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.

Infarto cerebral en pacientes jóvenes: características clínicas y evolución en una cohorte Argentina / Cerebral infarction in young patients: clinical character istics and evolution in an Argentine cohort

Resumen Introducción : El ataque cerebral (ACV) en adultos jó venes comprende aproximadamente el 10% de todos los eventos cerebrovasculares. La información disponible so bre 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 retros pectivo, 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 cerebro vascular. 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 neurovas culares ocurren predominantemente en pacientes con antecedentes de ACV/AIT previo, probablemente secun dario a enfermedades de difícil diagnóstico.

Abstract 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 previ ous stroke/TIA, probably secondary to diseases that are difficult to diagnose.

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.

Central Venous Reflux, a Rare Cause of Neurological Manifestations in Hemodialysis Patients: A Case Report and Literature Review.

Central venous disease (CVD) is a serious complication in hemodialysis patients. Neurological manifestations are rare. We describe a female with end-stage renal disease with throbbing headache accompanied by paresthesia, weakness, and abnormal posture of her right hand during dialysis sessions. Motor symptoms completely resolved after each dialysis session, although the headaches persisted for several hours. No neurological deficit was evidenced on physical examination. Digital subtraction angiography identified an incomplete thrombosis of the left brachiocephalic vein with retrograde flow in the internal jugular vein, sigmoid sinus, and transverse sinus on the left side. This case illustrates that cerebral venous congestion due to CVD can produce neurological symptoms. Furthermore, we systematically review the literature to identify the characteristics of the cases described so far. This allows clinicians to know the entity and have a high index of suspicion in a hemodialysis patient who develops neurological symptoms.

Perfil clínico de los pacientes con aneurisma cerebral atendidos en el Hospital Carlos Andrade Marín / Clinical profile of patients with cerebral aneurysms treated at Carlos Andrade Marín Hospital

INTRODUCCIÓN. En Ecuador, las enfermedades cerebrovasculares son la tercera causa de muerte en la población general; existen pocos datos en la literatura médica sobre la hemorragia subaracnoidea aneurismática y aneurismas no rotos, por lo que fue fundamental describir un perfil clínico. OBJETIVO. Caracterizar la clínica de los pacientes con diagnóstico de aneurisma cerebral. MATERIALES Y MÉTODOS. Estudio observacional descriptivo. Población de 450 y muestra de 447 pacientes con diagnóstico de aneurisma cerebral atendidos en la Unidad de Neurología del Hospital Carlos Andrade Marín, periodo enero 2010 a diciembre 2018. Los datos clínicos e imagenológicos fueron recolectados a través de la revisión de historias clínicas digitales obtenidas del sistema informático MIS/AS400, y se analizaron en el programa estadístico International Business Machines Statistical Package for the Social Sciences, versión 23. RESULTADOS. Se identificaron 605 aneurismas en 447 pacientes. El 80,5% (360; 447) presentó aneurismas rotos, de éstos el 81,4% (293; 360) tuvo un solo aneurisma. El factor de riesgo más frecuente fue la hipertensión arterial con el 44,3% (198; 447), las manifestaciones observadas fueron: hemorragia subaracnoidea con el 98,9% (356; 360) en aneurismas rotos; y cefalea con el 65,5% (57; 87) en aneurismas no rotos. DISCUSIÓN. Se encontró datos relevantes no coincidentes con la literatura científica mundial, como el bajo número de aneurismas asintomáticos y no rotos, comparados con su contraparte. CONCLUSIÓN. Se evidenció que los aneurismas intracraneales produjeron una amplia gama de manifestaciones clínicas, que fluctuaron desde la hemorragia subaracnoidea como la más frecuente en el grupo de aneurismas rotos, hasta pacientes completamente asintomáticos en el grupo de aneurismas no rotos.

INTRODUCTION. In Ecuador, cerebrovascular diseases are the third leading cause of death in the general population; there are few data in the medical literature on aneurysmal subarachnoid hemorrhage and unruptured aneurysms, so it was essential to describe a clinical profile. OBJECTIVE. To characterize the clinical profile of patients diagnosed with cerebral aneurysm. MATERIALS AND METHODS. Descriptive observational study. Population of 450 and sample of 447 patients with a diagnosis of cerebral aneurysm seen in the Neurology Unit of the Carlos Andrade Marín Hospital, period January 2010 to December 2018. Clinical and imaging data were collected through the review of digital medical records obtained from the MIS/AS400 computer system, and were analyzed in the statistical program International Business Machines Statistical Package for the Social Sciences, version 23. RESULTS. A total of 605 aneurysms were identified in 447 patients. A total of 80,5% (360; 447) had ruptured aneurysms, of which 81,4% (293; 360) had a single aneurysm. The most common risk factor was hypertension with 44,3% (198; 447), the manifestations observed were: subarachnoid hemorrhage with 98,9% (356; 360) in ruptured aneurysms; and headache with 65,5% (57; 87) in unruptured aneurysms. DISCUSSION. We found relevant data not consistent with the world scientific literature, such as the low number of asymptomatic and unruptured aneurysms, compared with its counterpart. CONCLUSION. It was evidenced that intracranial aneurysms produced a wide range of clinical manifestations, ranging from subarachnoid hemorrhage as the most frequent in the group of ruptured aneurysms, to completely asymptomatic patients in the group of unruptured aneurysms.

Perfil clínico y epidemiológico de los pacientes con diagnóstico de neuromielitis óptica / Clinical and epidemiological profile of patients with neuromyelitis optica diagnosis

INTRODUCCIÓN. El trastorno del espectro de neuromielitis óptica, enfermedad inflamatoria, desmielinizante, afecta al sistema nervioso central, frecuente en poblaciones no caucásicas como la ecuatoriana. El retraso en su diagnóstico y tratamiento provoca discapacidad que se puede prevenir. OBJETIVO. Determinar el perfil clínico y epidemiológico de los pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica. MATERIALES Y MÉTODOS. Estudio descriptivo transversal. Población de 45 Historias Clínicas y una muestra de 41 de pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica atendidos en la Unidad de Neurología del Hospital de Especialida-des Carlos Andrade Marín, período enero 2005 a diciembre 2019. Se realizó análisis univarial. Se aplicó el programa estadístico International Business Machines Statistical Package for the Social Sciences, versión 25. RESULTADOS. El 76,0% (31; 41) fueron mujeres. Datos promedios: edad 48,9 años; diagnóstico definitivo demoró 4,12 años, desde el inicio de los síntomas; tiempo de diagnóstico fue 3,17 años; 3,7 brotes en total; el 87,8% (36; 41) con un fenotipo recurrente. La media de duración de la enfermedad fue de 6,8 años. En el 70,7% (29; 41), se identificaron anticuerpos anti-AQP4 en suero mediante inmunofluorescencia directa, el 51,2% requirieron para la marcha apoyo uni o bilateral. El 43,9% (18; 41) debutó con neuritis óptica; el 31,7% (13; 41) presentaron mielitis como primer síntoma y el 24,4% (10; 41) la combinación de neuritis óptica y mielitis fueron los síntomas iniciales. CONCLUSIÓN. Se determinó el perfil clínico y epi-demiológico de los pacientes con diagnóstico de trastorno del espectro de neuromielitis óptica. Existió demora en el diagnóstico definitivo de los pacientes desde el inicio de los síntomas, lo que se tradujo en un aumento de la discapacidad.

INTRODUCTION. Neuromyelitis optica spectrum disorder, an inflammatory, demyelinating disease, affects the central nervous system, common in non-Caucasian popu-lations such as Ecuadorians. The delay in its diagnosis and treatment causes disabi-lity that can be prevented. OBJECTIVE. To determine the clinical and epidemiological profile of patients diagnosed with neuromyelitis optica spectrum disorder. MATERIALS AND METHODS. Cross-sectional descriptive study. Population of 45 Medical Records and a sample of 41 patients with a diagnosis of neuromyelitis optica spectrum disor-der seen at the Neurology Unit of the Carlos Andrade Marín Specialties Hospital, period from January 2005 to December 2019. Univariate analysis was performed. The statistical program International Business Machines Statistical Package for the Social Sciences, version 25 was used. RESULTS. 76,0% (31; 41) were women. Average data: age 48,9 years; definitive diagnosis took 4,12 years from the onset of symptoms; time to diagnosis was 3,17 years; 3,7 outbreaks in total; 87,8% (36; 41) with a recurrent phenotype. The average disease duration was 6,8 years. In 70,7% (29; 41), anti-AQP4 antibodies were identified in serum by direct immunofluorescence, 51,2% required uni- or bilateral su-pport for walking. Optic neuritis started in 43,9% (18; 41); 31,7% (13; 41) had myelitis as the first symptom and 24,4% (10; 41) the combination of optic neuritis and myelitis were the initial symptoms. CONCLUSION. The clinical and epidemiological profile of patients diagnosed with neuromyelitis optica spectrum disorder was determined. There was delay in the conclusive diagnosis of patients from the beginning of symptoms, which resulted in increased disability.

Impact of Rituximab on relapse rate and disability in an Ecuadorian cohort of patients with neuromyelitis optica spectrum disorders.

BACKGROUND: Neuromyelitis Optica Spectrum Disorder (NMOSD) is a severe inflammatory demyelinating disease of the central nervous system that often causes disability. Based on evidence from prospective and retrospective studies, Rituximab (RTX) has been used as the first-line of therapy in NMOSD. Nevertheless, evidence of the impact of RTX on relapse rate and disability in Ecuadorian patients with NMOSD is lacking. OBJECTIVE: To evaluate the impact of RTX in an Ecuadorian cohort of patients with NMOSD. MATERIALS AND METHODS: A retrospective study was conducted in a cohort of patients with NMOSD who received treatment with RTX in a third-level hospital in Quito, Ecuador. Digital medical records of NMOSD patients were reviewed to attain sociodemographic data, disease characteristics, and treatment with RTX. The annualized relapse rate ARR, as well as the degree of disability measured through the expanded disability scale (EDSS), was established before and after treatment. RESULTS: Twenty-three patients with NMOSD treated with RTX were included, the mean age of onset of the disease was 37.2 years (range, 13-64.5). The average duration of disease was 8.5 years (range, 1.3-34.4). Positivity for antibodies against aquaporin-4 (AQP4-IgG) was identified in 78% of the patients. The mean duration of the treatment with RTX was 40 months (range, 12-61). After the RTX therapy, the number of relapses was reduced in 91% (21/23) of cases. The annualized relapsed rate (ARR) was reduced with RTX from 1.89 to 0.12 (p <0.001). The mean EDSS was also reduced from 4.8 to 3.9 (p = 0.014). In all patients, the mean EDSS was reduced or stabilized with RTX. Overall, the drug was well tolerated, the most frequent adverse events were infections which were present in 65.2% of cases. CONCLUSIONS: Though with the limitations of and observational study, our data support RTX effectiveness and safety in an Ecuadorian cohort of patients with NMOSD.

Calidad de vida relacionada con la salud en pacientes con diagnóstico de tuberculosis pulmonar / Health-related quality of life in patients diagnosed with pulmonary tuberculosis

INTRODUCCIÓN. La tuberculosis es la novena causa de muerte a nivel mundial y la principal causa de defunción por un agente infeccioso. La localización pulmonar es frecuente, tiene síntomas inespecíficos y tendencia a la cronicidad. OBJETIVO. Evaluar la calidad de vida en una cohorte de pacientes con diagnóstico de tuberculosis pulmonar. MATERIALES Y MÉTODOS. Estudio observacional, analítico de casos y controles. Población y muestra conocida de 129 datos de Historias Clínicas, 43 casos y 86 controles sanos, en 15 Centros de Salud en Quito, de mayo a junio 2015. Criterios de inclusión (casos): mayor de 18 años, diagnóstico de tuberculosis pulmonar en tratamiento; (controles): no tener diagnóstico de tuberculosis pulmonar o extrapulmonar ni patologías crónicas. Los datos se tomaron de las Historias Clínicas, se aplicó una encuesta personalizada y el cuestionario de calidad de vida Short Form-36 versión 2. Se realizó un análisis univarial, bivarial y multivarial. Para el análisis de datos se utilizó el programa estadístico International Business Machines Statistical Package for the Social Sciences versión 23. RESULTADOS. Los casos tuvieron puntuaciones de calidad de vida bajos comparados con los contro- les (DM=34,11 IC 29,7-38,4). La dimensión más afectada fue función física (DM=46,5 IC 39,2­53,8) y la menos afectada fue dolor corporal (DM=24,1 IC 16,9­31,4). DISCUSIÓN. Se evidenció resultados similares de otros países con alta prevalencia de tuberculosis pulmonar que afectó la calidad de vida de los pacientes. CONCLUSIÓN. La tuberculosis pulmonar fue un factor determínate del deterioro de la calidad de vida.

INTRODUCTION. Tuberculosis is the ninth leading cause of death worldwide and the leading cause of death from an infectious agent. The pulmonary location is frequent, has nonspecific symptoms and a tendency to chronicity. OBJECTIVE. To assess the quality of life in a cohort of patients with a diagnosis of pulmonary tuberculosis. MATERIALS AND METHODS. Observational, analytical case-control study. Population and known sample of 129 data from Medical Records, 43 cases and 86 healthy controls, in 15 Health Centers in Quito, from may to june 2015. Inclusion criteria (cases): older than 18 years, diagnosis of pulmonary tuberculosis under treatment; (controls): not having a diagnosis of pulmonary or extrapulmonary tuberculosis or chronic pathologies. The data were taken from the Medical Records, a personalized survey and the Short Form-36 version 2 quality of life question- naire were applied. A univariate, bivariate and multivariate analysis was performed. For data analysis, the statistical program International Business Machines Statistical Package for the Social Sciences version 23 was used. RESULTS. The cases had low quality of life scores compared to the controls (MD=34,11 CI 29,7-38,4). The dimension most affected was physical function (MD=46,5 CI 39,2-53,8) and the least affected was body pain (MD=24,1 CI 16,9-31,4). DISCUSSION. Similar results were found in other countries with a high prevalence of pulmonary tuberculosis that affected the quality of life of patients. CONCLUSION. Pulmonary tuberculosis was a determining factor in the deterioration of quality of life.

El Perfil Epidemiológico Y Clínico De La Esclerosis Múltiple En El Ecuador / The Clinical And Epidemiological Profile Of Multiple Sclerosis In Ecuador

Resumen En el Ecuador ha habido un importante incremento en el número de publicaciones sobre Esclerosis Múltiple (EM) en los últimos años. Este interés por conocer el comportamiento clínico y epidemiológico de la enfermedad nos ha permitido establecer semejanzas y diferencias con otras cohortes de pacientes con EM que provienen de regiones en donde la prevalencia de la enfermedad es alta. El Ecuador sigue siendo un país de baja prevalencia, los estudios han demostrado que la misma fluctúa entre 3 a 5 casos por 100.000 habitantes. El comportamiento epidemiológico es muy similar a la de cohortes europeas por ejemplo el sexo femenino es el principalmente afectado. Sin embargo, el comportamiento clínico difiere en lo que respecta a deterioro cognitivo, fatiga siendo éstos menos frecuentes. Aún se desconoce el impacto de la vitamina D en nuestros pacientes debido a que, solo un estudio ha sido llevado a cabo. Al parecer, existe una alta prevalencia de deficiencia e insuficiencia de vitamina D en los pacientes ecuatorianos pero no se traduce en un incremento de prevalencia o discapacidad como ocurre en poblaciones europeas. A pesar de que tenemos una mejor comprensión de la enfermedad en el país, más estudios son necesarios y es imperativo incluir a todos los pacientes ecuatorianos con esclerosis múltiple con el fin de mejorar nuestro conocimiento sobre el comportamiento de esta patología en nuestra región.

Abstract In recent years, the number of publications on Multiple Sclerosis (MS) from Ecuador has seen a significant increase. As a result, the research on the clinical and epidemiological behaviour of the disease has allowed us to make comparisons with other cohorts of patients with MS that come from regions where the prevalence of the disease is high. Nevertheless, Ecuador is still a country in which the prevalence of MS is low with a prevalence that fluctuates between 3 to 5 cases per 100,000 inhabitants. The epidemiological behaviour of MS is very similar to that of european cohorts, for example female patients are the most affected. However, the clinical behaviour of multiple sclerosis differs in terms of cognitive impairment and fatigue being less frequent. The impact of vitamin D on patients with MS is still unknown as only one study has been carried out. This study show that there is a high prevalence of vitamin D deficiency and insufficiency in ecuadorian patients, but this does not translate into an increase in prevalence or disability as it does in european populations. Although we have a better understanding of the disease in the country, more studies are necessary, and it is imperative that all ecuadorian patients with MS be included in future studies in order to improve our knowledge about the behaviour of this disease in our region.

Factores pronósticos de la Esclerosis Múltiple / Prognostic factors in Multiple Sclerosis

Resumen La Esclerosis Múltiple es una enfermedad inflamatoria y degenerativa del Sistema Nervioso Central que afecta a la población adulta joven. La prevalencia de esta entidad es heterogénea en el mundo y baja en el Ecuador. El diagnóstico se basa en los criterios de McDonald 2017. Una vez que el diagnóstico se ha establecido, es necesario determinar si los pacientes tienen factores de mal pronóstico los cuales van a generar un impacto en el tipo de tratamiento a elegir. Al momento, se han estudiado factores pronósticos epidemiológicos, clínicos, biomoleculares y de imagen los cuales nos permiten predecir si la enfermedad tiene un comportamiento agresivo o por el contrario un curso benigno. El número de lesiones en las imágenes de resonancia magnética cerebral, la presencia de lesiones en tronco encefálico y médula espinal son los factores que han demostrado tener un impacto en la progresión de discapacidad. La presencia de bandas oligoclonales en el líquido cefalorraquídeo tiene un rol fundamental en la conversión de un síndrome clínico aislado en esclerosis múltiple clínicamente establecida. Los niveles bajos de vitamina D ha demostrado estar asociado con mal pronóstico pero su aplicabilidad en países como el Ecuador es aún tema de investigación.

Abstract Multiple sclerosis is an inflammatory and degenerative disease of the central nervous system which affects young adults. The prevalence of multiple sclerosis in the world is heterogeneous and is low in Ecuador. The diagnosis is based on the McDonald 2017 criteria. Once the diagnosis has been made, it is necessary that any negative factors which will impact the type of treatment used be identified. At this time, factors such as epidemiological, clinical, biomolecular, and magnetic resonance images, which will allow us to identify if the case is aggressive or benign, are studied. The number of lesions shown in a brain MRI, the presence of lesions in the brain stem and spinal cord are factors which have been demonstrated to have an impact on the progression of disability. The presence of oligoclonal bands in the cerebrospinal fluid has a fundamental role in the conversion of an isolated clinical syndrome to multiple sclerosis. Low levels of vitamin D have been associated with a negative prognosis, however how important vitamin D is in the prognosis of MS in countries such as Ecuador is still an area to be studied.