High-flow carotid cavernous fistula and 'white eye': A rare and easily misdiagnosed presentation - A case report and literature review.

The three classic symptoms of carotid cavernous fistula (CCF) are pulsating exophthalmos, bruit and conjunctival chemosis. Here, we present a clinical case of isolated abducens nerve palsy due to a high-flow CCF in an 84-year-old woman, without the typical congestive orbito-ocular features. It was a diagnostic challenge because, for patients older than 50 years with cardiovascular risk factors, ischaemic mononeuropathy is the most frequent aetiology. This case illustrates the least common type of CCF that can be easily misdiagnosed. Physicians should consider fistula as a possible diagnosis in a patient with isolated abducens nerve palsy even without the classic triad.

[Cerebral infarction in young patients: clinical characteristics and evolution in an Argentine cohort]. / Infarto cerebral en pacientes jóvenes: características clínicas y evolución en una cohorte argentina.

INTRODUCTION: Stroke (CVA) in young adults comprises approximately 10% of all cerebrovascular events. The information available on the recurrence of a new event in this population and particularly in Latin America is limited. Our objective was to examine the presence of stroke recurrence after having presented a stroke. METHODS: A retrospective cohort study was carried out, including patients with arterial cerebral infarctions between the ages of 18 and 55, between January 2005 and May 2020. The main outcome was the recurrence of a cerebrovascular attack. RESULTS: 138 patients were included during a median follow-up of 24 months. The 52.2% (n = 72) were male patients and 73.4% (n = 94) had an initial NIHSS score of less than 4. The 38% (n = 52) had a history of arterial hypertension and 13, 1% (n = 18) history of previous stroke / TIA. 13% (n = 18) presented recurrence during their follow-up. DISCUSSION: The recurrence of neurovascular events occurs predominantly in patients with a history of previous stroke/TIA, probably secondary to diseases that are difficult to diagnose.

Introducción: El ataque cerebral (ACV) en adultos jóvenes comprende aproximadamente el 10% de todos los eventos cerebrovasculares. La información disponible sobre la recurrencia de un nuevo evento en esta población y particularmente en Latinoamérica es limitada. Nuestro objetivo fue examinar la presencia de recurrencia de ACV luego de haber presentado un infarto cerebral. Métodos: Se realizó un estudio de cohorte retrospectivo, incluyendo pacientes con infartos cerebrales arteriales en edades comprendidas entre 18 y 55 años, entre enero de 2005 a mayo de 2020. El resultado principal fue la recurrencia de un ataque cerebrovascular. Resultados: Se incluyeron 138 pacientes durante una mediana de seguimiento de 24 meses. El 52.2% (n = 72) de sexo masculino y el 73,4% (n = 94) tuvo un NIHSS inicial menor a 4. El 38% (n = 52) tenía antecedentes de hipertensión arterial y 13,1% (n = 18) antecedentes de ACV / ataque isquémico transitorio (AIT) previo. El 13% (n = 18) presento recurrencia durante su seguimiento. Discusión: La recurrencia de los eventos neurovasculares ocurren predominantemente en pacientes con antecedentes de ACV/AIT previo, probablemente secundario a enfermedades de difícil diagnóstico.

Cyclosporine-induced erythromelalgia.

Erythromelalgia is a rare syndrome characterised by recurrent erythema, heat and burning pain in the extremities. There are two types: primary (genetic) and secondary (toxic, drug-related or associated with other diseases). We report a 42-year-old woman who developed erythromelalgia after taking cyclosporine for myasthenia gravis. Although exact mechanism for this rare adverse effect is unclear, it is reversible, and so clinicians should be aware of the association . Additional use of corticosteroids could aggravate cyclosporine's toxic effects.

[Clinical Practice Guidelines for diagnosis of organic involvement of amyloidosis: Part 3/3 Year 2020] / Guía de Práctica Clínica para el diagnóstico de compromiso orgánico en amiloidosis: Parte 3/3 Año 2020.

Method: Use the PICO format to generate a series of questions, focusing on the specificity and sensitivity of the amyloidosis diagnostic test. PubMed searches were conducted in English and Spanish from July to August 2019. The level of evidence and recommendation are based on the GRADE system (http://www.gradeworkinggroup.org/index.htm). The recommendations are graded according to their direction (for or against) and strength (strong and weak). Finally, it is recommended to use GLIA tools to evaluate the obstacles and facilitators in implementation. Suggested explanation A strong suggestion indicates a high degree of trust in support or opposition to the intervention. When defining a strong recommendation, this guide uses the "recommended" language. The weaker recommendations indicate that the outcome of the intervention (favorable or unfavorable) is doubtful. In this case, if a weak recommendation is defined, the "recommendation" language is used. How to use these guidelines: Recommendations must be explained within the scope of special care in validated diagnostic studies conducted by specially trained doctors. It is not assumed to change the coexistence conditions of the disease process. Presumably, the attending physician has a high degree of suspicion of amyloidosis. It assumes that diagnostic research is conducted by well-trained doctors using a validated standardized method. This guide is intended for health care professionals and those involved in health care policies to help ensure that the necessary agreements have been reached to provide appropriate care. Summary of recommendations For patients with suspected amyloidosis, it is recommended: Measured value of creatinine be used as a preliminary assessment for the diagnosis of renal involvement in patients with suspected renal amyloidosis. 24-hour proteinuria be measured and characterized to diagnose renal involvement in patients with suspected renal amyloidosis. Immunohistochemical staining of skin biopsy for patients genetically diagnosed with ATTR, for early diagnosis of neuropathy. The signs or symptoms of these patients suggest the presence of fine fiber neuropathy. Skin biopsy and immunohistochemical staining for early diagnosis of neuropathy. These patients show signs or symptoms suggesting fine fiber neuropathy. Conduct nerve conduction studies on motor and sensory fibers to diagnose total fiber neuropathy in patients who are diagnosed or suspected of having amyloidosis. Test (Sudoscan) is recommended for the early diagnosis of peripheral autonomic neuropathy (even in asymptomatic patients) in patients with suspected autonomic neuropathy due to amyloidosis. Ewing's standard to measure heart rate variability to diagnose autonomic hypofunction in patients with autonomic neuropathy suspected of having amyloidosis. Measure orthostatic hypotension to diagnose early autonomic hypotension for patients with amyloidosis or systemic amyloidosis suspected of autonomic neuropathy. It is suggested: QST test to diagnose neuropathy early for patients genetically diagnosed with ATTR, if they show signs or symptoms suggesting fine fiber neuropathy Measure alkaline phosphatase to initially assess liver involvement in patients with amyloidosis.

Métodos: Se generó un listado de preguntas con el formato PICO centradas en la especificidad y sensibilidad de las pruebas diagnósticas en amiloidosis. Se realizó la búsqueda en PubMed durante julio-agosto del 2019, en inglés y español. Los niveles de evidencia y los grados de recomendación se basan en el sistema GRADE (http://www.gradeworkinggroup.org/index.htm). Las recomendaciones se graduaron según su dirección (a favor o en contra) y según fuerza (fuertes y débiles). Las recomendaciones finales fueron evaluadas con la herramienta GLIA para barreras y facilitadores en la implementación de éstas. Interpretación de recomendaciones: Las recomendaciones fuertes indican alta confianza, ya sea a favor o en contra, de una intervención. En esta guía se utiliza el lenguaje "se recomienda" cuando se define una recomendación fuerte. Las recomendaciones débiles indican que los resultados para una intervención, favorable o desfavorable, son dudosos. En este caso, se utiliza el lenguaje "se sugiere", cuando se define una recomendación débil.Cómo utilizar estas pautas: Las recomendaciones deben ser interpretadas en el contexto de la atención especializada, con estudios diagnósticos validados y realizados por médicos entrenados. Se asume que el médico tratante tiene alto nivel de sospecha de amiloidosis. No asume condiciones coexistentes que modifican el curso de la enfermedad. Asume que los estudios diagnósticos son realizados por médicos entrenados con métodos validados y estandarizados. Esta guía es relevante para los profesionales de la salud y los involucrados en las políticas sanitarias, para ayudar a asegurar que existan los acuerdos necesarios para brindar la atención adecuada. Resumen de recomendaciones En pacientes con sospecha de amiloidosis se recomienda: Medición de la creatinina como evaluación inicial para el diagnóstico del compromiso renal en el paciente con sospecha de amiloidosis renal. Medición y caracterización de la proteinuria de 24 hs para el diagnóstico de compromiso renal en pacientes con sospecha de amiloidosis renal. Biopsia de piel con tinción inmunohistoquímica para el diagnóstico precoz de neuropatía en pacientes con diagnóstico genético de ATTR, que presenten signos o síntomas sugestivos de neuropatía de fibra fina. Biopsia de piel con tinción inmunohistoquímica para el diagnóstico precoz de neuropatía en pacientes con sospecha de amiloidosis, que presenten signos o síntomas sugestivos de neuropatía de fibra fina. Estudios de conducción nerviosa evaluando fibras motoras y sensitivas para el diagnóstico de neuropatía de fibras gruesas en pacientes con diagnóstico o sospecha de amiloidosis. Prueba de QST para el diagnóstico precoz de neuropatía en pacientes con diagnóstico genético de ATTR, que presenten signos o síntomas sugestivos de neuropatía de fibras finas. Test de cuantificación sudorípara (Sudoscan) para diagnóstico precoz de neuropatía autonómica periférica (incluso en asintomáticos) en pacientes con sospecha de neuropatía autonómica por amiloidosis. Medición de la variabilidad de la frecuencia cardiaca con criterios de Ewing para el diagnóstico de disautonomía en pacientes con sospecha de neuropatía autonómica por amiloidosis. Medición de hipotensión ortostática con técnica adecuadamente estandarizada para el diagnóstico precoz de compromiso autonómico en el paciente con sospecha de neuropatía autonómica por amiloidosis o diagnóstico de amiloidosis sistémica Se sugiere: Prueba de QST para el diagnóstico precoz de neuropatía en pacientes con amiloidosis o sospecha de amiloidosis, que presenten signos o síntomas sugestivos de neuropatía de fibras finas. Medición de fosfatasa alcalina para evaluación inicial del compromiso hepático en el paciente con amiloidosis.

Resultados del tratamiento quirúrgico en neoplasias epiteliales de timo / Outcomes of surgical treatment for thymic epithelial neoplasms

Resumen Las neoplasias epiteliales del timo (NET) son tumores infrecuentes. El manejo inicial de las mismas se basa en el análisis de la resecabilidad del tumor en los estudios por imágenes, siendo la resección completa el tratamiento estándar para cualquier estadio. El objetivo principal fue evaluar la supervivencia global y la supervivencia libre de enfermedad de los pacientes con diagnóstico de NET sometidos a tratamiento quirúrgico. El objetivo secundario fue comparar la supervivencia global de acuerdo al tipo de resección quirúrgica, el estadio de Masaoka-Koga y subtipo histológico. Estudio observacional descriptivo realizado en el Hospital Británico de Buenos Aires desde 2004 a 2020. Se incluyeron 42 pacientes operados de tumores en mediastino anterior con diagnóstico histopatológico definitivo de NET. Treinta y nueve eran timomas y 3 carcinomas tímicos. La cirugía realizada en todos los pacientes fue una timectomia total. La mediana de seguimiento fue de 63.5 meses (RIQ 32-97.5). La supervivencia global estimada a los cinco y diez años fue del 87% (95% IC, 0.69-0.95) y 78% (95% IC, 0.5-0.92), respectivamente. La supervivencia libre de enfermedad estimada a cinco y diez años fue del 90% (95% IC, 0.74-0.96). Los pacientes a los que se le realizó una resección completa y que tenían estadios tem pranos de Masaoka-Koga presentaron una supervivencia global superior en comparación con las resecciones incompletas y estadios avanzados (p = 0.0097 y p = 0.0028, respectivamente). Al contar con un bajo número de pacientes en el grupo de carcinomas tímicos, no pudimos sacar conclusiones en cuanto a supervivencia entre éstos y los timomas.

Abstract Thymic epithelial tumors are a group of rare neoplasms of the mediastinum. When resectable, complete resection is considered the gold standard for any stage. The primary endpoint was to evaluate overall survival and disease-free survival of patients with thymus epithelial tumors who underwent surgical treatment. The secondary endpoint was to evaluate and compare the overall survival according to: resection type (complete vs. incomplete), Masaoka-Koga stage and tumor histology according to WHO classification. This is a descriptive observational study from January 2004 to December 2020, in which 42 patients with a postoperative histopathological diagnosis of thymic epithelial tumours were included. Thirty-nine were thymomas (92.9%) and 3 were thymic carcinomas (7.1%). In all patients a total thymectomy was performed. The median follow-up was 63.5 months (IQR 32-97.5). The estimated overall survival at five and ten years was 87% (95% CI, 0.69-0.95) and 78% (95% CI, 0.5-0.92), respectively. Estimated disease-free survival at five and ten years was 90% (95% CI, 0.74-0.96). Patients who underwent complete resection and with early Masaoka-Koga stages had superior overall survival compared to incomplete resections and advanced Masaoka-Koga stages (p = 0.0097 and p = 0.0028, respectively). We found no differences in terms of survival between histological subtypes due to a low number of patients in the thymic carcinoma group.

[Outcomes of surgical treatment for thymic epithelial neoplasms]. / Resultados del tratamiento quirúrgico en neoplasias epiteliales de timo.

Thymic epithelial tumors are a group of rare neoplasms of the mediastinum. When resectable, complete resection is considered the gold standard for any stage. The primary endpoint was to evaluate overall survival and disease-free survival of patients with thymus epithelial tumors who underwent surgical treatment. The secondary endpoint was to evaluate and compare the overall survival according to: resection type (complete vs. incomplete), Masaoka-Koga stage and tumor histology according to WHO classification. This is a descriptive observational study from January 2004 to December 2020, in which 42 patients with a postoperative histopathological diagnosis of thymic epithelial tumours were included. Thirty-nine were thymomas (92.9%) and 3 were thymic carcinomas (7.1%). In all patients a total thymectomy was performed. The median follow-up was 63.5 months (IQR 32-97.5). The estimated overall survival at five and ten years was 87% (95% CI, 0.69-0.95) and 78% (95% CI, 0.5-0.92), respectively. Estimated diseasefree survival at five and ten years was 90% (95% CI, 0.74-0.96). Patients who underwent complete resection and with early Masaoka-Koga stages had superior overall survival compared to incomplete resections and advanced Masaoka-Koga stages (p = 0.0097 and p = 0.0028, respectively). We found no differences in terms of survival between histological subtypes due to a low number of patients in the thymic carcinoma group.

Las neoplasias epiteliales del timo (NET) son tumores infrecuentes. El manejo inicial de las mismas se basa en el análisis de la resecabilidad del tumor en los estudios por imágenes, siendo la resección completa el tratamiento estándar para cualquier estadio. El objetivo principal fue evaluar la supervivencia global y la supervivencia libre de enfermedad de los pacientes con diagnóstico de NET sometidos a tratamiento quirúrgico. El objetivo secundario fue comparar la supervivencia global de acuerdo al tipo de resección quirúrgica, el estadio de Masaoka-Koga y subtipo histológico. Estudio observacional descriptivo realizado en el Hospital Británico de Buenos Aires desde 2004 a 2020. Se incluyeron 42 pacientes operados de tumores en mediastino anterior con diagnóstico histopatológico definitivo de NET. Treinta y nueve eran timomas y 3 carcinomas tímicos. La cirugía realizada en todos los pacientes fue una timectomia total. La mediana de seguimiento fue de 63.5 meses (RIQ 32-97.5). La supervivencia global estimada a los cinco y diez años fue del 87% (95% IC, 0.69-0.95) y 78% (95% IC, 0.5-0.92), respectivamente. La supervivencia libre de enfermedad estimada a cinco y diez años fue del 90% (95% IC, 0.74-0.96). Los pacientes a los que se le realizó una resección completa y que tenían estadios tempranos de Masaoka-Koga presentaron una supervivencia global superior en comparación con las resecciones incompletas y estadios avanzados (p = 0.0097 y p = 0.0028, respectivamente). Al contar con un bajo número de pacientes en el grupo de carcinomas tímicos, no pudimos sacar conclusiones en cuanto a supervivencia entre éstos y los timomas.

Guía de práctica clínica de tratamiento de la polineuropatía amiloidótica familiar / Practice guideline for the treatment of familial amyloid polyneuropathy

Resumen Esta guía de práctica clínica de tratamiento de la polineuropatía amiloidótica familiar se basa en la mejor evidencia disponible de efectividad clínica. Se generó un listado de preguntas con formato PICO centradas en efectividad y seguridad del tratamiento de polineuropatía amiloidótica familiar. Se realizó la búsqueda en PubMed, Cochrane y Epistemonikos. Los niveles de evidencia y los grados de recomendación se basaron en el sistema GRADE. Las recomendaciones se graduaron según dirección y fuerza y se evaluaron con la herramienta GLIA para su implementación. Resumen de recomendaciones: En pacientes con polineuropatía amiloidótica familiar y neuropatía estadio I y II, se sugiere el tratamiento con inotersen 300 mg subcutáneo semanal o patisirán 0.3 mg/kg endovenoso una vez cada 3 semanas, dado que, probablemente, estabilicen o enlentezcan el avance de la neuropatía y el empeoramiento de la calidad de vida (calidad de la evidencia moderada; fuerza de la recomendación débil). En pacientes con polineruropatía amiloidótica familiar y neuropatía estadio I, se sugiere el tratamiento con tafamidis 20 mg vía oral, una vez por día, ya que podría enlentecer el avance de la neuropatía y el empeoramiento en la calidad de vida (calidad de la evidencia baja; fuerza de la recomendación débil), y aquellos con polineuropatía amiloidótica familiar y neuropatía sintomática y en ausencia de otros tratamientos con eficacia aprobada, se sugiere el tratamiento con diflunisal 250 mg dos veces al día, vía oral, ya que podría evitar la progresión de la neuropatía (calidad de la evidencia baja; fuerza de la recomendación débil).

Abstract. This clinical practice guideline for the treatment of familial amyloid polyneuropathy is based on the best available evi dence of clinical effectiveness. A list of questions was generated with a PICO format focused on the effectiveness and safety of the treatment of familial amyloid polyneuropathy. The search was carried out in PubMed, Cochrane and Epistemonikos. The levels of evidence and grades of recommendation were based on the GRADE system. Recommendations were graded according to their direction and their strength and were evaluated with the GLIA tool for their implementation. In patients with familial amyloid polyneuropathy and stage I and II neuropathy, it is suggested: inotersen 300 mg subcutaneous weekly or patisirán 0.3 mg/kg intravenously once every 3 weeks, since they probably stabilize or slow the progression of neuropathy and worsening quality of life (moderate qual ity of evidence; strength of recommendation weak). In patients with familial amyloid polyneuropathy and stage I neuropathy, treatment with tafamidis 20 mg orally, once a day, is suggested, as it could slow the progression of neuropathy and worsen quality of life (low quality of evidence; strength of recommendation weak). In patients with familial amyloid polyneuropathy and symptomatic neuropathy and in the absence of other treatments with approved efficacy, treatment with oral diflunisal 250 mg twice daily is suggested, as it could prevent the progres sion of neuropathy (quality evidence low; strength of recommendation weak).

[Practice guideline for the treatment of familial amyloid polyneuropathy]. / Guía de práctica clínica de tratamiento de la polineuropatía amiloidótica familiar.

This clinical practice guideline for the treatment of familial amyloid polyneuropathy is based on the best available evidence of clinical effectiveness. A list of questions was generated with a PICO format focused on the effectiveness and safety of the treatment of familial amyloid polyneuropathy. The search was carried out in PubMed, Cochrane and Epistemonikos. The levels of evidence and grades of recommendation were based on the GRADE system. Recommendations were graded according to their direction and their strength and were evaluated with the GLIA tool for their implementation. In patients with familial amyloid polyneuropathy and stage I and II neuropathy, it is suggested: inotersen 300 mg subcutaneous weekly or patisirán 0.3 mg/kg intravenously once every 3 weeks, since they probably stabilize or slow the progression of neuropathy and worsening quality of life (moderate quality of evidence; strength of recommendation weak). In patients with familial amyloid polyneuropathy and stage I neuropathy, treatment with tafamidis 20 mg orally, once a day, is suggested, as it could slow the progression of neuropathy and worsen quality of life (low quality of evidence; strength of recommendation weak). In patients with familial amyloid polyneuropathy and symptomatic neuropathy and in the absence of other treatments with approved efficacy, treatment with oral diflunisal 250 mg twice daily is suggested, as it could prevent the progression of neuropathy (quality evidence low; strength of recommendation weak).

Esta guía de práctica clínica de tratamiento de la polineuropatía amiloidótica familiar se basa en la mejor evidencia disponible de efectividad clínica. Se generó un listado de preguntas con formato PICO centradas en efectividad y seguridad del tratamiento de polineuropatía amiloidótica familiar. Se realizó la búsqueda en PubMed, Cochrane y Epistemonikos. Los niveles de evidencia y los grados de recomendación se basaron en el sistema GRADE. Las recomendaciones se graduaron según dirección y fuerza y se evaluaron con la herramienta GLIA para su implementación. Resumen de recomendaciones: En pacientes con polineuropatía amiloidótica familiar y neuropatía estadio I y II, se sugiere el tratamiento con inotersen 300 mg subcutáneo semanal o patisirán 0.3 mg/kg endovenoso una vez cada 3 semanas, dado que, probablemente, estabilicen o enlentezcan el avance de la neuropatía y el empeoramiento de la calidad de vida (calidad de la evidencia moderada; fuerza de la recomendación débil). En pacientes con polineruropatía amiloidótica familiar y neuropatía estadio I, se sugiere el tratamiento con tafamidis 20 mg vía oral, una vez por día, ya que podría enlentecer el avance de la neuropatía y el empeoramiento en la calidad de vida (calidad de la evidencia baja; fuerza de la recomendación débil), y aquellos con polineuropatía amiloidótica familiar y neuropatía sintomática y en ausencia de otros tratamientos con eficacia aprobada, se sugiere el tratamiento con diflunisal 250 mg dos veces al día, vía oral, ya que podría evitar la progresión de la neuropatía (calidad de la evidencia baja; fuerza de la recomendación débil).

Antecedent infections in Guillain-Barré syndrome in endemic areas of arbovirus transmission: A multinational case-control study.

Half of the world's population is at risk of arthropod-borne virus (arbovirus) infections. Several arbovirus infections have been associated with Guillain-Barré syndrome (GBS). We investigated whether arboviruses are driving GBS beyond epidemic phases of transmission and studied the antibody response to glycolipids. The protocol of the International Guillain-Barré syndrome Outcome Study (IGOS), an observational prospective cohort study, was adapted to a case-control design. Serum samples were tested for a recent infection with Zika virus (ZIKV), dengue virus (DENV), chikungunya (CHIKV) virus, hepatitis E virus, Epstein-Barr virus (EBV), cytomegalovirus (CMV), Campylobacter jejuni, and Mycoplasma pneumoniae, and for antibodies to glycolipids. Forty-nine patients were included from Brazil (63%), Argentina (14%), and Malaysia (22%). Evidence of a recent infection was found in 27/49 (55%) patients: C jejuni (n = 15, 31%), M pneumoniae (n = 5, 10%), CHIKV (n = 2, 4%), EBV (n = 1, 2%), C jejuni and M pneumoniae (n = 2, 4%), CMV and DENV (n = 1, 2%), and C jejuni and DENV (n = 1, 2%). In 22 patients, 35 paired controls were collected. Odds ratio for recent infections did not significantly differ between cases and controls. No typical anti-ganglioside antibody binding was associated with recent arbovirus infection. We conclude that arbovirus infections occur in GBS patients outside of epidemic viral transmission, although not significantly more than in controls. Broad infection and anti-ganglioside antibody serology are important to establish the most likely pathogenic trigger in GBS patients. Larger studies are necessary to determine the association between arboviruses and GBS.

AISYF: first national, prospective, multicenter study of young patients with stroke in Argentina.

Up to 15% of all strokes affect young patients and the incidence of ischemic stroke in this population is rising. Nevertheless, there is limited information of cerebrovascular events in this population both in our country and in Latin America. The aim of our study was to evaluate the clinical characteristics and risk factors of young adults with stroke in Argentina. This is a prospective, multicenter study of stroke in young adults (18 - 55 years) in Argentina. Patients presenting with a cerebrovascular event within 180 days were included. Stroke subtypes were classified according to TOAST criteria. A total number of 311 patients were enrolled (men 53.9%, mean age: 43.3 years). Ischemic strokes occurred in 91.8% (brain infarcts 82.6%, transient ischemic attack 9.2%) and hemorrhagic strokes in 8.2%. The most frequent vascular risk factors (including ischemic and hemorrhagic strokes) were: hypertension 120 (41%), smoking 92 (31.4%), dyslipidemia 81 (27.6%) and, overweight/obesity: 74 (25.3%). Stroke subtypes were: large artery disease 12.3%, cardioembolism 7.5%, small artery occlusion 11.5%, other defined etiology 27.1%, and undetermined etiology 41.6%. Our study demonstrates that vascular risk factors are very frequent in young adults with stroke. Our findings underline that urgent strategies are required for primary and secondary stroke prevention in this group of patients.

Aproximadamente un 15% de todos los ataques cerebrovasculares afectan a pacientes jóvenes y su incidencia estaría en aumento. Existe escasa información sobre el ataque cerebral en esta población tanto en nuestro país como en Latinoamérica. El objetivo de nuestro estudio fue evaluar las características clínicas y los factores de riesgo de los adultos jóvenes con ictus en Argentina. Realizamos un estudio prospectivo y multicéntrico en adultos jóvenes (18-55 años) en Argentina, que presentaron un evento cerebrovascular dentro de los 180 días previos. Los subtipos de ictus se clasificaron según los criterios de TOAST. Se incluyeron un total de 311 pacientes (hombres 53.9%, edad media: 43,3 años). Los ataques cerebrovasculares isquémicos ocurrieron en el 91.8% (infartos cerebrales 82.6%, ataque isquémico transitorio 9.2%) y los eventos hemorrágicos correspondieron al 8.2%. Los factores de riesgo vascular más frecuentes (incluyendo los eventos isquémicos y hemorrágicos) fueron: hipertensión 120 (41%), tabaquismo 92 (31.4%), dislipidemia 81 (27.6%) y sobrepeso/obesidad: 74 (25.3%). Los subtipos de ictus isquémicos fueron: arteriopatía de gran vaso 12.3%, cardioembolismo 7.5%, oclusión de pequeña arteria 11.5%, otra etiología definida 27.1% y etiología indeterminada 41.6%. Los factores de riesgo vascular son muy frecuentes en los adultos jóvenes con ictus. Nuestros hallazgos subrayan que se requieren estrategias urgentes para la prevención primaria y secundaria del ictus en este grupo particular de pacientes en nuestro país.

AISYF: first national, prospective, multicenter study of young patients with stroke in Argentina / AISYF: primer estudio nacional, prospectivo y multicéntrico de pacientes jóvenes con ataque cerebral en Argentina

Abstract Up to 15% of all strokes affect young patients and the incidence of ischemic stroke in this population is rising. Nevertheless, there is limited information of cerebrovascular events in this population both in our country and in Latin America. The aim of our study was to evaluate the clinical characteristics and risk factors of young adults with stroke in Argentina. This is a prospective, multicenter study of stroke in young adults (18 - 55 years) in Argentina. Patients presenting with a cerebrovascular event within 180 days were included. Stroke subtypes were classified according to TOAST criteria. A total number of 311 patients were enrolled (men 53.9%, mean age: 43.3 years). Ischemic strokes occurred in 91.8% (brain infarcts 82.6%, transient ischemic attack 9.2%) and hemorrhagic strokes in 8.2%. The most frequent vascular risk factors (including ischemic and hemorrhagic strokes) were: hypertension 120 (41%), smoking 92 (31.4%), dyslipidemia 81 (27.6%) and, over weight/obesity: 74 (25.3%). Stroke subtypes were: large artery disease 12.3%, cardioembolism 7.5, small artery occlusion 11.5%, other defined etiology 27.1%, and undetermined etiology 41.6%. Our study demonstrates that vascular risk factors are very frequent in young adults with stroke. Our findings underline that urgent strategies are required for primary and secondary stroke prevention in this group of patients.

Resumen Aproximadamente un 15% de todos los ataques cerebrovasculares afectan a pacientes jóvenes y su incidencia estaría en aumento. Existe escasa información sobre el ataque cerebral en esta población tanto en nuestro país como en Latinoamérica. El objetivo de nuestro estudio fue evaluar las características clínicas y los factores de riesgo de los adultos jóvenes con ictus en Argentina. Realizamos un estudio prospectivo y multicéntrico en adultos jóvenes (18-55 años) en Argentina, que presentaron un evento cerebrovascular dentro de los 180 días previos. Los subtipos de ictus se clasificaron según los criterios de TOAST. Se incluyeron un total de 311 pacientes (hombres 53.9%, edad media: 43,3 años). Los ataques cerebrovasculares isquémicos ocurrieron en el 91.8% (infartos cerebrales 82.6%, ataque isquémico transitorio 9.2%) y los eventos hemorrágicos correspondieron al 8.2%. Los factores de riesgo vascular más frecuentes (incluyendo los eventos isquémicos y hemorrágicos) fueron: hipertensión 120 (41%), tabaquismo 92 (31.4%), dislipidemia 81 (27.6%) y sobrepeso/obesidad: 74 (25.3%). Los subtipos de ictus isquémicos fueron: arteriopatía de gran vaso 12.3%, cardioembolismo 7.5%, oclusión de pequeña arteria 11.5%, otra etiología definida 27.1% y etiología indeterminada 41.6%. Los factores de riesgo vascular son muy frecuentes en los adultos jóvenes con ictus. Nuestros hallazgos subrayan que se requieren estrategias urgentes para la prevención primaria y secundaria del ictus en este grupo particular de pacientes en nuestro país.

Síndrome de Guillain-Barré asociado a infección por COVID-19: revisión de casos publicados / Guillain-Barré syndrome associated to COVID-19 infection: a review of published case reports

INTRODUCCIÓN: La pandemia por la enfermedad por coronavirus 2019 (COVID-19) es un importante problema para la salud mundial. Hay un incremento en las complicaciones neurológicas reconocidas por la COVID-19, incluyendo el síndrome de Guillain-Barré (SGB) y sus variantes. DESARROLLO: Se realizó una revisión de los casos publicados en los últimos meses de SGB asociado a infección por COVID-19. Incluimos a 48 pacientes (31 hombres; edad media: 56,4 años). Los síntomas de COVID-19 más comunes fueron tos (60,4%) y fiebre (56,3%). El tiempo promedio entre los síntomas de COVID-19 y el SGB fue de 12,1 días, pero nueve pacientes (18,8%) desarrollaron SGB en menos de siete días. Once pacientes (22,9%) presentaron afectación de los nervios craneales en ausencia de debilidad muscular, 36 presentaron la variante clásica sensitivomotora (75%) y uno tuvo una variante motora pura (2,1%). El patrón electrofisiológico se consideró desmielinizante en el 82,4% de las variantes generalizadas. La presencia de hiposmia/disgeusia estuvo asociada con una latencia menor a los siete días hasta el inicio de los síntomas del SGB (30 frente a 15,6%) y a la afectación de los nervios craneales en ausencia de debilidad (30,8 frente a 17,1%). La mayoría de los pacientes (87,5%) fueron tratados con inmunoglobulina endovenosa. La evolución neurológica fue favorable en el 64,6%, el 29,2% tuvo insuficiencia respiratoria y hubo un 4,2% de muertes. CONCLUSIONES: El SGB en pacientes con infección por SARS-CoV-2 es similar clínica y electrofisiológicamente a las formas clásicas. Se requieren más estudios para comprender si la frecuencia del SGB realmente aumentó debido a la pandemia por COVID-19 y explorar los mecanismos patógenos involucrados

INTRODUCTION: The coronavirus disease 2019 (COVID-19) pandemic is a major worldwide health disorder. There is an increasing number of neurological complications recognized with COVID-19 including patients with GBS and its variants. DEVELOPMENT: A review of the clinical cases of GBS associated to COVID-19 infection published in the last months has been developed. We included 48 patients (31 men, mean age 56.4 years). The most common COVID-19 symptoms were cough (60.4%) and fever (56.3%). Mean time from COVID-19 symptoms to neurologic manifestations was 12.1 days, but in nine patients (18.8%) developed GBS within seven days. Eleven patients (22.9%) presented cranial nerve involvement in the absence of muscle weakness; 36 presented the classic sensory motor variant (75%) and one had a pure motor variant (2.1%). The electrodiagnostic pattern was considered demyelinating in 82.4% of the generalized variants. The presence of hyposmia/dysgeusia was associated with a latency shorter than seven days to GBS onset of symptoms (30% vs 15.6%), and cranial nerve involvement in the absence of weakness (30.8% vs 17.1%). Most patients (87.5%) were treated with intravenous immunoglobulin. Neurological outcome was favorable in 64.6%; 29.2% had respiratory failure and 4.2% died shortly after being admitted. CONCLUSIONS: GBS in patients with SARS-CoV-2 infection resembles clinically and electrophysiology the classical forms. Further studies are necessary to understand whether GBS frequency is actually increased due to SARS-CoV-2 infection and explore pathogenic mechanisms

[Long-term psychosocial impact in young stroke survivors: a frequent but often under diagnosed complication]. / Impacto psicosocial del ataque cerebral en pacientes jóvenes: una complicación frecuente y habitualmente sub-diagnosticada.

INTRODUCTION: Approximately 15% of all strokes occur in young patients, affecting them in the most productive years of their lives. Currently, there is limited information (particularly in Latin America) regarding the long-term psychosocial consequences of stroke in young patients. Therefore, the objective of our study was to analyze the functional impact of stroke in this group of patients, regarding both cognitive and psychosocial aspects. MATERIAL AND METHODS: A Beck Depression Inventory (BDI) was administered to outpatients with ischemic stroke between 16 and 55 years of age in two centers of Argentina. The following variables were compared in depressed and non-depressed individuals: NIHSS, modified Rankin Score, Mini-Mental State Examination, Barthel Index, as well as clinical-demographic variables. A BDI score greater than 10 was considered as marker of depression. RESULTS: Thirty-four patients with cerebral infarction were included, 67% (n = 23) were women, mean age: 45.53 ± 9.78 years (range: 21-59). Eleven patients (33%) had depression; 50% of the population was unable to continue with their previous job and 15% divorced or separated from their partner after the vascular event. The stroke recurrence rate was 0.03%. CONCLUSIONS: A high proportion of patients with depressive symptoms was observed. It should be noted that, in the majority, symptoms of depression had not been recognized and the patients were under-treated. Likewise, depression spread persistently after several years of the cerebrovascular event. Likewise, a significant proportion of patients were not able to re-insert themselves into their usual work activity. Moreover, stroke also had an important impact on their affective relationships. Treatment of depression after stroke should be particularly considered in these individuals due to their long-term survival, and should be offered to achieve the highest possible quality of recovery after stroke.

Misdiagnosis of acute ischemic stroke in young patients.

Misdiagnosis is a challenging risk in young patients with ischemic stroke that leads to delayed recognition, prevents thrombolytic therapy and increases morbidity and mortality. To assess the frequency and reasons for misdiagnosis of ischemic stroke in young adults in the emergency department, we retrospectively evaluated 101 consecutive patients. The diagnosis required both the presence of an ischemic lesion in diffusion-weighted MRI as well as neurological symptoms. Twenty-three individuals were misdiagnosed (15 females, mean age 43 years [range 26-55 years]). The most common diagnostic errors included headache/neck pain n = 11 (48%) and peripheral vertigo n = 4 (17.3%), and the most common missed etiology was arterial dissection (AD) n = 15 (65.2%). The initial diagnosis was made by emergency physicians in 22 persons. The group with misdiagnosis had a significantly higher frequency of AD at 15 vs. 8 (p < 0.01), posterior territory involvement at 12 vs. 6 (p < 0.01), and female predominance. Misdiagnosis occurred in a third of young patients with an ischemic stroke and was more frequent in those with arterial dissection involving the posterior circulation and presenting with non-specific symptoms.

El error diagnóstico es una situación riesgosa en pacientes jóvenes con infarto cerebral, ya que conduce a demora en el reconocimiento, previene la administración de terapia trombolítica e incrementa la morbimortalidad. Para valorar la frecuencia y las razones de errores en esta población, evaluamos retrospectivamente 101 pacientes consecutivos. El diagnóstico de infarto cerebral requirió tanto la presencia de lesión isquémica aguda en resonancia magnética como también la presencia de síntomas neurológicos. Veintitrés pacientes fueron mal diagnosticados (15 mujeres, edad media 43 años, [rango 26-55 años]). El error más frecuente incluyó cefalea/cervicalgia n = 11 (48%) y vértigo periférico n = 4 (17.3%). La etiología menos sospechada fue disección arterial n = 15 (62.2%). La evaluación inicial en 22 pacientes fue realizada por emergentólogos. El grupo con errores tuvo significativamente mayor frecuencia de disección arterial 15 vs. 8 (p < 0.01), afectación del territorio posterior 12 vs. 6 (p < 0.01) y sexo femenino. En conclusión, los errores diagnósticos ocurrieron en un tercio de los pacientes jóvenes con infarto cerebral, y fueron más frecuentes en aquellos con disección arterial, compromiso del territorio posterior y síntomas de presentación inespecífica.

Misdiagnosis of acute ischemic stroke in young patients

Misdiagnosis is a challenging risk in young patients with ischemic stroke that leads to delayed recognition, prevents thrombolytic therapy and increases morbidity and mortality. To assess the frequency and reasons for misdiagnosis of ischemic stroke in young adults in the emergency department, we retrospectively evaluated 101 consecutive patients. The diagnosis required both the presence of an ischemic lesion in diffusion-weighted MRI as well as neurological symptoms. Twenty-three individuals were misdiagnosed (15 females, mean age 43 years [range 26-55 years]). The most common diagnostic errors included headache/ neck pain n = 11 (48%) and peripheral vertigo n = 4 (17.3%), and the most common missed etiology was arterial dissection (AD) n = 15 (65.2%). The initial diagnosis was made by emergency physicians in 22 persons. The group with misdiagnosis had a significantly higher frequency of AD at 15 vs. 8 (p < 0.01), posterior territory involvement at 12 vs. 6 (p < 0.01), and female predominance. Misdiagnosis occurred in a third of young patients with an ischemic stroke and was more frequent in those with arterial dissection involving the posterior circulation and presenting with non-specific symptoms.

El error diagnóstico es una situación riesgosa en pacientes jóvenes con infarto cerebral, ya que conduce a demora en el reconocimiento, previene la administración de terapia trombolítica e incrementa la morbimortalidad. Para valorar la frecuencia y las razones de errores en esta población, evaluamos retrospectivamente 101 pacientes consecutivos. El diagnóstico de infarto cerebral requirió tanto la presencia de lesión isquémica aguda en resonancia magnética como también la presencia de síntomas neurológicos. Veintitrés pacientes fueron mal diagnosticados (15 mujeres, edad media 43 años, [rango 26-55 años]). El error más frecuente incluyó cefalea/cervicalgia n = 11 (48%) y vértigo periférico n = 4 (17.3%). La etiología menos sospechada fue disección arterial n = 15 (62.2%). La evaluación inicial en 22 pacientes fue realizada por emergentólogos. El grupo con errores tuvo significativamente mayor frecuencia de disección arterial 15 vs. 8 (p < 0.01), afectación del territorio posterior 12 vs. 6 (p < 0.01) y sexo femenino. En conclusión, los errores diagnósticos ocurrieron en un tercio de los pacientes jóvenes con infarto cerebral, y fueron más frecuentes en aquellos con disección arterial, compromiso del territorio posterior y síntomas de presentación inespecífica.

Ischemic stroke of the "hand knob area": A case series and literature review.

Isolated hand paresis may reflect an infarction of the "hand knob area", which represents less than 1% of all ischemic strokes. In this type of stroke, a potential source of embolism is often identified. There are no large case series regarding this topic in Latin America. Herein we present the largest cohort of this entity in the region and we compare our results with those previously published. We analyzed all stroke patients admitted to our hospital between May 2015 - June 2018. Only patients with motor +/- sensory deficits restricted to the hand and ischemic stroke confirmed by MRI were included. We assessed stroke mechanism, clinical characteristics and outcome. From 339 patients admitted with ischemic stroke, 12 (3.53%) were included (9 men, 75%). Mean age: 60.4 years-old (range:24-79). Localization of stroke: 8 patients (66%) precentral gyrus, 3 (25%) postcentral; in 1 both gyri were affected. Stroke mechanism according to TOAST classification was as follows: two patients (16%) large artery atherosclerosis, two cardioembolic, one other determined etiology (thrombophilia), seven (58%) undetermined etiology (SUE). Nine patients (75%) received antiplatelets and statins, and three (25%) anticoagulants. The mean follow-up period was 11 months (range 1-26). Stroke recurrence was observed in one patient. At follow up, eight patients (66%) had a modified Rankin Score (mRS) of 0 and one a mRS of 1. In conclusion, in this series the most prevalent stroke mechanism was SUE, mainly embolic stroke of undetermined source. The outcome was excellent regardless of stroke mechanism.

Blood pressure evolution in young patients with acute ischemic stroke: a new model for understanding the natural course of spontaneous hypertension?

BACKGROUND: Approximately 80% of patients suffering an acute ischemic stroke develop transient hypertension. The physiopathological mechanism remains unclear. Due to the lack of vascular risk factors, young adults could be a useful model for understanding blood pressure (BP) evolution in this setting. METHODS: Patients between 18 and 55 years old admitted with an acute ischemic stroke were included. BP was evaluated during the following 48 h. Hypertension was defined as: systolic blood pressure (SBP) ≥140 mmHg or diastolic blood pressure (DBP) ≥90 mmHg. Patients were divided into two groups: RF group included those who had a previous vascular risk factor and/or other medical condition known to affect the autonomic function; noRF group included patients without any of the previously stated conditions. RESULTS: Thirty-nine patients were included: mean age: 44.26 years old, 21 were males (53.8%). Mean SBP and DBP at admission were 139.77 ± 5.35 mmHg (range: 90-243) and 84.44 ± 3.02 mmHg (range: 60-128), respectively; 43.58% patients were hypertensive at admission. SBP decreased significantly during the first 48 h (p = 0.044) for the entire population. RF group has higher SBP (p = 0.009) and DBP (p = 0.011) at admission than the noRF group. CONCLUSION: Most patients were normotensive at admission and BP fell spontaneously despite BP at admission. Young patients without medical conditions that could alter the autonomic system function could be a useful model for understanding acute hypertension in ischemic stroke.

Intraneural perineuriomas: diagnostic value of magnetic resonance neurography.

Intraneural perineurioma (IP) is an under-recognized hypertrophic peripheral nerve tumor. It affects young patients involving frequently the sciatic nerve and its branches and presents with a progressive, painless and predominantly motor deficit. Magnetic resonance neurography (MRN) is a useful tool to localize the lesion, evaluate its extension, and discriminate between different etiologies. We reviewed the clinical records of 11 patients with pathologically confirm IP. Eight patients were males with mean age 19 years. Initial complains were unilateral steppage (seven patients), bilateral steppage (one patient), unilateral gastrocnemius wasting (one patient), unilateral thigh atrophy (one patient), and unilateral hand weakness (one patient). Nine patients had mild painless sensory loss. Examinations revealed involvement of sciatic nerve extending into the peroneal nerve (eight patients), posterior tibial nerve (one patient), radial nerve (one patient), and femoral nerve (one patient). MRN revealed enlargement of the affected nerve isointense on T1-weighted, hyperintense on T2 fat-saturated images, and with avid enhancement on post-contrast imaging. In all patients, a nerve biopsy confirmed the diagnosis. MRN allows early and non-invasive identification of this tumor and is a key tool providing localization and differential diagnosis in patients with slowly progressive focal neuropathies.