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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.

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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.

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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.

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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.

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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.

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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.

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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.

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