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Thread: A Case of Copper-Deficiency Myeloneuropathy and Anemia

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    Arrow A Case of Copper-Deficiency Myeloneuropathy and Anemia

    A Case of Copper-Deficiency Myeloneuropathy and Anemia


    Summary

    Background: A 47-year-old woman with an otherwise unremarkable medical history was referred to the multiple sclerosis clinic by her primary neurologist for evaluation of a 2-3 year history of progressive knee and back pain, weakness, paresthesias, sensory loss, ataxia, and falls. During the same period, she had received blood transfusions for unexplained anemia and leukopenia. She had been wearing dentures for many years.
    Investigations: Physical examination, neurological examinations (assessments of reflexes, gait, proprioception, and sensitivity to temperature, pinprick and vibration), neurophysiological studies (visual and brainstem somatosensory evoked potentials, nerve conduction studies and electromyography), T2-weighted MRI of the brain and spine, cerebrospinal fluid analysis and serum evaluations.
    Diagnosis: Myeloneuropathy and anemia due to copper deficiency, secondary to zinc overload associated with long-term use of denture cream with a high zinc content.
    Management: Change to a low-zinc denture cream and oral copper replacement.
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    Case Details:



    A 47-year-old woman with an otherwise unremarkable medical history was referred to a multiple sclerosis (MS) clinic by her primary neurologist for investigation of a 2-3 year history of progressive knee and back pain, weakness, paresthesia, sensory loss, ataxia and falls. During the same period, she had been treated with wholeblood transfusions for unexplained anemia and leukopenia. Bone marrow biopsies demonstrated nonspecific changes.

    Investigations performed before her presentation to the MS clinic included an unremarkable serum work-up for infections, malignancy, vitamin deficiencies, and rheumatologic and collagen vascular diseases. Cerebrospinal fluid analysis showed increased myelin basic protein but no evidence of inflammation or infection.
    The patient self-reported that the results of a nerve conduction study and electromyography early in her disease course were normal. A T2-weighted MRI scan of the spine performed during this prior investigation had revealed a contiguous, longitudinal, subtly increased signal throughout the dorsal cervical and thoracic spine that did not show enhancement after administration of gadolinium (Figure 1A).

    MRI of the brain and electroencephalographic assessment of visual, as well as brainstem somatosensory evoked potentials had produced unremark¬able results. Posterior tibial somatosensory evoked potentials, recorded by electroencephalography, were abnormal above the level of the lumbar spinal cord.

    On presentation to the MS clinic, the patient's physical examination was notable for reduced muscle bulk and tone, associated with generalized muscle weakness of arms, legs and neck flexors (Medical Research Council grade 4 out of 5), which was worse distally (Medical Research Council grade 3 out of 5). Reflexes were normal in the patient's arms, but were decreased at the patellae, and absent at the ankles; a plantar flexion response was evident. Sensations of temperature, pinprick, vibration and proprioception were substantially reduced below the patient's knees. Sensitivity to temperature and pinprick was decreased in her hands, but intact to vibration and proprioception. Her gait was markedly ataxic and a Romberg test indicated peripheral ataxia.

    In the MS clinic we repeated the nerve conduction study and electromyography, which demonstrated a severe sensory neuropathy without radiculopathy or myopathy. The patient's hematological profile continued to show anemia, leukopenia and absolute neutropenia (Table 1). Serum analysis established low copper and ceruloplasmin levels, and high zinc levels; consequently, the patient was diagnosed as having copper-deficiency myeloneuropathy and anemia. On further questioning, the patient reported use of a denture cream that contained high levels of zinc (approximately 34,000 µg/g).


    The patient was advised to switch to a lowzinc denture cream and started oral copper gluconate supplementation at 8 mg daily, which was tapered to 2 mg daily over a period of 1 month (this regimen was suggested by N Kumar before publication of his research article on this topic[2]). Chelation therapy to reduce her zinc levels was not considered necessary. Her serum levels of copper, ceruloplasmin and zinc, and her blood counts had normalized by 2 months after initiation of treatment (Table 1). The increased, longitudinal, spinal-cord signal on MRI (Figure 1A) had improved at 6 months (Figure 1B) and had resolved fully by 18 months. Her neurological features stabilized, but these and her neurological signs still had not improved at her most recent visit, after 18 months of copper supplementation. Unfortunately, she did not tolerate symptomatic medications for pain. She was advised to continue indefinitely a daily multivitaminthat contained at least 2 mg of copper.

    Tables

    Table 1. Laboratory Findings for fhe Patient at Diagnosis of Copper Deficiency, and After 2 Months of Copper Supplementation


    Table 2. Distinguishing Features of, and Diagnostic Tests for, Disorders That Should Be Considered in the Differential Diagnosis of Copper-deficiency Myeloneuropathy



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    Last edited by trimurtulu; 02-13-2009 at 11:35 AM.

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