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.
A Case of Copper-Deficiency Myeloneuropathy and Anemia
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