Abstract |
Demyelinating polyneuropathy with anti- myelin associated glycoprotein (anti-MAG) antibodies is an immune mediated disorder characterized by proximal and distal symmetric weakness. Electrophysiological measurements depict features characteristic for demyelination, including prolonged distal latency, retarded conduction velocity, delayed or absent F-waves, and, rarely, partial conduction block. We report on a 65-year-old patient who was diagnosed with demyelinating polyneuropathy and anti-MAG antibodies five years before admission. Despite immunosuppressive agents and extracorporeal therapy ( plasmapheresis) the disease progressed as assessed by clinical symptoms and neurological tests. Laboratory results showed an increase of serum immunoglobulin M and anti-MAG antibodies over time. Because of progressive disease we decided to treat the patient with immunoadsorption followed by application of the anti-CD20 antibody, rituximab. Six cycles of selective immunoadsorption were performed over three-weekly intervals with a repetitively used column (Globaffin); each cycle consisted of four consecutive daily treatments. Starting at cycle 4 the anti-CD20 antibody rituximab was administered with 375 mg/m(2) after immunoadsorption. The pretreatment anti-MAG antibody level of 10,000 U/mL, indicating disease activity, initially increased during treatment to a maximum of 30,559 U/mL. However, after completion of the six cycles, the anti-MAG level had decreased to 2348 U/mL; 16 months after the last immunoadsorption cycle the anti-MAG level had increased to 4134 U/mL, while the conduction velocity and compound motor action potentials remained stabile. Immunoadsorption in combination with a monoclonal anti-CD20 antibody in patients with demyelinating polyneuropathy with anti-MAG is effective and can be used an alternative treatment option in patients with progressive disease.
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Authors | Jürgen Rech, Axel J Hueber, Stefan Kallert, Jan Leipe, Jochen R Kalden, Marcus Beck, Georg Schett, Hendrik Schulze-Koops |
Journal | Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy
(Ther Apher Dial)
Vol. 12
Issue 3
Pg. 205-8
(Jun 2008)
ISSN: 1744-9987 [Electronic] Australia |
PMID | 18503697
(Publication Type: Case Reports, Journal Article)
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Chemical References |
- Adrenal Cortex Hormones
- Antibodies, Monoclonal
- Antibodies, Monoclonal, Murine-Derived
- Autoantibodies
- Immunologic Factors
- Myelin-Associated Glycoprotein
- Rituximab
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Topics |
- Adrenal Cortex Hormones
(administration & dosage)
- Aged
- Antibodies, Monoclonal
(therapeutic use)
- Antibodies, Monoclonal, Murine-Derived
- Autoantibodies
(immunology)
- Demyelinating Diseases
(therapy)
- Humans
- Immunologic Factors
(therapeutic use)
- Immunosorbent Techniques
- Male
- Myelin-Associated Glycoprotein
(blood)
- Polyneuropathies
(therapy)
- Rituximab
- Treatment Outcome
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