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• W1731209403 abstract "OBJECTIVE: To study the neurological outcomes of Wilson disease (WD) following decoppering. BACKGROUND: Judicious decoppering can prevent WD related mortality and reverse neurological disability. DESIGN/METHODS: From 2005-2013 we prospectively recruited 100 consecutive patients with WD and WD related neurological disability. All received, penicillamine or trientine in recommmended doses (125 mg/day - up to 2 gm/day), in the initial decoppering phase of treatment. Treatment was tracked by using the standard WD specific scale, the Global Assessment Scale of Wilson Disease (GAS for WD). RESULTS: There was no mortality over the eight year study period. Of the 100 patients, 56 were men. The mean age at disease onset was 12.4 years (range 3-35). As per the three point disease severity scale (SS3), on recruitment, 43 % patients had severe neurological disability (defined as being bedbound, mute, requiring enteral feeding & bladder catheterization). While 37% had moderate and, 20% had mild neurological disability. Dystonia was the most frequent movement disorder (77%) followedby parkinsonism (53%) and tremor (47%). Of the 100 patients, 4 were lost to follow up after the initial visit and two were refractory to decoppering. All of the other 94 patients showed significant neurological recovery over a mean follow up of 20.7 months (range 4-36). Seventy per cent of the 94 patients (including 68% of patients with severe neurologic disability) showed complete neurological recovery and could resume normal lives at par with their peers. At last follow up, 30 % of the 94 patients exhibit on-going recovery. Promisingly, improvements were also seen in various movements disorders including dystonia and parkinsonism. Adverse events observed included, transient decoppering induced neurological deterioration (n=15), thrombocytopenia (n=20), emergent psychosis (n=10), skin rash (n=6), non-traumatic fracture (n=4), and penicillamine induced lupus (n=2). CONCLUSIONS: Our report reinforces that remarkable neurological recovery can be expected in patients with WD with judicious decoppering. Objective clinical assessments help in monitoring treatment and tracking resolution of disability. Disclosure: Dr. Aggarwal has received personal compensation for activities with Novartis. Dr. Aggarwal has received research support from the Seventh Framework Programme, and Marie Curie Actions. Dr. Bhatt has received research funding from Marie Curie Actions." @default.
• W1731209403 created "2016-06-24" @default.
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• W1731209403 date "2014-04-08" @default.
• W1731209403 modified "2023-09-25" @default.
• W1731209403 title "Complete Neurological Recovery in Wilson Disease: Experience With 100 Consecutive Patients Seen From 2005-2013 (S47.007)" @default.
• W1731209403 hasPublicationYear "2014" @default.
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