Abstract |
In this study we report a patient with chronic progressive sensory ataxia, proximal weakness, immunoglobulin M ( IgM) monoclonal gammopathy, and elevated protein levels in the cerebrospinal fluid, who showed a good response to prednisone. Electrophysiological study disclosed abnormalities predominantly of late responses (F waves and H reflexes), with no evidence of demyelination in the peripheral nerves, suggesting motor and preganglionic sensory nerve roots as the site of the lesion. An immune-mediated pathogenesis was considered and, to identify possible target antigens, we performed bidimensional electrophoresis and a Western blot study. Based on the suspected lesion site, we used human anterior and posterior root extracts. We identified IgM reactivity against peripheral nerve myelin protein P2. Enzyme-linked immunosorbent assay confirmed IgM reactivity toward one synthetic peptide from P2. To our knowledge, reactivity against P2 has not been reported previously in a paraproteinemic neuropathy. Furthermore, we demonstrated that bidimensional electrophoresis and Western blot of the tissue involved, as determined by clinical and electrophysiological studies, may be useful to establish clinical-immunological correlations in paraproteinemic neuropathies.
|
Authors | Ricard Rojas-Garcia, Eduard Gallardo, Carolina De La Torre, Lara Sanvito, Isabel Illa |
Journal | Muscle & nerve
(Muscle Nerve)
Vol. 38
Issue 1
Pg. 933-8
(Jul 2008)
ISSN: 0148-639X [Print] United States |
PMID | 18508345
(Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
|
Copyright | (c) 2008 Wiley Periodicals, Inc. |
Chemical References |
- Anti-Inflammatory Agents
- Myelin P2 Protein
- Nerve Tissue Proteins
- Prednisone
|
Topics |
- Aged
- Anti-Inflammatory Agents
(therapeutic use)
- Blotting, Western
- Chronic Disease
- Electrophoresis, Gel, Two-Dimensional
- Electrophysiology
- Enzyme-Linked Immunosorbent Assay
- Humans
- Male
- Myelin P2 Protein
(genetics, immunology)
- Nerve Tissue Proteins
(biosynthesis, genetics)
- Polyradiculopathy
(genetics, immunology, physiopathology)
- Prednisone
(therapeutic use)
- Proteomics
- Spinal Nerve Roots
(pathology)
|