title: [Magnetic Resonance Imaging Improvement in a Patient with Wilson's Disease Following Treatment with Trientine Hydrochloride and Zinc Acetate].
abstract: A 37-year-old male patient presented with psychiatric symptoms, dysarthria, limb dystonia, increased tendon reflexes, and a Kayser-Fleischer ring in his late teens. Laboratory examinations showed decreased concentrations of serum copper and ceruloplasmin, and increased urinary copper levels. Magnetic resonance imaging (MRI) showed high-signal-intensity lesions in the bilateral putamen, globus pallidus, thalamus, and brainstem on T2-weighted images (T2WI). Based on the MRI results and laboratory data, we diagnosed this patient with Wilson's disease (WD). He was treated with trientine hydrochloride and zinc acetate. Four months after the initiation of treatment, the patient'symptoms began to improve. On a follow-up MRI that was obtained 6 years after treatment, the high-signal-intensity lesions on the T2WI had disappeared completely. However, the low-signal-intensity lesions in the basal ganglia had spread to the caudate nuclei. Here, we discuss the characteristics of the MRI changes in WD following treatment. The Pathological basis for the low-signal-intensity lesions on T2WI in WD remains unclear. Our results suggest that this lesion may reflect the accumulation of materials other than copper.
authors.affiliation: Department of Neurology, Tokyo Metropolitan Neurological Hosptal.
authors.forename: Younhee
authors.initials: Y
authors.lastname: Kim
authors.forename: Reiji
authors.initials: R
authors.lastname: Koide
authors.forename: Akihiro
authors.initials: A
authors.lastname: Kawata
mesh_terms: D000328:Adult; D004338:Drug Combinations; D006527:Hepatolenticular Degeneration / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D014266:Trientine / Q000627:therapeutic use*; D019345:Zinc Acetate / Q000627:therapeutic use*
chemical_list: D004338:Drug Combinations; D019345:Zinc Acetate; D014266:Trientine