ana_22 Posted May 9, 2010 Report Share Posted May 9, 2010 I was just reading up on this as it can cause autonomic dysfunction, gut stuff and heat intolerance. Am thinking of asking the neuro next time I see them. Anyone been tested? Cardiovascular and sudomotor autonomic dysfunction in Wilson's disease?Limited correlation with clinical severityDilip Sonia, Garima Shukla, a, , Sumit Singha, Vinay Goyala and Madhuri BehariaaDepartment of Neurology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, IndiaReceived 16 April 2008; revised 10 June 2009; accepted 3 July 2009. Available online 8 August 2009. AbstractBackgroundWilson's disease, a disorder of copper metabolism, results in abnormal accumulation of copper in liver, brain, kidney and cornea giving rise to protean manifestations. Wilson's disease predominantly affects the basal ganglia and brain stem nuclei which may cause autonomic dysfunction. Disturbances of autonomic nervous system have not received attention in Wilson's disease due to its rarity.The aim of this study was to evaluate autonomic cardiovascular reflexes in patients of Wilson's disease and to look for any relationship between autonomic nervous system disturbances and clinical severity of Wilson's disease.Materials and methodsCardiovascular autonomic reflexes were evaluated clinically and electrophysiologically in 30 patients of Wilson's disease with neurological onset and compared with equal number of age and gender matched healthy controls.ResultsSignificantly abnormal response to the Valsalva maneuver and RR interval variation was seen in patients compared to controls (p < 0.05). Latency for sympathetic skin response was also significantly (p < 0.02) prolonged in patient group. No specific correlation with clinical severity of Wilson's disease and autonomic dysfunction could be established.ConclusionAutonomic dysfunction occurs in Wilson's disease and affects parasympathetic more than the sympathetic functions. Quote Link to comment Share on other sites More sharing options...
Noreen Posted May 9, 2010 Report Share Posted May 9, 2010 I was just reading up on this as it can cause autonomic dysfunction, gut stuff and heat intolerance. Am thinking of asking the neuro next time I see them. Anyone been tested? It seems that most Movement Disorder Clinics are running the blood test for Wilson's these days as an exclusion dx fo those presenting with autonomic dysfunction. I have not had the blood test done yet but my neuroopthalmologist checked fo Keischer Fleischer (sp?) rings and they were not present so I am not expecting any easy answers. Quote Link to comment Share on other sites More sharing options...
spaceorca Posted May 9, 2010 Report Share Posted May 9, 2010 My son was tested for Wilson's several years ago (at my request, after reading articles about the symptoms) but the results were negative. It's an easy blood test, so seems worth checking for anyone with autonomic symptoms. Even though few POTS patients will end up with this diagnosis, Wilson's and other diseases like it seem to point to the underlying physiology of POTS: something is affecting those basal ganglia or other parts of the nervous system. For a lot of patients, that something and how to treat it are still elusive. But maybe a researcher will figure it out, as they did with Wilson's. Quote Link to comment Share on other sites More sharing options...
sue1234 Posted May 9, 2010 Report Share Posted May 9, 2010 Before my POTS 4 yrs. ago, a naturopath had me request my MD check for serum and urinary copper levels. Both were at the very low end of normal. And I have looked before for the rings in my eyes, but none. It is interesting, though, and maybe worth me having my copper levels checked again. Quote Link to comment Share on other sites More sharing options...
flop Posted May 9, 2010 Report Share Posted May 9, 2010 Sue, people with Wilson's disease have very high copper levels so if your levels were previously low and your eyes are normal you don't need to worry about Wilson's disease. Flop Quote Link to comment Share on other sites More sharing options...
ana_22 Posted May 10, 2010 Author Report Share Posted May 10, 2010 Initially i thought serum copper in WD should be high but if you have Wilson's disease serum copper is LOW as all the copper has been sucked into the cells. So Sue it is worth def checking it out. Quote Link to comment Share on other sites More sharing options...
ana_22 Posted May 10, 2010 Author Report Share Posted May 10, 2010 High serum copper is not an indication of Wilson disease. Since most Wilson patients have a low ceruloplasmin they actually have a lower than normal serum copper. Ceruloplasmin is the protein that binds with copper to remove it from the body. It is the unbound (to ceruloplasmin) copper that is free to roam around the body and accumulate in organs causing Wilson disease damage. An elevated serum copper is more often due to an elevation of the level of serum ceruloplasmin since it contains ~90% of the circulating copper bound to it. Elevations of ceruloplasmin can occur with inflammation, in response to estrogen therapy and in pregnancy. Note: The exception to this is when there is severe liver injury (acute liver failure) caused by Wilson disease. This causes very large amounts of copper to be released into circulation and causes markedly elevated serum copper. When this occurs, patients are very ill and usually have jaundice (yellow eyes and skin color) and very abnormal lab results with respect to liver function and blood coagulation. Quote Link to comment Share on other sites More sharing options...
flop Posted May 11, 2010 Report Share Posted May 11, 2010 Sorry for my misleading post - memory failure!!Flop Quote Link to comment Share on other sites More sharing options...
sue1234 Posted May 12, 2010 Report Share Posted May 12, 2010 It's also interesting that copper is VERY involved in collagen building. I wonder if most of the EDS people have had their ceruloplasmin(?) levels checked?? I also know that copper is involved in the pigment melanin. I actually started getting grey hair when I was just 29 years old. Anyone else have any testing on copper or ceruloplasmin levels checked? Quote Link to comment Share on other sites More sharing options...
scarfgirl Posted May 12, 2010 Report Share Posted May 12, 2010 @flop - You're supposed to blame things on brain fog Quote Link to comment Share on other sites More sharing options...
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