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Netrin-1 receptor antibodies in thymoma-associated neuromyotonia with myasthenia gravis.
- Source :
-
Neurology [Neurology] 2017 Mar 28; Vol. 88 (13), pp. 1235-1242. Date of Electronic Publication: 2017 Mar 01. - Publication Year :
- 2017
-
Abstract
- Objective: To identify cell-surface antibodies in patients with neuromyotonia and to describe the main clinical implications.<br />Methods: Sera of 3 patients with thymoma-associated neuromyotonia and myasthenia gravis were used to immunoprecipitate and characterize neuronal cell-surface antigens using reported techniques. The clinical significance of antibodies against precipitated proteins was assessed with sera of 98 patients (neuromyotonia 46, myasthenia gravis 52, thymoma 42; 33 of them with overlapping syndromes) and 219 controls (other neurologic diseases, cancer, and healthy volunteers).<br />Results: Immunoprecipitation studies identified 3 targets, including the Netrin-1 receptors DCC (deleted in colorectal carcinoma) and UNC5A (uncoordinated-5A) as well as Caspr2 (contactin-associated protein-like 2). Cell-based assays with these antigens showed that among the indicated patients, 9 had antibodies against Netrin-1 receptors (7 with additional Caspr2 antibodies) and 5 had isolated Caspr2 antibodies. Only one of the 219 controls had isolated Caspr2 antibodies with relapsing myelitis episodes. Among patients with neuromyotonia and/or myasthenia gravis, the presence of Netrin-1 receptor or Caspr2 antibodies predicted thymoma ( p < 0.05). Coexisting Caspr2 and Netrin-1 receptor antibodies were associated with concurrent thymoma, myasthenia gravis, and neuromyotonia, often with Morvan syndrome ( p = 0.009). Expression of DCC, UNC5A, and Caspr2 proteins was demonstrated in paraffin-embedded thymoma samples (3) and normal thymus.<br />Conclusions: Antibodies against Netrin-1 receptors (DCC and UNC5a) and Caspr2 often coexist and associate with thymoma in patients with neuromyotonia and myasthenia gravis.<br />Classification of Evidence: This study provides Class III evidence that antibodies against Netrin-1 receptors can identify patients with thymoma (sensitivity 21.4%, specificity 100%).<br /> (© 2017 American Academy of Neurology.)
- Subjects :
- Adult
Aged
Calcium-Binding Proteins
Cell Adhesion Molecules, Neuronal genetics
Cell Adhesion Molecules, Neuronal metabolism
DCC Receptor
Electromyography
Female
HEK293 Cells
Humans
Immunoprecipitation
Magnetic Resonance Imaging
Male
Membrane Proteins genetics
Membrane Proteins metabolism
Middle Aged
Muscle, Skeletal pathology
Muscle, Skeletal physiopathology
Myasthenia Gravis complications
Myasthenia Gravis diagnostic imaging
Nerve Growth Factors genetics
Nerve Tissue Proteins genetics
Nerve Tissue Proteins metabolism
Netrin Receptors
Netrin-1
Neural Cell Adhesion Molecules
Receptors, Cell Surface genetics
Receptors, Cell Surface metabolism
Thymoma complications
Thymoma diagnostic imaging
Thymus Neoplasms complications
Thymus Neoplasms diagnostic imaging
Transfection
Tumor Suppressor Proteins genetics
Autoantibodies blood
Myasthenia Gravis blood
Nerve Growth Factors immunology
Nerve Growth Factors metabolism
Thymoma blood
Thymus Neoplasms blood
Tumor Suppressor Proteins immunology
Tumor Suppressor Proteins metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 1526-632X
- Volume :
- 88
- Issue :
- 13
- Database :
- MEDLINE
- Journal :
- Neurology
- Publication Type :
- Academic Journal
- Accession number :
- 28251919
- Full Text :
- https://doi.org/10.1212/WNL.0000000000003778