RESUMEN
Ischemic stroke (IS) results in the interruption of blood flow to the brain, which can cause significant damage. The pathophysiological mechanisms of IS include ionic imbalances, oxidative stress, neuroinflammation, and impairment of brain barriers. Brain barriers, such as the blood-brain barrier (BBB) and the blood-cerebrospinal fluid (CSF) barrier (B-CSF), protect the brain from harmful substances by regulating the neurochemical environment. Although the BBB is widely recognized for its crucial role in protecting the brain and its involvement in conditions such as stroke, the B-CSF requires further study. The B-CSF plays a fundamental role in regulating the CSF environment and maintaining the homeostasis of the central nervous system (CNS). However, the impact of B-CSF impairment during pathological events such as IS is not yet fully understood. In conditions like IS and other neurological disorders, the B-CSF can become compromised, allowing the entry of inflammatory substances and increasing neuronal damage. Understanding and preserving the integrity of the B-CSF are crucial for mitigating damage and facilitating recovery after ischemic stroke, highlighting its fundamental role in regulating the CNS during adverse neurological conditions.
Asunto(s)
Barrera Hematoencefálica , Accidente Cerebrovascular Isquémico , Barrera Hematoencefálica/fisiopatología , Humanos , Animales , Accidente Cerebrovascular Isquémico/fisiopatología , Estrés Oxidativo , Enfermedades Neuroinflamatorias/fisiopatología , Enfermedades Neuroinflamatorias/etiología , Accidente Cerebrovascular/fisiopatología , Encéfalo/fisiopatología , Encéfalo/irrigación sanguínea , Encefalopatías/fisiopatología , Encefalopatías/etiologíaAsunto(s)
Anticonvulsivantes , Antineoplásicos Alquilantes , Ifosfamida , Levetiracetam , Humanos , Levetiracetam/uso terapéutico , Anticonvulsivantes/efectos adversos , Anticonvulsivantes/uso terapéutico , Antineoplásicos Alquilantes/efectos adversos , Ifosfamida/efectos adversos , Piracetam/análogos & derivados , Piracetam/uso terapéutico , Piracetam/efectos adversos , Electroencefalografía , Masculino , Encefalopatías/inducido químicamente , Encefalopatías/diagnóstico por imagen , Encefalopatías/tratamiento farmacológico , FemeninoRESUMEN
Extracellular vesicles (EVs) hold promise as a source of disease biomarkers. The diverse molecular cargo of EVs can potentially indicate the status of their tissue of origin, even against the complex background of whole plasma. The main tools currently available for assessing biomarkers of brain health include brain imaging and analysis of the cerebrospinal fluid of patients. Given the costs and difficulties associated with these methods, isolation of EVs of neuronal origin (NEVs) from the blood is an attractive approach to identify brain-specific biomarkers. This perspective describes current key challenges in EV- and NEV-based biomarker research. These include the relative low abundance of EVs, the lack of validated isolation methods, and the difficult search for an adequate target for immunocapturing NEVs. We discuss that these challenges must be addressed before NEVs can fulfill their potential for biomarker research. HIGHLIGHTS: NEVs are promising sources of biomarkers for brain disorders. Immunocapturing NEVs from complex biofluids presents several challenges. The choice of surface target for capture will determine NEV yield. Contamination by non-EV sources is relevant for biomarkers at low concentrations.
Asunto(s)
Biomarcadores , Vesículas Extracelulares , Neuronas , Humanos , Vesículas Extracelulares/metabolismo , Biomarcadores/líquido cefalorraquídeo , Neuronas/metabolismo , Encéfalo , EncefalopatíasRESUMEN
Rhino-cerebral mucormycosis (RM) is a rare and opportunistic fungal infection observed in immune-compromised patients and metabolic imbalances such as Diabetes Mellitus. RM rapidly infiltrates blood vessels, leading to vascular thrombosis, subsequent tissue necrosis, and high mortality rates (23.6-60%). Due to its fast advancement, RM is a life-threatening condition requiring accurate clinical decisions by the medical and surgical teams. Based on the report of six cases, we emphasize the need for an early diagnosis and starting antifungal pharmacological therapy at the slightest suspicion of RM. Moreover, the restitution of metabolic balance and aggressive surgical debridement are vital steps to control RM, reducing the possibility of fatal outcomes.
Asunto(s)
Desbridamiento , Diagnóstico Precoz , Mucormicosis , Humanos , Mucormicosis/diagnóstico , Mucormicosis/terapia , Mucormicosis/microbiología , Mucormicosis/cirugía , Masculino , Persona de Mediana Edad , Femenino , Desbridamiento/métodos , Antifúngicos/uso terapéutico , Adulto , Anciano , Encefalopatías/diagnóstico , Encefalopatías/microbiología , Encefalopatías/terapia , Encefalopatías/cirugía , Enfermedades Nasales/diagnóstico , Enfermedades Nasales/microbiología , Enfermedades Nasales/terapia , Enfermedades Nasales/cirugíaRESUMEN
This article discusses a rare case of coexistent meningiomas and Primary familial brain calcification (PFBC). PFBC is a neurodegenerative disease characterized by brain calcifications and a variety of neuropsychiatric symptoms and signs, with pathogenic variants in specific genes. The study explores the potential link between PFBC and meningiomas, highlighting shared features like intralesional calcifications and common genes such as MEA6. The article also revisits PFBC patients developing other brain tumors, particularly gliomas, emphasizing the intersection of oncogenes like PDGFB and PDGFRB in both calcifications and tumor progression. In recent investigations, attention has extended beyond brain tumors to breast cancer metastasis, unveiling a noteworthy connection. These findings suggest a broader connection between brain calcifications and tumors, encouraging a reevaluation of therapeutic approaches for PFBC.
Asunto(s)
Neoplasias Encefálicas , Calcinosis , Meningioma , Humanos , Calcinosis/genética , Calcinosis/patología , Meningioma/genética , Meningioma/patología , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/patología , Neoplasias Encefálicas/metabolismo , Femenino , Neoplasias Meníngeas/genética , Neoplasias Meníngeas/patología , Encefalopatías/genética , Encefalopatías/patología , Encefalopatías/metabolismoRESUMEN
Thyroid storm is a rare but well-known life-threatening complication that occurs due to acute exacerbation of thyrotoxicosis with the increased levels of circulating thyroid hormones. Reports of metabolic encephalopathy associated with thyroid storm are scarce. We describe the case of a 23-year-old male patient with no previous history of abnormal thyroid function who had consumed excessive amounts of alcohol before disease onset. The patient was found unconscious and febrile on a roadside by a passerby and was admitted to our hospital's emergency department. His primary clinical presentation included hyperthermia (40.8 °C), nodal tachycardia (180 beats/min), seizures, coma, and hypoglycemia (2.18 mmol/L). The hypoglycemia was quickly corrected after admission, but his level of consciousness showed no improvement. With aggressive screening, the patient was found to have severe thyroid dysfunction (T3 = 6.67 nmol/L, T4 = 252.00 nmol/L, free T3 = 29.20 pmol/L, free T4 = 65.30 pmol/L, and TSH = 0.001 µIU/mL). After medical treatment, plasmapheresis, hemofiltration, and hemoperfusion, the patient showed substantial improvement in thyroid hormone levels and stabilization of vital signs, but the impaired consciousness and seizures persisted. Multiple computed tomography scans revealed brain abnormalities. Magnetic resonance imaging performed after tracheal extubation revealed bilateral frontal lobe lesions. We reported a case of metabolic encephalopathy in a patient with life-threatening thyroid storm and bilateral frontal lobe lesions. Hypoglycemia may have been involved in the development of encephalopathy in our patient. Health care providers should consider thyroid storm in the differential diagnosis of hyperthermia, seizures, and coma. Early plasmapheresis, hemofiltration, and hemoperfusion can lower T4 levels and improve prognosis in patients with thyroid storm and encephalopathy.
Asunto(s)
Lóbulo Frontal , Crisis Tiroidea , Humanos , Masculino , Crisis Tiroidea/complicaciones , Adulto Joven , Lóbulo Frontal/diagnóstico por imagen , Imagen por Resonancia Magnética , Encefalopatías/etiologíaRESUMEN
INTRODUCCIÓN La Encefalitis de Hashimoto (EH) es una encefalopatía de naturaleza autoinmune, con buena respuesta al tratamiento con corticoides, títulos séricos elevados de anticuerpos antitiroideos y de curso subagudo con recaídas-remisiones. Es una enfermedad poco frecuente, con una presentación clínica variable y fisiopatología aún desconocida. PRESENTACIÓN DEL CASO: Paciente femenina de 76 años con antecedentes de hipotiroidismo primario. Ingresó con un síndrome confusional agudo. Al examen físico vigil, Glasgow 13/15, subfebril (37.8°C) desorientada temporoespacialmente, ecolalia, pupilas isocóricas y reactivas, sin focalidad neurológica. Signos meníngeos negativos. Laboratorio: Hipocalcemia leve (7.8mg/dl), hipopotasemia (K 3,2 mmol/l), PCR 221.9 mg/L. Test rápido para VIH negativo. TC de encéfalo sin alteraciones. Punción lumbar líquido cristal de roca, proteínas 1 g/l, glucosa 0.67 g/l, láctico 1.3, leucocitos 77 células/microL (100% mononucleares). Se interpretó inicialmente como Encefalitis de etiología viral y se le indicó aciclovir. Presentó sensorio alternante, excitación psicomotriz y convulsión tónica clónica generalizada. Debido a deterioro súbito del sensorio, se realizó intubación orotraqueal y se trasladó a Unidad de Terapia Intensiva (UTI). Permaneció bajo asistencia mecánica ventilatoria y con vasopresores. Laboratorio: VDRL, p24 y anticuerpos HIV negativos, TSH 27,82, T4 0,41. PCR de LCR: Virus herpes simple 1 y 2, citomegalovirus y JC negativos. Hemocultivos negativos. Ante sospecha clínica de Encefalitis de Hashimoto, se solicitaron anticuerpos antitiroideo peroxidasa (aTPO), antitiroglobulina (aTG) y Anticuerpos Anti-Receptor de TSH (TRABS), que resultaron positivos. Recibió tratamiento con levotiroxina endovenosa e hidrocortisona. Normaliza valores. Por fallo en el weaning, se realizó traqueostomía. Luego de 21 días de internación en Terapia Intensiva pasó a clínica con posterior alta hospitalaria. Discusión: La EH se puede considerar como diagnóstico, solo después de descartar otras causas. En el caso expuesto se llegó al diagnóstico luego de descartar otras causas posibles, con anticuerpos antitiroideos positivos en altas concentraciones y respuesta al tratamiento con corticoides. Conclusión: Se destaca la necesidad de ampliar el conocimiento de esta patología con el fin de disminuir el subdiagnóstico y promover un inicio precoz del tratamiento, mejorando así su progresión y calidad de vida de los pacientes.
INTRODUCTION: Hashimoto's Encephalitis (HE) is an autoimmune encephalopathy, with a good response to treatment with corticosteroids, high serum titers of antithyroid antibodies and a subacute course with relapses-remissions. It is a rare disease, with a variable clinical presentation and still unknown pathophysiology. CASE REPORT: A 76-year-old female patient with a history of primary hypothyroidism. She was admitted with acute confusional syndrome. On physical examination, she was awake, she was Glasgow 13/15, she was subfebrile (37.8°C), disoriented temporally, echolalia, isochoric and reactive pupils, without neurological focality. Negative meningeal signs. Laboratory: Mild hypocalcemia (7.8 mg/dl), hypokalemia (K 3.2 mmol/l), CRP 221.9 mg/L. Rapid test for HIV negative. Brain CT without alterations. Lumbar puncture rock crystal liquid, proteins 1 g/l, glucose 0.67 g/l, lactic acid 1.3, leukocytes 77 cells/microL (100% mononuclear). It was initially interpreted as Encephalitis of viral etiology and acyclovir was prescribed. He presented alternating sensory, psychomotor excitement, and generalized tonic-clonic seizure. Due to sudden deterioration of the sensorium, orotracheal intubation was performed and he was transferred to the Intensive Care Unit. He remained under mechanical ventilatory assistance and with vasopressors. Laboratory: VDRL, p24 and HIV antibodies negative, TSH 27.82, T4 0.41. CSF PCR: Herpes simplex virus 1 and 2, cytomegalovirus and JC negative. Negative blood cultures. Due to clinical suspicion of Hashimoto's Encephalitis, anti-thyroid peroxidase (aTPO), anti-thyroglobulin (aTG) antibodies and Anti-TSH Receptor Antibodies (TRABS) were requested, which were positive. She was treated with intravenous levothyroxine and hydrocortisone. Normalized values. Due to weaning failure, a tracheostomy was performed. After 21 days of hospitalization in the Intensive Care Unit, she was admitted to the clinic and subsequently discharged from the hospital. Discussion: HD can be considered as a diagnosis, only after ruling out other causes. In the case presented, the diagnosis was made after ruling out other possible causes, with positive antithyroid antibodies in high concentrations and response to treatment with corticosteroids. Conclusion: The need to expand knowledge of this pathology is highlighted in order to reduce underdiagnosis and promote early initiation of treatment, thus improving its progression and quality of life of patients.
Asunto(s)
Encefalopatías , Enfermedad de Hashimoto , Enfermedades Autoinmunes , Enfermedades de la Tiroides , Informes de Casos , DiagnósticoRESUMEN
Hypoxia plays a significant role in the development of various cerebral diseases, many of which are associated with the potential risk of recurrence due to mitochondrial damage. Conventional drug treatments are not always effective for hypoxia-related brain diseases, necessitating the exploration of alternative compounds. In this study, we investigated the potential of diphenyl diselenide [(PhSe)2] to ameliorate locomotor impairments and mitigate brain mitochondrial dysfunction in zebrafish subjected to hypoxia. Additionally, we explored whether these improvements could confer resistance to recurrent hypoxia. Through a screening process, an appropriate dose of (PhSe)2 was determined, and animals exposed to hypoxia received a single intraperitoneal injection of 100 mg/kg of the compound or vehicle. After 1 h from the injection, evaluations were conducted on locomotor deficits, (PhSe)2 content, mitochondrial electron transport system, and mitochondrial viability in the brain. The animals were subsequently exposed to recurrent hypoxia to assess the latency time to hypoxia symptoms. The findings revealed that (PhSe)2 effectively crossed the blood-brain barrier, attenuated locomotor deficits induced by hypoxia, and improved brain mitochondrial respiration by modulating complex III. Furthermore, it enhanced mitochondrial viability in the telencephalon, contributing to greater resistance to recurrent hypoxia. These results demonstrate the beneficial effects of (PhSe)2 on both hypoxia and recurrent hypoxia, with cerebral mitochondria being a critical target of its action. Considering the involvement of brain hypoxia in numerous pathologies, (PhSe)2 should be further tested to determine its effectiveness as a potential treatment for hypoxia-related brain diseases.
Asunto(s)
Encefalopatías , Compuestos de Organoselenio , Animales , Pez Cebra , Mitocondrias , Derivados del Benceno/farmacología , Derivados del Benceno/uso terapéutico , Compuestos de Organoselenio/farmacología , Compuestos de Organoselenio/uso terapéutico , Hipoxia/tratamiento farmacológicoRESUMEN
Although some studies have shown neuroimaging and neuropsychological alterations in post-COVID-19 patients, fewer combined neuroimaging and neuropsychology evaluations of individuals who presented a mild acute infection. Here we investigated cognitive dysfunction and brain changes in a group of mildly infected individuals. We conducted a cross-sectional study of 97 consecutive subjects (median age of 41 years) without current or history of psychiatric symptoms (including anxiety and depression) after a mild infection, with a median of 79 days (and mean of 97 days) after diagnosis of COVID-19. We performed semi-structured interviews, neurological examinations, 3T-MRI scans, and neuropsychological assessments. For MRI analyses, we included a group of non-infected 77 controls. The MRI study included white matter (WM) investigation with diffusion tensor images (DTI) and functional connectivity with resting-state functional MRI (RS-fMRI). The patients reported memory loss (36%), fatigue (31%) and headache (29%). The quantitative analyses confirmed symptoms of fatigue (83% of participants), excessive somnolence (35%), impaired phonemic verbal fluency (21%), impaired verbal categorical fluency (13%) and impaired logical memory immediate recall (16%). The WM analyses with DTI revealed higher axial diffusivity values in post-infected patients compared to controls. Compared to controls, there were no significant differences in the functional connectivity of the posterior cingulum cortex. There were no significant correlations between neuropsychological scores and neuroimaging features (including DTI and RS-fMRI). Our results suggest persistent cognitive impairment and subtle white matter abnormalities in individuals mildly infected without anxiety or depression symptoms. The longitudinal analyses will clarify whether these alterations are temporary or permanent.
Asunto(s)
Encefalopatías , COVID-19 , Disfunción Cognitiva , Sustancia Blanca , Humanos , Adulto , Imagen de Difusión Tensora/métodos , Estudios Transversales , COVID-19/complicaciones , Disfunción Cognitiva/diagnóstico por imagen , Disfunción Cognitiva/etiología , Encéfalo/diagnóstico por imagen , Sustancia Blanca/diagnóstico por imagen , Trastornos de la Memoria , Fatiga/etiologíaRESUMEN
BACKGROUND: Hepatorenal syndrome is a condition that occurs in people with chronic liver disease (such as alcoholic hepatitis, advanced cirrhosis, or fulminant liver failure) and portal hypertension. The prognosis is dismal, often with a survival of weeks to months. Hepatorenal syndrome is characterised by the development of intense splanchnic vasodilation favouring ascites and hypotension leading to renal vasoconstriction and acute renal failure. Therefore, treatment attempts focus on improving arterial pressure through the use of vasopressors, paracentesis, and increasing renal perfusion pressure. Several authors have reported that the placement of transjugular intrahepatic portosystemic shunts (TIPS) may be a therapeutic option because it decreases portal pressure and improves arterial and renal pressures. However, the evidence is not clearly documented and TIPS may cause adverse events. Accordingly, it is necessary to evaluate the evidence of the benefits and harms of TIPS to assess its value in people with hepatorenal syndrome. OBJECTIVES: To evaluate the benefits and harms of transjugular intrahepatic portosystemic shunts (TIPS) in adults with hepatorenal syndrome compared with sham, no intervention, conventional treatment, or other treatments. SEARCH METHODS: We used standard, extensive Cochrane search methods. The latest search date was 2 June 2023. SELECTION CRITERIA: We included only randomised clinical trials with a parallel-group design, which compared the TIPS placement with sham, no intervention, conventional therapy, or other therapies, in adults aged 18 years or older, regardless of sex or ethnicity, diagnosed with chronic liver disease and hepatorenal syndrome. We excluded trials of adults with kidney failure due to causes not related to hepatorenal syndrome, and we also excluded data from quasi-randomised, cross-over, and observational study designs as we did not design a separate search for such studies. DATA COLLECTION AND ANALYSIS: We used standard Cochrane methods. Our primary outcomes were 1. all-cause mortality, 2. morbidity due to any cause, and 3. serious adverse events. Our secondary outcomes were 1. health-related quality of life, 2. non-serious adverse events, 3. participants who did not receive a liver transplant, 4. participants without improvement in kidney function, and 5. length of hospitalisation. We performed fixed-effect and random-effects meta-analyses using risk ratio (RR) or Peto odds ratio (Peto OR), with 95% confidence intervals (CI) for the dichotomous outcomes and mean difference (MD) or standardised mean difference (SMD) for the continuous outcomes. We used GRADE to assess certainty of evidence. MAIN RESULTS: We included two randomised clinical trials comparing TIPS placement (64 participants) versus conventional treatment (paracentesis plus albumin 8 g/L of removed ascites) (66 participants). The co-interventions used in the trials were dietary treatment (sodium less than 60 mmoL/day), spironolactone (300 mg/day to 400 mg/day), and furosemide (120 mg/day). Follow-up was up to 24 months. Both were multicentre trials from Spain and the USA, and Germany, conducted between 1993 and 2002. Most participants were men (aged 18 to 75 years). We are uncertain about the effect of TIPS placement compared with conventional treatment, during the first 24 months of follow-up, on all-cause mortality (RR 0.88, 95% CI 0.55 to 1.38; 2 trials, 130 participants; I2 = 58%; very low-certainty evidence) and on the development of any serious adverse event (RR 1.60, 95% CI 0.10 to 24.59; 2 trials, 130 participants; I2 = 78%; very low-certainty evidence). The use of TIPS may or may not result in a decrease in overall morbidity such as bacterial peritonitis, encephalopathy, or refractory ascites, during the first 24 months of follow-up, compared with the conventional treatment (RR 0.95, 95% CI 0.77 to 1.18; 2 trials, 130 participants; I2 = 0%; low-certainty evidence). We are uncertain about the effect of TIPS placement versus conventional treatment on the number of people who did not receive a liver transplant (RR 1.03, 95% CI 0.93 to 1.14; 2 trials, 130 participants; I2 = 0%; very low-certainty evidence) or on the length of hospitalisation (MD -20.0 days, 95% CI -39.92 to -0.08; 1 trial, 60 participants; very low-certainty evidence). Kidney function may improve in participants with TIPS placement (RR 0.53, 95% CI 0.27 to 1.02; 1 trial, 70 participants; low-certainty evidence). No trials reported health-related quality of life, non-serious adverse events, or number of participants with improvement in liver function associated with the TIPS placement. Funding No trials reported sources of commercial funding or conflicts of interest between researchers. Ongoing studies We found one ongoing trial comparing TIPS with conventional therapy (terlipressin plus albumin) and listed one study as awaiting classification as no full-text article could be found. AUTHORS' CONCLUSIONS: TIPS placement was compared with conventional treatment, with a follow-up of 24 months, in adults with hepatorenal syndrome type 2. Based on two trials with insufficient sample size and trial limitations, we assessed the overall certainty of evidence as low or very low. We are unsure if TIPS may decrease all-cause mortality, serious adverse events, the number of people who did not receive a liver transplant, and the days of hospitalisation because of the very low-certainty evidence. We are unsure if TIPS, compared with conventional treatment, has better effects on overall morbidity (bacterial peritonitis, encephalopathy, or refractory ascites). TIPS may improve kidney function, but the certainty of evidence is low. The trials included no data on health-related quality of life, non-serious adverse events, and liver function associated with the TIPS placement. We identified one ongoing trial and one study awaiting classification which may contribute to the review when information becomes available.
Asunto(s)
Encefalopatías , Síndrome Hepatorrenal , Peritonitis , Derivación Portosistémica Intrahepática Transyugular , Adulto , Humanos , Albúminas , Ascitis/etiología , Ascitis/cirugía , Encefalopatías/etiología , Síndrome Hepatorrenal/etiología , Síndrome Hepatorrenal/cirugía , Peritonitis/etiología , Derivación Portosistémica Intrahepática Transyugular/efectos adversos , Calidad de Vida , Ensayos Clínicos Controlados Aleatorios como AsuntoRESUMEN
OBJECTIVE: To evaluate the use of a large magnetic resonance imaging (MRI) normative dataset to quantify structural brain anomalies that may improve diagnostic sensitivity for atypical brain volume in youth with fetal alcohol spectrum disorder (FASD). STUDY DESIGN: Participants included 48 children with prenatal alcohol exposure (PAE) and 43 controls, ages 8-17 years, from the longitudinal Collaborative Initiative on FASD s. Recently published lifespan brain charts were used to quantify participants' (per)centile for brain volumes (cortical and subcortical gray matter and cortical white matter), providing an index of (dis)similarity to typically developing individuals of the same age and sex. RESULTS: Participants with PAE demonstrated lower mean centile scores compared with controls. Participants with PAE and scores ≤ 10th centile on at least 1 brain volume metric demonstrated significantly lower performance on measures of intellectual function and aspects of executive functioning compared with participants with PAE and "typical" volumes (>10th centile). Brain volume centiles explained a greater amount of variance in IQ and improved sensitivity to brain volume anomalies in FASD compared with the most commonly used diagnostic criterion of occipitofrontal circumference (OFC) ≤ 10th. CONCLUSION: Age- and sex-adjusted brain volumes based on a large normative dataset may be useful predictors of functional outcomes and may identify a greater number of individuals with FASD than the currently used criterion of OFC.
Asunto(s)
Encefalopatías , Trastornos del Espectro Alcohólico Fetal , Efectos Tardíos de la Exposición Prenatal , Embarazo , Niño , Adolescente , Femenino , Humanos , Trastornos del Espectro Alcohólico Fetal/diagnóstico por imagen , Encéfalo/diagnóstico por imagen , Imagen por Resonancia MagnéticaRESUMEN
[RESUMEN]. El síndrome urémico hemolítico (SUH) es una entidad endémica en nuestro país y constituye la primera causa de insuficiencia renal aguda en la edad pediátrica y la segunda de insuficiencia renal crónica. Se define por la aparición simultánea de anemia hemolítica microangiopática, trombocitopenia y daño parenquimatoso renal. Las manifestaciones iniciales incluyen insuficiencia renal aguda, hipertensión arterial y sintomatología neurológica hasta en un 30% de los casos aproximadamente. Dentro de las manifestaciones del sistema nervioso central se encuentran las convulsiones, irritabilidad, letargo, encefalopatía y coma. (1,2) Realizamos un estudio descriptivo, retrospectivo a partir de la revisión de historias clínicas de 53 pacientes menores de 16 años de edad, con diagnóstico de SUH derivados al servicio de pediatría, tanto cuidados intermedios como UTIP del Hospital El Cruce en el periodo de tiempo de enero del 2010 hasta septiembre del 2023 con el objetivo de describir la tasa de afectación neurológica y evaluar secuelas a largo plazo. Conclusiones: De los 53 niños con diagnóstico de SUH, 14 presentaron manifestaciones a nivel del SNC, es decir un 26% del total. Las manifestaciones que prevalecieron fueron las convulsiones y el estatus convulsivo. 3 de 14 pacientes permanecieron con secuelas neurológicas, los 11 restantes presentaron recuperación completa (89%). Si bien la afectación neurológica es menos común que la afectación renal, sigue siendo una causa de mortalidad aguda y discapacidad a largo plazo entre los pacientes con SUH.
[ABSTRACT]. Hemolytic uremic syndrome is an endemic entity in our country which establishes the main cause of acute kidney failure in children. It also represents the second cause of chronic kidney failure. Its main clinical manifestations are microangiopathic hemolytic anemia, thrombocytopenia and kidney injury. Early clinical manifestations are characterized by hypertension, acute kidney failure and neurological involvement in up to 30% of children with HUS. Seizures, irritability, lethargy, encephalopathy, and coma are the most common central nervous system manifestations. (1,2) We identified 53 children under the age of 16, between January 2010 and September 2023 with a confirmed diagnosis of SUH at El Cruce hospital . Patients came from intermediate care or had PICU admission. Our objective was to describe the neurological involvement rate in HUS and long term sequels. Of the 53 patients with HUS, 14 had central nervous system manifestations, which represents 26% of children with the diagnosis. Most frequent manifestations included seizures and convulsive status. 3 of 14 patients remained with neurological sequels, the last 11 showed total recovery. Even though Neurological involvement is less common than renal injury, it still represents one of the causes of acute mortality and long term disability in patients with HUS.
Asunto(s)
Síndrome Hemolítico-Urémico , Convulsiones , Encefalopatías , Manifestaciones NeurológicasRESUMEN
BACKGROUND: Neurological immune-related adverse events associated with immune checkpoint inhibitors can have several clinical manifestations, but the syndromes and prognostic factors are still not well known. We aimed to characterise and group the clinical features, with a special focus in patients presenting with encephalopathy, and to identify predictors of response to therapy and survival. METHODS: This retrospective observational study included patients with neurological immune-related adverse events from 20 hospitals in Spain whose clinical information, serum samples, and CSF samples were studied at Hospital Clinic de Barcelona, Barcelona, Spain. Patients with pre-existing paraneoplastic syndromes or evidence of alternative causes for their neurological symptoms were excluded. We reviewed the clinical information, classified their clinical features, and determined the presence of neural antibodies. Neurological status was assessed by the treating physician one month after adverse event onset (as improvement vs no improvement) and at the last evaluation (complete recovery or modified Rankin Scale score decrease of at least 2 points, indicating good outcome, vs all other modified Rankin Scale scores, indicating poor outcome); if the participant had died, the date and cause of death were recorded. We used Fisher's exact tests and Mann-Whitney U tests to analyse clinical features, and multivariable logistic regression to analyse prognostic factors. FINDINGS: From Jan 1, 2018, until Feb 1, 2023, 83 patients with suspected neurological immune-related adverse events after use of immune checkpoint inhibitors were identified, of whom 64 patients were included. These patients had a median age of 67 years (IQR 59-74); 42 (66%) were male and 22 (34%) were female. The predominant tumours were lung cancer (30 [47%] patients), melanoma (13 [21%] patients), and renal cell carcinoma (seven [11%] patients). Neural antibodies were detected in 14 (22%) patients; 52 (81%) patients had CNS involvement and 12 (19%) had peripheral nervous system involvement. Encephalopathy occurred in 45 (70%) patients, 12 (27%) of whom had antibodies or well defined syndromes consistent with definite paraneoplastic or autoimmune encephalitis, 24 (53%) of whom had encephalitis without antibodies or clinical features characteristic of a defined syndrome, and nine (20%) of whom had encephalopathy without antibodies or inflammatory changes in CSF or brain MRI. Nine (14%) of 64 patients had combined myasthenia and myositis, five of them with myocarditis. Even though 58 (91%) of 64 patients received steroids and 31 (48%) of 64 received additional therapies, 18 (28%) did not improve during the first month after adverse event onset, and 11 of these 18 people died. At the last follow-up for the 53 remaining patients (median 6 months, IQR 3-13), 20 (38%) had a poor outcome (16 deaths, one related to a neurological immune-related adverse event). Mortality risk was increased in patients with lung cancer (vs those with other cancers: HR 2·5, 95% CI 1·1-6·0) and in patients with encephalopathy without evidence of CNS inflammation or combined myocarditis, myasthenia, and myositis (vs those with the remaining syndromes: HR 5·0, 1·4-17·8 and HR 6·6, 1·4-31·0, respectively). INTERPRETATION: Most neurological immune-related adverse events involved the CNS and were antibody negative. The presence of myocarditis, myasthenia, and myositis, of encephalopathy without inflammatory changes, or of lung cancer were independent predictors of death. Most deaths occurred during the first month of symptom onset. If our findings are replicated in additional cohorts, they could confirm that these patients need early and intensive treatment. FUNDING: The Instituto de Salud Carlos III and the European Union.
Asunto(s)
Encefalopatías , Neoplasias Pulmonares , Miocarditis , Miositis , Humanos , Masculino , Femenino , Persona de Mediana Edad , Anciano , Inhibidores de Puntos de Control Inmunológico/efectos adversos , Estudios Retrospectivos , España , Miocarditis/complicaciones , Miocarditis/tratamiento farmacológico , Neoplasias Pulmonares/complicaciones , Neoplasias Pulmonares/tratamiento farmacológico , Neoplasias Pulmonares/patología , Síndrome , Estudios Observacionales como AsuntoRESUMEN
Malaria is a wide-spread disease in tropical areas. The severe form is characterized by organic involvement and/or hyperparasitaemia. Criteria for early monitoring in intensive care rooms are defined; without a timely and early treatment, severe malaria has a 100% mortality. Although the literature in these cases is not extensive, extracorporeal therapy used sequentially for hepatic and renal detoxification is a useful and safe tool that can be used in intensive care. We describe the case of a 36-year-old man with a diagnosis of severe malaria according to WHO criteria. He began treatment with intravenous artesunate and due to a torpid evolution, a sudden increase in bilirubinemia with encephalopathy, parameters of acute kidney injury and acute pulmonary edema, undergoes extracorporeal sequential treatment, coupled with plasma filtration adsorption, high-exchange plasmapheresis, and continuous hemodiafiltration with favorable evolution. This case shows that extracorporeal support in trained hands and in a timely manner is effective when organ failure evolves rapidly to achieve stability and provide necessary time for definitive treatment, in this case rapid action antimalarials until parasitemia becomes negative.
La malaria es una enfermedad con amplia distribución en áreas tropicales. En su forma grave se caracteriza por afección orgánica y/o hiperparasitemia. Se definen los criterios para el monitoreo temprano en las salas de terapia intensiva, debido a que sin tratamiento oportuno y precoz la malaria grave tiene una mortalidad de 100%. Si bien no es amplia la literatura en este aspecto la terapia extracorpórea en forma secuencial para detoxificación hepática y renal es una herramienta útil y segura que puede ser utilizada en terapia intensiva. Se describe un caso de un varón de 36 años con diagnóstico de malaria grave según criterio de la Organización Mundial de la Salud (OMS) que comenzó con tratamiento con artesunato endovenoso y por evolución tórpida, ascenso brusco de bilirrubinemia con encefalopatía, parámetros de lesión renal aguda y edema agudo de pulmón, realiza tratamiento extracorpóreo secuencial, plasma filtración acoplada a adsorción, plasmaféresis de alto intercambio y hemodiafiltración continua con evolución favorable. En conclusión, el caso presentado nos demuestra que el rol del sostén extracorpóreo en manos entrenadas y en forma oportuna es crucial cuando el fallo de órganos evoluciona rápidamente para lograr dar estabilidad y otorgar el tiempo necesario para la acción del tratamiento definitivo en este caso, los antimaláricos de acción rápida hasta negativización de la parasitemia.
Asunto(s)
Antimaláricos , Encefalopatías , Malaria Falciparum , Malaria , Masculino , Humanos , Adulto , Malaria Falciparum/diagnóstico , Malaria Falciparum/tratamiento farmacológico , Malaria/tratamiento farmacológico , Antimaláricos/uso terapéutico , Artesunato/uso terapéutico , Encefalopatías/tratamiento farmacológicoRESUMEN
INTRODUCCIÓN: La enfermedad cerebral de pequeño vaso es una causa principal de pérdida funcional, discapacidad y deterioro cognitivo. OBJETIVO: Determinar la prevalencia de la enfermedad de pequeño vaso y características clínicas que se asocian a mayor deterioro funcional, cognitivo y afectivo en adultos mayores con enfermedad cerebrovascular atendidos en el Servicio de Neurología del Hospital Carlos Andrade Marín en el período 2020 2021. METODOLOGÍA: Estudio observacional, analítico transversal con 80 pacientes mayores de 65 años con enfermedad cerebrovascular previamente diagnosticada. Se determinó cuáles presentaban enfermedad cerebral de pequeño vaso. Se compararon los dos grupos el de enfermedad cerebro vascular isquémico con y sin enfermedad cerebral de pequeño vaso. Se midió el grado de deterioro funcional con escala de Barthel; Lawton y Brody. El deterioro cognitivo con test de Montreal Cognitive Assessment Basic, estado afectivo con escala de Yesavage. Se utilizó razón de momios y se consideró significativo un valor p <0,05. Se utilizó el programa Statistical Package for Social Sciences versión 25. RESULTADOS: Los hombres representaron el 51,2%. La edad promedio fue 76,2 años. Prevalencia de enfermedad cerebral de pequeño vaso (87,5%). Escala de Fazekas grado 1 (46,3%), Factores asociados con enfermedad cerebral de pequeño vaso: tabaquismo [RR: 7,27; IC 95%: 1,69-31,3); enfermedad renal crónica [RR: 4,0; IC 95%: 1,01-15,7]. Dependencia moderada [RR: 6,42; IC 95%: 1,02-40,3]. Factores asociados con pérdida funcionalidad: gravedad del ictus. Factores asociados con deterioro cognitivo: infarto con doble territorio. Factores asociados con deterioro afectivo: infarto con doble territorio y síndrome metabólico (p<0,05). CONCLUSIÓN: La enfermedad cerebral de pequeño vaso tiene una elevada prevalencia entre los adultos mayores con enfermedad cerebrovascular y representó un deterioro cognitivo, funcional y afectivo considerable, en relación a los pacientes sin esta enfermedad.
INTRODUCTION: Cerebral small vessel disease is a leading cause of functional loss, disability, and cognitive impairment. OBJECTIVE: To determine the prevalence of small vessel disease and clinical characteristics associated with greater functional, cognitive and affective impairment in older adults with cerebrovascular disease attended at the Neurology Service of the Carlos Andrade Marín Hospital in the period 2020 - 2021. METHODOLOGY: Observational, analytical cross-sectional study with 80 patients over 65 years of age with previously diagnosed cerebrovascular disease. It was determined which patients had cerebral small vessel disease. The two groups of ischemic cerebrovascular disease with and without cerebral small vessel disease were compared. The degree of functional impairment was measured with the Barthel, Lawton and Brody scales. Cognitive impairment was measured with the Montreal Cognitive Assessment-Basic test, and affective state with the Yesavage scale. Odds ratio was used and a p value <0,05 was considered significant. Statistical Package for Social Sciences version 25 was used. RESULTS: Males represented 51,2%. Mean age was 76,2 years. Prevalence of cerebral small vessel disease (87,5%). Fazekas scale grade 1 (46,3%), Factors associated with cerebral small vessel disease: smoking [RR: 7,27; 95% CI: 1,69-31,3); chronic kidney disease [RR: 4,0; 95% CI: 1,01-15,7]. Moderate dependence [RR: 6,42; 95% CI: 1,02-40,3]. Factors associated with loss of function: severity of stroke. Factors associated with cognitive impairment: infarction with double territory. Factors associated with affective impairment: dual territory infarction and metabolic syndrome (p<0.05). CONCLUSION: Cerebral small vessel disease has a high prevalence among older adults with cerebrovascular disease and represented a considerable cognitive, functional and affective deterioration, in relation to patients without this disease.
Asunto(s)
Humanos , Masculino , Femenino , Anciano , Encefalopatías , Anciano , Disfunción Cognitiva , Porencefalia , Accidente Cerebrovascular Isquémico , Estado Funcional , Ecuador , GeriatríaRESUMEN
La Enfermedad Cerebrovascular Isquémica (ECV-Isquémica) provoca alteraciones neurológicas agudas, causadas por la dis-función del flujo sanguíneo cerebral, lo que determina la pre-sencia de injuria neuronal.1Los factores de riesgo se clasifican en modificables y no modi-ficables entre estos últimos, los más frecuentes son: la hiperten-sión arterial, diabetes mellitus, obesidad, tabaco y sedentarismo, y su frecuencia es notablemente mayor después de los 65 años de edad (Anexo 1).1La Enfermedad Cerebrovascular Isquémica se caracteriza por ser la segunda causa de mortalidad a nivel mundial, y la ter-cera en causar discapacidad. En 2019, según el reporte del Ins-tituto Nacional de Estadísticas y Censos (INEC), se registraron 4577 fallecimientos producto de esta patología; y se reportó como la tercera causa de fallecimiento en hombres y mujeres en Ecuador.2El impacto económico que genera la ECV-Isquémica es con-siderable, puesto que se ha evidenciado que aproximadamente supone un gasto promedio de 4330 dólares en los primeros 3 meses posterior a presentar esta patología, sin considerar otras consecuencias como la pérdida laboral.3
Ischemic Cerebrovascular Disease (Ischemic-CVD) causes acute neurological alterations, caused by cerebral blood flow dysfunction, which determines the presence of neuronal injury.1Risk factors are classified as modifiable and non-modifiable, among the latter, the most frequent are: arterial hypertension, diabetes mellitus, obesity, smoking and sedentary lifestyle, and their frequency is notably higher after 65 years of age (Anexo 1).1Ischemic Cerebrovascular Disease is characterized as the second leading cause of mortality worldwide, and the third leading cause of disability. In 2019, according to the report of the National Ins-titute of Statistics and Census (INEC), 4577 deaths were regis-tered as a result of this pathology; and it was reported as the third leading cause of death in men and women in Ecuador.2The economic impact of CVD-ischemic stroke is considerable, since it has been shown that approximately US$ 4330 is spent on average in the first 3 months after the onset of this pathology, without considering other consequences such as loss of work.3
Asunto(s)
Humanos , Masculino , Femenino , Adolescente , Adulto , Persona de Mediana Edad , Anciano , Adulto Joven , Atención Terciaria de Salud , Isquemia Encefálica , Infarto Encefálico , Cuidados Críticos , Accidente Cerebrovascular Isquémico , Neurología , Encefalopatías , Activador de Tejido Plasminógeno , Accidente Cerebrovascular , EcuadorRESUMEN
INTRODUCTION: Cerebral venous sinus thrombosis (CVST) is a well-known, although underestimated, cause of stroke in childhood. Its diagnosis requires a high index of suspicion, a correct interpretation of neuroimaging studies and an interrelation between clinicians and radiologists. The clinical features, risk factors and neuroimaging of children under 15 years of age with CVST were analyzed. METHODS: multicenter, retrospective, descriptive, study of a consecutive series of cases of children under 15 years of age, who were admitted due to CVST between January 1st, 2010, and March 1st, 2022. RESULTS: The study included 51 patients: 39% with acute symptoms and 59% with subacute symptoms. Newborns predominantly presented encephalopathic symptoms and seizures, while children exhibited signs of intracranial hypertension (ICH). Risk factors were identified in 90% of the cases. Magnetic resonance with angiography in venous time confirmed the diagnosis in 80% of the patients, with the straight sinus being the most affected in newborns and the lateral sinus in children. Hemorrhagic complications were found in 30.5%, being more frequent in newborns. Anticoagulation was initiated in 82% without complications. Sequelae were present in 44.4% of newborns and 37.9% of children, being more frequent and severe in newborns. CONCLUSIONS: To make an early diagnosis, it is essential to consider CVST in newborns with encephalopathic symptoms and/or seizures, and in children with signs of ICH in the presence of predisposing or triggering conditions.
Introducción: La trombosis de venas y senos venosos cerebrales (TVSC) constituye una causa conocida, aunque subestimada de ictus en la infancia. Su diagnóstico requiere un alto índice de sospecha, una correcta interpretación de la neuroimagen e interrelación entre el clínico y el radiólogo. OBJETIVO: Analizar las manifestaciones clínicas, factores de riesgo y neuroimagen de recién nacidos (RN) y niños menores de 15 años con TVSC. Métodos: Estudio descriptivo, retrospectivo, multicéntrico, de una serie consecutiva de casos de menores de 15 años que ingresaron por TVSC entre el 1 de enero del 2010 y el 1 de marzo de 2022. RESULTADOS: El estudio incluyó 51 pacientes: 39% con síntomas agudos y 59% subagudos. En los RN predominaron síntomas encefalopáticos y convulsiones, mientras en los niños elementos de hipertensión endocraneana (HTEC). Se identificaron factores de riesgo en el 90% de los casos. La resonancia magnética con angiografía en tiempo venoso confirmó el diagnóstico en el 80%, siendo el seno recto el más afectado en RN y el seno lateral en niños. Se encontraron complicaciones hemorrágicas en 30.5%, siendo más frecuentes en los RN. Se inició anticoagulación en el 82% sin complicaciones. Las secuelas estuvieron presentes en 44.4% de RN y 37.9% de niños, siendo más frecuentes y graves en los RN. CONCLUSIONES: Para realizar un diagnóstico precoz es fundamental pensar en TVSC en RN con síntomas encefalopáticos y/o convulsiones y en mayores con clínica de HTEC en presencia de enfermedades predisponentes o desencadenantes.