Clinical and radiological profile of neuromyelitis optica spectrum disorders in an Ecuadorian cohort.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is a complex disease characterized by a severe inflammation of the central nervous system (CNS). This disease typically manifests with recurrent optic neuritis (ON) and acute transverse myelitis (ATM). The clinical and radiological spectrum of NMOSD is little known in Latin America (LATAM) and few reports have been published in the literature so far. In Ecuador, no reports on NMOSD have been published. For this reason we aimed to assess the demographic, clinical and imaging characteristics of patients with NMOSD from third level hospitals from Ecuador. METHODS: This is a descriptive study in which we assessed medical reports of patients with inflammatory demyelinating diseases who were attended in third level hospitals from Ecuador in 2017. Then we applied the 2015 diagnostic criteria, those patients who met the new NMOSD diagnostic criteria were selected and analyzed. Additionally, exploratory sub-analyses were subsequently carried out. RESULTS: We identified 59 patients with NMOSD, the relative frequency of NMOSD was 15.9%. The multiple sclerosis (MS) /NMOSD ratio was 5.2:1. Twenty four percent of patients were newly defined as having NMOSD when 2015 criteria was applied. The median time to diagnoses was shorter by the 2015 criteria than 2006 criteria (p<0.001). NMOSD was more prevalent in women (female/male ratio 4.4:1). The disease onset was more frequent at the fourth decade of life. The most common symptoms at the disease onset were ON and the association of ON with ATM. The mean of expanded disability status scale (EDSS) was 4.8 (SD±1.8). Concomitant autoimmune diseases were infrequent in this population (11.9%). The brain magnetic resonance imaging (MRI) abnormalities were present in 25.7% of patients at disease onset. Spinal cord MRI showed longitudinally extensive transverse myelitis (LETM) in 91.5% of cases. Recurrent NMOSD was frequent in this cohort (88%). Positivity for antibodies against aquaporin-4 (AQP4-IgG) which was measured through indirect immunofluorescence assay (IIF) was identified in 81% of the patients tested. Patients with seronegative AQP4-IgG had higher grade of disability than seropositive patients (p<0.05). Ninety eight percent of patients received treatment with immunosuppressive drugs. Three patients died due to gastric cancer (1 patient) and infectious diseases (2 patients). CONCLUSIONS: This is the first descriptive study in an Ecuadorian cohort of patients with NMOSD. We show a wide epidemiological, clinical and radiological spectrum of NMOSD.

Prevalence of multiple sclerosis in Cuenca, Ecuador.

BACKGROUND: Cuenca, a city in the Andean Region of southern Ecuador, has 591,996 inhabitants. A decade-old study showed the prevalence of multiple sclerosis in Cuenca was 0.75 cases per 100,000 inhabitants but no new epidemiological studies in this city have been performed since then. The aim of this study, conducted in 2016, was to update the prevalence records of multiple sclerosis in Cuenca. METHODS: We performed a descriptive cross-sectional study in which we investigated prevalence rates in November of 2016. We estimated the prevalence of multiple sclerosis by cross-matching registries from the two neurological referral hospitals in Cuenca. RESULTS: A total of 23 records were obtained from the two sources. The estimated prevalence was 3.88 per 100,000 inhabitants (95% confidence interval: 3.83-3.94). The disease was predominant among women (60%). The mean age of this cohort was 37 years (standard deviation ±12.4). Of the cases, 78% were relapsing-remitting multiple sclerosis. The mean Expanded Disability Status Scale score was 2.5. CONCLUSIONS: This study is an update to the first study conducted 10 years ago and shows the prevalence of multiple sclerosis in Cuenca has increased. However, the prevalence of multiple sclerosis is still low and very similar to that reported in neighbouring countries.

Validity of a PCR assay in CSF for the diagnosis of neurocysticercosis.

OBJECTIVE: To prospectively evaluate the validity of a PCR assay in CSF for the diagnosis of neurocysticercosis (NC). METHODS: We conducted a multicenter, prospective case-control study, recruiting participants from 5 hospitals in Cuenca, Ecuador, from January 2015 to February 2016. Cases fulfilled validated diagnostic criteria for NC. For each case, a neurosurgical patient who did not fulfill the diagnostic criteria for NC was selected as a control. CT and MRI, as well as a CSF sample, were collected from both cases and controls. The diagnostic criteria to identify cases were used as a reference standard. RESULTS: Overall, 36 case and 36 control participants were enrolled. PCR had a sensitivity of 72.2% (95% confidence interval [CI] 54.8%-85.8%) and a specificity of 100.0% (95% CI 90.3%-100.0%). For parenchymal NC, PCR had a sensitivity of 42.9% (95% CI 17.7%-71.1%), and for extraparenchymal NC, PCR had a sensitivity of 90.9% (95% CI 70.8%-98.9%). CONCLUSIONS: This study demonstrated the usefulness of this PCR assay in CSF for the diagnosis of NC. PCR may be particularly helpful for diagnosing extraparenchymal NC when neuroimaging techniques have failed. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that CSF PCR can accurately identify patients with extraparenchymal NC.

[Treatment with vitamin D and slowing of progression to severe stage of Alzheimer's disease]. / Tratamiento con vitamina D y enlentecimiento de la progresión a estadio severo en enfermedad de Alzheimer.

The aim of the study was to analyze the impact of treatment with vitamin D in the progression of Alzheimer's disease. We performed a retrospective study including patients with mild stage of Alzheimer's disease with more than four years of follow-up. The presence of cardiovascular risk factors, osteoporosis, treatment with memantine, acetylcholinesterase inhibitors drugs and vitamin D were analyzed as independent variables. Time of progression to moderate and severe Alzheimer's disease was analyzed as dependent variable. The analysis was done using multivariate linear regression model, Kaplan Meier analysis, Chi-square and T test. Two hundred and two patients met the inclusion criteria. 11% of the patients (n = 23) remained in the mild stage of the disease, 54% (n = 110) developed the moderate form in a mean time of 3 ± 1.4 years while 35% (n = 69) developed the severe form in a mean time of 4.6 ± 1.4 years. Time of progression to severe stage of Alzheimer's disease was slower in patients under treatment with vitamin D compared with those without treatment (5.4 ± 0.4 years vs. 4.4 ± 0.16 years respectively, p=0.003). Treatment with vitamin D may be an independent protecting factor in the progression of Alzheimer's disease.

Tratamiento con vitamina D y enlentecimiento de la progresión a estadio severo en enfermedad de Alzheimer. / [Treatment with vitamin D and slowing of progression to severe stage of Alzheimers disease].

The aim of the study was to analyze the impact of treatment with vitamin D in the progression of Alzheimers disease. We performed a retrospective study including patients with mild stage of Alzheimers disease with more than four years of follow-up. The presence of cardiovascular risk factors, osteoporosis, treatment with memantine, acetylcholinesterase inhibitors drugs and vitamin D were analyzed as independent variables. Time of progression to moderate and severe Alzheimers disease was analyzed as dependent variable. The analysis was done using multivariate linear regression model, Kaplan Meier analysis, Chi-square and T test. Two hundred and two patients met the inclusion criteria. 11

of the patients (n = 23) remained in the mild stage of the disease, 54

(n = 110) developed the moderate form in a mean time of 3 ± 1.4 years while 35

(n = 69) developed the severe form in a mean time of 4.6 ± 1.4 years. Time of progression to severe stage of Alzheimers disease was slower in patients under treatment with vitamin D compared with those without treatment (5.4 ± 0.4 years vs. 4.4 ± 0.16 years respectively, p=0.003). Treatment with vitamin D may be an independent protecting factor in the progression of Alzheimers disease.

Tratamiento con vitamina D y enlentecimiento de la progresión a estadio severo en enfermedad de Alzheimer / [Treatment with vitamin D and slowing of progression to severe stage of Alzheimer’s disease].

The aim of the study was to analyze the impact of treatment with vitamin D in the progression of Alzheimer’s disease. We performed a retrospective study including patients with mild stage of Alzheimer’s disease with more than four years of follow-up. The presence of cardiovascular risk factors, osteoporosis, treatment with memantine, acetylcholinesterase inhibitors drugs and vitamin D were analyzed as independent variables. Time of progression to moderate and severe Alzheimer’s disease was analyzed as dependent variable. The analysis was done using multivariate linear regression model, Kaplan Meier analysis, Chi-square and T test. Two hundred and two patients met the inclusion criteria. 11

of the patients (n = 23) remained in the mild stage of the disease, 54

(n = 110) developed the moderate form in a mean time of 3 ± 1.4 years while 35

(n = 69) developed the severe form in a mean time of 4.6 ± 1.4 years. Time of progression to severe stage of Alzheimer’s disease was slower in patients under treatment with vitamin D compared with those without treatment (5.4 ± 0.4 years vs. 4.4 ± 0.16 years respectively, p=0.003). Treatment with vitamin D may be an independent protecting factor in the progression of Alzheimer’s disease.

[Validation of a questionnaire for epilepsy diagnosis in primary care services]. / Validación de un cuestionario para el diagnóstico de la epilepsia en servicios de atención primaria.

OBJECTIVE: To evaluate the usefulness of a questionnaire in primary health care services for establishing the diagnosis of epilepsy in children from 1 to 10 years old and for classifying their epileptic seizures. METHODS: This cross-sectional observational study was conducted in 2004. There were 204 children in the study (102 diagnosed with epilepsy and 102 without epilepsy). The children were randomly selected at the Regional Center of Epilepsies and the Vincent Corral Moscoso Hospital, which are both in the city of Cuenca, Ecuador. For the study, each child with epilepsy was matched with a child without epilepsy, in two age groups: 1 to 5 years old and 6 to 10 years old. Four students who were in their last academic year in the School of Medicine of Cuenca and who did not know the child's diagnosis utilized the questionnaire with a family member or other guardian of the child. The validity, sensitivity, specificity, positive predictive value, and negative predictive value of the questionnaire were calculated, with 95% confidence intervals (95% CIs). The clinical diagnosis carried out by specialists was used as the gold standard. RESULTS: The assessment showed that the questionnaire had good sensitivity (95.10%; 95% CI: 94.58%-95.61%) and good specificity (97.06%; 95% CI: 96.58%-97.59%). Validity was 96.08% (95% CI: 95.84%-96.36%), with a positive predictive value of 97.00% (95% CI: 96.48%-97.52%) and a negative predictive value of 95.19% (95% CI: 94.74%-95.74%). The level of agreement in the classification of the epileptic seizures carried out by the neurologists and by the medical students who used the questionnaire was satisfactory for the generalized seizures (kappa = 0.67). Upon testing for interobserver agreement among the specialists, the kappa value for the diagnoses was 0.80 among the neurologists and 0.89 among the pediatricians. CONCLUSIONS: The diagnostic questionnaire that was assessed has good sensitivity and adequate specificity, and, after brief training, primary health care general practitioners can use it to help them diagnose epileptic seizures.

Validación de un cuestionario para el diagnóstico de la epilepsia en servicios de atención primaria / Validation of a questionnaire for epilepsy diagnosis in primary care services

OBJETIVOS: Evaluar la utilidad de un cuestionario para establecer el diagnóstico de epilepsia en niños de 1 a 10 años de edad y clasificar las crisis epilépticas en los servicios de atención primaria. MÉTODOS: Estudio observacional transversal; participaron 204 niños de 1 a 10 años de edad (102 con diagnóstico de epilepsia y 102 sin epilepsia) escogidos al azar en el Centro Regional de Epilepsias y en el Hospital Vicente Corral Moscoso, ambos de la ciudad de Cuenca, Ecuador. Los niños fueron pareados por grupos de edad (de 1 a 5 y de 6 a 10 años). Cuatro estudiantes del último año de la Escuela de Medicina de esa ciudad que desconocían el diagnóstico del niño aplicaron el cuestionario a un familiar del niño o a su representante. Se evaluó el grado de discriminación diagnóstica (validez) del cuestionario, la sensibilidad, la especificidad y el valor diagnóstico de un resultado positivo o negativo, con un intervalo de confianza de 95 por ciento (IC95 por ciento). Como criterio de referencia se utilizó el diagnóstico clínico emitido por especialistas. RESULTADOS: La evaluación realizada demostró que el cuestionario de diagnóstico estudiado tiene buena sensibilidad (95,10 por ciento; IC95 por ciento: 94,58 a 95,61) y especificidad (97,06 por ciento; IC95 por ciento: 96,58 a 97,59). El índice de validez fue de 96,08 (IC95 por ciento: 95,84 a 96,36), con un valor pronóstico de un resultado positivo de 97,00 por ciento (IC95 por ciento: 96,48 a 97,52) y un valor pronóstico de un resultado negativo de 95,19 por ciento (IC95 por ciento: 94,74 a 95,74). El grado de concordancia de la clasificación de las crisis epilépticas realizadas por los neurólogos y los estudiantes de medicina que utilizaron el cuestionario fue satisfactorio para las crisis generalizadas (índice k: 0,67). Según la prueba de reproducibilidad de resultados entre observadores, el índice k para el diagnóstico de los neurólogos fue de 0,80 y para el diagnóstico de los pediatras de 0,89. CONCLUSIONES: El cuestionario de diagnóstico evaluado posee una buena sensibilidad y una adecuada especificidad y puede ayudar a los médicos generales, después de un breve entrenamiento, a diagnosticar las crisis epilépticas en los servicios de atención primaria.