Drug reaction with eosinophilia and systemic symptoms (DRESS) induced by phenytoin re-exposure: case report and systematic review.

Introduction: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is an adverse and severe skin reaction due to patients' susceptibility to medications, including phenytoin. The objective was to explore the characteristics of patients with DRESS secondary to phenytoin through a systematic review.Methods: We describe a case of DRESS syndrome secondary to phenytoin in a patient with previous exposure to this drug. A systematic literature review of cases of phenytoin-induced DRESS syndrome was conducted in PubMed/Medline, Scopus and Web of Science until May 2019.Results: 37 articles describing 40 cases of DRESS syndrome were selected. Mean age of onset was 33 years, without gender difference. Symptoms started between two and 90 days (mean ± 23 days). Liver and respiratory tract were most frequently involved, and eosinophilia was a common feature, it was presented with an average value of 9.7%. A discussion of the case and qualitative synthesis of the evidence reported in the literature were made.Conclusion: Patients and presentation characteristics will mostly be presented according to the criteria used by RegiSCAR. It is recommended to consider adequate monitoring of adverse reactions to antiepileptic drugs such as DRESS syndrome, given its severity and high lethality.

Is Surfer's myelopathy an acute hyperextension-induced myelopathy? A systematic synthesis of case studies and proposed diagnostic criteria.

BACKGROUND: Surfer's myelopathy is a rare complication of spinal hyperextension originally described in novice surfers. However, reports from patients practicing different activities had risen. AIM: To systematically synthesize the epidemiological and clinical evidence on acute hyperextension-induced myelopathy ("Surfer's myelopathy") and propose new diagnostic criteria. METHODS: We systematically searched four databases for all observational and case studies on the topic. We performed a narrative synthesis to propose diagnostic criteria and tested the criteria retrospectively on the included cases. A case report is also presented. RESULTS: Forty-two articles reporting 104 cases (median age 19 years, slightly male predominance) were included. All cases reported a nontraumatic hyperextension event (58% after surfing). All of the cases presented pain of hyperacute onset. The most frequent clinical feature was bladder or bowel dysfunction (84%). The thoracic region was the most frequently affected (87%) with longitudinal involvement until the conus (67%). At discharge or follow-up, 52% partially recovered. We propose five diagnostic criteria with three levels of certainty (definite, probable, and possible): (1) nontraumatic spine hyperextension activity (in individuals with no pre-existent spinal disease); (2) hyperacute onset (with acute pain onset); (3) spinal cord injury clinic (motor, sensory, or autonomic deficit); (4) MRI findings with central spinal cord abnormalities (multiple segments); and (5) no other alternative diagnosis. We identified 88% definite and 12% probable/possible cases. CONCLUSION: The acute hyperextension-induced myelopathy could occur not only during surfing but also during other activities. Therefore, increased awareness and education among sports communities and general physicians are needed.

Chronic meningitis as an initial presentation of Sjögren syndrome / La meningitis crónica como presentación inicial del síndrome de Sjögren

ABSTRACT Sjögren syndrome (SS) is an autoimmune disease, rarely affecting the central nervous system. The case is reported on a 68-year-old male patient who presented with keratocon-junctivitis sicca and xerostomia, during neuropsychological evaluation showed moderate to severe multidomain cognitive impairment. The magnetic resonance showed evidence of hypertrophic pachymeningitis. The CSF study showed pleocytosis, mild proteinuria, and negative cultures. The immune positive profile, Anti SS-A, SS-B and salivary gland biopsy was compatible with SS. The treatment was started with methylprednisolone pulses at a dose of 1g every 24 h for three days, and controlled with maintenance therapy, showing a favorable response. Chronic meningitis is a diagnostic challenge; among the less common causes is hypertrophic pachymeningitis in SS, and less frequently in male patients (1/9). Non-specific neurological manifestations should be considered, such as headaches or neuropsychiatric symptoms, as on some occasions rare cases may be encountered, such as the one described.

RESUMEN El síndrome de Sjögren (SS) es una enfermedad autoinmune que rara vez afecta al sistema nervioso central. En este informe de caso describimos a un paciente varón, de 68 años, que presentó queratoconjuntivitis seca y xerostomía, y a la evaluación neuropsicológica mostró deterioro cognitivo multidominio en grado de moderado a grave. La resonancia magnética mostró evidencia de paquimeningitis hipertrófica. El estudio del LCR mostró pleocitosis, proteinuria leve y cultivos negativos; el perfil inmunológico positivo anti SS-A, SS-B y la biopsia de la glándula salival compatible con SS. El tratamiento se inició con pulsos de metilprednisolona a una dosis de 1 g/cada 24 h/durante 3 días, control con terapia de mantenimiento, mostrando una respuesta favorable. La meningitis crónica es un desafío para el diagnóstico, entre las causas menos frecuentes está la paquimeningitis hipertrófica en el SS, y con menor frecuencia en los pacientes varones (1/9). Se deben considerar manifestaciones neurológicas inespecíficas, como dolores de cabeza o síntomas neuropsiquiátricos, ya que en algunas ocasiones podemos enfrentarnos a casos raros como el descrito.

Neurological diseases in Peru: a systematic analysis of the global burden disease study.

BACKGROUND: Disease burden indicators assess the impact of disease on a population. They integrate mortality and disability in a single indicator. This allows setting priorities for health services and focusing resources. OBJECTIVE: To analyze the burden of neurological diseases in Peru from 1990-2015. METHODS: A descriptive study that used the epidemiological data published by the Institute for Health Metrics and Evaluation of Global Burden of Diseases from 1990 to 2015. Disease burden was measured using disability-adjusted life years (DALY) and their corresponding 95% uncertainty intervals (UIs), which results from the addition of the years of life lost (YLL) and years lived with disability (YLD). RESULTS: The burden of neurological diseases in Peru were 9.06 and 10.65%, in 1990 and 2015, respectively. In 2015, the main causes were migraine, cerebrovascular disease (CVD), neonatal encephalopathy (NE), and Alzheimer's disease and other dementias (ADD). This last group and nervous system cancer (NSC) increased 157 and 183% of DALY compared to 1990, respectively. Young population (25 to 44 years old) and older (>85 years old) were the age groups with the highest DALY. The neurological diseases produced 11.06 and 10.02% of the national YLL (CVD as the leading cause) and YLD (migraine as the main cause), respectively. CONCLUSION: The burden of disease (BD) increased by 1.6% from 1990 to 2015. The main causes were migraine, CVD, and NE. ADD and NSC doubled the DALY in this period. These diseases represent a significant cause of disability attributable to the increase in the life expectancy of our population among other factors. Priority actions should be taken to prevent and treat these causes.

Neurological diseases in Peru: a systematic analysis of the global burden disease study / Enfermedades neurológicas en el Perú: un análisis sistemático del Global Burden Disease Study

ABSTRACT Background: Disease burden indicators assess the impact of disease on a population. They integrate mortality and disability in a single indicator. This allows setting priorities for health services and focusing resources. Objective: To analyze the burden of neurological diseases in Peru from 1990-2015. Methods: A descriptive study that used the epidemiological data published by the Institute for Health Metrics and Evaluation of Global Burden of Diseases from 1990 to 2015. Disease burden was measured using disability-adjusted life years (DALY) and their corresponding 95% uncertainty intervals (UIs), which results from the addition of the years of life lost (YLL) and years lived with disability (YLD). Results: The burden of neurological diseases in Peru were 9.06 and 10.65%, in 1990 and 2015, respectively. In 2015, the main causes were migraine, cerebrovascular disease (CVD), neonatal encephalopathy (NE), and Alzheimer's disease and other dementias (ADD). This last group and nervous system cancer (NSC) increased 157 and 183% of DALY compared to 1990, respectively. Young population (25 to 44 years old) and older (>85 years old) were the age groups with the highest DALY. The neurological diseases produced 11.06 and 10.02% of the national YLL (CVD as the leading cause) and YLD (migraine as the main cause), respectively. Conclusion: The burden of disease (BD) increased by 1.6% from 1990 to 2015. The main causes were migraine, CVD, and NE. ADD and NSC doubled the DALY in this period. These diseases represent a significant cause of disability attributable to the increase in the life expectancy of our population among other factors. Priority actions should be taken to prevent and treat these causes.

RESUMEN Introducción: Los indicadores de carga de la enfermedad evalúan el impacto que producen las enfermedades en una población. Integran la mortalidad y discapacidad en un solo indicador. Esto permite fijar prioridades para los servicio de salud y enfocar los recursos. Objetivo: Analizar los datos de carga de enfermedades neurológicas en Perú para el periodo 1990-2015. Métodos: Estudio descriptivo de datos del The Institute for Health Metrics and Evaluation de carga de enfermedad global desde el año 1990 al 2015. La medida de carga de la enfermedad utilizada fue los años de vida ajustados por discapacidad y sus correspondientes intervalos de incertidumbre (95%), que resultan de la suma de los años de vida perdidos debido a muerte prematura y los vividos con discapacidad. Resultados: La carga de enfermedades neurológicas en Perú fue de 9,06 y 10,65% en 1990 y 2015, respectivamente. En 2015, las principales causas fueron: migraña, enfermedad cerebrovascular, encefalopatía neonatal, enfermedad de Alzheimer y otras demencias. Este último grupo y el cáncer del sistema nervioso presentaron un incremento del 157 y 183% de los años de vida ajustados por discapacidad con respecto a 1990, respectivamente. Los adultos jóvenes (25 a 44 años) y adultos mayores (>85 años) fueron los grupos de edad con más años de vida ajustado por discapacidad. Las enfermedades neurológicas produjeron el 11.06 y el 10.02% de los años de vida perdidos debido a muerte prematura (enfermedad cerebrovascular como la causa principal) y años vividos con discapacidad (migraña como la causa principal), respectivamente. Conclusiones: La carga de la enfermedad aumentó un 1,6% entre 1990 y 2015. Las principales causas fueron migraña, la enfermedad cerebrovascular y la encefalopatía neonatal. La enfermedad de Alzheimer y otras demencias y cáncer del sistema nervioso duplicaron los años de vida ajustados por discapacidad en este período. Estas enfermedades representan una causa importante de discapacidad atribuible al aumento de la esperanza de vida de nuestra población, entre otros factores. Se deben tomar medidas prioritarias para prevenir y tratar estas causas.

Neuro-Behçet's disease in Peru: a case report and literature review. / Enfermedad de neuro-Behçet en Perú: reporte de caso y revisión de la literatura.

Behçet's disease is a vasculitis that can cause inflammatory lesions in multiple organs or systems including the nervous system. Most cases worldwide have been reported along what is called the "Silk Route" from the Mediterranean region to Japan, so it is considered a rare disease in Latin American countries. The frequency of neurological involvement ranges from 5 to 13%. We present the case of a young adult woman with diagnostic criteria for Behçet's disease and manifestations of neurological disease, as well as a review of the literature.

La enfermedad de Behçet es una vasculitis que puede ocasionar lesiones inflamatorias en múltiples órganos o sistemas como el neurológico. El mayor número de casos a nivel mundial se han reportado a lo largo de la llamada Ruta de la Seda, que va desde la región mediterránea hasta Japón, siendo considerado una enfermedad rara en países latinoamericanos. La frecuencia de afectación neurológica oscila en un rango entre 5 y 13%. Se presenta el caso de una mujer adulta joven con criterios diagnósticos de enfermedad de Behçet y manifestaciones de afectación neurológica, así como una revisión de la literatura en Perú.