dc.contributor.author |
Graus Ribas, Francesc |
dc.contributor.author |
Saiz Hinajeros, Albert |
dc.contributor.author |
Lai, Marina |
dc.contributor.author |
Bruna, Jordi |
dc.contributor.author |
López, Francisca |
dc.contributor.author |
Sabater, Lidia |
dc.contributor.author |
Blanco, Yolanda |
dc.contributor.author |
Rey, Maria Jesús |
dc.contributor.author |
Ribalta, Teresa |
dc.contributor.author |
Dalmau Obrador, Josep |
dc.date |
2019-01-23T10:53:58Z |
dc.date |
2019-01-23T10:53:58Z |
dc.date |
2008-09-16 |
dc.date |
2019-01-23T10:53:58Z |
dc.identifier.citation |
0028-3878 |
dc.identifier.citation |
612250 |
dc.identifier.uri |
http://hdl.handle.net/2445/127545 |
dc.format |
7 p. |
dc.format |
application/pdf |
dc.language.iso |
eng |
dc.publisher |
Lippincott, Williams & Wilkins. Wolters Kluwer Health |
dc.relation |
Reproducció del document publicat a: https://doi.org/10.1212/01.wnl.0000325917.48466.55 |
dc.relation |
Neurology, 2008, vol. 71, num. 12, p. 930-936 |
dc.relation |
https://doi.org/10.1212/01.wnl.0000325917.48466.55 |
dc.rights |
(c) American Academy of Neurology, 2008 |
dc.rights |
info:eu-repo/semantics/openAccess |
dc.subject |
Encefalitis |
dc.subject |
Immunoglobulines |
dc.subject |
Immunoteràpia |
dc.subject |
Encephalitis |
dc.subject |
Immunoglobulins |
dc.subject |
Immunotheraphy |
dc.title |
Neuronal surface antigen antibodies in limbic encephalitis: clinical-immunologic associations |
dc.type |
info:eu-repo/semantics/article |
dc.type |
info:eu-repo/semantics/publishedVersion |
dc.description.abstract |
Objective: To report the frequency and type of antibodies against neuronal surface antigens (NSA-ab) in limbic encephalitis (LE). Methods: Analysis of clinical features, neuropathologic findings, and detection of NSA-ab using immunochemistry on rat tissue and neuronal cultures in a series of 45 patients with paraneoplastic (23) or idiopathic (22) LE. Results: NSA-ab were identified in 29 patients (64%; 12 paraneoplastic, 17 idiopathic). Thirteen patients had voltage-gated potassium channels (VGKC)-ab, 11 novel NSA (nNSA)-ab, and 5 NMDA receptor (NMDAR)-ab. nNSA-ab did not identify a common antigen and were more frequent in paraneoplastic than idiopathic LE (39% vs 9%; p = 0.03). When compared with VGKC-ab or NMDAR-ab, the nNSA associated more frequently with intraneuronal antibodies (11% vs 73%; p = 0.001). Of 12 patients (9 nNSA-ab, 2 VGKC-ab, 1 NMDAR-ab) with paraneoplastic LE and NSA-ab, concomitant intraneuronal antibodies occurred in 9 (75%). None of these 12 patients improved with immunotherapy. The autopsy of three of them showed neuronal loss, microgliosis, and cytotoxic T cell infiltrates in the hippocampus and amygdala. These findings were compatible with a T-cell mediated neuronal damage. In contrast, 13 of 17 (76%) patients with idiopathic LE and NSA-ab (8 VGKC-ab, 4 NMDAR-ab, 1 nNSA-ab) and 1 of 5 (20%) without antibodies had clinical improvement (p = 0.04). Conclusions: In paraneoplastic limbic encephalitis (LE), novel antibodies against neuronal surface antigens (nNSA-ab) occur frequently, coexist with antibodies against intracellular antigens, and these cases are refractory to immunotherapy. In idiopathic LE, the likelihood of improvement is significantly higher in patients with NSA-ab than in those without antibodies. |