Thank you very much!!! This gives me hope! I want a cure so bad!
izzetbesi <izzetbesi@...> wrote:
Hi everybody.I found this article.It is hopefull isn't it:
Treatment with Intravenous Immunoglobulin Improves Muscle Strength in a Patient with Dysferlin-Deficient Muscular Dystrophy
Simone Spuler, Joanna Zabojszcza, Male Friedrich-Freksa, Berlin, Germany
OBJECTIVE: To investigate whether ivig improves muscle strength in dysferlin-deficient muscular dystrophy. BACKGROUND: Recently, we reported that complement inhibitory factor CD55 is downregulated in skeletal muscle of mice and patients with dysferlin-deficient muscular dystrophy (LGMD2B). The absence of CD55 increased susceptibility of dysferlin-deficient human myotubes to complement attack in vitro (Wenzel et al. J Immunol.
2005 Nov 1;175:6219-25). One of the possible therapeutic effects of intravenous immunoglobulines (ivig) is inhibition of complement factors C4 and C5. We therefore tested whether ivig might be beneficial in LGMD2B. DESIGN/METHODS: As compassionate use a single female 32 year old patient with dysferlin-deficient muscular dystrophy was treated with ivig (Intraglobin, Biotest AG, Taunus, Germany) for six months after informed consent was obtained. After an induction cycle of 0.4 g/kg body weight for 5 days ivig was applied once a month at 0.4 g/kg body weight. Muscle strength was tested by manual muscle testing. A muscle biopsy specimen was analysed before and after treatment for deposition of sarcolemmal C5b9 membrane attack complex. RESULTS: Ivig was tolerated well. After three months the patient reported a marked improvement in her ability to walk and in her strength when holding a heavy bag. Muscle strength improved
further until treatment-month six. All muscle groups tested improved during treatment, some muscles such as the tibial anterior muscle improved from grade of paresis of 2/5 to almost full strength at 4-5/5. C5b9 membrane attack complex, present on almost all muscle fibres in the pretreatment muscle specimen, were nearly absent in muscle obtained after treatment. CONCLUSIONS/RELEVANCE: The effect of ivig in LGMD2B should be tested in a controlled clinical trial.
That's good news... I am wondering what happend to the rest of the group... Nobody posts lately. Maybe everybody got cured and they run away... Was a lady in...
Yes, I thoroughly agree that it is very hopeful, thanks for sharing it with the members. I had come across this abstract a few weeks ago. Since then, I have ...
Hi. I agree that anything new is exciting. I was previously diagnosed with Polymyositis & had a week long course of Intravenous Immunoglobulin as part of...
I've been lurking on this list--too busy to post anything lately! Although granted, it's hard to define "lurking" on list with such few posts. Brad Williams...
Four years ago, when I was diagnosed with LGMD 2B, Dr. Karpati from Montreal prescribed IVIG. I had two back to back treatments, but then I moved and my new...
Amanda, That's very interesting, and thanks for telling about your experience with IVIg. Since right now we're trying to figure out if some type of trial is ...
Hi Brad, I can't remember if Dr. Karpati treated other dysferlinopathy patients with IVIg but I wouldn't be surprised if he had. He is among the world's...
Amanda thank you for sharing your experience.It is very important and im agree.I think it was ivig.I want to ask some quesions.Can you reply at here or to my...
That is correct, at the time my condition was not 100% confirmed. My biopsy showed a possible depletion of the dysferlin signal. As my file says: "In view of...
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