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Table of Contents    
Year : 2014  |  Volume : 62  |  Issue : 4  |  Page : 434-435

A new differential diagnosis for acquired demyelinating neuropathy: Copper deficiency

Department of Neurology, Acibadem University School of Medicine, Istanbul, Turkey

Date of Web Publication19-Sep-2014

Correspondence Address:
Geysu Karlikaya
Department of Neurology, Acibadem University School of Medicine, Istanbul
Geysu Karlikaya
Department of Neurology, Acibadem University School of Medicine, Istanbul
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.141219

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How to cite this article:
Karlikaya G, Kaya D, Karlikaya G, Kaya D. A new differential diagnosis for acquired demyelinating neuropathy: Copper deficiency . Neurol India 2014;62:434-5

How to cite this URL:
Karlikaya G, Kaya D, Karlikaya G, Kaya D. A new differential diagnosis for acquired demyelinating neuropathy: Copper deficiency . Neurol India [serial online] 2014 [cited 2020 Oct 23];62:434-5. Available from:


Copper is a very important trace element. It is required for basic cell functioning and is a cofactor in a number vital enzymes necessary for the nervous system. [1],[2] The first case of copper deficiency-related myelopathy in humans was reported in 2001. [3],[4],[5] Clinically, a presentation similar to B12 deficiency myelopathy has been described, with neurological examination findings suggestive of pyramidal and posterior column abnormalities. Commonly, spinal cord magnetic resonance imaging (MRI) studies display abnormal T2 signals in the posterior cord and EMG studies demonstrate an axonal peripheral neuropathy. We report a case of acquired demyelinating neuropathy due to copper deficiency.

A 31-year-old male patient presented with a 2-month history of tingling in his legs, followed by in his hands. Medical history was positive for a gastrointestinal hemorrhage and partial gastrectomy 5 years prior to the present admission. Family history was negative for any systemic or neurological disorder. Neurological examination revealed mild proximal muscle weakness (+4/5 MRC grade) during hip flexion. Upper limb reflexes were hypoactive while knee and ankle reflexes were abnormally brisk with bilateral ankle clonus. Sensory examination displayed glove-stocking paresthesias and hypoesthesia, and normal sense of position and vibration. Cervical, thoracal, and lumbosacral MRI studies with contrast were all normal. Electrodiagnostic studies showed features consistent with acquired demyelinating neuropathy-conduction slowing in the motor (22-23 m/s in lower extremities, 34 m/s in the upper extremities) and sensory (33-36 m/s) nerves, prolonged motor distal latencies, temporal dispersion, and motor conduction blocks bilaterally in tibial and median nerves [Figure 1], [Table 1] and [Table 2]. The compound muscle action potential (CMAP) and sensory nerve action potential (SNAP) amplitudes were mildly reduced. All F latencies were prolonged (53-67 ms in the upper extremities; 70-75 ms in the lower extremities; [Figure 2]). Temporal dispersion, conduction blocks, low nerve conduction velocities, and prolonged F latencies fulfilled the electrodiagnostic criteria for an acquired demyelinating neuropathy.
Figure 1: Right tibial nerve conduction study displaying a conduction block between ankle and popliteal (1,37 mV amplitude at the ankle-0,41 mV amplitude at poplitea, nerve conduction velocity of 30,3 m/s)

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Figure 2: Left median nerve F wave study displaying, min F latency 58 ms

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Table 1: Nerve conduction studies displaying prolonged distal latencies, slow nerve conduction velocities, conduction block in the right median nerve and prolonged distal latencies

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Table 2: Sensorial nerve conduction studies displaying slow nerve conduction velocities

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Cerebrospinal fluid analysis showed 42.9 mg/dl protein, 76 mg/dl glucose, and no abnormal cells. Further neuropathic work up including RF, ACE, Ana, Anti ds DNA, anca, TSH, protein and immunfixation electrophoresis, and gangilosid profile was normal. His serum copper level was low 30 μg/dl (N: 70-150 μg/dl). Serum ceruloplasmin and urinary copper levels were normal. In search of a cause for his low serum copper level, his zinc level was checked and found to be normal. Celiac disease investigations were also negative. The patient was treated with oral copper supplementation, 6 mg/day elemental copper for 1 week, 4 mg/d for the second week, and 2 mg/d thereafter. At 2-month follow up, his serum copper level was 52 μg/dl, and he reported a decrease of the paresthetic symptoms. His motor strength was normal and his nerve conduction studies showed improved distal latencies and conduction velocities.

Acquired copper deficiency is quite rare since a normal diet has a daily amount of 1-5 mg copper and is usually sufficient to meet the daily requirement of 0.9 mg/day. [1],[2] Gastric pH is necessary to free copper from ingested food, and then it is absorbed primarily in the duodenum, stomach, and ileum. Therefore, the most common cause of copper deficiency is malabsorbtion, most commonly related to a history of gastric surgery; up to 10-15% have been reported following bariatric surgery. Other rare causes are malabsorbtion syndromes such as celiac disease, prolonged total parenteral nutrition, and excessive zinc ingestion. [2],[6]

Copper deficiency has been reported to be a relatively late complication of gastric surgery and has even been reported 23 years after a total gastrectomy. [7],[8],[9] In our patient, the symptoms developed after 5 years. Our case demonstrates the importance of recommending not only vitamin B12 and thiamine but also copper to patients following surgery and obtaining periodical blood works. Kumar studied 13 patients with acquired copper deficiency; he reported that only 3 out of 10 patients with a positive Babinski's sign had increased T2 signal on the spinal cord MRI. [10] Two years later he described 25 patients with copper deficiency myelopathy and neuroimaging was positive in only 11/25 patients. [6] In our patient, the neurological signs were suggestive of a myelopathy. Although there were no signal changes in his spinal cord MRI studies, examination of the upper motor neuron findings (brisk tendon reflexes and clonus) suggests spinal cord involvement. The neuropathy related to copper deficiency was previously described as an axonal type polyneuropathy, as it was in the study by Kumar et al. An axonal peripheral neuropathy was found in 21 out of 24 patients diagnosed with copper deficiency myelopathy. [6]

To our best of our knowledge, this is the first case of copper deficiency presenting with electrophysiological findings typical for an acquired demyelinating polyneuropathy. This patient represents the importance of work-up for copper deficiency in patients with history of gastric surgery and acquired demyelinating neuropathy.

 » References Top

1.Lazarchick J. Update on anemia and neurtopenia in copper deficiency. Curr Opin Hematol 2012;19:58-60.  Back to cited text no. 1
2.Miller NG. Copper deficiency after bariatric surgery. Clin Nutr Insight 2013;39:1-4.  Back to cited text no. 2
3.Schleper B, Stuerenburg HJ. Copper deficiency associated myelopathy in a 46 year old women. J Neurol 2001;248:705-6.  Back to cited text no. 3
4.Reyes CV. Polyneuropathy and pancytopenia secondary to copper deficiency. Fed Pract 2011;28:23-6.  Back to cited text no. 4
5.Tormoehlen LM, Kumar N. Neurotoxicology five new things. Neurol Clin Pract 2012;2:301-8.  Back to cited text no. 5
6.Kumar N. Copper deficiency myelopathy (human swayback). Mayo Clin Proc 2006;81:1371-84.  Back to cited text no. 6
7.Rudnicki SA. Prevention and treatment of peripheral neuropathy after bariatric surgery. Curr Treat Options Neurol 2010;12:29-36.  Back to cited text no. 7
8.Inaba M, Torii T, Shinoda K, Yamasaki R, Ohyagi Y, Kira J. Peripheral neuropathy, myelopathy, cerebellar ataxia, and subclinical optic neuropathy associated with copper deficiency occuring 23 years after total gastrectomy. Rinsho Shinkeigaku 2011;51:412-6.  Back to cited text no. 8
9.Wu J, Ricker M, Muench J. Copper deficiency as cause of unexplained hematolologic and neurologic deficits in patient with prior gastrointestinal surgery. J Am Board Fam Med 2006;19:191-4.  Back to cited text no. 9
10.Kumar N, Gross JB Jr, Ahlskog JE. Copper deficiency myelopathy produces a clinical picture like subacute combined degeneration. Neurology 2004;63:33-9.  Back to cited text no. 10


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