Research article| Volume 119, ISSUE 1, P85-90, October 1993

Copper-64 metabolism in two patients with non-Wilsonian movement disorders and copper deficiency

  • A.S. Wierzbicki
    Correspondence to: Dr. A.S. Wierzbicki, Department of Chemical Pathology, Chelsea and Westminster Hospital, 369 Fulham Road, London SW10 9NH, UK. Tel.: (81) 746-8086; Fax: (81) 746-8085.
    Department of Chemical Pathology, Charing Cross and Westminster Medical School, Chelsea and Westminster Hospital, London SW10 9NH, UK

    Department of Chemical Pathology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK
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  • N. Patel
    Department of Chemical Pathology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK
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  • K. Evans
    Department of Medical Physics, Hospital for Sick Children, Great Ormond Street, London WC1N 3JH, UK
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  • P.T. Lascelles
    Department of Chemical Pathology, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK
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      Copper-64 studies are presented of 2 patients with non-Wilsonian movement disorder and with abnormal copper handling. Both patients differred from the usual phenotype of non-Wilsonian low copper movement disorder as they had choreiform movement disorders with an onset in the first decade; one patient lacked significant intellectual impairment. Both patients had reduced serum total copper and marginal free copper and caeruloplasmin levels, and both patients were capable of incorporating 64Cu2+ into caeruloplasmin but the second case did so at markedly reduced level. Both showed slightly increased basal and stimulated urinary copper loss compared to normal controls with the rate in patient 1 being capable of leading to copper depletion. Liver copper content was normal in both cases. These 2 patients add to the reports of cases with copper deficiency and movement disorder in whom copper chelation therapy is unlikely to be beneficial.


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        • Danks D.M.
        Hereditary diseases of copper metabolism.
        in: Stanbury J.B. Wyngaarden J.B. Fredericksen J.S. Goldstein J.L. Brown M.S. The Metabolic Basis of Inherited Disease. 6th edn. McGraw-Hill, 1989: 1251-1268 (1989)
        • Fujii T.
        • Okuno T.
        • Ito M.
        • et al.
        Non-Menke's-type copper deficiency with regression, lactic acidosis and granulocytopenia.
        Neurology. 1991; 41: 1263-1266
        • Gibbs K.
        • Hanka R.
        • Walshe J.M.
        The urinary excretion of radiocopper in presymptomatic and symptomatic Wilson's disease, heterozygotes and controls: its significance in diagnosis and management.
        Quart. J. Med. 1978; 247: 349-364
        • Gibbs K.
        • Walshe J.M.
        A study of caeruloplasmin concentrations found in 75 patients with Wilson's disease, their kinship and various control groups.
        Quart. J. Med. 1979; 248: 447-463
        • Godwin-Austen R.B.
        • Robinson A.
        • Evans K.
        • et al.
        An unusual neurological disorder of copper metabolism clinically resembling Wilson's disease but biochemically a distinct entity.
        J. Neurol. Sci. 1978; 39: 85-89
        • Haas R.H.
        • Robinson A.
        • Evans K.
        • et al.
        An X-linked disease of the nervous system with disorder of copper metabolism and features of Menke's disease.
        Neurology. 1981; 31: 852-859
        • Hamer D.H.
        ‘Kinky hair’ disease sheds light on copper metabolism.
        Nature Genetics. 1993; 3: 3-5
        • Heckmann J.
        • Saffer D.
        Abnormal copper metabolism: another non-Wilson's case.
        Neurology. 1988; 38: 1493-1495
        • Lau J.Y.
        • Lai C.L.
        • Wu P.C.
        • et al.
        Wilson's disease: 35 years experience.
        Quart. J. Med. 1990; 278: 897
        • Meret S.
        • Henkin R.I.
        Simultaneous direct estimation of atomic absorption spectrophotometry of copper and zinc in serum, urine and cerebrospinal fluid.
        Clin. Chem. 1971; 17: 369-373
        • Ono S.
        • Kurisaki H.
        An unusual neurological disorder with abnormal copper metabolism.
        J. Neurol. 1981; 235: 397-399
        • Osborn S.B.
        • Walshe J.M.
        Studies with radiocopper (64Cu) in Wilson's disease: the liver/thigh ratio Clin.
        Sci. 1964; 27: 319-328
        • Pall H.S.
        • William A.C.
        • Blake D.R.
        • et al.
        Movement disorder associated with abnormal copper metabolism and decreased blood anti-oxidants.
        J. Neurol. Neurosurg. Psychiat. 1981; 50: 1234-1235
        • Quinn N.P.
        • Marsden C.D.
        Coincidence of Wilson's disease with other movement disorders in the same family.
        J. Neurol. Neurosurg. Psychiat. 1986; 49: 221-222
        • Robertson M.
        • Evans K.
        • Robinson A.
        • Trimble M.
        • Lascelles P.
        Abnormalities of copper in Gilles de la Tourette syndrome.
        Biol. Psychiat. 1987; 22: 968-978
        • Tauxe W.N.
        • Goldstein N.P.
        • Randall R.V.
        • Gross J.B.
        Radiocopper studies in patients with Wilson's disease and their relatives.
        Am. J. Med. 1966; 41: 375-380
        • Vulpe C.
        • Levinson B.
        • Whitney S.
        • Packman S.
        • Gitschier J.
        Isolation of a candidate gene for Menke's disease and evidence it encodes a copper transporting ATPase.
        Nature Genetics. 1993; 3: 7-13
        • Willvonseder R.
        • Goldstein N.P.
        • McCall J.T.
        • et al.
        A hereditary disorder with dementia, spastic dysarthria, vertical eye movement paresis, gait disturbance, splenomegaly and abnormal copper metabolism.
        Neurology. 1973; 23: 1039-1049