Short-segment transverse myelitis lesions in a cohort of Latin American patients with neuromyelitis optica spectrum disorders.

STUDY DESIGN: Multicenter retrospective study. OBJECTIVES: The aim was to determine the frequency and magnetic resonance imaging (MRI) features of short-segment transverse myelitis (STM) in patients with neuromyelitis optica spectrum disorders (NMOSD) during a myelitis attack. SETTING: Latin American diagnostic centres (Neuroimmunology Unit). A multicenter study from Argentina, Brazil and Venezuela was performed. METHODS: Seventy-six patients with NMOSD were included. We analyzed 346 attacks and reviewed spinal cord MRIs performed within 30 days from spinal attack onset. Sagittal and axial characteristics on cervical and thoracic MRI (1.5 tesla) were observed. Demographics, clinical, serological, and disability data were collected. RESULTS: Among the 76 patients with NMOSD, isolated STM was observed in 8% (n = 6), multisegmental lesions (longitudinally extensive transverse myelitis (LETM) + STM) in 28% (n = 21; 13 had at least one STM), LETM in 42% (n = 32), and normal spinal MRI in 22% (n = 17). However, isolated STM was increased by 10% in patients with NMOSD with spinal lesions (6 out of 59) with mean attacks of 2.5 (±0.83) and last follow-up expanded disability status scale (EDSS) of 3.1 (±2.63). Positive aquaporin 4 antibodies (AQP4-ab) were found in 50%. Upper-cervical lesion was most frequently observed (5 out of 6). Myelitis was preceded by ON in all isolated patients with STM. Only one had a positive gadolinium lesion and none of these had asymptomatic spinal cord lesion. CONCLUSION: Isolated STM does not exclude NMOSD diagnosis. Therefore, APQ4-ab testing could be useful during a myelitis attack with STM.

Status of the neuromyelitis optica spectrum disorder in Latin America.

BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) is an increasing diagnostic and therapeutic challenge in Latin America (LATAM). Despite the heterogeneity of this population, ethnic and socioeconomic commonalities exist, and epidemiologic studies from the region have had a limited geographic and population outreach. Identification of some aspects from the entire region are lacking. OBJECTIVES: To determine ethnic, clinical characteristics, and utilization of diagnostic tools and types of therapy for patients with NMOSD in the entire Latin American region. METHODS: The Latin American Committee for Treatment and Research in MS (LACTRIMS) created an exploratory investigational survey addressed by Invitation to NMOSD Latin American experts identified through diverse sources. Data input closed after 30 days from the initial invitation. The questionnaire allowed use of absolute numbers or percentages. Multiple option responses covering 25 themes included definition of type of practice; number of NMOSD cases; ethnicity; utilization of the 2015 International Panel criteria for the diagnosis of Neuromyelitis optica (IPDN); clinical phenotypes; methodology utilized for determination of anti-Aquaporin-4 (anti- AQP4) antibodies serological testing, and if this was performed locally or processed abroad; treatment of relapses, and long-term management were surveyed. RESULTS: We identified 62 investigators from 21 countries reporting information from 2154 patients (utilizing the IPDN criteria in 93.9% of cases), which were categorized in two geographical regions: North-Central, including the Caribbean (NCC), and South America (SA). Ethnic identification disclosed Mestizos 61.4% as the main group. The most common presenting symptoms were concomitant presence of optic neuritis and transverse myelitis in 31.8% (p=0.95); only optic neuritis in 31.4% (more common in SA), p<0.001); involvement of the area postrema occurred in 21.5% and brain stem in 8.3%, both were more frequent in the South American cases (p<0.001). Anti-AQP4 antibodies were positive in 63.9% and anti-Myelin Oligodendrocyte Glycoprotein (MOG) antibodies in 4.8% of total cases. The specific laboratorial method employed was not known by 23.8% of the investigators. Acute relapses were identified in 81.6% of cases, and were treated in 93.9% of them with intravenous steroids (IVS); 62.1% with plasma exchange (PE), and 40.9% with intravenous immunoglobulin-G (IVIG). Therapy was escalated in some cases due to suboptimal initial response. Respondents favored Rituximab as long-term therapy (86.3%), whereas azathioprine was also utilized on 81.8% of the cases, either agent used indistinctly by the investigators according to treatment accessibility or clinical judgement. There were no differences among the geographic regions. CONCLUSIONS: This is the first study including all countries of LATAM and the largest cohort reported from a multinational specific world area. Ethnic distributions and phenotypic features of the disease in the region, challenges in access to diagnostic tools and therapy were identified. The Latin American neurological community should play a determinant role encouraging and advising local institutions and health officials in the availability of more sensitive and modern diagnostic methodology, in facilitating the the access to licensed medications for NMOSD, and addressing concerns on education, diagnosis and management of the disease in the community.

Frequency of brain MRI abnormalities in neuromyelitis optica spectrum disorder at presentation: A cohort of Latin American patients.

BACKGROUND: Brain magnetic resonance imaging (BMRI) lesions were classically not reported in neuromyelitis optica (NMO). However, BMRI lesions are not uncommon in NMO spectrum disorder (NMOSD) patients. OBJECTIVE: To report BMRI characteristic abnormalities (location and configuration) in NMOSD patients at presentation. METHODS: Medical records and BMRI characteristics of 79 patients with NMOSD (during the first documented attack) in Argentina, Brazil and Venezuela were reviewed retrospectively. RESULTS: BMRI abnormalities were observed in 81.02% of NMOSD patients at presentation. Forty-two patients (53.1%) showed typical-NMOSD abnormalities. We found BMRI abnormalities at presentation in the brainstem/cerebellum (n = 26; 32.9%), optic chiasm (n = 16; 20.2%), area postrema (n = 13; 16.4%), thalamus/hypothalamus (n = 11; 13.9%), corpus callosum (n = 11; 13.9%), periependymal-third ventricle (n = 9; 11.3%), corticospinal tract (n = 7; 8.8%), hemispheric white matter (n = 1; 1.2%) and nonspecific areas (n = 49; 62.03%). Asymptomatic BMRI lesions were more common. The frequency of brain MRI abnormalities did not differ between patients who were positive and negative for aquaporin 4 antibodies at presentation. CONCLUSION: Typical brain MRI abnormalities are frequent in NMOSD at disease onset.

Management of relapsing-remitting multiple sclerosis in Latin America: practical recommendations for treatment optimization.

The Latin American MS Experts' Forum has developed practical recommendations on the initiation and optimization of disease-modifying therapies in patients with relapsing-remitting multiple sclerosis (RRMS). The recommendations reflect the unique epidemiology of MS and the clinical practice environment in Latin American countries. Treatment response may be evaluated according to changes in relapses; progression, as assessed by the Expanded Disability Status Scale and the Timed 25-foot Walk; and lesion number on magnetic resonance imaging. Follow-up assessments are recommended every six months, or annually for stable patients. Cognitive function should be evaluated in all RRMS patients at baseline and annually thereafter. These recommendations are intended to assist clinicians in Latin America in developing a rational approach to treatment selection and sequencing for their RRMS patients.

Pediatric multiple sclerosis in Venezuela.

OBJECTIVE: To describe the epidemiological and clinical characteristics of Venezuelan pediatric patients with multiple sclerosis (MS). METHODS: Database records from the National Program for MS were searched for patients with an established diagnosis of MS whose first symptoms appeared before age 18. RESULTS: The national database held records of 1.710 patients; 3.8% had onset of the first symptoms before age 18. 46.7% were boys, yielding an F:M ratio of 1.13:1. Many children had a disease onset characterized by motor impairment (30.7%), brainstem/cerebellum and spinal cord affectation (27.6%), headache (26%). Less frequent symptoms were sensory symptoms (8%) and optic neuritis (7%). DISCUSSION: Pediatric MS patients in Venezuela represent a significant proportion of all MS cases. The clinical pattern is characterized by motor symptoms at onset, and predominantly monosymptomatic presentation with a relapsing-remitting pattern. This is the first systematic attempt to estimate the prevalence of pediatric MS in Venezuela.

Pediatric multiple sclerosis in Venezuela / Esclerosis múltiple pediátrica en Venezuela

OBJECTIVE: To describe the epidemiological and clinical characteristics of Venezuelan pediatric patients with multiple sclerosis (MS). METHODS: Database records from the National Program for MS were searched for patients with an established diagnosis of MS whose first symptoms appeared before age 18. RESULTS: The national database held records of 1.710 patients; 3.8% had onset of the first symptoms before age 18. 46.7% were boys, yielding an F:M ratio of 1.13:1. Many children had a disease onset characterized by motor impairment (30.7%), brainstem/cerebellum and spinal cord affectation (27.6%), headache (26%). Less frequent symptoms were sensory symptoms (8%) and optic neuritis (7%). DISCUSSION: Pediatric MS patients in Venezuela represent a significant proportion of all MS cases. The clinical pattern is characterized by motor symptoms at onset, and predominantly monosymptomatic presentation with a relapsing-remitting pattern. This is the first systematic attempt to estimate the prevalence of pediatric MS in Venezuela.

OBJETIVO: Describir las características clínicas y epidemiológicas de pacientes pediátricos venezolanos con EM. MÉTODOS: Se revisó la base de datos del Programa Nacional para EM, identificando pacientes con diagnóstico establecido de EM antes de los 18 años de edad. RESULTADOS: La base de datos registró 1.710 pacientes y 3,8% tenía una edad de aparición menor de 18 años. De estes, 46,7% eran varones, dando una relación F:M de 1.13:1. Muchos niños tuvieron una aparición de la enfermedad caracterizada por déficit motor (30,7%), afectación del tronco encefálico-cerebelo y la médula espinal (27,6%), dolor de cabeza (26%). Síntomas menos frecuentes fueron sensoriales (8%) y neuritis óptica (7%). DISCUSIÓN: Los pacientes pediátricos venezolanos con MS representan una proporción significativa de todos los casos de MS. El patrón clínico está caracterizado por síntomas motores y presentación predominantemente monosintomática con patrón brote-remisión. Este es el primer intento sistemático para estimar la prevalencia de MS pediátrica en Venezuela.

Consenso venezolano para el uso de la resonancia magnética en el diagnóstico y seguimiento de pacientes con esclerosis múltiple / Venezuelan consensus guidelines on the use of magnetic resonance in diagnosis and follow up of patients with multiple sclerosis

Introducción. El uso de la resonancia magnética (RM) en el diagnóstico y seguimiento de pacientes con esclerosis múltiple (EM) se ha incrementado considerablemente durante los últimos años. Diversos grupos de trabajo internacionales han intentado clarificar y normativizar el uso de la RM tanto en el momento del diagnóstico como durante el seguimiento de los pacientes. Sin embargo, en muchas ocasiones se extrapolan datos de otras regiones que no contemplan la realidad de cada lugar o son difíciles de implementar. Objetivo. Elaborar un consenso venezolano para el uso de la RM en el diagnóstico y seguimiento de pacientes con EM. Desarrollo. Un grupo de expertos de Venezuela, conformado por neurólogos y radiólogos, mediante metodología de ronda de encuestas a distancia y reuniones presenciales, llevó adelante la elaboración del consenso pretendido para el uso de la RM en el diagnóstico y seguimiento de pacientes con EM en Venezuela. Se establecieron 17 recomendaciones basadas en la evidencia publicada y en el criterio de los expertos que participaron. Las recomendaciones se enfocaron en el papel de las técnicas convencionales de RM, así como en el de la medición de la atrofia cerebral en pacientes con EM, tanto en el momento del diagnóstico como durante el seguimiento. Conclusión. Las recomendaciones establecidas en el presente consenso permitirán optimizar el cuidado y el seguimiento de los pacientes con EM en Venezuela (AU)

Introduction. The clinical use of magnetic resonance (MR) in patients with multiple sclerosis (MS) has advanced markedly over the past few years. Several groups around the world have developed consensus guidelines about the role of MR in MS at diagnosis and during follow up. However, in some regions is difficult to extrapolate the recommendations. Aim. To provide recommendations for the implementation of MR in MS patients at diagnosis and follow up in Venezuela. Development. A group of experts from Venezuela that included neurologists and radiologists, by using the online surveys methodology as well as face to face meetings developed the intended consensus for the use of MR during the diagnosis and follow up of MS patients in Venezuela. Seventeen recommendations were established based on published evidence and the expert opinion. Recommendations focused on the role of conventional MR techniques and brain atrophy measurement in MS patients both at diagnosis and during follow-up. Conclusions. The recommendations of this consensus guidelines attempts to optimize the health care and management of patients with MS in Venezuela (AU)

Prevalence and clinical characteristics of postictal psychiatric symptoms in partial epilepsy.

OBJECTIVE: S: To identify the prevalence and clinical characteristics of postictal psychiatric (PPS) and cognitive (PCS) symptoms in patients with refractory partial epilepsy and to investigate whether interictal psychiatric and cognitive symptoms worsened in severity during the postictal period. METHODS: Using a 42-item questionnaire, the authors determined the prevalence and clinical characteristics of PPS and PCS that occurred after >50% of seizures in 100 of 114 consecutive patients with refractory partial epilepsy during a 3-month period. The postictal period was defined as the 72 hours that followed a seizure. The prevalence of all interictal psychiatric and cognitive symptoms was identified and the frequency with which they worsened postictally determined. RESULTS: A mean of 2.8 +/- 1.8 PCS (median = 3) and 5.9 +/- 5.3 PPS (median = 5) was identified, which included postictal symptoms of depression (PSD) in 43 patients, anxiety (PSA) in 45, postictal psychotic symptoms (PIP) in 7, hypomanic symptoms in 22, neurovegetative symptoms in 52, and fatigue in 37. Most patients experienced more than one type of PPS. Independently of the occurrence of PPS, 38 patients reported a worsening of interictal psychiatric and cognitive symptoms postictally. A history of depression and anxiety significantly increased the number of PSD, PSA, and PIP. CONCLUSIONS: Postictal psychiatric symptoms are common among patients with refractory partial epilepsy, and the severity of interictal psychiatric and cognitive symptoms commonly worsens during the postictal period.

The localizing yield of sphenoidal and anterior temporal electrodes in ictal recordings: a comparison study.

PURPOSE: To compare the localizing yield of sphenoidal electrodes placed under fluoroscopic guidance (SEs) and anterior temporal electrodes (ATEs) in ictal recordings from a group of patients with seizure disorders of anterior temporal origin. METHODS: We compared ictal recordings of 156 seizures obtained with SEs and ATEs from 40 consecutive patients with seizures of anterior temporal origin. Four electroencephalographers reviewed ictal recordings independently and blind to the patients' identity, presurgical data, and inclusion of ATEs or SEs. Outcome variables included (a) number of correctly localized seizures with SE and ATE recordings by at least three raters; (b) number of ictal foci in which all seizures were localized only with SEs; and (c) number of seizures in which SEs identified the ictal onset > or =5 s earlier than ATEs. RESULTS: Interrater agreement among the four raters was significantly greater with SE than with ATE recordings (p < 0.0001). The number of seizures correctly localized was significantly greater with SEs (n = 144) than with ATEs (n = 99; p < 0.0001). All the seizures [n = 36 (23%)] originating from 14 ictal foci (29%) in 11 patients (27.5%) were localized only with SEs. Finally, the ictal onset was detected at SEs > or =5 s earlier than at ATEs in 67 (43%) seizures originating from 33 (69%) foci in 30 (75%) patients. CONCLUSIONS: SEs improve interrater agreement in the localization of seizures of anterior temporal origin, and in about one fourth of patients, SEs add ictal data not identified by ATEs.

Pseudotumor cerebri / Cerebri pseudotumor

El pseudotumor cerebri (Hipertensión intracraneal ideopática) es una entidad patológica de etiología no bien precisada, caracterizada por un aumento de la presión intracraneana, asociada a síntomas y signos como cefalea, tinnitus, obscurecimientos transitorios, diplopia pero sin hallazgos neurológicos focalizados, ni debe encontrarse evidencias de obstrucción o deformidad del sistema ventricular y los estudios neurodiagnósticos son normales, exceptuando la elevación de la presión intracraneal. Se revisa la estadística de la Unidad de Neuroftalmología del Hospital Domingo Luciani del I.V.S.S., Caracas, desde agosto de 1992 hasta mayo de 1995, encontrándose 10 casos de Pseudotumor Cerebri (P.C), estableciéndose el diagnóstico de acuerdo a los criterios modificados de Dandy. De los 10 casos evaluados, el 80 por ciento correspondieron a la tercera y cuarta década de la vida. Igualmente un 70 por ciento correspondió al sexo femenino. Las manifestaciones clínicas más frecuentemente encontradas fueron cefalea y diplopia en un 90 y 80 por ciento de los casos. la obesidad constituyó la patología sistémica más observada. El tratamiento recibido fue a base de dieta y diuréticos en el 100 por ciento de los casos. La evolución fue a mejoría en un 80 por ciento de casos y estacionario en el 20 por ciento restante. Ningún caso requirió tratamiento quirúrgico

Neuritis óptica / Optics neuritis

La neuritis óptica primaria es una enfermedad caracterizada por inflamación y desmielinización del nervio óptico usualmente de origen temporal, que cursa con disminución de la agudeza visual (A/V), en adultos jóvenes menores de 45 años y rara vez en niños. Se evaluaron 12 pacientes de la consulta neuroftalmología del Hospital Dr. Domingo Luciani entre agosto de 1992 hasta mayo de 1995, encontrándose 12 pacientes que cumplían con la clínica de neuritis óptica. Se les realizó examen clínico oftalmológico y neurológico, además hematología VSG, química sanguínea, VDRL, HIV, TAC, RM, potenciales visuales evocados. LCR (citoquímico, inmunología). El tratamiento médico fue a base de esteroides y 2 casos no recibieron esteroides. Se clasificó el material en edad, sexo, síntomas y signos más frecuentes, formas clínicas de presentación, tratamiento recibido, agudeza visual de ingreso, recuperación de la agudeza visual y evolución a esclerósis múltiple definitiva

Culinary practices and consumption characteristics of common beans at the rural home level

Con el propósito de recolectar información sobre la selección de procedimientos de cocción, prácticas culinarias y consumo de frijol en los hogares de las áreas rurales localizadas en cuatro departamentos del noreste de Guatemala, se llevó a cabo una encuesta en las amas de casa de las áreas rurales productoras de frijol. La muestra del estudio incluyó 10 familias, seleccionadas al azar, de cada una de 18 comunidades. El cuestionario que se utilizó había sido evaluado previamente en dos comunidades rurales no incluidas en el estudio. Según se ha demostrado anteriormente a través de otras encuestas de esta naturaleza llevadas a cabo por el INCAP, se encontró que todas las familias consumían frijoles negros y, con raras excepciones, frijoles de otro color o se remojan los granos antes de cocinar y que son pocas las amas de casa que agregan sal al principio de la cocción. Por lo general, se obtienen dos fracciones: los frijoles cocidos y el caldo de cocción. Otro hallazgo fue que los frijoes se consumen en cuatro diferentes formas: cocidos enteros, enteros y fritos, colados y colados y fritos (volteados). Los adultos y los niños consumen el caldo de frijos, por lo que prefieren un empienzan a ingerir el caldo desde los dos meses de edad, y los frijoles a los 14 meses. La cantidad promedio de caldo consumido fue de 62 g y de frijoles cocidos, 70 g. Las madres entrevistadas indicaron que administraban caldo a sus hijos porque: era nutritivo (56% de las madres); para enseñarle a sus hijos a...

Enfermedad de Creutzfeldt-Jakob en Venezuela: informe de 10 casos y revisión de la literatura / The Creutzfeldt-Jakob disease in Venezuela: report of 10 cases and revition of the literature

La enfermedad de Creutzfeldt-Jakob (ECJ) es una encefalopatía espongiforme transmisible infrecuente y de amplia distribución mundial. Su incidencia en Venezuela es desconocida y sólo 5 casos previos han sido publicados. Se presentan en este informe 10 casos estudiados en los últimos 27 años. La confirmación del diagnóstico fue realizada mediante el estudio histológico y estructural de muestras del cerebro y/o cerebelo, obtenidas por biopsia en 3 casos y por autopsia en 7. El promedio de edad fue de 60,1 años. El intervalo entre el inicio de los síntomas y muerte fue de 4,5 meses. Todos los pacientes presentaron un síndrome demencial rápidamente progresivo asociado a signos piramidales y extrapiramidales, mioclonos y cambios en el electroencefalograma. El examen histológico reveló en todos los casos degeneración espongiosa, astrogliosis y pérdida neuronal en la sustancia gris de la corteza cerebral, núcleos basales y cerebelo. Desde el punto de vista estructural, las áreas afectadas en 4 casos estudiados mostraron vacuolización extensa del neurópilo, degeneración neuronal y prolongaciones astrogliales con numerosos filamentos intermedios. Ningún caso tenía antecedente familiar ni causa iatrogénica probable de la enfermedad. Se plantea la posibilidad de un subregistro de la ECJ en Venezuela y la importancia epidemiológica que tiene el conocimiento de esta entidad clínico-patológica en el diagnóstico diferencial de los diferentes tipos de trastornos demenciales