Estandarización de la batería neuropsicológica de funciones ejecutivas y lóbulos frontales BaNFE-2 en muestra paraguaya con esclerosis múltiple tipo recaída remisión

Introducción: la esclerosis múltiple (EM), enfermedad crónica del sistema nervioso (SNC), compromete significativamente la cognición. Su prevalencia en Paraguay es 9,2/100.000 habitantes, 72% con recaída remisión (EMRR) e incidencia de 2-3mujeres/hombre, afecta más a personas en edad productiva, con altos costos económicos y afectivos. Objetivos: caracterizar al paciente con EMRR, evaluar sus funciones ejecutivas (FEs) con BaNFE-2; establecer valores de corte ajustados al país. Metodología: con fundamento en teoría de la neurociencia cognitiva, diseño no experimental, cuantitativo, descriptivo, empírico, retrospectivo y transversal; técnica psicométrica y entrevista neuropsicológica en una muestra por conveniencia de 40 pacientes, 82,5% mujeres y edad 25-55 años (37,78±7,89). Resultados: se reportaron datos demográficos y clínicos, se caracterizaron las escalas de BaNFE-2 cuya consistencia interna resultó significativa. Se obtuvo 52,5% de alteración en la escala prefrontal y 42,5% en FEs; relaciones significativas con escolaridad, discapacidad física (DF), cantidad de brotes y deterioro cognitivo (DC); diferencias significativas por sexo, escolaridad, DF y DC. Se calcularon puntuaciones tipificadas por escolaridad, z<-1 establece el corte entre normalidad y alteración. Conclusión: las pruebas de BaNFE-2 perfilan la afectación del tiempo de ejecución, atención, memoria de trabajo y FEs. Este estudio aporta baremos ajustados al país y abre una novedosa línea de investigación aplicando BaNFE-2 en EM.

Introduction: Multiple sclerosis (MS) is a chronic, neurodegenerative, inflammatory disease of the central nervous system (CNS) that significantly compromises cognitive functions. In Paraguay, it occurs with a prevalence of 9.2/100,000 inhabitants, 72% in the clinical form of relapsing remission (RRMS) and an incidence of 2 to 3 women/men, affecting more people of productive age with high economic and emotional costs. Objectives: To characterize the Paraguayan patient with RRMS, to assess the state of their executive functions (EFs) with the BaNFE-2 battery, and to establish cut-off values adjusted to the country. Methods: Research based on the theory of cognitive neuroscience of non-experimental, quantitative and descriptive design for analytical purposes. It is empirical, retrospective and cross-sectional. The psychometric technique and neuropsychological interview were used in a convenience sample of 40 patients aged 25 to 55 (37.78 ± 7.89) and 82.5% women. Results: Demographic and clinical data of the participants were reported. The BaNFE-2 coded and normalized scales were statistically characterized, the internal consistency of which was significant. 52.5% of the alteration was obtained in the anterior prefrontal scale and 42.5% in FEs; there are significant relationships with schooling, physical disability (PD), number of outbreaks and cognitive impairment (CI); there were also significant differences by sex, education, PD and CI. Standardized scores adjusted for schooling were calculated such that z<-1 establishes the cutoff between normality and abnormality. Conclusion: BaNFE-2 battery tests profile the affectation of execution time, attention, working memory, and FEs. This study provides the adjusted scales for the country and opens a new line of research applying the BaNFE-2 battery in people with MS.

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.

Central Nervous System Idiopathic Inflammatory Demyelinating Disorders in South Americans: A Descriptive, Multicenter, Cross-Sectional Study.

The idiopathic inflammatory demyelinating disease (IIDD) spectrum has been investigated among different populations, and the results have indicated a low relative frequency of neuromyelitis optica (NMO) among multiple sclerosis (MS) cases in whites (1.2%-1.5%), increasing in Mestizos (8%) and Africans (15.4%-27.5%) living in areas of low MS prevalence. South America (SA) was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing. The current study analyzed the IIDD spectrum in SA after accounting for the ethnic heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals followed in 2011 with a confirmed diagnosis of IIDD using new diagnostic criteria were considered eligible. Patients' demographic, clinical and laboratory data were collected. In all, 1,917 individuals from 22 MS centers were included (73.7% female, 63.0% white, 28.0% African, 7.0% Mestizo, and 0.2% Asian). The main disease categories and their associated frequencies were MS (76.9%), NMO (11.8%), other NMO syndromes (6.5%), CIS (3.5%), ADEM (1.0%), and acute encephalopathy (0.4%). Females predominated in all main categories. The white ethnicity also predominated, except in NMO. Except in ADEM, the disease onset occurred between 20 and 39 years old, early onset in 8.2% of all cases, and late onset occurred in 8.9%. The long-term morbidity after a mean disease time of 9.28±7.7 years was characterized by mild disability in all categories except in NMO, which was scored as moderate. Disease time among those with MS was positively correlated with the expanded disability status scale (EDSS) score (r=0.374; p=<0.001). This correlation was not observed in people with NMO or those with other NMO spectrum disorders (NMOSDs). Among patients with NMO, 83.2% showed a relapsing-remitting course, and 16.8% showed a monophasic course. The NMO-IgG antibody tested using indirect immunofluorescence (IIF) with a composite substrate of mouse tissues in 200 NMOSD cases was positive in people with NMO (95/162; 58.6%), longitudinally extensive transverse myelitis (10/30; 33.3%) and bilateral or recurrent optic neuritis (8/8; 100%). No association of NMO-IgG antibody positivity was found with gender, age at onset, ethnicity, early or late onset forms, disease course, or long-term severe disability. The relative frequency of NMO among relapsing-remitting MS (RRMS) + NMO cases in SA was 14.0%. Despite the high degree of miscegenation found in SA, MS affects three quarters of all patients with IIDD, mainly white young women who share similar clinical characteristics to those in Western populations in the northern hemisphere, with the exception of ethnicity; approximately one-third of all cases occur among non-white individuals. At the last assessment, the majority of RRMS patients showed mild disability, and the risk for secondary progression was significantly superior among those of African ethnicity. NMO comprises 11.8% of all IIDD cases in SA, affecting mostly young African-Brazilian women, evolving with a recurrent course and causing moderate or severe disability in both ethnic groups. The South-North gradient with increasing NMO and non-white individuals from Argentina, Paraguay, Brazil and Venezuela confirmed previous studies showing a higher frequency of NMO among non-white populations.