Link between BFS and Mentioned?

Highlights Only
PNH is the cause of invountary muscular twitching, cramps etc. BFS is one of the many names given to invountary muscular twitching. Think of it as having BFS caused by PNH.

Steve.
 
I am reminded of the Sufi parable where 3 people are led into a darkened room with an elephant inside. They have never seen an elephant before and so when they touch it, each one has a very different idea of what an elephant is. One touches it’s back and says, “ Ah, surely an elephant is like a temple, broad and high.” Another touches it’s leg and says, “It would seem to me that an elephant is similar to an oak tree, sturdy and strong.” The 3rd person touches the elephants trunk and says, “ No, no surely an elephant is like a large snake.” The speaker of the parable ponders what would have happened if they had bothered to light a candle, would they then not have seen the elephant as it really was, instead of what it appeard to be. In other words, in reducing something to the sum of it’s parts we lose sight of the whole thing.

Does it really matter what we call the syndrome/disorder/bag of symptoms that we all share. Has the distinction between BFS and PNH brought anyone closer to discovering the cause of their fasciculations? What is important, I think, is that if a neuro has made a dx of something that is benign, based on his/her considerable knowledge as a learned physician, the diagnostic tools, and perhaps, more importantly, the empirical evidence that has been garnered through many years of witnessing pathology: believe it!

That we share a commonality of symptoms is not debatable, what the cause of these symptoms is, would seem to be elusive. You say tomahto, I say tomayto. Let’s call the whole thing off!

Cheers,
Basso
 
Steve do you have VGKC antibodies?

The research is a bit concerning, although going by the amount of people suffering this syndrome that haven't developed anything nasty is quite reassuring.

I have only skimmed through but will read more carefully later. however, would I be right to conclude that all of us here have PNH, but it has been described to us in different ways? And it is likely that many of us could have an autoimmune channelopathy?

I realise that you have been diagnosed with PNH and you have perhaps received better lay-man explanation of the syndrome. I would very much like to hear your views/ hypotheses on this.
Cheers

Diego4Life
 
Diego4Life.

I've a couple of PNH emails that came through the web site which have been waiting for what will be, a long reply and I must get this done. On top of this I'm building a database for the Saluki welfare rescue society and trying to get a new web site up and running which hopefully should have some input in layman's terms, from Dr Hart. So at the moment I'm struggling to keep up. My legs pain me when I'm sat for a while which is a hindrance and I wish I could type faster, so if you can just bare with me I'll get back to you as soon as I can

Regards Steve.
 
Diego4Life.

First an apology to all on the board is required. Since being diagnosed in 2000 I have been under the impression that I was included in the 60 patient study and that I had tested positive for the VGKC antibodies and I have said this many times in the past. However, during a casual conversation with Hart on Monday where I was enquiring whether this study was still on going, it emerged that I was not in the study, missing out by a few months. I then asked about my VGKC test results and was informed that I had tested negative, although he added that due to the insensitivity of the technique that is commonly used to look for these antibodies he strongly suspected that they where present. Therefore, to answer your first question, I tested negative for the antibodies but my neuro is pretty sure they are present, which doesn't really answer your question at all.

I was originally diagnosed with Neuromyotonia, based on clinical and EMG findings (3 within 8 weeks) but I am now classed as having PNH. Would you be right in concluding that everyone has PNH? That depends on how you interpret the article but in my view that would be correct. If you read the article you will observe that there is no mention of BFS only CFS (Cramp fasciculation syndrome). To my way of thinking BFS is the same as CFS but cramps are a more prominent feature in this variant. Given that CFS along with the rest of the names that have been used to describe this condition, are now classed as PNH and having asked my neuro the question, then BFS is classed as PNH.

To my mind at least, the term PNH is a far more descriptive name for this condition of involuntary muscular activity and I don't think anyone can dispute that this is what we're all experiencing. I'm unaware of any other way that the muscles can constantly twitch other than by receiving a signal sent via a nerve that tells them to do so. Nerve cells create the signals and it would appear that this is where the fault lies due to a potassium channelopathy that causes them to become excitory of their own accord. The way Hart explained this to me in layman's terms when we first met was as follows. Inside the nerve there are two channels one containing potassium, the other sodium, which cross back and forth during the production of a signal, through what are known as gates. Due to a blockage affecting the potassium gate, the sodium is prohibited from crossing over and therefore starts to build up until a point is reached whereby the only means of release it to fire the signal that causes the muscle to twitch and then the process starts again.

When it comes to the VGKC antibodies there is no way of knowing how many of us have these. I don't think that they are routinely tested for by neuro's when someone presents with twitching and cramps and, as of yet there is no test procedure that can identify them every time. Even though I fell outside the study, because Hart's research interests are antibody-mediated autoimmune neurolologic disorders, he tests for VGKC antibodies as an established practice.

Because research has shown that PNH can be associated with other autoimmune disorders such as MG along with small cell carcinoma and thymoma, it would make sense that anyone presenting with twitching and cramps should be investigated for these disorders. He made the analogy on Monday that you wouldn't want to go to your Dr's complaining of headaches to be told you have a headache without any further investigations being made. Therefore, preferably, I would want my neuro to be aware of PNH and these associations evidenced by research, because the earlier they are detected the better. As an example, thyroid antibodies where picked up during routine blood tests aproximately 3 years after first attending my neuro's clinic. I've no sysmptoms at the moment but I've been told to expect them. I appreciate that neuro's cannot possible keep up with every piece of research that is published but can they also remember all the names for what is to my mind the same disorder. I would be surprised if BFS is so completly different from CFS as for it not to be caused by PNH, but should this turn out to be the case then it would tend to leave people out in the cold as far research goes.

Steve
 
From listening to Steve and reading the research by Hart, it would seem to me that amongst all of our diverse symptoms there are two identifiable types of twitchers: Those who twitch randomly all over with a hotspot in one place for a few hours/ days and then it moves somewhere else and then there are the twitchers who have localised twitching constantly in one or two areas. The most popular manifestation seems to be the calves and feet.

I wonder if it could be said then that the people who twitch constantly for example in the calves are more likely to fall into the potassium channelopathy PNH diagnosis? And those who randomly twitch would not.

Diego4Life
 

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