[Rituximab for the treatment of generalised myasthenia gravis: experience in clinical practice]. / Rituximab para el tratamiento de la miastenia grave generalizada: experiencia en la práctica clínica.

INTRODUCTION AND AIMS: Rituximab (RTX) is an anti-CD20 monoclonal antibody that has been used in cases of refractory myasthenia gravis (MG). The aim of this work is to analyse the efficacy and safety of RTX in MG in real clinical practice in a tertiary hospital. PATIENTS AND METHODS: A retrospective study was conducted with patients with MG treated with RTX in our centre from March 2014 to September 2020. Demographic and serological data, together with information about previous immunomodulatory treatment, clinical response and adverse effects are collected. RESULTS: Twenty patients with MG - 100% generalised: 70% late-onset MG (LOMG) and 30% early-onset MG (EOMG) - were given RTX (mean age: 66.8 years; 70% male). A total of 90% are seropositive, 16 of them with positive anti-acetylcholine receptor antibodies and two with positive muscle-specific tyrosine kinase (anti-MuSK) antibodies. All had failed previous treatments: 100% with steroids, 100% with intravenous immunoglobulins and/or plasmapheresis, 55% with other immunosuppressants (25% with one previous immunosuppressant, 10% with two, 15% with three and 5% with four) and 35% with thymectomy. After RTX, 75% of patients showed a clinical response (12 patients with complete remission and the possibility of steroid withdrawal without recurrence; and three patients with partial remission and the possible reduction of steroid dosage) and 25% therapeutic failure; in all these cases RTX was withdrawn. All the anti-MuSK+ patients (100%) and 92.8% of the LOMG patients responded to RTX, while 66% of EOMG patients failed. Only three patients reported adverse effects, all of which were mild and did not require RTX withdrawal. CONCLUSION: In our experience, rituximab is a safe and effective treatment in aggressive generalised MG with anti-MuSK or late-onset MG (LOMG).

TITLE: Rituximab para el tratamiento de la miastenia grave generalizada: experiencia en la práctica clínica.Introducción y objetivos. El rituximab (RTX) es un anticuerpo monoclonal anti-CD20 que se ha utilizado en casos de miastenia grave (MG) refractaria. El objetivo de este trabajo es analizar la eficacia y la seguridad del RTX en la MG en la práctica clínica real en un hospital terciario. Pacientes y métodos. Se realiza un estudio retrospectivo de pacientes con MG tratados con RTX en nuestro centro de marzo de 2014 a septiembre de 2020. Se recogen datos demográficos, serológicos, tratamiento inmunomodulador previo, respuesta clínica y efectos adversos. Resultados. Veinte pacientes con MG ­el 100%, generalizada: el 70%, MG de inicio tardío (LOMG), y el 30%, MG de inicio temprano (EOMG)­ han recibido RTX (edad media: 66,8 años; 70%, varones). El 90% son seropositivos ­16 con anticuerpos antirreceptor de la acetilcolina positivos y dos con anticuerpos antitirosincinasa muscular específica (anti-MuSK) positivos­. Todos habían fracasado con tratamientos previos: el 100% con esteroides, el 100% con inmunoglobulinas intravenosas y/o con plasmaféresis, el 55% con otros inmunosupresores (25%, un inmunosupresor previo; 10%, dos; 15%, tres; y 5%, cuatro) y el 35% con timectomía. Tras el RTX, presentó respuesta clínica el 75% de los pacientes (12 pacientes, remisión completa con posibilidad de la retirada de los esteroides sin recurrencia; y tres parcial, con posibilidad de la reducción de la dosis de esteroides) y fracaso terapéutico el 25%; en todos estos casos se retiró el RTX. El 100% de los pacientes anti-MuSK+ y el 92,8% de los de LOMG presentaron respuesta al RTX, mientras que el 66% de los pacientes con EOMG fracasaron. Sólo tres pacientes presentaron efectos adversos, todos leves, que no requirieron la retirada del RTX. Conclusión. En nuestra experiencia, el rituximab es un tratamiento seguro y eficaz en la MG generalizada agresiva con anticuerpos anti-MuSK o de inicio tardío (LOMG).

[Recurrent cerebral embolism as the main sign of atrial myxoma]. / Embolismos cerebrales de repetición como manifestación principal del mixoma auricular.

Atrial myxomas (AM) are the most frequent cardiac tumors. Between 25-45% of the cases present neurologic manifestations, generally owing to cerebral embolic infarcts or intracranial haemorrhages. Cerebral embolism can precede cardiac or constitutional symptoms, but recurrent embolisms as the only disturbance are infrequent. We present the case of two patients with 66 and 70 years that suffered recurrent cerebral embolisms as the only manifestation of a left AM. Magnetic resonance (MR) studies showed several isquemic infarcts in different vascular territories. Echocardiagrams showed the presence of heterogeneous masses in left atrial that suggested AM. These were removed in both cases and the diagnosis of AM was confirmed by anatomopathologic exam. The presence of recurrent isquemic episodes in different cerebral territories must point the diagnosis to a cardioembolic source. AM must be taken into account in these cases, despite the lack of constitutional or cardiac symptoms, older age or concomitant carotid pathology.