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.