Encephalopathy in severe SARS-CoV2 infection: Inflammatory or infectious?

Concerning the letter by Moriguchi et al., we describe our experience with a case of encephalopathy with and atypical damage on magnetic resonance imaging (MRI) in a patient with severe infection due to the SARS-CoV2 virus. A 56-year-old woman, without previous pathologies, developed cough, fever, and respiratory failure for five days, after returning from a 6-day trip to Venice. Chest radiography shows a large bilateral interstitial infiltrate. In the first 24 hours, she was admitted to the Intensive Care Unit (ICU) for severe respiratory failure and positive protein chain reaction-PCR in nasal exudate. She needed intubation for ten days. In the first 48 hours outside the ICU, she developed an acute confusional syndrome (hyperactive delirium). Neurological examination showed temporal-spatial disorientation and incoherent fluent speech. An electroencephalogram (EEG) showed generalized hypovoltaic activity. Cranial magnetic resonance imaging showed a bilateral and symmetrical increase in the supratentorial white matter's signal intensity, with a discrete thickening of both temporal lobes, with a slight increase in signal intensity and a sequence of normal diffusion. The lumbar puncture showed no changes (glucose 71 mg/dL, protein 30 mg/dL, 1 leukocyte). Within 72 hours of starting symptoms, she was neurologically asymptomatic. Our final diagnosis was an inflammatory encephalopathy related to a SARS-CoV2 infection.

Guía clínica para el diagnóstico y seguimiento de la distrofia miotónica tipo 1, DM1 o enfermedad de Steinert / Clinical guide for the diagnosis and follow-up of myotonic dystrophy type 1, MD1 or Steinert's disease

Antecedentes y objetivos: La enfermedad de Steinert o distrofia miotónica tipo 1 (DM1), (OMIM 160900) es la miopatía más prevalente en el adulto. Es una enfermedad multisistémica con alteración de prácticamente todos los órganos y tejidos y una variabilidad fenotípica muy amplia, lo que implica que deba ser atendida por diferentes especialistas que dominen las alteraciones más importantes. En los últimos años se ha avanzado de manera exponencial en el conocimiento de la enfermedad y en su manejo. El objetivo de la guía es establecer recomendaciones para el diagnóstico, el pronóstico, el seguimiento y el tratamiento de las diferentes alteraciones de la DM1. Material y métodos: Esta guía de consenso se ha realizado de manera multidisciplinar. Se ha contado con neurólogos, neumólogos, cardiólogos, endocrinólogos, neuropediatras y genetistas que han realizado una revisión sistemática de la literatura. Recomendaciones: Se recomienda realizar un diagnóstico genético con cuantificación precisa de tripletes CTG. Los pacientes con DM1 deben seguir control cardiológico y neumológico de por vida. Antes de cualquier cirugía con anestesia general debe realizarse una evaluación respiratoria. Debe monitorizarse la presencia de síntomas de disfagia periódicamente. Debe ofrecerse consejo genético a los pacientes con DM1 y a sus familiares. Conclusión: La DM1 es una enfermedad multisistémica que requiere un seguimiento en unidades especializadas multidisciplinares

Background and objectives: Steinert's disease or myotonic dystrophy type 1 (MD1), (OMIM 160900), is the most prevalent myopathy in adults. It is a multisystemic disorder with dysfunction of virtually all organs and tissues and a great phenotypical variability, which implies that it has to be addressed by different specialities with experience in the disease. The knowledge of the disease and its management has changed dramatically in recent years. This guide tries to establish recommendations for the diagnosis, prognosis, follow-up and treatment of the complications of MD1. Material and methods: Consensus guide developed through a multidisciplinary approach with a systematic literature review. Neurologists, pulmonologists, cardiologists, endocrinologists, neuropaediatricians and geneticists have participated in the guide. Recommendations: The genetic diagnosis should quantify the number of CTG repetitions. MD1 patients need cardiac and respiratory lifetime follow-up. Before any surgery under general anaesthesia, a respiratory evaluation must be done. Dysphagia must be screened periodically. Genetic counselling must be offered to patients and relatives. Conclusion: MD1 is a multisystemic disease that requires specialised multidisciplinary follow-up

Clinical guide for the diagnosis and follow-up of myotonic dystrophy type 1, MD1 or Steinert's disease. / Guía clínica para el diagnóstico y seguimiento de la distrofia miotónica tipo 1, DM1 o enfermedad de Steinert.

BACKGROUND AND OBJECTIVES: Steinert's disease or myotonic dystrophy type 1 (MD1), (OMIM 160900), is the most prevalent myopathy in adults. It is a multisystemic disorder with dysfunction of virtually all organs and tissues and a great phenotypical variability, which implies that it has to be addressed by different specialities with experience in the disease. The knowledge of the disease and its management has changed dramatically in recent years. This guide tries to establish recommendations for the diagnosis, prognosis, follow-up and treatment of the complications of MD1. MATERIAL AND METHODS: Consensus guide developed through a multidisciplinary approach with a systematic literature review. Neurologists, pulmonologists, cardiologists, endocrinologists, neuropaediatricians and geneticists have participated in the guide. RECOMMENDATIONS: The genetic diagnosis should quantify the number of CTG repetitions. MD1 patients need cardiac and respiratory lifetime follow-up. Before any surgery under general anaesthesia, a respiratory evaluation must be done. Dysphagia must be screened periodically. Genetic counselling must be offered to patients and relatives. CONCLUSION: MD1 is a multisystemic disease that requires specialised multidisciplinary follow-up.

Do not do in COPD: consensus statement on overuse.

Background: To identify practices that do not add value, cause harm, or subject patients with chronic obstructive pulmonary disease (COPD) to a level of risk that outweighs possible benefits (overuse). Methods: A qualitative approach was applied. First, a multidisciplinary group of healthcare professionals used the Metaplan technique to draft and rank a list of overused procedures as well as self-care practices in patients with stable and exacerbated COPD. Second, in successive consensus-building rounds, description files were created for each "do not do" (DND) recommendation, consisting of a definition, description, quality of supporting evidence for the recommendation, and the indicator used to measure the degree of overuse. The consensus group comprised 6 pulmonologists, 2 general practitioners, 1 nurse, and 1 physiotherapist. Results: In total, 16 DND recommendations were made for patients with COPD: 6 for stable COPD, 6 for exacerbated COPD, and 4 concerning self-care. Conclusion: Overuse poses a risk for patients and jeopardizes care quality. These 16 DND recommendations for COPD will lower care risks and improve disease management, facilitate communication between physicians and patients, and bolster patient ability to provide self-care.

¿Sabrías qué no hay que hacer en EPOC? / No disponible

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Valor diagnóstico de la poligrafía respiratoria en pacientes con baja probabilidad de síndrome de apneas e hipopneas durante el sueño / Diagnostic value of respiratory polygraphy in patients with low probability of obstructive sleep apnea syndrome

Introducción y objetivo: La polisomnografía (PSG) es el método estándar para el diagnóstico del síndrome de apneas e hipopneas del sueño (SAHS). Es una técnica cara, compleja y de poca disponibilidad, por lo que la poligrafía respiratoria (PR) es de uso habitual. La PR no está validada en casos de baja probabilidad; sin embargo, la normativa vigente contempla el tratamiento conservador en caso de PR negativa. Nos hemos propuesto estudiar la prevalencia y gravedad del SAHS mediante PSG, en una muestra de pacientes con baja probabilidad y PR negativa. Material y métodos: Estudio retrospectivo, observacional, descriptivo y analítico de pacientes con baja probabilidad de SAHS y PR negativa a los que se les realizó posteriormente una PSG. Se registraron datos antropométricos, clínicos y características del sueño. Resultados: Ochenta y dos pacientes fueron incluidos. En el registro de la PSG se observó un incremento de hipopneas (137,8 ± 70,1 frente a 51,2 ± 38,4 [p < 0,05]) y del índice de apneas e hipopneas (27,8 ± 15,6 frente a 11,7 ± 7,1 [p < 0,05]), así como un aumento del 17% en la prevalencia de SAHS, de un 35% de casos graves y una disminución de un 41% de los casos leves. Conclusión: De acuerdo con los resultados de este estudio, la PR subestima de forma estadísticamente significativa la prevalencia y gravedad del SAHS en pacientes con baja probabilidad. Es necesario un adecuado proceso de estratificación de riesgo para la correcta indicación de pruebas diagnósticas, y recomendable realizar una PSG cuando se ha realizado una PR con resultado negativo en estos pacientes (AU)

Introduction and objective: Polysomnography (PSG) is the gold standard technic for the diagnosis of obstructive sleep apnea syndrome (OSAS). It is an expensive, complex and not always available technic, meaning that respiratory polygraphy (RP) has become usual. Although RP is not validated in low probability patients, Spanish guidelines recommend conservative treatment in patients with negative RP. We intended to study the prevalence and severity of OSAS through PSG in a sample of patients with low probability and negative RP. Material and methods:Retrospective, observational, descriptive and analytic study of low probability OSAS patients with negative RP in whom a PSG was performed. Anthropometric, clinical and sleep data were collected. Results: Eighty-two patients were included. After PSG, a greater number of hypopneas (137.8 ± 70.1 vs. 51.2 ± 38.4 [P < .05]) and apnea hypopnea index (27.8 ± 15.6 vs. 11.7 ± 7.1 [P < .05]) was observed, as well as an increment in OSAS prevalence of 17%, which was 35% in severe OSAS. In mild OSAS, there was a decrement of 41%. Conclusion: According with the results of this study, RP significantly underestimates the prevalence and severity of OSAS in low probability patients. While it is necessary to adequately stratify the OSAS probability in order to correctly indicate diagnosis tests, we recommend performing a PSG in low probability patients with negative RP (AU)

[Diagnostic value of respiratory polygraphy in patients with low probability of obstructive sleep apnea syndrome]. / Valor diagnóstico de la poligrafía respiratoria en pacientes con baja probabilidad de síndrome de apneas e hipopneas durante el sueño.

INTRODUCTION AND OBJECTIVE: Polysomnography (PSG) is the gold standard technic for the diagnosis of obstructive sleep apnea syndrome (OSAS). It is an expensive, complex and not always available technic, meaning that respiratory polygraphy (RP) has become usual. Although RP is not validated in low probability patients, Spanish guidelines recommend conservative treatment in patients with negative RP. We intended to study the prevalence and severity of OSAS through PSG in a sample of patients with low probability and negative RP. MATERIAL AND METHODS: Retrospective, observational, descriptive and analytic study of low probability OSAS patients with negative RP in whom a PSG was performed. Anthropometric, clinical and sleep data were collected. RESULTS: Eighty-two patients were included. After PSG, a greater number of hypopneas (137.8±70.1 vs. 51.2±38.4 [P<.05]) and apnea hypopnea index (27.8±15.6 vs. 11.7±7.1 [P<.05]) was observed, as well as an increment in OSAS prevalence of 17%, which was 35% in severe OSAS. In mild OSAS, there was a decrement of 41%. CONCLUSION: According with the results of this study, RP significantly underestimates the prevalence and severity of OSAS in low probability patients. While it is necessary to adequately stratify the OSAS probability in order to correctly indicate diagnosis tests, we recommend performing a PSG in low probability patients with negative RP.