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Resumen La enfermedad por COVID-19 se ha extendido por el mundo desde diciembre de 2019. Los sintomas neurologicos forman parte de su espectro clinico. Objetivo Conocer las manifestaciones neurologicas en pacientes infectados por COVID-19 en Argentina. Metodos Estudio multicentrico realizado en adultos, desde mayo de 2020 a enero de 2021, con COVID-19 confirmado y sintomas neurologicos. Se consignaron variables demograficas, existencia de comorbilidades sistemicas o neurologicas, la forma de comienzo de la infeccion, alteracion en estudios complementarios y el grado de severidad de los sintomas neurologicos. Resultados Se incluyeron 817 pacientes de todo el pais, 52% varones, edad promedio 38 anos. La mayoria sin comorbilidades ni patologia neurologica previa. El primer sintoma de la infeccion fue neurologico en 56,2% de los casos, predominando la cefalea (69%), luego anosmia/ageusia (66%). Tambien se reportaron mialgias (52%), alodinia/hiperalgesia (18%), astenia (6%). Un 3,2% mostro compromiso difuso del SNC como encefalopatia o convulsiones. Un 1,7% tuvo complicaciones cerebrovasculares. Los trastornos del sueno se observaron en 3,2%. Se reportaron seis pacientes con sindrome de Guillain-Barre (GBS), neuropatia periferica (3,4%), parestesias en lengua (0,6%), hipoacusia (0,4%), plexopatia (0,3%). La severidad de sintomas neurologicos se correlaciono con la edad y la existencia de comorbilidades. Conclusiones Nuestros resultados, similares a los de otros paises, muestran dos tipos de sintomas neurologicos asociados a COVID-19: algunos potencialmente incapacitantes o mortales como el GBS o la encefalitis, y otros menos devastadores, pero mas frecuentes, como cefalea o anosmia que demandan en forma creciente atencion a largo plazo.

Introduction: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease of unknown cause, characterized by the simultaneous involvement of the upper and lower motor neurons. Epidemiological studies have estimated its annual incidence between 0.31 and 3.2 and its prevalence between 0.8 and 8.5 cases per 100,000 inhabitants. The epidemiological information in our country is limited to specialized centers. The present study presents the results of an epidemiological study in ELA performed in the Autonomous City of Buenos Aires (CABA). Methods: A multicentric retrospective study was conducted. Patients with defined and probable ALS according to the El Escorial Criteria, evaluated between January 1, 2012 and December 31, 2013, who lived in the CABA at the onset of symptoms, were included. The calculation of the incidence was based on the 2010 census. Results: We included 103 patients (55 men), with a mean age of 64 years. The onset of symptoms was in the lower limbs at 39%, upper extremities at 25% and bulbar at 26%. The initial symptom was weakness in 58% and dysarthria in 20%; 9% had dementia associated with ALS. The mean time to diagnosis was 14.5 months. Thirty new cases/patients were diagnosed between 01/06/2012 and 01/06/2013, with an incidence rate of 1.04 per 100,000 inhabitants. Conclusions: The epidemiological characteristics of ALS in CABA are similar to those reported in the universal literature. Further studies are needed to determine if these findings are applicable to the rest of the Argentine population. Introduction: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease of unknown cause, characterized by the simultaneous involvement of the upper and lower motor neurons. Epidemiological studies have estimated its annual incidence between 0.31 and 3.2 and its prevalence between 0.8 and 8.5 cases per 100,000 inhabitants. The epidemiological information in our country is limited to specialized centers. The present study presents the results of an epidemiological study in ELA performed in the Autonomous City of Buenos Aires (CABA). Methods: A multicentric retrospective study was conducted. Patients with defined and probable ALS according to the El Escorial Criteria, evaluated between January 1, 2012 and December 31, 2013, who lived in the CABA at the onset of symptoms, were included. The calculation of the incidence was based on the 2010 census. Results: We included 103 patients (55 men), with a mean age of 64 years. The onset of symptoms was in the lower limbs at 39%, upper extremities at 25% and bulbar at 26%. The initial symptom was weakness in 58% and dysarthria in 20%; 9% had dementia associated with ALS. The mean time to diagnosis was 14.5 months. Thirty new cases/patients were diagnosed between 01/06/2012 and 01/06/2013, with an incidence rate of 1.04 per 100,000 inhabitants. Conclusions: The epidemiological characteristics of ALS in CABA are similar to those reported in the universal literature. Further studies are needed to determine if these findings are applicable to the rest of the Argentine population. Fil: Pérez Akly, Manuel. Sociedad Neurológica; Argentina Fil: Schiava, Marianela. Unidad Asistencial Doctor César Milstein; Argentina Fil: Melcom, Mario. Fundación para la Investigación en Neuroepidemiología; Argentina Fil: Rodríguez, Gabriel. Sociedad Neurológica; Argentina Fil: Gargiulo Monachelli, Gisella Mariana. Sociedad Neurológica; Argentina Fil: Bettini, Mariela. Sociedad Neurológica; Argentina Fil: Reisin, Ricardo. Sociedad Neurológica; Argentina Fil: Bendersky, Mariana. Sociedad Neurológica; Argentina Fil: Barroso, Fabio. Sociedad Neurológica; Argentina Fil: Brand, Patricio. Fundación para la Lucha Contra las Enfermedades Neurológicas de la Infancia. Instituto de Investigaciones Neurológicas "Raúl Carrea"; Argentina Fil: de Ambrosi, Bruno. Sociedad Neurológica; Argentina Fil: Di Egidio, Marianna. Sociedad Neurológica; Argentina Fil: Fiorotto, Luis. Sociedad Neurológica; Argentina Fil: Jáuregui, Agustín. Sociedad Neurológica; Argentina Fil: Landriscina, Paula. Sociedad Neurológica; Argentina Fil: Marchesoni, Cintia. Sociedad Neurológica; Argentina Fil: Mazia, Claudio. Sociedad Neurológica; Argentina Fil: Rey, Roberto. Sociedad Neurológica; Argentina Fil: Rugiero, Marcelo. Sociedad Neurológica; Argentina Fil: Salutto, Valeria Luján. Sociedad Neurológica; Argentina Fil: Tillard, Belén. Sociedad Neurológica; Argentina Fil: Fulgenzi, Ernesto. Sociedad Neurológica; Argentina

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated neuropathy. It presents with a course of progression which may be slow and steady or step-wise or relapsing. Sensory ataxic polyneuropathy may be the only clinical manifestation of this disease. Treatment with interferon beta1a (INF beta1a) has been tried with different results in patients who were refractory to other, more conventional, immunomodulatory therapies. Here we report on a patient who had a relapsing form of pure sensory ataxic CIDP and who failed to respond to intravenous human immunoglobulin. He was put on INF beta1a for 3 years. During this period he suffered no relapses while his condition stabilized.

Objective To evaluate the oxidative state in patients with familial amyloidotic polyneuropathy type 1 (FAP1). Design From 3 unrelated families, patients with FAP1 carrying a transthyretin Met-30 mutation were studied. The diagnosis was confirmed by genetic analysis. Eleven of 21 patients carried the mutation; all were symptomatic and were clinically assessed using a clinical score. All of the patients were evaluated for copper-zinc superoxide dismutase type 1 activity in red blood cells using spectrophotometry. Plasma total reactive antioxidant potential was studied using a chemiluminescent method. The results were compared with those obtained from an age-matched control group. Setting A public and academic multidisciplinary research clinic. Results Six of the 11 FAP1-positive patients disclosed superoxide dismutase type 1 activity values greater than 55 U/mg of protein (upper control limit), whereas 9 of 10 patients in whom total reactive antioxidant potential was measured had values below the lower limit of the control group. No relationship was found between the levels of superoxide dismutase type 1 activity and the severity of the clinical involvement. Conclusions Oxidative stress may be part of the mechanisms leading to tissue damage in patients with FAP1. The lack of correlation between the laboratory findings and the severity of clinical involvement may signal that oxidative processes are at work throughout the natural history of the disease.