Múltiples infartos cerebrales en zona limítrofe y encefalopatía como forma de presentación del COVID19 en un paciente con CADASIL

Introducción: El virus SARS-Cov-2 se ha asociado a múltiples manifestaciones neurológicas, incluyendo accidente cerebrovascular agudo. La manifestación cerebrovascular reportada con mayor secuencia es accidente cerebrovascular secundario a trombosis de grandes vasos. La arteriopatía cerebral autosómica dominante con infartos subcorticales y leucoencefalopatía (CADASIL, por las siglas en inglés), es la enfermedad genética más frecuente asociada a lesiones de sustancia blanca e infartos lacunares múltiples. Se desconoce como el SARS-Cov-2 podría afectar en los pacientes con CADASIL. El objetivo de este trabajo es reportar la manifestación neurológica del COVID-19 en un paciente con CADASIL. Métodos: Se buscó información clínica y de laboratorio en los registros clínicos. Información adicional se obtuvo del informe de la evaluación de neuropsicología y de la entrevista con el paciente. Resultados: Paciente en la sexta década de la vida consulta a servicio de urgencias por confusión, desorientación, dificultad para expresarse y debilidad en su mano y pierna a derecha. La resonancia magnética cerebral demostró lesiones subcorticales múltiples agudas en áreas limítrofe y lesiones de sustancia blanca crónicas en capsula externa y polos de los lóbulos temporales, ambas típicas del CADASIL. El examen genético arrojó mutación sin sentido en el gen NOTCH3. El paciente fue seguido durante 10 meses, aunque presentó mejoría de su condición neurológica, persistió el déficit cognitivo con repercusión en sus actividades instrumentales de la vida diaria. Conclusión: Los pacientes con CADASIL que se infectan con SARS-Cov-2 pueden manifestarse con accidentes cerebrovasculares de zona limítrofe y encefalopatía. El COVID19 podría acelerar la declinación cognitiva descripta en los pacientes con CADASIL.

Introduction: The SARS-Cov-2 is associated with many neurological manifestations, including acute cerebrovascular disease. The most common reported stroke manifestation is ischemic stroke secondary to large vessels occlusion. The cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the most frequent genetic disease associated with white matter disease and multiple lacunar strokes. How the SARS-Cov-2 could affect CADASIL patients is unknown. Our aim is to report the neurologic presentation of COVID19 in a CADASIL patient. Method: With searched laboratory data and patient history in clinical registers. Additional information was obtained from the neuropsychologic report and patient's family interview. Results: A patient on his fifties consulted to the emergency department for disorientation, difficulty with language and weakness of his right arm and leg. The magnetic resonance showed multiple acute subcortical border zone lesions and other chronic white matter lesions affecting the pole of the temporal lobes and external capsule, both typical of CADASIL. The genetic examination confirmed a missense mutation on the NOTCH3 gene. The patient was followed up for 10 months and although there was an improvement in his neurologic condition, he remained with cognitive deficits that impacted in his instrumental activities of daily living. Conclusion: CADASIL patients infected with SARS-Cov-2 can suffer of multiple border zone infarcts and encephalopathy. The COVID19 could accelerated the cognitive decline of CADASIL patients.

Hipopituitarismo postraumatismo encefalocraneano: revisión de la literatura y algoritmo de estudio y abordaje terapéutico

Hypopituitarism after moderate or severe traumatic brain injury (TBI) is usually underdiagnosed and therefore undertreated. Its course can be divided in an acute phase during the first 14 days after TBI with 50 to 80% risk of hypopituitarism, and a chronic phase, beginning three months after the event, with a prevalence of hypopituitarism that ranges from 2 to 70%. Its pathophysiology has been addressed in several studies, suggesting that a vascular injury to the pituitary tissue is the most important mechanism during the acute phase, and an autoimmune one during chronic stages. In the acute phase, there are difficulties to correctly interpret pituitary axes. Hence, we propose a simple and cost-effective algorithm to detect and treat a potential hypothalamic-pituitary-adrenal axis impairment and alterations of sodium homeostasis, both of which can be life-threatening. In the chronic phase, post-concussion syndrome is the most important differential diagnosis. Given the high prevalence of hypopituitarism, we suggest that all pituitary axes should be assessed in all patients with moderate to severe TBI, between 3 to 6 months after the event, and then repeated at 12 months after trauma by a specialized team in pituitary disease.

La criticidad de las Unidades de Intensivo. Ampliando las Unidades en tiempos de pandemia / Expanding ICU facilities in a pandemic scenario

Our country is suffering the effects of the ongoing pandemic of coronavirus disease (COVID-19). Because the vulnerability of healthcare systems, especially the intensive care areas they can rapidly be overloaded. That challenge the ICUs simultaneously on multiple fronts making urgent to increase the number of beds, without lowering the standards of care. The purpose of this article is to discuss some aspects of the national situation and to provide recommendations on the organizational management of intensive care units such as isolation protocols, surge in ICU bed capacity, ensure adequate supplies, protect and train healthcare workers maintaining quality clinical management.

Efectos Adversos del Tratamiento con Vigabatrina en Síndrome de West: reporte de 2 casos / Adverse Effects of Vigabatrin Use for West Syndrome treatment: a report of 2 Clinical Cases

El síndrome de West (SW) es un síndrome epiléptico de la infancia temprana. Dentro de los fármacos de primera línea utilizados para su tratamiento se encuentran la hormona adrenocorticotropa (ACTH) y Vigabatrina. Estudios sugieren igual efectividad en el uso a largo plazo de ambos para controlar el SW. En Chile, el uso de Vigabatrina ha aumentado dada su mayor disponibilidad, facilidad de uso y menor costo. Se describen 2 casos clínicos presentando complicaciones agudas infrecuentes secundarias a su uso. Ambos pacientes con antecedentes de SW y trisomía 21. Primer caso: Lactante de 11 meses que inicia tratamiento con 100 mg/kg/día de Vigabatrina a los 7 meses, aumentando a 150 mg/kg/día por mala respuesta. Evolucionó con un síndrome extrapiramidal, con alteraciones radiológicas características. Segundo caso: Lactante de 7 meses, que tras iniciar tratamiento con vigabatrina (100 mg/kg/día) desarrolla rash facial sugerente de hipersensibilidad a fármacos antiepilépticos (FAEs), sin compromiso mucoso ni alteraciones sistémicas. Ambas regresan a su basal luego de suspensión o disminución de dosis del medicamento. Destaca la importancia de la monitorización de efectos adversos en el uso de FAEs y atender la aparición de reacciones poco conocidas. Las alteraciones imagenológicas por Vigabatrina son conocidas, no así el síndrome extrapiramidal asociado (primer caso). Por otra parte, las reacciones cutáneas están ampliamente descritas para múltiples FAEs, pero no para Vigabatrina (segundo caso). Dado el uso frecuente de Vigabatrina para tratar SW y otras epilepsias, es fundamental conocer y manejar reacciones adversas poco conocidas como las aquí presentadas. Palabras claves: Síndrome de West, Síndrome de Down, espasmos infantiles, vigabatrina, reacciones adversas, toxicidad, alergia, rash.

West Syndrome is an epileptic syndrome which typically presents in early childhood. In regard to treatment, the first line includes adrenocorticotropic hormone (ACTH) and Vigabatrin. Studies suggest similar response in the long term to both treatments. In Chile, Vigabatrin is being used more frequently as it is more available, of easier administration and lower cost. We present in the following report 2 clinical cases that presented acute infrequent complications secondary to its use in patients with both Down and West Syndrome. First case: 11-month-old infant who was initially treated with 100mg/kg/day of Vigabatrin at 7 months of age and increased to 150mg/kg/day due to lack of response. She evolved with an extrapyramidal syndrome with radiological manifestations. The second case: 7-month old toddler who initiated treatment with 100mg/kg/day of Vigabatrin and developed a facial rash, suggestive of hypersensitivity to antiepileptic drugs, with no mucosal or systemic involvement. Both patients returned to their previous condition shortly after Vigabatrin was decreased or discontinued. We emphasize the importance of the early monitorization of adverse effects in the use of antiepileptic drugs and awareness of less common reactions. Radiological findings associated with the use of Vigabatrin are well known, but not the clinical evolution with symptomatic extrapyramidal symptoms, as in the first case. Allergic reactions to the use of antiepileptic drugs have also been reported to several drugs, but not to Vigabatrin (second case). As Vigabatrin is being used more frequently to treat WS and other epilepsies it is important to know and manage uncommon adverse reactions as the ones presented in this report. Keywords: West Syndrome, Down Syndrome, infantile spasms, vigabatrin, adverse reactions, toxicity, allergy, rash

Características y evolución de los pacientes que ingresan a una Unidad de Cuidados Intensivos de un hospital público / Characteristics and evolution of patients admitted to a public hospital intensive care unit

Background: The epidemiology of critical patients in Chile could differ from that reported in international studies. Aim: To describe the causes of admission and evolution of patients who were admitted to the ICU (Intensive Care Unit) of a general hospital in Chile in a two-year period (2012-2013). Patients and Methods: A retrospective study was carried out using the ICU database. The following variables were registered: admission diagnosis, APACHE II (Acute Physiology and Chronic Health Evaluation), days of mechanical ventilation (MV), ICU length of stay and ICU and hospital survival. Results: We analyzed data from 1075 aged 54 ± 18 years (55% males), representing 75% of the admissions during the study period. The median ICU and MV lengths were 5 and 3 days respectively (92% of patients required MV). APACHE II was 20.5 ± 8.2. The ICU and hospital mortality rate were 19.4% and 31%, respectively. Critical neurological diseases were the most common diagnoses requiring ICU, representing 26.8% of the admissions. No differences were found between 2012 and 2013 in age, APACHE II, ICU or hospital survival. A longer post ICU length of stay was found during 2013, both for patients who survived and those who died at the hospital. Conclusions: This study highlights the high percentage of patients that required MV and the high percentage critical neurological conditions requiring ICU admission. The characteristics and evolution of patients admitted to the ICU did not differ during 2012 and 2013.

Utilidad del estudio contrastado precoz del tracto digestivo superior después de cirugía bariátrica: ¿se justifica su uso rutinario? / Usefulness of upper gastrointestinal series to detect leaks in the early postoperative period of bariatric surgery

Background: Postoperative leaks are the most undesirable complication of bariatric surgery and upper gastrointestinal (GI) series are routinely ordered to rule them out. Despite the published literature recommending against its routine use, it is still being customarily used in Chile. Aim: To examine the usefulness of routine upper GI series using water-soluble iodinated contrast media for the detection of early postoperative leaks in patients undergoing bariatric surgery. Material and Methods: A cohort of 328 patients subjected to bariatric surgery was followed from October 2012 to October 2013. Most of them underwent sleeve gastrectomy. Results: Upper GI series on the first postoperative day were ordered to 308 (94%) patients. Postoperative leaks were observed in two patients, with an incidence of 0.6%. The sensitivity for upper GI series detection of leak was 0% and the negative predictive value was 99%. Conclusions: Routine upper GI series after bariatric surgery is not useful for the diagnosis of postoperative leak, given the low incidence of this complication and the low sensitivity of the technique.

Manejo del potencial donante cadáver / Management of the potential organ donor

Solid organ transplantation is limited by donor availability. The loss of brain function produces hemodynamic, respiratory, hormonal and metabolic changes that lead to hypotension and organ dysfunction. Management of a potential donor is similar to any critically ill patient. Cardiovascular stability and protective ventilatory support must be pursued, aimed at minimizing the local and systemic inflammatory response that is triggered by brain death. There is no consensus on protocols for hormonal supplementation. The administration of vasopressin analogues and steroids may be beneficial under certain conditions. Appropriate medical management helps to optimize the function of different organs prior to transplantation. This may increase the number of harvested organs and improve their functional outcome in the recipient.

Características de los pacientes traumatizados que ingresan a la UCI de un hospital general en Chile / Characteristics of trauma patients admitted to the intensive care unit of a general hospital in Chile

Background: Trauma is an important cause of death among young adults. Aim: To determine the characteristics, treatments and evolution of trauma patients admitted to an intensive care unit (ICU) of a public hospital in Santiago, Chile. Material and Methods: All polytrauma (PT) and severely traumatized (ST) patients admitted to ICU were included. We recorded the type of trauma along with demographic and hemodynamic variables, treatments and complications. The evolution and treatments received by PT and ST patients were compared. Results: We recorded data from 72 patients aged 43 ± 21 years (93% males). Sixty two percent were PT and 24% had penetrating injuries. TBI (Trauma Brain Injury) was the most common trauma. On admission, acute Physiology and Chronic Health Evaluation II (APACHE II) score was 18.7 + 7.3, and Injury Severity Score (ISS) was 32.8 + 20.1. ICU stay was 7.8 + 6 days. Sixty seven per cent of patients required surgery and 58% received blood transfusions. No differences were found between PT and ST. ICU and hospital mortality rates were 15 and 25% respectively. Conclusions: The characteristics and evolution of PT and ST of this series of patients are similar to those described abroad. Mortality was in agreement with ISS and APACHE II scores.

Evolución del compromiso cardiovascular de pacientes insuficientes renales, en hemodiálisis, sin bloqueo del eje renina-angiotensina / Cardiovascular assessment of non diabetic patients on hemodialysis

Background: Diabetes mellitus is an important risk factor for cardiovascular complications among patients on hemodialysis. However, the incidence of these complications among non diabetic patients on hemodialysis is not well known. Aim: To assess the incidence of cardiovascular complications in non diabetic patients on hemodialysis. Patients and methods: Seventy five non diabetic patients aged 55.6 ± 17 years (48 males), receiving hemodialysis three times a week were evaluated with laboratory tests, echocardiogram anda carotid ultrasound. In 26 patients, interleukin 6, tumor necrosis factor alpha, and intercellular adhesión molecule (ICAM-1) were also measured. Patients were followed during two years. Results: The mean lapse of dialysis therapy was 6.5 ±5 years. The main cause of renal failure was hypertension. Sixty two percent had systolic hypertension, 86 percent had concentric left ventricular hypertrophy, 43 percent had atrial dilatation and 60 percent had calcifications in the thoracic aorta. Compared with normal controls, patients had higher levels of interleukin 6, tumor necrosis factor alpha and ICAM-1. Carotid media thickness was also higher and increased in the two years of follow up. No correlations were found between ventricular hypertrophy and dialysis lapse, packed red cell volume, calcium phosphorus product, parathormone levels or median arterial pressure. No cardiovascular events were recorded during the follow up period. Conclusions: Non diabetic patients on chronic hemodialysis have a high frequency of ventricular hypertrophy, carotid media thickening, aortic calcifications and an increase in proinflammatory cytokines.