Multiple sclerosis in Central America and Caribbean countries: frequency and clinical characterization of an emergent disease.

Background: Multiple Sclerosis (MS) is a common neurological disease among white populations of European origin. Frequencies among Latin Americans continue to be studied, however, epidemiologic, and clinical characterization studies lack from Central American and Caribbean countries. Ethnicity in these countries is uniformly similar with a prevalent Mestizo population. Methods and results: Data from January 2014 to December 2019 from Guatemala, El Salvador, Honduras, Nicaragua, Costa Rica, Panama, Dominican Republic, and Aruba on demographic, clinical, MRI and phenotypic traits were determined in coordinated studies: ENHANCE, a population-based, retrospective, observational study on incidence and clinical characteristics, and from the subgroup with MS national registries (Aruba, Dominican Republic, Honduras, and Panama), data on prevalence, phenotypes and demographics. Expanded Disability Status Scale (EDSS), and therapeutic schemes were included. ENHANCE data from 758 patients disclosed 79.8% of Mestizo ethnicity; 72.4% female; median age at onset 31.0 years and 33.2 at diagnosis. The highest incidence rate was from Aruba, 2.3-3.5 × 100,000 inhabitants, and the lowest, 0.07-0.15 × 100,000, from Honduras. Crude prevalence rates per 100,000 inhabitants fluctuated from 27.3 (Aruba) to 1.0 (Honduras). Relapsing MS accounted for 87.4% of cases; EDSS <3.0 determined in 66.6% (mean disease duration: 9.1 years, SD ± 5.0); CSF oligoclonal bands 85.7%, and 87% of subjects hydroxyvitamin D deficient. Common initial therapies were interferon and fingolimod. Switching from interferon to fingolimod was the most common escalation step. The COVID-19 pandemic affected follow-up aspects of these studies. Conclusion: This is the first study providing data on frequencies and clinical characteristics from 8 countries from the Central American and Caribbean region, addressing MS as an emergent epidemiologic disorder. More studies from these areas are encouraged.

Neuromyelitis Optica Spectrum Disorder in Central America and the Caribbean: A Multinational Clinical Characterization Study.

Here, a study of NMOSD in Central America and the Caribbean with a multinational collaborative, multicentric and descriptive approach involving 25 institutions from 9 countries is presented. Demographics, clinical manifestations, expanded disability scale status (EDSS), brain and spinal cord MRI, serological anti-AQP4-IgG and anti-MOG-IgG antibodies, and cerebrospinal fluid (CSF) oligoclonal bands were included. A central serological repository utilized the cell-based assay. The specimens outside of this network employed diverse methodologies. Data were collected at the Gorgas Commemorative Institute of Health Studies (ICGES), Panama, and included 186 subjects, of which 84% were females (sex ratio of 5.6:1). Mestizos constituted 72% of the study group. The median age was 42.5 years (IQR: 32.0-52.0). Associated autoimmune diseases (8.1%) were myasthenia gravis, Sjögren's syndrome and systemic lupus erythematosus. The most common manifestation was optic neuritis-transverse myelitis (42.5%). A relapsing course was described in 72.3% of cases. EDSS scores of 0-3.5 were reported in 57.2% of cases and higher than 7.0 in 14.5%. Positive anti-AQP4-IgG antibody occurred in 59.8% and anti-MOG-IgG antibody in 11.5% of individuals. Antibody testing was lacking for 13.4% of patients. The estimated crude prevalence of NMOSD from Panama and the Dominican Republic was 1.62/100,000 (incidence of 0.08-0.41) and 0.73/100,000 (incidence 0.02-0.14), respectively. This multinational study contributes additional insights and data on the understanding of NMOSD in this Latin American region.

Encefalitis por Anticuerpos contra el Receptor NMDA: un diagnóstico diferencial neuropsiquiátrico

La Encefalitis por Anticuerpos contra el Receptor NMDA es un reto debido a la amplia lista de posibilidades diagnósticas a las que se asemeja la sintomatología inicial. Es una enfermedad cuya fisiopatología está dada por la generación y acción de anticuerpos, inducidos mayormente por agentes externos (virus) e internos (algunos tumores), sobre NMDAR (receptor N-metil-D-aspartato) que puede cursar con alteraciones neurológicas, psiquiátricas y autonómicas, usualmente afectando a población femenina adulta joven y que en ocasiones forma parte de un síndrome paraneoplásico. Su manejo se basa en inmunoterapia con corticosteroides, inmunoglobulina intravenosa o en casos refractarios, plasmaféresis. La eficacia de estas terapias aumenta con el diagnóstico oportuno, sin embargo con frecuencia el tratamiento se aplica tardíamente por lo difícil del acierto diagnóstico. Presentamos el caso clínico de una femenina de 22 años, que debutó con manifestaciones neuropsiquiátricas, inicialmente medicada con antipsicóticos y que desarrolló rigidez, aumento de creatina quinasa y estatus epiléptico, por lo cual se sospechó síndrome neuroléptico maligno, ameritando hospitalización en la unidad de cuidados intensivos. Ante la nula mejoría, se replanteó el diagnóstico, con sospecha de encefalitis autoinmune y se instauró el tratamiento específico, con lo cual la paciente pudo retornar a su vida diaria sin déficit. (provisto por Infomedic International)