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Progressive encephalomyelitis with rigidity and myoclonus: a syndrome with diverse clinical features and antibody responses.
- Source :
-
European neurology [Eur Neurol] 2013; Vol. 69 (5), pp. 257-62. Date of Electronic Publication: 2013 Feb 20. - Publication Year :
- 2013
-
Abstract
- Background/aims: To better characterize progressive encephalomyelitis with rigidity and myoclonus (PERM) syndrome and identify novel PERM phenotypes.<br />Methods: The clinical features and antibody status of PERM patients were investigated using immunoblots, cell-based assays, RIA, protein macroarray and ELISA.<br />Results: Two patients with supratentorial involvement showed abnormal PET or EEG findings. One patient was discovered to have renal cell carcinoma, and protein macroarray revealed Ma3-antibodies. Another patient with leucine-rich, glioma-inactivated 1 (LGI1) and glutamic acid decarboxylase (GAD) antibodies showed a good response to immunotherapy.<br />Conclusion: The heterogeneity of the immunological features suggests that PERM is caused by diverse pathogenic mechanisms. Seropositivity to well-characterized neuronal cell surface antigens might indicate a good treatment response.<br /> (Copyright © 2013 S. Karger AG, Basel.)
- Subjects :
- Aged
Encephalomyelitis drug therapy
Enzyme-Linked Immunosorbent Assay
Glutamate Decarboxylase immunology
HEK293 Cells
Humans
Immunosuppressive Agents therapeutic use
Intracellular Signaling Peptides and Proteins
Ion Channels immunology
Male
Membrane Proteins immunology
Middle Aged
Muscle Rigidity drug therapy
Myoclonus therapy
Nerve Tissue Proteins immunology
Protein Array Analysis
Proteins immunology
Transfection
Autoantibodies blood
Encephalomyelitis blood
Encephalomyelitis complications
Muscle Rigidity blood
Muscle Rigidity complications
Myoclonus blood
Myoclonus complications
Subjects
Details
- Language :
- English
- ISSN :
- 1421-9913
- Volume :
- 69
- Issue :
- 5
- Database :
- MEDLINE
- Journal :
- European neurology
- Publication Type :
- Academic Journal
- Accession number :
- 23429048
- Full Text :
- https://doi.org/10.1159/000342237