due to dorsal column dysfunction and lower limb spasticity. All patients had polyneuropathy. A high or high-normal serum zinc level was seen in 7 of the 11 patients for whom this information was available. Somatosensory evoked potential studies done in eight patients showed impaired conduction in central proprioceptive pathways. Dorsal column signal change on spine MRI was present in three patients. An initial clue to the diagnosis was a very low ceruloplasmin level; further tests of Copper metabolism excluded Wilson disease. The cause remained unexplained in most patients. Oral Copper supplementation restored normal or near-normal Copper levels in 7 of the 12 patients in whom adequate follow-up data were available; parenteral supplementation restored normal level in 3 further patients. Copper supplementation prevented further neurologic deterioration, but the degree of actual improvement was variable. Conclusions: Unrecognized Copper Deficiency appears to be a common cause of idiopathic myelopathy in adults. The clinical picture bears striking similarities to the syndrome of subacute combined degeneration associated with vitamin B 12 Deficiency. Early recognition and Copper supplementation may prevent neurologic deterioration.
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Copper Deficiency myelopathy
2004Co-Authors: Neeraj Kumar, Brian A Crum, Ronald C Petersen, Steven Vernino, Eric J AhlskogAbstract:Background In humans, Menkes disease is the well-recognized neurological disorder due to inherited Copper Deficiency. Myelopathy due to acquired Copper Deficiency is not a well-recognized entity in humans, although myelopathy due to Copper Deficiency is well documented in some animal species. Patients We describe 3 patients who developed a progressive spastic-ataxic gait with proprioceptive deficits. All patients had a severe reduction in serum ceruloplasmin and Copper levels. Results All patients had evidence of posterior column dysfunction clinically and on somatosensory evoked potential studies. Two had a signal change in the posterior column on magnetic resonance imaging of the spinal cord. Conclusion Patients presenting with otherwise unexplained myelopathies should have their serum ceruloplasmin level measured.
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myelopathy due to Copper Deficiency
2003Co-Authors: Neeraj Kumar, John B Gross, Eric J AhlskogAbstract:Copper is an essential trace metal and plays key roles in the structure and function of the nervous system, vascular, and skeletal tissues and in hematopoiesis and catecholamine metabolism. Inherited Copper Deficiency (Menkes’ disease) results in intellectual deterioration, failure to thrive, seizures, abnormal hair, and connective tissue abnormalities. Due to the ubiquitous distribution of Copper and the low daily requirement, acquired Copper Deficiency is extremely rare in humans. Ataxic myelopathy due to Copper Deficiency does occur in ruminants and is called swayback.1 The hematologic manifestations of acquired Copper Deficiency are well described,2 but the neurologic manifestations of acquired Copper Deficiency in humans are not widely appreciated. We describe a case of myelopathy that occurred in the setting of Copper Deficiency. A 65-year-old man was evaluated for a 5-year history of progressive gait difficulty. For 1 year prior to evaluation, he had been using a cane and had paresthesias involving the feet and hands. He had been taking 200 to 400 mg of zinc a day for cold prevention for 22 years (recommended daily allowance of zinc, 15 mg/day). Neurologic examination …
J. Eric Ahlskog - One of the best experts on this subject based on the ideXlab platform.
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Imaging features of Copper Deficiency myelopathy: a study of 25 cases
2006Co-Authors: Neeraj Kumar, J. Eric Ahlskog, Christopher J. Klein, John D. PortAbstract:Acquired Copper Deficiency presents with a spastic gait and sensory ataxia. Spinal cord magnetic resonance imaging (MRI) in patients with Copper Deficiency myelopathy may show increased T2 signal, most commonly in the dorsal midline cervical and thoracic cord. These imaging findings may be reversible with normalization of serum Copper. The clinical and imaging picture is very similar to the subacute combined degeneration seen in patients with vitamin B12 Deficiency. Neuroradiologists should consider this possibility when a long segment of symmetric dorsal spinal cord T2-hyperintensity is identified.
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Myelopathy Due to Copper Deficiency Following Gastrointestinal Surgery
2003Co-Authors: Neeraj Kumar, Kathleen M. Mcevoy, J. Eric AhlskogAbstract:Background Ataxic myelopathy due to Copper Deficiency has been described in ruminant animals and is called swayback. Neurological manifestations due to inherited Copper Deficiency secondary to the failure of intestinal Copper absorption is well recognized as Menkes disease. The neurological consequences of acquired Copper Deficiency in humans are not well described. Objective To report 2 cases where patients developed a myelopathy with Copper Deficiency after gastrointestinal surgery. Patients Two patients developed a myelopathy many years after gastrointestinal surgery. Both had severe Copper Deficiency, which was the likely cause of the myelopathy. Conclusions Acquired Copper Deficiency may present as a myelopathy. Gastrointestinal surgery and resulting decreased Copper absorption may be causative.
Thomas R Ziegler - One of the best experts on this subject based on the ideXlab platform.
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optic neuropathy myelopathy anemia and neutropenia caused by acquired Copper Deficiency after gastric bypass surgery
2014Co-Authors: Shadi S Yarandi, Vivian M Zhao, Daniel P Griffith, Rahul A Sharma, Arun Mohan, Thomas R ZieglerAbstract:Malabsorptive bariatric surgery is rapidly becoming a major cause of Copper Deficiency given the increasing prevalence of these procedures for morbid obesity. Acquired Copper Deficiency can present with clinically significant hematologic and neurological manifestations. Although hematologic manifestations of Copper Deficiency are rapidly reversible, significant neurological improvement after Copper supplementation therapy is unusual and many patients remain debilitated and may only experience, at best, stabilization of the neurological manifestations. Here we present a case of an undiagnosed Copper Deficiency several years after bariatric gastric bypass surgery, in a patient who concomitantly used zinc-containing denture cream for several years, associated with anemia, neutropenia, myelopathy, respiratory failure, and bilateral optic neuropathy, which caused major vision loss. This patient was also a heterozygote carrier of the 5,10-methylenetetrahydrofolate reductase A1298C gene polymorphism, which may affect Copper metabolism. Intravenous Copper repletion resulted in rapid correction of hematologic indices. However, neurological manifestations, including vision loss responded only modestly to Copper supplementation, despite achieving normal blood Copper concentrations. Clinicians should consider Copper Deficiency in patients at risk, as in this case, as a delayed diagnosis can lead to irreversible disability due to neurological manifestations.
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incidence and prevalence of Copper Deficiency following roux en y gastric bypass surgery
2012Co-Authors: Nana Gletsumiller, M Broderius, Jennifer K Frediani, Vivian M Zhao, Daniel P Griffith, Scott S Davis, John F Sweeney, Edward Lin, J R Prohaska, Thomas R ZieglerAbstract:The frequency of Copper Deficiency and clinical manifestations following roux-en-y gastric bypass (RYGB) surgery is not yet clear. Objectives were to determine the prevalence and incidence of Copper Deficiency in patients who have undergone RYGB. We sought to determine the number of RYGB patients undergoing medical and nutritional follow-up visits at the Emory Bariatric Center who experienced Copper Deficiency and associated hematological and neurological complaints (n=136). Separately, in patients followed longitudinally before and during 6 and 24 months following RYGB surgery, we obtained measures of Copper status (n=16). Systemic blood cell counts and measures of Copper, zinc and ceruloplasmin were determined using standardized assays in reference laboratories including atomic absorption spectrometry and immunoassays. Thirteen patients were identified to have Copper Deficiency suggesting a prevalence of Copper Deficiency of 9.6%, and the majority of these had concomitant complications including anemia, leukopenia and various neuro-muscular abnormalities. In the longitudinal study, plasma Copper concentrations and ceruloplasmin activity decreased over 6 and 24 months following surgery, respectively (P<0.05), but plasma zinc concentrations did not change. A simultaneous decrease in white blood cells was observed (P<0.05). The incidence of Copper Deficiency in these subjects was determined to be 18.8%. The prevalence and incidence of Copper Deficiency following RYGB surgery was determined to be 9.6% and 18.8%, respectively, with many patients experiencing mild-to-moderate symptoms. Given that Copper Deficiency can lead to serious and irreversible complications if untreated, frequent monitoring of the Copper status of RYGB patients is warranted.
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incidence and prevalence of Copper Deficiency following roux en y gastric bypass surgery
2012Co-Authors: Nana Gletsumiller, M Broderius, Jennifer K Frediani, Vivian M Zhao, Daniel P Griffith, Scott S Davis, John F Sweeney, Edward Lin, J R Prohaska, Thomas R ZieglerAbstract:Introduction and Objectives The frequency of Copper Deficiency and clinical manifestations following roux-en-y gastric bypass (RYGB) surgery is not yet clear. Objectives were to determine the prevalence and incidence of Copper Deficiency in patients who have undergone RYGB.
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acquired Copper Deficiency a potentially serious and preventable complication following gastric bypass surgery
2009Co-Authors: Daniel P Griffith, Thomas R Ziegler, David Liff, Gregory J Esper, Elliott F WintonAbstract:Copper is an essential cofactor in many enzymatic reactions vital to the normal function of the hematologic, vascular, skeletal, antioxidant, and neurologic systems. Copper Deficiency in the United States is believed to be relatively rare but has been described in the setting of zinc supplementation, myelodysplastic syndrome, use of parenteral nutrition and chronic tube feeding, and in various malabsorptive syndromes, including following gastrectomy and gastric bypass surgery. Features of Copper Deficiency include hematologic abnormalities (anemia, neutropenia, and leukopenia) and myeloneuropathy; the latter is a rarer and often unrecognized complication of Copper Deficiency. We here describe two patients who presented with severe gait abnormalities and anemia combined with neutropenia several years after roux-en-Y gastric bypass (RYGB) surgery for obesity who were found to be severely Copper deficient. Intravenous Copper repletion resulted in the rapid correction of hematologic indices; combined intravenous and oral Copper supplementation and eventual oral Copper supplements alone normalized serum Copper levels in each patient, but resulted in only partial resolution of the neurologic deficits. This report serves to alert physicians of the association between RYGB procedures and subsequent Copper Deficiency in order to avoid diagnostic delays and to improve treatment outcomes.
Brent P. Goodman - One of the best experts on this subject based on the ideXlab platform.
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clinical physiological and pathological characterisation of the sensory predominant peripheral neuropathy in Copper Deficiency
2017Co-Authors: Neeraj Kumar, Brent P. Goodman, Christopher J. Klein, Sean W Taylor, Ruple S Laughlin, Peter J Dyck, James P B DyckAbstract:Introduction Myelopathy is considered the most common neurological complication of Copper Deficiency. Concurrent peripheral neuropathy has been recognised in association with Copper Deficiency but has not been well characterised. Objectives To characterise the clinical, physiological and pathological features of Copper-deficient peripheral neuropathy. Methods Patients with simultaneous Copper Deficiency ( Results 34 patients were identified (median age 55 years, range 36–78) including 24 women and 10 men. Myelopathy was found in 21 patients. Median serum Copper level was 0.11 μg/mL (range 0–0.58). The most frequent clinical and electrophysiological pattern of neuropathy was a sensory predominant length-dependent peripheral neuropathy (71%). Somatosensory evoked potentials demonstrated central slowing supporting myelopathy (96%). Quantitative sensory testing demonstrated both small and large fibre involvement (100%). Autonomic reflex screens (77%) and thermoregulatory sweat test (67%) confirmed sudomotor dysfunction. 14 cutaneous nerve biopsies revealed loss of myelinated nerve fibres (86%), increased regenerative clusters (50%), increased rates of axonal degeneration (91%) and increased numbers of empty nerve strands (73%). 71% of biopsies demonstrated epineurial perivascular inflammation. Conclusions An axonal, length-dependent sensory predominant peripheral neuropathy causing sensory ataxia is characteristic of Copper Deficiency usually co-occurring with myelopathy. Neurophysiological testing confirms involvement of large, greater than small fibres. The pathological findings suggest axonal degeneration and repair. Inflammatory infiltrates are common but are small and of doubtful pathological significance.
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Copper Deficiency myeloneuropathy due to occult celiac disease
2009Co-Authors: Brent P. Goodman, Deven H Mistry, Shabana F Pasha, Peter E BoschAbstract: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.
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Clinical and electrodiagnostic findings in Copper Deficiency myeloneuropathy
2008Co-Authors: Brent P. Goodman, E P Bosch, Mark A. Ross, Charlene Hoffman-snyder, D D Dodick, Benn E. SmithAbstract:Introduction: Copper Deficiency is an increasingly recognised cause of neurological impairment. This retrospective review highlights clinical and electrodiagnostic findings in patients diagnosed at our institution with Copper Deficiency. Methods: Clinical, radiographic and electrodiagnostic findings were reviewed in patients with evidence of Copper Deficiency. Patients with other potential causes of myelopathy or neuropathy were excluded. Results: The predominant clinical feature in all six patients was a sensory ataxia, resulting in marked gait unsteadiness. Nerve conduction studies and needle EMG were performed in all patients and revealed a mild to moderate distal, axonal, sensorimotor peripheral neuropathy. Median and tibial somatosensory evoked potentials were abnormal in all five patients in which it was performed, showing impaired conduction in central or proximal peripheral somatosensory pathways. Conclusions: This pattern of electrodiagnostic findings suggests that impairment in somatosensory pathways demonstrated by somatosensory evoked potential testing is the main cause of the sensory ataxia in patients with Copper Deficiency.
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Copper Deficiency myeloneuropathy resembling b12 Deficiency partial resolution of mr imaging findings with Copper supplementation
2006Co-Authors: Brent P. Goodman, Brian W Chong, Ameet Patel, Geoffrey P Fletcher, Benn E. SmithAbstract:SUMMARY: Copper Deficiency has been associated with a clinical syndrome, myeloneuropathy. Radiographic changes resembling B12 Deficiency in the cervical spinal cord have been described. We present a case of Copper Deficiency myeloneuropathy, with cervical MR imaging findings resembling B12 Deficiency, which partially reversed following Copper supplementation. This is, to our knowledge, the first described case of radiographic improvement with Copper supplementation.