Impact of SARS-CoV-2 infection and COVID-19 pandemic on the morbidity and mortality of amyotrophic lateral sclerosis patients in Valencia, Spain.

BACKGROUND AND PURPOSE: The purpose was to describe the risk of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, hospitalization for coronavirus disease 2019 (COVID-19) and related death and to assess the impact of the pandemic in the survival of amyotrophic lateral sclerosis (ALS) patients. METHODS: The risk of SARS-CoV-2 infection, hospitalization for COVID-19 and related death was assessed in ALS patients alive between March 2020 and July 2022. To evaluate its impact in the overall survival of ALS patients, the survival of patients who died before and during the pandemic was compared. RESULTS: Amongst 263 ALS patients alive during the pandemic, 62 got infected during the study period (infection rate 14.34 per 100 person-years). Most infections (68%) occurred during the sixth wave (November 2021 to January 2022) and most patients (67%) were vaccinated at the time of infection. The hospitalization rate due to COVID-19 was 4.16 per 100 person-years. The multivariable model confirmed non-invasive ventilation (NIV) use prior to infection as a risk factor for hospitalization (odds ratio [OR] = 7.96, p = 0.003) and COVID-19 vaccination as a protective factor (OR = 0.093, p = 0.025) independent of age, sex and gastrostomy. Within 30 days after infection, 7% of non-ventilated patients started NIV and five patients (8.06%) died, of whom four were previously ventilated. The median survival of ALS patients was similar before and during the pandemic and no effect was found in the Cox regression model (hazard ratio 1.02, p = 0.89). CONCLUSIONS: This study shows a high risk of severe COVID-19 amongst ALS patients requiring NIV. Nevertheless, the pandemic showed no impact in the overall survival of ALS patients, probably due to a high vaccination rate and an adequate access to healthcare resources.

Vocal cord paresis and diaphragmatic dysfunction are severe and frequent symptoms of GDAP1-associated neuropathy.

Cranial nerve involvement in Charcot-Marie-Tooth disease (CMT) is rare, though there are a number of CMT syndromes in which vocal cord paralysis is a characteristic feature. CMT disease due to mutations in the ganglioside-induced differentiation-associated protein 1 gene (GDAP1) has been reported to be associated with vocal cord and diaphragmatic palsy. In order to address the prevalence of these complications in patients with GDAP1 mutations we evaluated vocal cord and respiratory function in nine patients from eight unrelated families with this disorder. Hoarseness of the voice and inability to speak loudly were reported by eight patients and one had associated symptoms of respiratory insufficiency. Patients were investigated by means of peripheral and phrenic nerve conduction studies, flexible laryngoscopy, pulmonary function studies and polysomnography. Nerve conduction velocities and pathological studies were compatible with axonal CMT (CMT2). Flexible laryngoscopy showed left vocal cord palsy in four cases, bilateral cord palsies in four cases and was normal in one case. Restrictive respiratory dysfunction was seen in the eight patients with vocal cord paresis who were all chair-bound. These eight had confirmed phrenic nerve dysfunction on neurophysiology evaluation. The patient with normal vocal cord and pulmonary function had a less severe clinical course.This study shows that CMT patients with GDAP1 mutations develop severe disability due to weakness of limb muscles and that laryngeal and respiratory muscle involvement occurs late in the disease process when significant proximal upper limb weakness has developed. The early and predominant involvement of the left vocal cord innervated by the longer left recurrent laryngeal nerve suggests a length dependent pattern of nerve degeneration. In GDAP1 neuropathy, respiratory function should be thoroughly investigated because life expectancy can be compromised due to respiratory failure.

[Predictive value of chest X-ray and electrocardiographic abnormalities in patients with pulmonary embolism in relation to mortality and recurrences]. / Papel de las alteraciones radiográficas y del electrocardiograma en la predicción de mortalidad y recidivas en pacientes con embolia de pulmón.

BACKGROUND AND OBJECTIVE: Radiological and electrocardiographic (EKG) abnormalities are very frequent in patients with pulmonary embolism (PE). Our main objective was to evaluate, in patients with PE, whether any of those abnormalities was correlated with an increased risk of relapses or mortality until 3 months after the acute episode. PATIENTS AND METHOD: Multicenter observational study of patients included in the Venous Thromboembolic Disease Register (RIETE). Demographic, clinical, radiological and EKG data were collected from all patients. Statistics was made with the Pearson correlation and multiple linear regression analysis. RESULTS: We found a significant but non determinant association between mortality and the presence of radiological and/or EKG abnormalities and no relation between them and relapses of PE. CONCLUSIONS: Radiological or EKG manifestations could not predict relapses or mortality 3 months after PE in our patients.

[Usefulness of basic tests in patients with pulmonary embolism]. / Utilidad de las pruebas básicas en el paciente con embolia pulmonar.

There are common, specific noninvasive and invasive tests for the diagnosis of pulmonary embolism (PE). Arterial blood gases, lung radiography and electrocardiography belong to the first group and should be performed in patients with suspected PE. Among the second group, D-dimer determination is essential. Ventilation-perfusion scintigraphy was formerly the most frequently used test but has been relegated to a second-line position by the development of multislice computed tomography (CT) angiography, which provides the possibility of simultaneously studying the lower limbs to detect deep venous thrombosis (DVT). SPECT and magnetic resonance imaging can be used as an alternative to multislice CT angiography, but there is less experience with these techniques. Transthoracic echocardiography has, above all, predictive value and lower limb ultrasonography is the most common non-invasive method to detect DVT. Invasive tests, the gold standard, are often unavailable and their use is therefore limited to selected patients. In conclusion, noninvasive management (clinical probability, D-dimer and multislice CT) is feasible in most patients with suspected PE.

[Early complications in patients with pulmonary embolism]. / Complicaciones precoces en pacientes con embolia de pulmón.

BACKGROUND AND OBJECTIVE: Mortality, recurrences and hemorrhages are the most serious early complications of pulmonary embolism (PE). We intended to ascertain the frequency and mechanisms of complications within the first 10 days after PE was diagnosed. PATIENTS AND METHOD: We included patients suspected of suffering PE between December 2003 and August 2004 from 8 hospitals. We performed a multicenter observational prospective study of 8 Spanish hospitals with consecutive outpatients diagnosed with PE. Database of clinical variables: computerized Registry of Patients with Venous Thromboembolism (RIETE). Statistic analysis included chi2 and Student's t test to compare the 2 groups of patients (with and without complications). RESULTS: Six hundred eighty one patients were included, 336 men and 345 women (mean age 66 and 70, respectively). During the first 10 days, 33 (4.8%) of them died, and 22 (3.2%) had an hemorrhage yet no recurrences appeared. Previous immobilization and the presence of respiratory failure, cancer or renal failure were significantly associated with early complications while previous surgery and thoracic pain were not associated with those. CONCLUSIONS: Early complications and some of the associated factors were in agreement with published data. Other situations such as previous surgery or thoracic pain appeared to be protective factors.

Papel de las alteraciones radiográficas y del electrocardiograma en la predicción de mortalidad y recidivas en pacientes con embolia de pulmón / Predictive value of chest X-ray and electrocardiographic abnormalities in patients with pulmonary embolism in relation to mortality and recurrences

Fundamento y objetivo: es sabido que las alteraciones radiológicas y electrocardiográficas son muy frecuentes y diversas en los pacientes con embolia de pulmón (EP), pero se desconoce si pueden ser factores pronósticos a largo plazo (hasta los 3 meses). El objetivo principal del presente estudio ha sido evaluar si las alteraciones en la radiografía de tórax y en el electrocardiograma (ECG) podrían predecir la aparición de recidivas o la muerte en esta enfermedad. Pacientes y método: se ha realizado un estudio observacional y multicéntrico de pacientes diagnosticados de EP incluidos en el Registro Informatizado de la Enfermedad Tromboembólica venosa en España (RIETE). Se recogieron las variables demográficas, clínicas y exploratorias incluidas en el registro. Se aplicó un test de correlación de Pearson para averiguar la relación entre las variables, y con las variables significativas encontradas se realizó una regresión lineal múltiple, y se evaluaron las ecuaciones de predicción según supuestos de independencia, homocedasticidad y normalidad. Resultados: los hallazgos de la radiografía y el ECG se asociaron con la mortalidad por EP, pero ninguno de ellos fue determinante en el fallecimiento de los pacientes. No se encontró relación alguna entre estas variables y la aparición de recidivas. Conclusiones: la aparición o el tipo de alteraciones en la radiografía de tórax y el ECG no constituyeron factores predictivos de recidivas o de mortalidad en los primeros 3 meses del episodio agudo de EP (AU)

Background and objective: Radiological and electrocardiographic (EKG) abnormalities are very frequent in patients with pulmonary embolism (PE). Our main objective was to evaluate, in patients with PE, whether any of those abnormalities was correlated with an increased risk of relapses or mortality until 3 months after the acute episode. Patients and method: Multicenter observational study of patients included in the Venous Thromboembolic Disease Register (RIETE). Demographic, clinical, radiological and EKG data were collected from all patients. Statistics was made with the Pearson correlation and multiple linear regression analysis. Results: We found a significant but non determinant association between mortality and the presence of radiological and/or EKG abnormalities and no relation between them and relapses of PE. Conclusions: Radiological or EKG manifestations could not predict relapses or mortality 3 months after PE in our patients (AU)

Utilidad de las pruebas básicas en el paciente con embolia pulmonar / Usefulness of basic tests in patients with pulmonary embolism

Se clasifican en pruebas genéricas, específicas no invasivas y específicas invasivas. La gasometría arterial, la radiografía de tórax y el electrocardiograma pertenecen al primer grupo, y es recomendable su realización ante un paciente con sospecha de embolia pulmonar. Dentro del segundo grupo, la determinación del dímero D es imprescindible en el algoritmo diagnóstico; la gammagrafía de ventilaciónperfusión ha sido la más utilizada hasta la actualidad, pero el desarrollo de la angio-TC multidetectora, que además ofrece la posibilidad del estudio simultáneo de las extremidades inferiores para la detección de una trombosis venosa profunda, la ha desplazado a un segundo término; la resonancia magnética o la tomografía computarizada por emisión monofotónica (SPECT) pueden ser una alternativa a la angio-TC, aunque la experiencia con ellas es menor; la ecocardiografía transtorácica tiene sobre todo valor pronóstico y la eco-Doppler de las extremidades inferiores es el método no invasivo por excelencia para diagnosticar una trombosis venosa profunda. Las pruebas invasivas son poco accesibles y sus indicaciones, muy limitadas hoy en día. Se puede concluir que es posible el manejo no invasivo (probabilidad clínica, dímero D y angio-TC de tórax y extremidades inferiores) en la mayor parte de los pacientes con sospecha de embolia pulmonar

There are common, specific noninvasive and invasive tests for the diagnosis of pulmonary embolism (PE). Arterial blood gases, lung radiography and electrocardiography belong to the first group andshould be performed in patients with suspected PE. Among the second group, D-dimer determination is essential. Ventilation-perfusion scintigraphy was formerly the most frequently used test but has been relegated to a second-line position by the development of multislicecomputed tomography (CT) angiography, which provides the possibility of simultaneously studying the lower limbs to detect deep venous thrombosis (DVT). SPECT and magnetic resonance imaging can be used as an alternative to multislice CT angiography, but there is lessexperience with these techniques. Transthoracic echocardiography has, above all, predictive value and lower limb ultrasonography is the most common non-invasive method to detect DVT. Invasive tests, thegold standard, are often unavailable and their use is therefore limited to selected patients. In conclusion, noninvasive management (clinical probability, D-dimer and multislice CT) is feasible in most patientswith suspected PE

Complicaciones precoces en pacientes con embolia de pulmón / Early complications in patients with pulmonary embolism

Fundamento y objetivo: Las complicaciones precoces más graves de la embolia pulmonar (EP) son la mortalidad, las recidivas y las hemorragias. Los objetivos de este estudio fueron conocer cuáles son las complicaciones más frecuentes y los factores a ellas asociados en los primeros 10 días siguientes al diagnóstico de EP. Pacientes y método: Se incluyó prospectivamente en el estudio a todos los pacientes ingresados por sospecha de EP entre diciembre de 2003 y agosto de 2004, procedentes de 8 hospitales. Se evaluó la aparición de complicaciones durante los primeros 10 días. Las variables clínicas recogidas se incluyeron en el Registro Informatizado de la Enfermedad Tromboembólica en España (RIETE). El análisis estadístico se realizó mediante las pruebas de la *2 y de la t de Student para la comparación de los grupos según presentaran o no complicaciones; se efectuó un análisis multivariado para averiguar qué factores estaban relacionados con la aparición de complicaciones. Resultados: El número de pacientes fue de 681, 336 hombres y 345 mujeres, edad media 66 y 70 años, respectivamente. En los primeros 10 días, 33 (4,8%) fallecieron y 22 (3,2%) presentaron alguna hemorragia. No hubo ninguna recidiva de la EP. El antecedente de inmovilización y la presencia de insuficiencia respiratoria, de un cáncer o de insuficiencia renal fueron las variables asociadas con más frecuencia a la aparición de complicaciones. La cirugía y el dolor torácico fueron las asociadas a menos complicaciones. Conclusiones: La frecuencia de presentación de complicaciones precoces de la EP y una parte de los factores asociados a ellas coinciden con los ya conocidos. Sin embargo, este estudio demuestra que otros factores como la cirugía previa o el dolor torácico protegen a los pacientes frente a su aparición

Background and objective: Mortality, recurrences and hemorrhages are the most serious early complications of pulmonary embolism (PE). We intended to ascertain the frequency and mechanisms of complications within the first 10 days after PE was diagnosed. Patients and method: We included patients suspected of suffering PE between December 2003 and August 2004 from 8 hospitals. We performed a multicenter observational prospective study of 8 Spanish hospitals with consecutive outpatients diagnosed with PE. Database of clinical variables: computerized Registry of Patients with Venous Thromboembolism (RIETE). Statistic analysis included *2 and Student's t test to compare the 2 groups of patients (with and without complications). Results: Six hundred eighty one patients were included, 336 men and 345 women (mean age 66 and 70, respectively). During the first 10 days, 33 (4.8%) of them died, and 22 (3.2%) had an hemorrhage yet no recurrences appeared. Previous immobilization and the presence of respiratory failure, cancer or renal failure were significantly associated with early complications while previous surgery and thoracic pain were not associated with those. Conclusions: Early complications and some of the associated factors were in agreement with published data. Other situations such as previous surgery or thoracic pain appeared to be protective factors