0

[Magnetic Resonance Imaging Improvement in a Patient With Wilson's Disease Following Treatment With Trientine Hydrochloride and Zinc Acetate]

Younhee Kim, Reiji Koide, Akihiro Kawata

Brain Nerve. 2015 May;67(5):635-8.

PMID: 25957210

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.

Chemicals Related in the Paper:

Catalog Number Product Name Structure CAS Number Price
AP38260014-A Trientine hydrochloride Trientine hydrochloride 38260-01-4 Price
qrcode