Extended composite index of anthropometric failure in Argentinean preschool and school children.

OBJECTIVE: The Composite Index of Anthropometric Failure (CIAF) can only be applied to children under 5 years of age and does not contemplate obesity. The aim of this study was to propose an Extended CIAF (ECIAF) that combines the characterization of malnutrition due to undernutrition and excess weight, and apply it in six Argentine provinces. DESIGN: ECIAF excludes children not in anthropometric failure (group A) and was calculated from a percentage of children included in malnutrition categories B: wasting only; C: wasting and underweight; D: wasting, stunting and underweight; E: stunting and underweight; F: stunting only; Y: underweight only; G: only weight excess; and H: stunting and weight excess. SETTING: Cross-sectional study conducted in Buenos Aires, Catamarca, Chubut, Jujuy, Mendoza and Misiones (Argentina). PARTICIPANTS: 10 879 children of both sexes aged between 3 and 13·99. RESULTS: ECIAF in preschool children (3 to 4·99 years) was 15·1 %. The highest prevalence was registered in Mendoza (16·7 %) and the lowest in Misiones (12·0 %). In school children (5 to 13·99 years) ECIAF was 28·6 %. Mendoza also recorded the highest rate (30·7 %), while Catamarca and Chubut had the lowest values (27·0 %). In the whole sample, about 25 % of the malnutrition was caused by undernutrition and 75 % by excess weight. CONCLUSIONS: The ECIAF summarizes anthropometric failure by both deficiency and excess weight and it highlights that a quarter of the malnutrition in the Argentine population was caused by undernutrition, although there are differences between Provinces (P < 0·05). ECIAF estimates are higher than those of CIAF or under-nutrition.

DIET QUALITY OF MOROCCAN ADOLESCENTS LIVING IN MOROCCO AND IN SPAIN.

Intra-population socioeconomic changes and migration are powerful factors in changing eating habits. Changes in eating habits could affect the nutritional status, growth, development and health of adolescents. The aim of this study was to compare the diet of adolescents of Moroccan origin living in Spain with that of adolescents living in Morocco. The sample comprised 428 Moroccan adolescents aged from 12 to 19 recruited in high schools: 327 living in Ouarzazate (Morocco) and 101 living in Madrid (Spain). The variables studied were energy intake (kcal/day), diet quality indicators (adherence to the Mediterranean Adequacy Index (MAI); cholesterol intake (mg/day); fibre intake (g/day) and energy profile)); and indicators of keeping traditional customs (halal meat consumption, bread made at home). Teenagers from Morocco living in Madrid consumed more calories, proteins, saturated fats and simple sugars (p<0.001) than those living in Morocco. Their diet was of lower quality than that of their peers in Morocco. This difference was more marked in boys than in girls. Changes in eating habits associated with migration from the south to the north Mediterranean basin can benefit young migrants in an immediate way (through greater availability of energy and nutrients), but later in life it could have negative consequences for their health, increasing the risk of overweight, obesity and cardiovascular and metabolic problems.

Leptomeningeal Interfoliar Enhancement on Vessel Wall MR Imaging as a Unique Radiologic Finding of Susac Syndrome.

Susac syndrome is a rare disorder affecting the brain, retina, and inner ear, probably triggered by an immune-mediated endotheliopathy. Diagnosis is based on clinical presentation and ancillary test findings (brain MR imaging, fluorescein angiography, and audiometry). Recently, vessel wall MR imaging has shown increased sensitivity in the detection of subtle signs of parenchymal, leptomeningeal, and vestibulocochlear enhancement. In this report, we describe a unique finding identified using this technique in a series of 6 patients with Susac syndrome and discuss its potential value for diagnostic work-up and follow-up.

Therapeutic plasma exchange in MS refractory relapses: Long-term outcome.

INTRODUCTION: Therapeutic plasma exchange (TPE) is considered a treatment option for steroid-refractory multiple sclerosis (MS) relapses. Our objective was to assess long-term clinical response to TPE in MS steroid-refractory exacerbations. METHODS: Retrospective study of relapsing remitting MS (RRMS) patients presenting intravenous methylprednisolone (IVMPS)-refractory relapses, who underwent TPE. Response to TPE was assessed at 1, 3, 6, 12 and 24-months post-treatment, and compared to a second group of RRMS patients with similar demographic and clinical characteristics presenting, IVMPS-refractory relapses but not treated with TPE. Multivariate regression analysis was used to assess potential predictors of significant clinical response. RESULTS: Between 2011 to 2020, a total of 23 RRMS patients were treated with TPE. Twenty-one patients not receiving the treatment served as controls. No differences in demographic or clinical characteristics, or predictors of clinical improvement after TPE were detected between groups. Seventy-eight percent of patients treated with TPE presented clinical improvement at 24 months. TPE-treated patients presented lower EDSS scores at 6 and at 24 months. Younger age, presence of gadolinium-enhancing lesions and TPE treatment were associated with better clinical outcomes. No life-threatening side effects were reported. CONCLUSIONS: TPE is a safe and well tolerated procedure that decreases long-term disability in RRMS patients with IVMPS-refractory relapses.

[Lambert-Eaton myasthenic syndrome]. / Síndrome miasteniforme de Lambert-Eaton.

INTRODUCTION: Early diagnosis based on clinical findings, neurophysiological studies and serum antibody titres allows early initiation of symptomatic treatment and oncological screening. Reports of patients with LEMS in Latin America are scarce. AIM: This article aims to describe the characteristics of patients with LEMS from a private centre in Buenos Aires, Argentina, and to compare them with those of other series that have been published. PATIENTS AND METHODS: The medical records of 13 patients with LEMS with clinical findings, compatible electromyogram and/or positive antibodies were reviewed. Follow-up was performed until associated neoplasia was ruled out or confirmed according to the recommended algorithms. RESULTS: Four patients were diagnosed with T-LEMS, two of them with small-cell lung carcinoma. Of the nine patients with NT-LEMS, five had a DELTA-P score of 3 and 4. Nine patients presented with the classic clinical triad from the onset of the disease. All patients had electromyogram findings compatible with presynaptic neuromuscular plaque defect. Of the total, 70% improved symptomatically with pyridostigmine. CONCLUSIONS: The clinical findings, together with compatible neurophysiological studies, are sufficient for the diagnosis of LEMS. The relationship between the DELTA-P score and the risk of small-cell lung carcinoma could not be replicated. Symptomatic treatment with pyridostigmine represents an effective therapeutic alternative.

TITLE: Síndrome miasteniforme de Lambert-Eaton.Introducción. El síndrome miasteniforme de Lambert-Eaton (LEMS) es una patología paraneoplásica (T-LEMS) o idiopática autoinmunitaria (NT-LEMS) ocasionada por autoanticuerpos contra los canales de calcio dependientes del voltaje presinápticos de la unión neuromuscular. El 60% de los T-LEMS se asocia a carcinoma de pulmón de células pequeñas. Una puntuación Dutch-English LEMS Tumor Association Prediction (DELTA-P) mayor de 3 denota un riesgo elevado de dicha asociación. El diagnóstico precoz fundado en los hallazgos clínicos, estudios neurofisiológicos y dosificación de títulos de anticuerpos en el suero permite iniciar tempranamente el tratamiento sintomático y la búsqueda oncológica. Son escasos los informes de pacientes con LEMS en Latinoamérica. Objetivo. Describir las características de pacientes con LEMS de un centro privado de Buenos Aires, Argentina, y compararlas con las de otras series publicadas. Pacientes y métodos. Se revisaron historias clínicas de 13 pacientes con LEMS con hallazgos clínicos, electromiograma compatible y/o anticuerpos positivos. Se realizó seguimiento hasta descartar o confirmar una neoplasia asociada de acuerdo con los algoritmos recomendados. Resultados. Cuatro pacientes presentaron diagnóstico de T-LEMS, dos de ellos con carcinoma de pulmón de células pequeñas. De los nueve pacientes con NT-LEMS, cinco presentaron una puntuación DELTA-P de 3 y 4. Nueve pacientes presentaron la tríada clínica clásica desde el inicio. Todos los pacientes presentaron en el electromiograma hallazgos compatibles con defecto de placa neuromuscular presináptico. El 70% mejoró sintomáticamente con piridostigmina. Conclusiones. Los hallazgos clínicos, junto con los estudios neurofisiológicos compatibles, resultan suficientes para el diagnóstico de LEMS. No pudo replicarse la relación entre puntuación DELTA-P y riesgo de carcinoma de pulmón de células pequeñas. El tratamiento sintomático con piridostigmina representa una alternativa terapéutica eficaz.

Vasculitic Peripheral Neuropathy, Differences Between Systemic and Non-Systemic Etiologies: A Case Series and Biopsy Report.

BACKGROUND: Vasculitic peripheral neuropathy (VPN) is caused by vessel inflammation leading to peripheral nerve injury of acute-to-subacute onset. When VPN occurs in the context of systemic disease it is classified as Systemic Vasculitic Neuropathy (SVN) and as Non-Systemic Vasculitic Neuropathy (NSVN) when restricted to the nerves. OBJECTIVE: This study aimed to compare the clinical characteristics, biopsy findings and disease outcome in patients with VPN. METHODS: Clinical records of adult patients with VPN diagnosed at our institution between June-2002 and June-2019 were retrospectively reviewed. Demographic characteristics, clinical manifestations, nerve conduction studies, nerve biopsies, treatment and clinical evolution were analyzed in all patients with at least 6 months follow-up. RESULTS: Twenty-five patients with VPN were included (SVN, n = 10; NSVN, n = 15). No significant differences in demographic or clinical features were found between groups. The median delay between symptom onset and nerve biopsy was significantly longer in NSVN patients (10 vs 5.5 months, p = 0.009). Erythrocyte sedimentation rate (ESR) values over 20 mm/h were significantly more common in SVN patients (100% vs. 60%, p = 0.024). Nerve biopsies showed active lesions more frequently in treatment-naive patients compared to those who had received at least 2 weeks of corticosteroids (92% vs 38%; p = 0.03), with a higher proportion of definite VPN cases (92 vs 46%; p = 0.04). CONCLUSIONS: Although the clinical manifestations are similar, ESR is an important tool to help distinguish between both conditions. Early nerve biopsy in untreated patients increases diagnostic accuracy, avoiding misdiagnosis.

Differential diagnosis in acute inflammatory myelitis.

INTRODUCTION: Establishing differential diagnosis between different inflammatory causes of acute transverse myelitis (ATM) can be difficult. The objective of this study was to see which clinical, imaging or laboratory findings best contribute to confirm ATM etiology. METHODS: We reviewed clinical history, MRI images, CSF and serum laboratory tests in a retrospective study of patients presenting ATM. Univariate and multivariate multinomial logistic regression analysis was performed for each of the items listed above. RESULTS: One hundred and seventy-two patients were analyzed in the study: 68 with multiple sclerosis (MS), 67 presenting idiopathic myelitis (IM; 23 of which were recurrent), 21 who developed positive systemic-antibodies associated myelitis (SAb-M) and 16 with neuromyelitis optica spectrum disorders (NMOSD). The following factors were associated with increased risk of developing MS: lower values in the modified Rankin scale at admission; positive oligoclonal bands (OCB); higher spinal cord lesion load; presence of brain demyelinating lesions; and disease recurrence. Longitudinally extended (LE) lesions, brain demyelinating lesions, and recurrences also contributed to final diagnosis of NMOSD. Multivariate multinomial logistic regression analysis showed presence of LE lesions increased risk of NMOSD and recurrence of ATM. Whereas, brain demyelinating lesions, and presence of OCB increased risk of MS. CONCLUSIONS: ATM etiology may be clarified on the basis of spinal cord and brain MRI findings, together with CSF biochemistry and serum laboratory test results, allowing more timely and exact diagnosis as well as specific therapy for cases of uncertain origin.

Disease activity impacts disability progression in primary progressive multiple sclerosis.

BACKGROUND: Although solid information on the natural history of primary progressive multiple sclerosis (PPMS) is available, evidence regarding impact of disease activity on PPMS progression remains controversial. OBJECTIVE: To describe the clinical characteristics, presence or absence of MRI activity, and natural history of a PPMS cohort from two referral centers in Argentina and assess whether clinical and/or radiological disease activity correlated with disability worsening. METHODS: Retrospective study conducted at two MS clinics in Buenos Aires, Argentina, through comparative analysis of patients with and without evidence of disease activity. RESULTS: Clinical and/or radiologic activity was presented in 56 (31%) of 178 patients. When stratified by age at onset, we found that for every 10 years of increase in age at onset, risk of reaching EDSS scores of 4 and 6 increased by 26% and 31%, respectively (EDSS 4: HR 1.26, CI 95%: 1.06-1.50; EDSS 6: HR 1.31, CI 95%: 1.06-1.62). Patients who presented clinical exacerbations reached EDSS scores of 6, 7 and 8 faster than those without associated exacerbations (p = 0.009, p = 0.016 and p = 0.001, respectively). Likewise, patients who presented gadolinium-enhancing lesions during the course of disease reached EDSS scores of 7 earlier (p = 0.002). CONCLUSION: Older age at onset and presence of clinical and/or radiological disease activity correlated with accelerated disability progression in this cohort of PPMS patients.

Clinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system.

INTRODUCTION: To assess clinical and/or imaging features useful to distinguish between Susac syndrome (SuS) and primary angiitis of central nervous system (PACNS). METHODS: Multicenter retrospective analysis of two cohorts of Argentine patients diagnosed with SuS and PACNS. RESULTS: 13 patients diagnosed with SuS (6 women and 7 men, mean age 35 ±â€¯10 years) and 15 with PACNS (10 women and 5 men, mean age 44 ±â€¯18 years) were analyzed. Cognitive impairment (11 out of 13 patients vs. 5 out of 15, p = .006), ataxia (7 out of 13 vs. 2 out of 15, p = .042) and auditory disturbances (7 out of 13 vs. 0 out of 15, p = .003) were more frequent in SuS patients; whereas seizures were more frequent in PACNS patients (8 out of 15 vs. 1 out of 13, p = .035). On MRI, corpus callosum (CC) involvement was observed more often in SuS, with abnormalities in CC genu, in 13 out of 13 SuS patients vs. only 2 out of 15 PACNS patients (p < .001); in CC body these were present in 13 out of 13 SuS patients vs. 1 out of 15 PACNS patients, (p < .001); and in CC splenium in 12 out of 13 Sus patients vs. 1 of 15 PACNS, p < .001). Cortical lesions were more frequent in PACNS patients (10 out of 15 vs. 3 out of 13 SuS patients, p = .02), as were hemorrhages (5 out of 15 vs. 0 out of 13 SuS, p = .04) and multiple basal ganglia infarcts (7 out of 15 vs. 1 out of 13 Sus, p = .037). CONCLUSION: Specific clinical and/or MRI findings may help distinguish SuS from PACNS with potential therapeutic implications.

Brain and spinal cord lesion criteria distinguishes AQP4-positive neuromyelitis optica and MOG-positive disease from multiple sclerosis.

OBJECTIVE: Test the ability of a brain and spinal cord MRI criteria to differentiate neuromyelitis optica spectrum disorders and MOG-disease from MS. MRI criteria was further tested in patients with CIS and pediatric MS. BACKGROUND: MOG-disease and neuromyelitis optica spectrum disorders can present clinical and radiological features strikingly similar to those of MS. Previously, diagnostic criteria based on brain MRI have been proposed to distinguish between these demyelinating diseases (Matthews-Jurynczik criteria), but spinal cord imaging and its relevance in CIS have not been evaluated. Simple brain and spinal cord MRI criteria may help separate these three inflammatory CNS diseases both in adults and children, aiding in early diagnostic decision-making, such as need for antibody testing. DESIGN/METHODS: We included 150 participants (23 with aquaporin-4-positive neuromyelitis optica spectrum disorder, 14 with MOG-disease, 20 with aquaporin-4-negative neuromyelitis optica spectrum disorder, 48 with adult-onset relapsing remitting MS, 24 with pediatric-onset MS and 21 with clinically isolated syndrome). Brain and spinal cord MRI scans were anonymised and scored by 2 separate raters, based on two sets of criteria: one previously described by Matthews and colleagues (including presence of at least one lesion adjacent to the body of lateral ventricle and in the inferior temporal lobe, or presence of subcortical U-fiber lesion or a Dawson's finger-type lesion), and an extended version including spinal cord features (non-longitudinally extensive cervical lesion). RESULTS: Extended MRI brain and spinal cord lesion criteria were able to separate adult-onset relapsing remitting MS with 100% sensitivity and 87% specificity from aquaporin-4-positive neuromyelitis optica spectrum disorder; and with 100% sensitivity and 79% specificity from MOG-disease. Additionally, brain and spinal cord criteria showed 100% sensitivity and specificity in patients presenting optic neuritis. Brain and spinal cord criteria were less sensitive in patients with CIS and in pediatric MS patients. CONCLUSIONS: Our data suggest radiological criteria can be useful to separate MS from MOG- and aquaporin-4-positive neuromyelitis optica spectrum disorders, in particular in patients with optic neuritis. Further work is needed to support their use in CIS.

Development of subcutaneous fat in Spanish and Latin American children and adolescents: Reference values for biceps, triceps, subscapular and suprailiac skinfolds.

Subcutaneous fat skinfolds represent a reliable assessment instrument of adiposity status. This study provides current percentile references for four subcutaneous skinfolds (biceps, triceps, subscapular, suprailiac) applicable to children and adolescents in Spain and in Latin American countries where data are scarce. The design consisted of a cross-sectional multicenter study performed with identical methods in 5 countries (Argentina, Cuba, Mexico, Spain and Venezuela). Total sample comprised 9163 children and youths (boys 4615 - girls 4548) aged 6-18 years, healthy and without apparent pathologies. Percentiles 3, 5, 10, 25, 50, 75, 90, 95 and 97 were calculated by the LMS method. Sexual dimorphism was assessed using the t-test and age differences with ANOVA. Normalized growth percentile references were obtained according to sex and age for each skinfold. The mean values of four skinfolds were significantly greater in girls than boys (p<0.001) and, in both sexes, all skinfolds show statistical differences through age (p<0.001) with different magnitudes. Except triceps in girls, peaks between 11 and 12 years of age are more noticeable in boys than in girls. Although the general model of growth is known, the skinfold measurements show variability among populations and differences of magnitude are presented according to the analyzed population. Therefore, these age and sex-specific reference percentile values for biceps, triceps, subscapular and suprailiac skinfolds, derived from a large sample of Spanish and Latin American children and adolescents, are a useful tool for adiposity diagnosis in this population for which no reference values were available.

Body mass index through self-reported data and body image perception in Spanish adults attending dietary consultation.

OBJECTIVES: The aim of this study was to explore, based on sex and age, knowledge regarding weight, height, and the perception of body shape in Spanish adults who attend dietary consultation. We also wanted to determine the participants' desired body shapes and what they considered their best health status. METHODS: The sample consisted of 8100 women and 1220 men from Spain. They were between the ages of 18 and 75 y. Weight (kg) and height (cm) were measured and body mass index (BMI) was calculated. Participants were nutritionally classified following the cutoffs proposed by the World Health Organization. Each individual was asked about his or her weight and height and self-reported BMI was calculated. They also answered a test of body image perception through drawings of human silhouettes that corresponded to an exact BMI. With this, perceived BMI, desired BMI, and BMI considered healthy were estimated. Parametric statistic tests for contrast of mean and percentages were applied. RESULTS: Self-reported and perceived BMI underestimate the BMI obtained through anthropometry. Differences between measured and self-reported BMI are lower in women and increase with age in both sexes. The same result was obtained when comparing measured BMI with perceived BMI through silhouette test. On average, desired BMI and healthy BMI were in the limits of normal weight for all ages and both sexes. However, the difference between them was also lower in women. CONCLUSION: Age and sex influence the perception of excess weight and body image. This could condition the demand of dietary treatment to improve the nutritional status.

Body composition of rural and urban children from the central region of Spain.

Body composition components are considered in a cross-sectional sample of 2564 children (age range 6-14 years) from Central Spain. The sample was divided depending on rural or urban residence. Weight, and skinfold at the triceps, biceps, subscapular and suprailiac site were measured. Body composition was represented by percentage fat (%F), lean body mass (LBM), fat mass (FM) and sum of skinfolds (SSK). Sex differences were found to be highly significant for all variables and rural-urban differences were small. Rural boys show slightly larger fat content, in %F, FM and SSK, than their urban peers, but significant (p < 0.05) differences appear only for SSK. No statistically significant environmental effect was found for LBM. The improvement of living conditions, the urban influence on the rural area and the degree of urbanization of the urban settlement are suggested as the main determinants influencing obtained results.

Trend in menarcheal age in Spain: rural and urban comparison during a recent period.

Menarcheal age of a sample of rural (n = 342) and urban (n = 469) girls with ages ranging from 9 to 16 years from Madrid province was analysed. Results, obtained through the status quo method and probit analysis (age 12.79 SD 1.04 in Madrid city and 12.90 SD 1.03 in rural sample), were compared with data from previous studies carried out in the same area, as well as those from other regions of the Iberian Peninsula. The aim of these comparisons was to establish the variability of menarche in the recent past, considering physical environment, degree of urbanization and socio-economic development. Decline in menarcheal age was detected and, in general, a tendency towards similar means among Spanish populations in the period studied.

Human genetic variation in the Sierra de Gredos mountain (central Spain): study of several polymorphisms.

The variation in the third component of human complement (C3) and orosomucoid (ORM1) serum proteins, and 6-phosphogluconate dehydrogenase (6PGD), adenosine deaminase (ADA), esterase D (ESD), and acid phosphatase (ACP1) red cell enzymes was examined in two samples from autochthonous populations living on either side of the Sierra de Gredos range in Central Spain. These results support only a moderate differentiation between Gredos subpopulations, which is discussed in relation to other Iberian Peninsula groups. Allele frequencies in Gredos show a remarkable heterogeneity as contrasted with other Iberian populations for most of the markers examined; relatively high C3*S, ORM1*F, ADA*1, ESD*2, and ACP1*C gene frequencies are characteristics of Gredos samples. This differentiation was more marked for the Northern Gredos population and could be related to the geographical peculiarities of this region.