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12474_9274278_thymomae1053.full.pdf (493.55 KB)

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2021-07-23

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cc by-nc-nd (c) Guasp, Mar et al., 2021
Si us plau utilitzeu sempre aquest identificador per citar o enllaçar aquest document: https://hdl.handle.net/2445/182720

Thymoma and Autoimmune Encephalitis: Clinical Manifestations and Antibodies

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https://doi.org/10.1212/nxi.0000000000001053

Resum

To report the clinical, neuroimaging, and antibody associations in patients with autoimmune encephalitis (AE) and thymoma.A retrospective cohort study of 43 patients was conducted. Antibody determination and immunoprecipitation to characterize novel antigens were performed using reported techniques.Patients' median age was 52 years (range: 23-88 years). Forty (93%) had neuronal surface antibodies: gamma-aminobutyric acid receptor A (GABAAR) (15), amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR) (13), contactin-associated protein-like 2 (CASPR2) (4), leucine-rich, glioma inactivated 1 (LGI1) (3), glycine receptor (GlyR) (3), and unknown antigens (2). Concurrent antibodies against intracellular antigens occurred in 13 (30%; 9 anti-collapsin response mediator protein 5 [CRMP5]) and were more frequent in anti-AMPAR encephalitis (54% vs 20%; p = 0.037). The most common clinical presentation was encephalitis with multiple T2/fluid-attenuated inversion recovery hyperintense lesions in 23 (53%) patients (15 GABAAR, 5 AMPAR, and 1 unknown neuropil antibody), followed by encephalitis with peripheral nerve hyperexcitability in 7 (16%; 4 CASPR2, 2 LGI1, and 1 unknown antibody), limbic encephalitis in 6 (14%; 4 AMPAR, 1 LGI1, and 1 antibody negative), progressive encephalomyelitis with rigidity and myoclonus in 4 (9%; 3 GlyR and 1 AMPAR antibodies), and encephalitis with normal MRI in 3 (7%; AMPAR antibodies). Anti-GABAAR encephalitis was more prevalent in Japanese patients compared with Caucasians and other ethnicities (61% vs 16%; p = 0.003). In anti-AMPAR encephalitis, 3/4 patients with poor and 0/6 with good outcome had concurrent CRMP5 antibodies (p = 0.033). Immunoprecipitation studies identified metabotropic glutamate receptor 3 antibodies that were additionally found in 5 patients (3 with and 2 without encephalitis).AE in patients with thymoma include several clinical-radiologic syndromes that vary according to the associated antibodies. Anti-GABAAR encephalitis was the most frequent AE and occurred more frequently in Japanese patients.

Matèries

Encefalitis, Estudi de casos, Immunologia

Matèries (anglès)

Encephalitis, Case studies, Immunology

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Articles publicats en revistes (Medicina)
Articles publicats en revistes (IDIBAPS: Institut d'investigacions Biomèdiques August Pi i Sunyer)
Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
Articles publicats en revistes (Institut de Neurociències (UBNeuro))

Citació

GUASP, Mar, LANDA MEDRANO, Jon, MARTÍNEZ HERNÁNDEZ, Eugenia, SABATER BAUDET, Lidia, IIZUKA, Takahiro, SIMABUKURO, Mateus, NAKAMURA, Masataka, KINOSHITA, Makoto, KAIDA, Kenichi, BRUNA, Jordi, KAPETANOVIC, Solange, SÁNCHEZ, Pedro, RUIZ GARCÍA, Raquel, NARANJO, Laura, PLANAGUMÀ, Jesús, MUÑOZ LOPETEGI, Amaia, BATALLER, Luis, SAIZ HINAREJOS, Albert, DALMAU OBRADOR, Josep, GRAUS RIBAS, Francesc, KURIHARA, Masanori. Thymoma and Autoimmune Encephalitis: Clinical Manifestations and Antibodies. _Neurology-Neuroimmunology & Neuroinflammation_. 2021. Vol. 8, núm. 5, pàgs. e1053. [consulta: 21 de gener de 2026]. ISSN: 2332-7812. [Disponible a: https://hdl.handle.net/2445/182720]

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