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In Vivo Electrophysiological Measurement of Compound Muscle Action Potential from the Forelimbs in Mouse Models of Motor Neuron Degeneration
Published on: June 15, 2018
Axonal sensory motor neuropathy in copper-deficient Wilson's disease
Alexandra Foubert-Samier1, Annabelle Kazadi, Marie Rouanet
1Department of Neurology, University Hospital Bordeaux, Haut-Lévêque, Avenue Magellan, 33604 Pessac Cedex, France.
Abstract:
Copper deficiency may cause myeloneuropathy or progressive limb weakness. By contrast, Wilson's disease (WD) is characterized by progressive copper accumulation with hepatic and neurological impairment and requires life-long treatment with zinc and/or chelator agents. We report a WD patient who developed axonal sensory motor neuropathy in the context of copper deficiency due to his treatment with zinc and chelators. Exhaustive testing for other etiologies was negative. After treatment adjustment, only mild clinical improvement was noted during long-term follow-up. Muscle Nerve 40: 294-296, 2009.
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