Internal Medicine (Tokyo, 1992) 2012-01-01

Resolution of cranial MRI and SPECT abnormalities in a patient with Wilson's disease following oral zinc monotherapy.

Shimon Ishida, Yoshimitsu Doi, Kazushi Yamane, Masakazu Sugino, Fumiharu Kimura, Toshiaki Hanafusa, Hideo Fukui, Hiroshi Tamai

Index: Intern. Med. 51(13) , 1759-63, (2012)

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Abstract

A 38-year-old woman with Wilson's disease developed neurological deterioration after 25 years of low-dose penicillamine administration. She showed an akinetic-rigid syndrome and cerebellar motor ataxia. Brain MRI showed increased signal intensity at the bilateral pons, midbrain, putamen, and thalamus. 123I-IMP-SPECT revealed a diffuse reduction of cerebral blood flow at the bilateral cerebral hemisphere including the basal ganglia. After the patient's regimen was changed to zinc therapy, her neurological condition gradually improved, and she showed almost complete recovery within two years. Serial MRI and SPECT studies showed a marked improvement in the lesions.