Worsening of neurologic syndrome in patients with Wilson's disease with initial penicillamine therapy
G. J. Brewer, C. A. Terry, A. M. Aisen and G. M. Hill
We describe a patient with Wilson's disease who presented with neurologic
disease, was treated with D-penicillamine, and suffered sudden neurologic
deterioration coincident with therapy. Replicate brain magnetic resonance
imaging examinations after six weeks and 11 months of penicillamine therapy
documented the development of new brain lesions during this period, while
liver biopsy specimen data disclosed that excellent hepatic decoppering had
occurred. To develop information on the relative rarity or frequency of
neurologic worsening with the initiation of penicillamine therapy, we
conducted a retrospective survey of 25 additional patients with Wilson's
disease who met the criteria of presenting with neurologic disease and
having been treated with penicillamine. The replies indicate that, at least
from the patient's viewpoint, this syndrome occurs frequently. We suggest
that the cause of this distressing syndrome, and ways to mitigate or
circumvent it, must be discovered.
Treatment of Wilson Disease With Ammonium Tetrathiomolybdate: IV. Comparison of Tetrathiomolybdate and Trientine in a Double-blind Study of Treatment of the Neurologic Presentation of Wilson Disease.
Brewer et al.
Arch Neurol 2006;63:521-527.
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Prognostic factors in patients presenting with severe neurological forms of Wilson's disease
Prashanth et al.
QJM 2005;98:557-563.
ABSTRACT
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Treatment of Wilson Disease With Ammonium Tetrathiomolybdate: III. Initial Therapy in a Total of 55 Neurologically Affected Patients and Follow-up With Zinc Therapy
Brewer et al.
Arch Neurol 2003;60:379-385.
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Copper Control as an Antiangiogenic Anticancer Therapy: Lessons from Treating Wilson's Disease
Brewer
Exp. Biol. Med. 2001;226:665-673.
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Recognition, Diagnosis, and Management of Wilson's Disease
Brewer
Exp. Biol. Med. 2000;223:39-46.
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A homesick student
McDonnell and Esmonde
Postgrad. Med. J. 1999;75:375-378.
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Neurology and the liver
Jones and Weissenborn
J. Neurol. Neurosurg. Psychiatry 1997;63:279-293.
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