Relapses, Comorbidities, and Predictors of Outcome in Anti-GABAA Receptor Encephalitis.
Обработка статьи · этапы, ошибки и управление
Ошибки и ограничения
История · было → стало
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Papi Claudia, Milano Chiara, Marmolejo Laura, Serafim Ana Beatriz, Aguilar Esther, Guasp Mar, Fonseca Elianet, Simabukuro Mateus Mistieri, Iorio Raffaele, Fukami Yuki, Santos Maria Lucia Schmitz Ferreira, Miwa Takashi, Araga Takashi, Uchida Yuto, Kurihara Masanori, Okada Satoshi, Garcia Luz Victoria, Kaida Kenichi, Cunha Natalia Spinola Costa da, Vogrig Alberto, Lamblin Florian, de Vries Juna M, Dutra Livia Almeida, Höftberger Romana, Titulaer Maarten J, Planagumà Jesús, Sabater Lidia, Martinez-Hernandez Eugenia, Armangué Thais, Graus Francesc, Iizuka Takahiro, Dalmau Josep, Spatola Marianna, Anti‐GABAAR encephalitis study group
Аннотация
METHODS: GABAAR antibodies were confirmed by 2 techniques in serum or cerebrospinal fluid. Long-term outcomes were defined as good (modified Rankin scale, mRS = 0-1) or poor (mRS 2-5) at ≥12 months. LMO5 antibodies were assessed by cell-based assays and Western blot.
RESULTS: Thirty-three patients were identified (4 children, 29 adults; median age, 5.5 and 60 years; 61% male). Ten patients (10/32, 31%) had concurrent systemic autoimmunity. Adults presented with seizures and cognitive/behavioral symptoms, often with thymoma, gastrointestinal, or other tumors (18/33, 55%), whereas children frequently had seizures and ataxia with cerebellar MRI lesions. Multifocal T2/fluid-attenuated inversion recovery hyperintensities were present at onset in 23 of 31 (74%) or developed later in those with absent or single lesions. Lesions showed dynamic changes, suggesting ongoing inflammation even without clinical correlate. Relapses occurred in 17 of 31 (55%, all adults) and were associated with older age (p = 0.02) and lack of second-line immunotherapy (p = 0.02). Four patients (4/33, 12%) died. After a 32.5-month median follow-up, 9 of 20 (45%) had persistent cognitive deficits, and 6 of 20 (30%) had a poor outcome, which was associated with relapses (p = 0.04). LMO5 antibodies were absent in patients and controls.
INTERPRETATION: Anti-GABAAR encephalitis shows age-dependent presentations, most commonly seizures. MRI reveals dynamic changes consistent with an ongoing "clinically silent" inflammation. Relapses and cognitive sequelae are common and associate with not receiving second-line immunotherapy. LMO5 antibodies lack tumor-predictive value. ANN NEUROL 2026;100:139-150.