Comparison between a handheld ultrasound device and a traditional ultrasound for performing transcranial sonography in patients with Parkinson's disease.

OBJECTIVE: The aim of this study is to compare a portable ultrasound (US) device and a traditional US for performing transcranial ultrasonography (CCT) in patients with Parkinson's disease (PD). METHODS: This is a cross-sectional, observational, and analytical study. The study recruited a total of 129 individuals from two public hospitals in the city of Rio de Janeiro in a prospective and non-randomized manner between September 2019 and July 2021 as follows: group A with 31 patients with PD, group B with 65 patients with PD, and group C with 64 healthy individuals. Group A was used to collect data to establish the agreement analysis of the TCS measurements between the two devices. Groups B and C provided data for constructing the receiver operating characteristic curve for the handheld US. The subjects underwent the assessment of the transtemporal bone window (TW) quality, the mesencephalon area, the size of the third ventricle, and the substantia nigra (SN) hyperechogenicity area. RESULTS: There was a good agreement between the methods regarding the quality of the TW-Kappa concordance coefficient of 100% for the right TW and 83% for the left, the midbrain area-intraclass correlation coefficient (ICC) of 69%, the SN area ICC = 90% for the right SN and 93% for the left and the size of the third ventricle ICC = 96%. The cutoff point for the SN echogenic area in the handheld US was 0.20 cm2 . CONCLUSIONS: The handheld US is a viable imaging method for performing TCS because it shows good agreement with the measurements performed with traditional equipment, and the measurement of SN echogenic area for PD diagnosis presents good sensitivity and specificity.

Evaluation of pattern-reversal visual evoked potential in patients with neuromyelitis optica.

BACKGROUND: The visual evoked potential (VEP) is used in the evaluation of multiple sclerosis (MS) patients, showing a delay in P100 wave latency with no changes in amplitude in 60-100% of cases. In the last decade, the recurrent form of neuromyelitis optica (NMO) has been recognized, and clinically characterized by acute events of transverse myelitis (TM) and optic neuritis (ON), differing from MS in clinical and laboratory criteria. Despite these differences, so far, the VEP parameters described in MS have been used in the evaluation of patients with NMO. The objective of this study was to investigate VEP responses in NMO. METHODS: Patients with NMO underwent pattern-reversal visual stimulation. Nineteen patients were selected for the study. RESULTS: Among the 38 eyes examined, 18 (47.4%) had no visual evoked responses and 13 (34.2%) had a reduction of P100 wave amplitude with normal latency. Only two (5.3%) had the pattern described in MS and five (13.2%) were normal. CONCLUSION: Evaluation of VEP in patients with definite NMO revealed a pattern that is different from that of MS in 81.6% of eyes examined, characterized by the absence of responses, or decreased amplitude with normal latency.

Documento de consenso de LACTRIMS para el tratamiento farmacológico de la esclerosis múltiple y sus variantes clínicas / LACTRIMS consensus document for the pharmacological treatment of the multiple sclerosis and its clinical

Introducción. La esclerosis múltiple (EM) dejó de ser una enfermedad rara en Latinoamérica. Casi todos los países de la región han comunicado cifras de mediana o baja prevalencia. Sin embargo, no existen guías de manejo terapéutico en la mayoría de países, por lo que el Comité Ejecutivo del Comité Latinoamericano para el Tratamiento e Investigación en Esclerosis Múltiple (LACTRIMS) elabora este documento de consenso para presentar recomendaciones y pautas específicas para su tratamiento. Desarrollo. El Comité Ejecutivo de LACTRIMS convocó a un grupo de expertos en el tratamiento e investigación de la EM para redactar un documento de consenso que sirva como instrumento para Latinoamérica, especialmente para aquellos países en los que no existen guías de manejo. Se organizaron grupos de trabajo para cada una de las variables clínicas. Estos grupos estuvieron conformados por neurólogos y neuropediatras de la mayoría de los países y coordinados por expertos, quienes prepararon un documento preliminar que se revisó y redactó en la ciudad de Quito durante los días 8 y 9 de julio de 2011. Finalmente, el documento de consenso fue enviado a cada uno de los representantes de los diferentes países, quienes, en un tiempo fijo, hicieron las últimas recomendaciones incluidas en este documento final. Conclusiones. Basándose en los niveles de evidencia y aplicando los criterios AGREE, se revisan las diferentes variantes clínicas de la enfermedad y se hacen recomendaciones para el uso de los diferentes agentes modificadores de la enfermedad y otros medicamentos (AU)

Introduction. Multiple sclerosis (MS) it is not considered any more a rare disease in Latin America. Most of the Latin American countries have reported moderate or lower prevalence data. However only very few countries have developed therapeutic guidelines. LACTRIMS prepared this consensus document with specific recommendations for the treatment of the disease. Development. Experts on treatment and clinical research on MS were invited by LACTRIMS in order to generate a initial document to be discussed in Quito, Ecuador. Several groups were organized in relation of the different clinical variants. These groups were coordinated by experts leaders and prepared a preliminary document that was discussed in Quito during July 8th and 9th, 2011. Finally the final version was submitted to the members and delegates of LACTRIMS in most of the Latin American countries who were able to make modifications and suggest changes to the final manuscript. Conclusions. Based on the different evidence levels and the AGREE criteria, the clinical variants were reviewed and recommendations were made for the use of drugs and different modifying disease therapeutic agents (AU)

Haplotype analysis of the CAG and CCG repeats in 21 Brazilian families with Huntington's disease.

We studied the allelic profile of CAG and CCG repeats in 61 Brazilian individuals in 21 independent families affected by Huntington's disease (HD). Thirteen individuals had two normal alleles for HD, two had one mutable normal allele and no HD phenotype, and forty-six patients carried at least one expanded CAG repeat allele. Forty-five of these individuals had one expanded allele and one individual had one mutable normal allele (27 CAG repeats) and one expanded allele (48 CAG repeats). Eleven of these forty-five subjects had a mutant allele with reduced penetrance, and thirty-four patients had a mutant allele with complete penetrance. Inter- and intragenerational investigations of CAG repeats were also performed. We found a negative correlation between the number of CAG repeats and the age of disease onset (r=-0.84; P<0.001) and no correlation between the number of CCG repeats and the age of disease onset (r=0.06). We found 40 different haplotypes and the analysis showed that (CCG)(10) was linked to a CAG normal allele in 19 haplotypes and to expanded alleles in two haplotypes. We found that (CCG)(7) was linked to expanded CAG repeats in 40 haplotypes (95.24%) and (CCG)(10) was linked to expanded CAG repeats in only two haplotypes (4.76%). Therefore, (CCG)(7) was the most common allele in HD chromosomes in this Brazilian sample. It was also observed that there was a significant association of (CCG)(7) with the expanded CAG alleles (χ(2)=6.97, P=0.0084). Worldwide, the most common CCG alleles have 7 or 10 repeats. In Western Europe, (CCG)(7) is the most frequent allele, similarly to our findings.

Clinical aspects of amyotrophic lateral sclerosis in Rio de Janeiro/Brazil.

OBJECTIVE: The clinical and epidemiological profile of sporadic amyotrophic lateral sclerosis (ALS), a chronic, degenerative, progressive motor neuron disease of unknown etiology, was described and evaluated in the city of Rio de Janeiro. METHOD: Patients with a diagnosis definite of ALS according to the revised criteria of the El Escorial World Federation of Neurology were included in this retrospective, descriptive study (n=227). Demographic data, clinical variables, mortality and survival of these patients were assessed. RESULTS: Of the 227 included cases, 143 (63%) were male and 84 (37%) were female, resulting in a male/female ratio of 1.7:1. Mean age at onset of the disease was 53.6 ± 12.1 years, overall median survival time was 49 months (95%CI: 42.4-55.5) and the majority of patients (71.4%) were white, black patients 15.9% and mulattos 12.8%. The most common forms of the disease were classic and bulbar ALS. CONCLUSION: Taking classic and bulbar ALS together, the disease was more common in white, male patients of 50 to 70 years of age. When analyzed separately, the bulbar form was more common in women and in older patients. Survival of patients with bulbar ALS was shorter compared to that of patients with classic ALS.

Acidente vascular cerebral / cerebral vascular accident

O acidente vascular encefálico (AVE) é uma das doença mais comuns nos serviços de emergência, corresponde a terceira maior causa de mortalidade nos EUA e é responsável por um custo médio de 19 bilhöes de dólares/ano no que diz respeito a perda de produtividade e despesas médicas secundárias a sua morbidade.Este trabalho tem como objetivo principal a compreensäo e descriçäo das principais condutas para o tratamento do acidente vascular cerebral (AVC) em sua fase aguda; dando-se ênfase aos principais procedimentos realizados na Unidade de Emergência do Hospital Central do Exército/RJ. Existem dúvidas e controvérsias no que diz respeito ao assunto, entretanto alguns aspectos referentes ao correto manejo do AVC em sua fase aguda já säo rotina de vários centros de tratamento de AVC. Na maioria das vezes, os primeiros socorros ao paciente com déficit neurológico sugestivo de AVC näo säo feitos pelo neurologistas, fato este que reflete a importância do treinamento e correto preparo de médicos generalistas que trabalham em emrgência e/ou UTI.au