These are a few of my favorite things...

General Topics

Moderators: JohnV, Arron, garym

These are a few of my favorite things...

Postby Angie on February 9th, 2008, 10:25 am

Raindrops on roses and whiskers on kittens
Bright copper kettles and warm woolen mittens…
And this article by John Newsom-Davis.

Since we have some new members that may not have seen it, I thought I would repost it because it's the best article in regards to explaining an autoimmune cause for some cases of BCFS. It also lists the main researchers. For a neurology paper, it's one of the easiest to read which only goes to show how brilliant Newsom-Davis was. It also mentions anxiety which obviously a lot of us have had and states that our condition is treatable.

Neuromyotonia: A diverse disorder.(Invited Commentaries)
Byline: John Newsom-Davis

Peripheral nerve hyperexcitability at its least severe causes muscle cramps and fasciculations termed the Cramp-Fasciculation syndrome by Tahmoush et al. [1] In more severe cases myokymia (visible muscle twitching that can have an undulating characteristic), pseudomyotonia (failure to relax after voluntary contraction) and muscle hypertrophy can be observed and some patients experience painful sensory symptoms and feelings of anxiety. Several names have been applied to this latter syndrome including Isaacs' syndrome and neuromyotonia. The electromyographic changes, first described by Denny Brown, are characterised by myokymic discharges (doublet or multiplet motor unit discharges) and by spontaneous (neuromyotonic) motor unit discharges.

There are several known conditions that cause neuromyotonia. These include axonal damage (e.g., from local nerve irradiation or toxins), timber rattle snake envenomation, certain idiopathic or inherited neuropathies and genetic mutations affecting potassium channels. In the remainder, who constitute the majority of patients, the disorder was regarded as idiopathic until studies dating from 1991 showed that many of these patients have strong immunological associations and respond to plasma exchange.[2] The neurophysiological abnormalities can be transferred to experimental animals by injection of neuromyotonic IgG,[3] unequivocally pointing to an antibody-mediated disorder and implicating voltage-gated potassium channels (VGKCs) as their target. It was later shown that antibodies to VGKCs could be detected in about 40% of patients by radioimmunoassay using [sup] 125a-dendrotoxin.[4] By down-regulating VGKCs, the antibodies would be expected to interfere with nerve repolarisation and lead to repetitive discharges.

Neuromyotonia is now increasingly being recognized as a potentially treatable disorder and the study[5] is remarkable for the large number of patients in their series. The disorder was characterised by electromyography in all of them, but measurement of VGKC antibodies was only possible in one patient in whom the titre was markedly elevated. It cannot be assumed that the disorder in all the remaining 19 patients was due to VGKC antibodies. First, VGKC antibodies are only detected in about 40% of patients. Second, neuromyotonia can associate with inflammatory neuropathies presumably as a result of damage to juxta-nodal VGKCs by an unknown disease process that may not involve an autoantibody attack. Thirdly and importantly, 11 of the 20 patients had been receiving Ayurvedic medications that, as the authors point out, could have been the incriminating factor in these patients. This important observation needs to be widely known and the authors should be congratulated on bringing this to the attention of neurologists.

References

1. Tahmoush AJ, Alonso RJ, Tahmoush GP, Heiman-Patterson TD. Cramp-fasciculation syndrome: A treatable hyperexcitable peripheral nerve disorder. Neurology 1991;41:1021-4.

2. Newsom-Davis J, Mills KR. Immunological associations of acquired neuromyotonia (Isaacs syndrome). Report of five cases and literature review. Brain 1993;116:453-69.

3. Shillito P, Molenaar PC, Vincent A, Leys K, Zheng W, van den Berg RJ, et al. Acquired neuromyotonia: Evidence for autoantibodies directed against K+ channels of peripheral nerves. Ann Neurol 1995;38:714-22.

4. Hart IK, Maddison P, Newsom-Davis J, Vincent A, Mills KR. Phenotypic variants of autoimmune peripheral nerve hyperexcitability. Brain 2002;125:1887-95.

5. Panagariya A, Kumar H, Mathew V, Sharma B. Neuromyotonia: Clinical profile of twenty cases from North West India. Neurol India 2006;54:382-6.

So;

When I’m twitching
When my foot cramps
When I'm feeling sad
I simply remember my favorite journal article
And then I don't feel so bad
Cramp-fasciculation syndrome: a treatable hyperexcitable peripheral nerve disorder.
Neurology. 1991 Jul;41(7):1021-4 Tahmoush AJ, et al.
"muscle aching, cramps, stiffness, exercise intolerance, and peripheral nerve hyper excitability"
User avatar
Angie
Saint
Saint
 
Posts: 946
Joined: March 30th, 2007, 1:35 pm
Location: PA

These are a few of my favorite things...

Sponsor

Sponsor
 

Return to General Topics

Who is online

Users browsing this forum: No registered users and 5 guests