Epidemiología de la miastenia gravis en la provincia de Ourense (Galicia, noroeste de España) / Epidemiology of myasthenia gravis in the province of Ourense (Galicia, Spain)

Introducción: La miastenia gravis (MG) es un enfermedad autoinmune que afecta a la transmisión nerviosa a nivel de la unión neuromuscular causando debilidad muscular típicamente fluctuante. Los estudios epidemiológicos constatan un aumento de las tasas de prevalencia de la MG y es especialmente evidente en la población anciana.ObjetivoRealizar un estudio epidemiológico retrospectivo para conocer las tasas de incidencia y prevalencia en la provincia de Ourense (Galicia) caracterizada por el envejecimiento poblacional.Material y métodosLos pacientes fueron reclutados de nuestra base de datos clínica de enfermedades neuromusculares y a través de la búsqueda de pacientes con prescripción activa de bromuro de piridostigmina. La tasa de incidencia se estimó entre los años 2009-2018. Se estableció la fecha de prevalencia al 31/12/2018. El censo de la provincia de Ourense al 1/1/2019 era de 307.651 habitantes, de los que 96.544 (31,4%) tenían una edad ≥ de 65 años.ResultadosSe identificaron 80 casos de MG. La prevalencia fue de 260 casos/1.000.000 habitantes (IC95%: 202,7-316,4), y en la población ≥ 65 años de 517,9/1.000.000 habitantes (IC95%: 363,2-672,9). La incidencia acumulada en el periodo de estudio fue de 15,4 casos/1.000.000 habitantes-año. El inicio precoz (≤ 50 años) ocurrió en el 29,1% de los casos.ConclusiónLa prevalencia de la MG en nuestra área sanitaria es de las más altas entre las cifras previamente reportadas, y es una enfermedad muy prevalente en la población anciana. (AU)

Introduction: Myasthenia gravis (MG) is an autoimmune disease affecting nerve transmission at the level of the neuromuscular junction, and typically causes fluctuating muscle weakness. Epidemiological studies show an increase in MG prevalence, particularly among the older population.ObjectiveWe performed a retrospective epidemiological study to determine the incidence and prevalence of MG in the province of Ourense (Galicia, Spain), characterised by population ageing.Material and methodsPatients were selected from our clinical neuromuscular diseases database by searching for patients with an active prescription for pyridostigmine bromide. Incidence was estimated for the period 2009-2018. We calculated prevalence at 31/12/2018. According to census data for the province of Ourense, the population on 1/1/2019 was 307,651, of whom 96,544 (31.4%) were aged ≥ 65 years.ResultsWe identified 80 cases of MG, with a prevalence rate of 260 cases/1 000 000 population (95% CI, 202.7-316.4), rising to 517.9/1 000 000 population in those aged ≥ 65 (95% CI, 363.2-672.9). Cumulative incidence in the study period was 15.4 cases per 1 000 000 person-years. Early onset (≤ 50 years) was recorded in 29.1% of cases.ConclusionThe prevalence of MG in our health district is one of the highest published figures, and the disease is highly prevalent in the older population. (AU)

Epidemiology of myasthenia gravis in the province of Ourense (Galicia, Spain).

INTRODUCTION: Myasthenia gravis (MG) is an autoimmune disease affecting nerve transmission at the level of the neuromuscular junction, and typically causes fluctuating muscle weakness. Epidemiological studies show an increase in MG prevalence, particularly among the older population. OBJECTIVE: We performed a retrospective epidemiological study to determine the incidence and prevalence of MG in the province of Ourense (Galicia, Spain), characterised by population ageing. MATERIAL AND METHODS: Patients were selected from our clinical neuromuscular diseases database by searching for patients with an active prescription for pyridostigmine bromide. Incidence was estimated for the period 2009-2018. We calculated prevalence at 31/12/2018. According to census data for the province of Ourense, the population on 1/1/2019 was 307 651, of whom 96 544 (31.4%) were aged ≥ 65 years. RESULTS: We identified 80 cases of MG, with a prevalence rate of 260 cases/1 000 000 population (95% CI, 202.7-316.4), rising to 517.9/1 000 000 population in those aged ≥ 65 (95% CI, 363.2-672.9). Cumulative incidence in the study period was 15.4 cases per 1 000 000 person-years. Early onset (≤ 50 years) was recorded in 29.1% of cases. CONCLUSION: The prevalence of MG in our health district is one of the highest published figures, and the disease is highly prevalent in the older population.

[Clinical and radiological effectiveness of natalizumab extended dosage interval in patients with relapsing multiple sclerosis]. / Efectividad clínica y radiológica del intervalo extendido de dosis con natalizumab en pacientes con esclerosis múltiple recurrente.

INTRODUCTION: Natalizumab (NTZ) is a very effective treatment approved for highly active multiple sclerosis. The main risk of treatment with NTZ is the possibility of developing progressive multifocal leukoencephalopathy, which is related to JC virus positivity and the number of NTZ infusions. This risk decreases with the extended dosage interval (EDI), which involves 9 or fewer infusions/year. However, it is a matter of controversy as to whether EDI remains effective in reducing recurrences and the presence of new lesions in magnetic resonance imaging (MRI). PATIENTS AND METHODS: A prospective observational study was conducted from 1 April 2019 to 30 June 2021, following up patients on NTZ treatment who switched to EDI. Patients should have at least one MRI six months after the start of EDI. The presence of attacks or MRI activity (new lesions in T2) during the EDI was recorded. RESULTS: Twenty-three patients with a mean age of 43.5 ± 9.4 years were included. The median number of NTZ infusions was 68 (minimum, 25; maximum, 127). The median interval between the start of the EDI and the last MRI was 14 months (minimum, 6; maximum, 25), and 23 months from the last medical follow-up visit (minimum, 7; maximum, 28). Two patients (8.7%) presented with attacks and two others (8.7%) showed MRI activity. CONCLUSIONS: EDI with NTZ maintains high clinical and activity effectiveness in MRI.

TITLE: Efectividad clínica y radiológica del intervalo extendido de dosis con natalizumab en pacientes con esclerosis múltiple recurrente.Introducción. El natalizumab (NTZ) es un tratamiento de alta eficacia aprobado para la esclerosis múltiple de alta actividad. El principal riesgo del tratamiento con NTZ es la posibilidad de desarrollar una leucoencefalopatía multifocal progresiva, que está en relación con la positividad al virus JC y el número de infusiones del NTZ. Este riesgo disminuye con el intervalo extendido de dosis (IED), lo que supone 9 infusiones/año o menos. Sin embargo, es materia de controversia si el IED mantiene la efectividad sobre la reducción de las recurrencias y la presencia de nuevas lesiones en la resonancia magnética (RM). Pacientes y métodos. Se ha realizado un estudio observacional prospectivo desde el 1 de abril de 2019 hasta el 30 de junio de 2021, siguiendo a los pacientes en tratamiento con NTZ que se pasaron al IED. Los pacientes deberían tener al menos una RM a los seis meses del inicio del IED. Se registró la presencia de brotes o de actividad en la RM (nuevas lesiones en T2) durante el IED. Resultados. Se incluyó a 23 pacientes con una media de edad de 43,5 ± 9,4 años. La mediana de infusiones de NTZ fue de 68 (mínimo, 25; máximo, 127). La mediana del intervalo entre el inicio del IED y la última RM fue de 14 meses (mínimo, 6; máximo, 25), y de 23 meses respecto a la última visita de seguimiento médico (mínimo, 7; máximo, 28). Dos pacientes (8,7%) presentaron brotes y otros dos pacientes (8,7%) presentaron actividad en la RM. Conclusiones. El IED de NTZ mantiene una alta efectividad clínica y de actividad en la RM.

Efectividad clínica y radiológica del intervalo extendido de dosis con natalizumab en pacientes con esclerosis múltiple recurrente / Clinical and radiological effectiveness of natalizumab extended dosage interval in patients with relapsing multiple sclerosis

Introducción: El natalizumab (NTZ) es un tratamiento de alta eficacia aprobado para la esclerosis múltiple de alta actividad. El principal riesgo del tratamiento con NTZ es la posibilidad de desarrollar una leucoencefalopatía multifocal progresiva, que está en relación con la positividad al virus JC y el número de infusiones del NTZ. Este riesgo disminuye con el intervalo extendido de dosis (IED), lo que supone 9 infusiones/año o menos. Sin embargo, es materia de controversia si el IED mantiene la efectividad sobre la reducción de las recurrencias y la presencia de nuevas lesiones en la resonancia magnética (RM). Pacientes y métodos: Se ha realizado un estudio observacional prospectivo desde el 1 de abril de 2019 hasta el 30 de junio de 2021, siguiendo a los pacientes en tratamiento con NTZ que se pasaron al IED. Los pacientes deberían tener al menos una RM a los seis meses del inicio del IED. Se registró la presencia de brotes o de actividad en la RM (nuevas lesiones en T2) durante el IED. Resultados: Se incluyó a 23 pacientes con una media de edad de 43,5 ± 9,4 años. La mediana de infusiones de NTZ fue de 68 (mínimo, 25; máximo, 127). La mediana del intervalo entre el inicio del IED y la última RM fue de 14 meses (mínimo, 6; máximo, 25), y de 23 meses respecto a la última visita de seguimiento médico (mínimo, 7; máximo, 28). Dos pacientes (8,7%) presentaron brotes y otros dos pacientes (8,7%) presentaron actividad en la RM. Conclusiones: El IED de NTZ mantiene una alta efectividad clínica y de actividad en la RM.(AU)

Introduction: Natalizumab (NTZ) is a very effective treatment approved for highly active multiple sclerosis. The main risk of treatment with NTZ is the possibility of developing progressive multifocal leukoencephalopathy, which is related to JC virus positivity and the number of NTZ infusions. This risk decreases with the extended dosage interval (EDI), which involves 9 or fewer infusions/year. However, it is a matter of controversy as to whether EDI remains effective in reducing recurrences and the presence of new lesions in magnetic resonance imaging (MRI). Patients and methods: A prospective observational study was conducted from 1 April 2019 to 30 June 2021, following up patients on NTZ treatment who switched to EDI. Patients should have at least one MRI six months after the start of EDI. The presence of attacks or MRI activity (new lesions in T2) during the EDI was recorded. Results: Twenty-three patients with a mean age of 43.5 ± 9.4 years were included. The median number of NTZ infusions was 68 (minimum, 25; maximum, 127). The median interval between the start of the EDI and the last MRI was 14 months (minimum, 6; maximum, 25), and 23 months from the last medical follow-up visit (minimum, 7; maximum, 28). Two patients (8.7%) presented with attacks and two others (8.7%) showed MRI activity. Conclusions: EDI with NTZ maintains high clinical and activity effectiveness in MRI.(AU)

Epidemiology of myasthenia gravis in the province of Ourense (Galicia, Spain). / Epidemiología de la miastenia gravis en la provincia de Ourense (Galicia, noroeste de España).

INTRODUCTION: Myasthenia gravis (MG) is an autoimmune disease affecting nerve transmission at the level of the neuromuscular junction, and typically causes fluctuating muscle weakness. Epidemiological studies show an increase in MG prevalence, particularly among the older population. OBJECTIVE: We performed a retrospective epidemiological study to determine the incidence and prevalence of MG in the province of Ourense (Galicia, Spain), characterised by population ageing. MATERIAL AND METHODS: Patients were selected from our clinical neuromuscular diseases database by searching for patients with an active prescription for pyridostigmine bromide. Incidence was estimated for the period 2009-2018. We calculated prevalence at 31/12/2018. According to census data for the province of Ourense, the population on 1/1/2019 was 307,651, of whom 96,544 (31.4%) were aged ≥ 65 years. RESULTS: We identified 80 cases of MG, with a prevalence rate of 260 cases/1 000 000 population (95% CI, 202.7-316.4), rising to 517.9/1 000 000 population in those aged ≥ 65 (95% CI, 363.2-672.9). Cumulative incidence in the study period was 15.4 cases per 1 000 000 person-years. Early onset (≤ 50 years) was recorded in 29.1% of cases. CONCLUSION: The prevalence of MG in our health district is one of the highest published figures, and the disease is highly prevalent in the older population.

[Isolated dysarthria and dysphagia as the presenting symptoms of simultaneous bilateral acute lagunar ischaemic stroke]. / Disartria y disfagia aisladas como forma de presentacion de un ictus isquemico agudo lacunar bilateral y simultaneo.

TITLE: Disartria y disfagia aisladas como forma de presentacion de un ictus isquemico agudo lacunar bilateral y simultaneo.

[Trigeminal-autonomic cephalgia secondary to a spontaneous intra-orbital haematoma]. / Cefalea trigeminoautonomica secundaria a un hematoma intraorbitario espontaneo.

TITLE: Cefalea trigeminoautonomica secundaria a un hematoma intraorbitario espontaneo.

[Experience in the treatment of multiple sclerosis with interferon beta in Galicia]. / Experiencia del tratamiento con interferón beta en la esclerosis múltiple en Galicia.

OBJECTIVE: To analyze the experience in daily clinical practice of interferon-beta (IFN-beta) treatment in relapsing-remitting (RR) and secondary progressive (SP) multiple sclerosis (MS) in Galicia (Spain). PATIENTS AND METHODS: Patients with RR-MS and SP-MS treated with IFN-beta1a and 1b between 1995 and December/2000, analyzing demographic and clinical data. RESULTS: 313 patients were included, with a mean age of 38.2 years. A total of 296 patients (94.6%) were clinically defined MS and 17 (5.4%) were laboratory supported (Poser criteria); 84.6% of the patients were RR and 15.4% were SP. The mean duration of the disease prior to treatment was 7.06 years. Betaferon was used in 52.4% patients (115 RR-MS and 47 SP-MS), Avonex in 26% and Rebif in 21.6%. Relapse rate was reduced in 68.8% for the RR-MS for Betaferon-treated patients, 73.3% for Avonex treated and 35.7% for Rebif-treated patients. Betaferon reduced relapse rate in 50% for SP-MS. The global EDSS remained stable during IFN-beta treatment. During treatment, 33% of Betaferon, 60.5% of Avonex and 54.5% of Rebif-treated patients remained relapse-free. Treatment was suspended in 12.9% of Betaferon, 6.2% of Avonex, and 3% Rebif-treated patients. The most frequent causes of treatment suspension were increase in disability and in relapse count. CONCLUSIONS: The present study supports the benefits of IFN-beta treatment in RR MS and SP MS in daily clinical practice, with reduction in relapses count and incapacity, good over-all tolerance and low incidence of serious adverse side-effects.

Experiencia del tratamiento con interferón B en la esclerosis múltiple en Galicia / Experience in the treatment of multiple of multiple sclerosis with interferón beta in Galicia

Objetivo. Analizar la experiencia en la práctica clínica diaria del tratamiento con interferón b (IFN b) en la esclerosis múltiple (EM) remitenterecidivante (RR) y secundaria progresiva (SP) en Galicia. Pacientes y métodos. Se analizaron los datos clínicos y demográficos de pacientes con EM RR y EM SP tratados con IFN b 1a y 1b entre 1995 y diciembre de 2000. Resultados. Se incluyeron 313 pacientes, con una media de edad de 38,2 años. Un total de 296 caso (94,6%) fueron EM clínicamente definidos y 17 (5,4%) fueron EM apoyados por laboratorio (criterios Poser); el 84,6% de los pacientes padecían EM RR y el 15,4%, EM SP. La duración media de la enfermedad antes del tratamiento fue 7,06 años. Se empleó Betaferón â en el 52,4% de los pacientes (115 EM RR y 47 EM SP), Avonex â en el 26% y Rebif â en el 21,6%. La tasa de brotes se redujo en un 68,8% para la EM RR en los pacientes tratados con Betaferón, en un 73,3% en los tratados con Avonex y en un 35,7% en los tratados con Rebif. El Betaferón redujo la tasa de brotes en un 50% para la EM SP. Durante el tratamiento, el EDSS global permaneció estable, y el 33% de los pacientes tratados con Betaferón, el 60,5% de los tratados con Avonex y el 54,5% de los tratados con Rebif permanecieron libres de brotes. El tratamiento se suspendió en el 12,9% de los pacientes tratados con Betaferón, el 6,2% de los tratados con Avonex, y el 3% de los tratados con Rebif, y las causas más frecuentes fueron el incremento de la discapacidad y el aumento de brotes. Conclusiones. Este estudio apoya el beneficio del tratamiento con IFN b en la EM RR y la EM SP en la práctica clínica diaria, pues promueve la reducción de los brotes, la tasa de brotes y la discapacidad, con una buena tolerancia global y una baja incidencia de efectos secundarios graves (AU)

Objective. To analyze the experience in daily clinical practice of interferonbeta (IFN b) treatment in relapsing remitting (RR) and secondary progressive (SP) multiple sclerosis (MS) in Galicia (Spain). Patients and methods. Patients with RRMS and SPMS treated with IFN b 1a and 1b between 1995 and December/2000, analyzing demographic and clinical data. Results. 313 patients were included, with a mean age of 38,2 years. A total of 296 patients (94,6%) were clinically defined MS and 17 (5,4%) were laboratory supported (Poser criteria); 84,6% of the patients were RR and 15,4% were SP. The mean duration of the disease prior to treatment was 7,06 years. Betaferon â was used in 52,4% patients (115 RRMS and 47 SPMS), Avonex â in 26% and Rebif â in 21,6%. Relapse rate was reduced in 68,8% for the RRMS for Betaferontreated patients, 73,3% for Avonex treated and 35,7% for ebiftreated patients. Betaferon reduced relapse rate in 50% for SPMS. The global EDSS remained stable during IFN b treatment. During treatment, 33% of Betaferon, 60,5% of Avonex and 54,5% of Rebiftreated patients remained relapsefree. Treatment was suspended in 12,9% of Betaferon, 6,2% of Avonex, and 3% Rebiftreated patients. The most frequent causes of treatment suspension were increase in disability and in relapse count. Conclusions. The present study supports the benefits of IFN b treatment in RR MS and SP MS in daily clinical practice, with reduction in relapses count and discapacity, good overall tolerance and low incidence of serious adverse sideeffects (AU)