A Ranjan1, J Kalita2, V Kumar1, U K Misra1. 1. Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India. 2. Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India. Electronic address: jayanteek@yahoo.com.
Abstract
BACKGROUND AND AIM: There is no report of MRI correlation with neurological worsening following chelating treatment in Wilson disease with neurological manifestation (WDN). We report radiological changes in four patients with WDN who worsen after penicillamine. METHODS: WDN was diagnosed on the basis of clinical, KF ring, serum ceruloplasmin and 24h urinary copper. Hematological, biochemical and cranial MRI were repeated at the time of clinical deterioration following chelating treatment. RESULTS: Four WDN patients had neurological deterioration within 4-8 weeks of penicillamine therapy. This was associated with new lesions in white matter, thalamus, pons and mid brain and these lesions showed diffusion restriction. The neurologic deterioration was associated with increased free serum copper and malanodialdehyde and reduced glutathione. Clinical conditions stabilized after few weeks of penicillamine discontinuation. CONCLUSION: Neurological worsening was associated with new lesions on MRI which revealed diffusion restriction. Increased free copper induced oxidative stress may be responsible for these changes.
BACKGROUND AND AIM: There is no report of MRI correlation with neurological worsening following chelating treatment in Wilson disease with neurological manifestation (WDN). We report radiological changes in four patients with WDN who worsen after penicillamine. METHODS: WDN was diagnosed on the basis of clinical, KF ring, serum ceruloplasmin and 24h urinary copper. Hematological, biochemical and cranial MRI were repeated at the time of clinical deterioration following chelating treatment. RESULTS: Four WDN patients had neurological deterioration within 4-8 weeks of penicillamine therapy. This was associated with new lesions in white matter, thalamus, pons and mid brain and these lesions showed diffusion restriction. The neurologic deterioration was associated with increased free serum copper and malanodialdehyde and reduced glutathione. Clinical conditions stabilized after few weeks of penicillamine discontinuation. CONCLUSION: Neurological worsening was associated with new lesions on MRI which revealed diffusion restriction. Increased free copper induced oxidative stress may be responsible for these changes.