Wilson's disease: MRI with clinical correlation

• Published in: Neuroradiology Vol. 36; no. 2; p. 97
• Main Authors: Magalhaes, A C, Caramelli, P, Menezes, J R, Lo, L S, Bacheschi, L A, Barbosa, E R, Rosemberg, L A, Magalhaes, A
• Format: Journal Article
• Language: English
• Published: Germany 01.02.1994
• Subjects: Adolescent; Adult; Brain - pathology; Child; Female; Hepatolenticular Degeneration - pathology; Hepatolenticular Degeneration - physiopathology; Humans; Magnetic Resonance Imaging; Male; Middle Aged
• ISSN: 0028-3940
• DOI: 10.1007/BF00588068

We tried to establish possible correlations between clinical data and MRI in a group of patients with Wilson's disease. Eleven patients (6 male, 5 female), aged between 11 and 50 years old, with a duration of illness from 5 months to 32 years, were submitted to MRI on a 1.5 T System. Three patients were asymptomatic, two had mild neurological disturbances, two were moderately affected and the remaining four had a severe form of the disease. All were receiving D-penicillamine at the time of the study. In the most symptomatic patients there were abnormalities in five or more sites on MRI. The putamen was affected in all symptomatic patients, including five with dystonia. A striking feature was the peripheral location of high signal putaminal lesions on T2-weighted images. In five cases, lesions in the corpus striatum or substantia nigra explained the patient's Parkinsonian features. MRI is an efficient method for studying involvement of the central nervous system in Wilson's disease, and allows some interesting anatomoclinical correlations.