Forums » Open clinical » Neurology » 100 years ago: 1911, Wilson described lenticular neurodegeneration and liver cirrhosis
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100 years ago: 1911, Wilson described lenticular neurodegeneration and liver cirrhosis
posted at 14/10/2011 5:01 AM BST
on bmj.com
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Posts: 24
First: 14/5/2009 Last: 7/4/2013 |
wilson's disease became the first neurodegenerative disorder of which the cause would be discovered: a disorder of copper metabolism leading to copper toxicosis. wilson's disease became also the first neurodegeneratve disorder for which a causal therapy was developed: 50 years ago: 1961, Schouwink described that oral zinc therapy might be a safe and effective treatment of copper toxicosis in Wilson's disease. Wilson's disease is a rare disorder, a typical orphan disease, but we can learn a lot from it, the more so since we discovered that abnormalities in copper metabolism play a role in Alzheimer's diseaese.
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Re: 100 years ago: 1911, Wilson described lenticular neurodegeneration and liver cirrhosis
posted at 17/7/2012 1:51 PM BST
on bmj.com
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Posts: 312
First: 2/6/2012 Last: 10/5/2013 |
wilson disease neurologically presented as young onset parkinsonism,seizure,dystonia and mental retardation, psychiatric problem.as far my knowledge goes, it not to cause dementia.defective CU metabolism without chromasomal involvement , rarely cause wilson and AD has different pathogenesis. |
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Re: 100 years ago: 1911, Wilson described lenticular neurodegeneration and liver cirrhosis
posted at 10/12/2012 11:35 PM GMT
on bmj.com
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Posts: 24
First: 14/5/2009 Last: 7/4/2013 |
Evidence based zinc therapy in ‘Wilson's free copper disease’ Wilson's disease is a rare hereditary disorder of copper metabolism, which leads to toxic free copper levels that may harm different organs including the liver and brain. Lacking well designed clinical trial, Wilson's disease has been treated on expert opinions. For years, resultant injuries were contributed to copper accumulation. However, according to the available evidence, the accumulation is believed to be a protective mechanism against copper intoxication. Evidence based medicine urges physicians to practise on the best available evidence. Consequently, in this short review the primary literature has been critically analysed to search for the best available evidence for the treatment of copper impairment, based on a rational evidence based reasoning. According to the evidence, free copper intoxication in Wilson's disease can safely and effectively be treated with zinc monotherapy, which is very affordable and patient-oriented choice, and unscientific chelation-based treatment, which causes further toxicity, should be rooted out. |
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Re: 100 years ago: 1911, Wilson described lenticular neurodegeneration and liver cirrhosis
posted at 7/2/2013 4:57 AM GMT
on bmj.com
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Posts: 312
First: 2/6/2012 Last: 10/5/2013 |
I think zink is adjunct therapy in maontainance phase and as monotherapy it is indicated until contraindication of penicillamine or others. |
Forums » Open clinical » Neurology » 100 years ago: 1911, Wilson described lenticular neurodegeneration and liver cirrhosis
