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NEUROLOGY 2006;67:878-880
© 2006 American Academy of Neurology
Brief Communications

Subcortical white matter abnormalities related to drug resistance in Wilson disease

D. Aikath, MBBS*, A. Gupta, MSc*, I. Chattopadhyay, PhD, M. A. Hashmi, MD (Radio), P. K. Gangopadhyay, DM (Neuro), S. K. Das, DM (Neuro) and K. Ray, PhD

From the Human Genetics and Genomics Division (D.A., A.G., I.C., K.R.), Indian Institute of Chemical Biology, Bangur Institute of Neurology (P.K.G., S.K.D.), and Eko CT and MRI Centre (M.A.H.), Medical College Hospitals, Kolkata, India is currently with the Institute of Neuroscience, University of Oregon, Eugene.

Address correspondence and reprint requests to Dr. K. Ray, Human Genetics and Genomics Division, Indian Institute of Chemical Biology, 4 Raja S C Mullick Road, Jadavpur, Kolkata 700 032, India; e-mail: thisiskr{at}rediffmail.com and kunalray{at}gmail.com

Wilson disease (WD) produces typical lesions in the brain, which can aid in diagnosis and therapy. The authors present a drug-resistant WD case with atypical cerebral lesions with marked involvement of white matter as visualized on MRI scans. The diagnosis was confirmed by identification of mutations in the ATP7B gene. The case demonstrates an uncommon pathology-related cerebral copper accumulation and emphasizes the importance of genetic screening in the diagnosis of WD.

*These authors contributed equally to the work.

Supported by a research grant (K.R.) and a fellowship (D.A.) from Indian Council of Medical Research (ICMR), Government of India. A.G. and I.C. are supported by fellowships from Council of Scientific and Industrial Research, India.

Disclosure: The authors report no conflicts of interest.

Received December 20, 2005. Accepted in final form May 9, 2006.






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