Resumo A síndrome de Cockayne é doença multissistêmica autossômica recessiva devido à falha no reparo do DNA. Originalmente descrita em 1936 em crianças com baixa estatura, atrofia retiniana e surdez, é caracterizada por nanismo, caquexia, fotossensibilidade, envelhecimento acelerado e déficits neurológicos. O mais típico é a fácies, descrita como similar à de um pássaro: maxila proeminente, atrofia do coxim adiposo bucal, olhos profundos, orelhas grandes e nariz fino. Tem sido descrita dificuldade no manejo da via aérea com estreitamento subglótico e risco de aspiração gástrica. Embora as características clínicas da síndrome de Cockayne sejam bem relatadas em publicações pediátricas, há apenas um relato de anestesia em paciente obstétrica na literatura. Relatamos o caso de gestante com diagnóstico de síndrome de Cockayne, submetida com sucesso a raquianestesia para parto cesariano por desproporção cefalopélvica. Diante da difícil decisão entre induzir anestesia geral em paciente com provável via aérea difícil ou anestesia neuroaxial, em meio a limitações cardiovasculares, respiratórias e neurocognitivas da paciente, conduta individualizada é sugerida para alcançar os melhores resultados para a gestante e o neonato.

Abstract Cockayne syndrome is an autosomal recessive multi-systemic disorder due to DNA repair failure. It was originally described in 1936 in children of small stature, retinal atrophy and deafness, characterized by dwarfism, cachexia, photosensitivity, premature aging and neurologic deficits. The most typical feature is described as birdlike facies: protruding maxilla, facial lipoatrophy, sunken eyes, large ears and thin nose. Difficult airway management with subglottic stenosis and risk of gastric content aspiration has been described. Although the clinical characteristics of Cockayne syndrome have been well described in pediatric publications, there is only one report in the literature on anesthesia for an obstetric patient. We report the case of a pregnant patient diagnosed with Cockayne syndrome, submitted successfully to spinal anesthesia for a cesarean section due to cephalopelvic disproportion. In view of the difficult decision between inducing general anesthesia in a patient with a likely difficult airway, or neuraxial anesthesia in a patient with cardiovascular, respiratory and neurocognitive limitations, we suggest tailored management to reach the best results for the mother and newborn.

OBJECTIVE: The aim of this study was to assess the relationship between the degree of unilateral spatial neglect during the acute phase of stroke and long-term functional independence. METHODS: This was a prospective study of right ischemic stroke patients in which the independent variable was the degree of spatial neglect and the outcome that was measured was functional independence. The potential confounding factors included sex, age, stroke severity, topography of the lesion, risk factors, glycemia and the treatment received. Unilateral spatial neglect was measured using the line cancellation test, the star cancellation test and the line bisection test within 48 hours of the onset of symptoms. Functional independence was measured using the modified Rankin and Barthel scales at 90 days after discharge. The relationship between unilateral spatial neglect and functional independence was analyzed using multiple logistic regression that was corrected for confounding factors. RESULTS: We studied 60 patients with a median age of 68 (34-89) years, 52% of whom were male and 74% of whom were Caucasian. The risk for moderate to severe disability increased with increasing star cancellation test scores (OR=1.14 [1.03-1.26], p=0.01) corrected for the stroke severity, which was a confounding factor that had a statistically positive association with disability (OR=1.63 [1.13-2.65], p=0.01). The best chance of functional independence decreased with increasing star cancellation test scores (OR=0.86 [0.78-0.96], p=0.006) corrected for the stroke severity, which was a confounding factor that had a statistically negative association with independence (OR=0.66 [0.48-0.92], p=0.017). CONCLUSION: The severity of unilateral spatial neglect in acute stroke worsens the degree of long-term disability and functional independence.

RESUMO: O potencial evocado visual (PEV) é técnica eletrodiagnóstica que permite avaliação das vias visuais. O PEV de 20 cães e 20 ovinos adultos, de ambos os sexos, foram obtidos para estudo normativo. Foram utilizados eletrodos de agulha monopolar posicionados em O1 e O2 (ativos), em Fpz (referência comum) e em Cz (terra). O estímulo foi na forma de flash a 1Hz. Dezessete cães e 17 ovinos foram incluídos no estudo, pois apresentaram potenciais auditivos com boa qualidade técnica. Os resultados da avaliação dos dois olhos foram agrupados, totalizando 34 exames em cada espécie. O achado mais consistente em todos os exames foi uma deflexão eletropositiva, com latência média de 55,4ms em cães (P55) e 63,75ms em ovinos (P63). Outras duas ondas eletronegativas também foram identificadas: N31 e N75 em cães; e N42 e N86 em ovinos. A metodologia utilizada neste estudo permitiu obtenção de potenciais visuais com pequena variabilidade, sendo útil para avaliação de animais com suspeita de alteração nas vias visuais centrais.

ABSTRACT: The visual evoked potential (VEP) is an electro-diagnostic technique that allows assessment of visual pathways. The VEP from 20 adult dogs and 20 adult sheep of both gender was obtained for normative study. Monopolar electrode needles were placed in O1 and O2 (active), Fpz (common reference) and Cz (ground). The stimuli were flash at 1Hz. Seventeen dogs and 17 sheep were included in the study because they had auditory potentials with good technical quality. The results from both eyes were grouped, totaling 34 results in each species. The most consistent result in all tests was an electropositive deflection, with a mean latency 55.4ms in dogs (P55) and 63.75ms in sheep (P63). Two negative deflections were also identified: N31 and N75 in dogs, and N42 and N86 in sheep. The methodology used in this study allowed to obtain potential similar to those described in other studies, with little variability, and is useful for evaluation of animals with suspected changes in the central visual pathways.

OBJECTIVE: The face-hand test is a simple, practical, and rapid test to detect neurological syndromes. However, it has not previously been assessed in a Brazilian sample; therefore, the objective of the present study was to standardize the face-hand test for use in the multi-cultural population of Brazil and identify the sociodemographic factors affecting the results. METHODS: This was a cross sectional study of 150 individuals. The sociodemographic variables that were collected included age, gender, race, body mass index and years of education. Standardization of the face-hand test occurred in 2 rounds of 10 sensory stimuli, with the participant seated to support the trunk and their vision obstructed in a sound-controlled environment. The face-hand test was conducted by applying 2 rounds of 10 sensory stimuli that were applied to the face and hand simultaneously. The associations between the face-hand test and sociodemographic variables were analyzed using Mann-Whitney tests and Spearman correlations. Binomial models were adjusted for the number of face-hand test variations, and ROC curves evaluated sensitivity and specificity of sensory extinction. RESULTS: There was no significant relationship between the sociodemographic variables and the number of stimuli perceived for the face-hand test. There was a high relative frequency of detection, 8 out of 10 stimuli, in this population. Sensory extinction was 25.3%, which increased with increasing age (OR=1.4[1:01–1:07]; p=0.006) and decreased significantly with increasing education (OR=0.82[0.71-0.94]; p=0.005). CONCLUSION: In the Brazilian population, a normal face-hand test score ranges between 8–10 stimuli, and the results indicate that sensory extinction is associated with increased age and lower levels of education.

RESUMO Objetivo Relacionar as ondas-F com exames clínicos e laboratoriais na fase aguda do acidente vascular cerebral (AVC). Os critérios de inclusão para este estudo transversal foram: hemiplegia, ausência de trauma craniano, miopatia, diabetes, alcoolismo ou outra causa conhecida de neuropatia periférica, além de condução sensorial e motora normal. O National Institutes of Health Stroke Scale (NIHSS), glicemia, hemoglobina glicada e CPK foram obtidos na admissão por meio de exames de rotina. Após a admissão hospitalar, a latência e persistência das ondas-F foram obtidas por meio da estimulação do nervo fibular profundo utilizando técnicas simétricas. Foram avaliados 20 indivíduos – média de idade 66 anos, 50% homem e 85% caucasianos – apresentaram associação univariada da persistência das ondas-F com glicemia (r = 0.71; p < 0.001) e NIHSS categorizado (NIHSS 1–7 = 65.0 x NIHSS 9-23 = 100; p = 0.004). Na regressão multivariada foi encontrado associação somente entre persistência de ondas-F com glicemia β = 0.59(0.44–0.74); p < 0.001. Conclusão O aumento da persistência de ondas-F está associado com maior nível de glicemia na fase aguda do AVC.

ABSTRACT Objective To relate F-waves with clinical and laboratory exams in the acute phase of stroke. Methods Inclusion criteria for this cross-sectional study were: hemiplegia, absence of previous cranial trauma, myopathy, diabetes, alcoholism or other known causes of peripheral neuropathy, and normal sensory and motor conduction. The National Institutes of Health Stroke Scale (NIHSS) score, glycemia, glucosilate hemoglobin, and CPK were obtained at admission by routine blood exams. After hospital admission, the F-wave latencies and persistence were obtained from the deep peroneal nerve using symmetrical techniques. Results Evaluation of 20 individuals – mean age 66 years, 50% male and 85% Caucasian – showed association of F-wave persistence with glycemia (r = 0.71; p < 0.001) and NIHSS categorized (NIHSS 1-7 = 65.0 x NIHSS 9-23 = 100; p = 0.004). Multivariate analysis found only association of F-wave persistence with glycemia β = 0.59 (0.44–0.74); p < 0.001. Conclusion The increase in the persistence of F-waves are associated with hyperglycemia in the acute phase of stroke.

RESUMO: O Potencial Evocado Auditivo de Tronco Encefálico (BAEP-Brainstem Auditory Evoked Potential) avalia a integridade das vias auditivas até o tronco encefálico. O objetivo da pesquisa foi padronizar o uso do BAEP em 21 equinos, sem aparente anormalidade auditiva. Os animais foram sedados com cloridrato de detomidina (0,013mg.kg-1 de PV). Foram utilizados fones de inserção,cliques de rarefação a 90 dB e um ruído de mascaramento de 40 dB. Foram identificadas as latências das ondas I, II, III, IV e V e dos interpicos I-III, III-V e I-V. As médias das latências das ondas foram: onda I 2,24ms; onda II 2,74ms; onda III 3,61ms; onda IV 4,61ms; e onda V 5,49ms. As médias das latências dos interpicos foram: I-III 1,37ms; III-V 1,88ms; e I-V 3,26ms. Este é o primeiro estudo utilizando o BAEP em equinos no Brasil e as latências observadas serão utilizadas como dados normativos para interpretação de exames de cavalos com anormalidades auditivas ou neurológicas.

ABSTRACT: The brainstem auditory evoked potential (BAEP) evaluates the integrity of the auditory pathways to the brainstem. The aim of this study was to evoke BAEPs in 21 clinically normal horses. The animals were sedated with detomidine hydrochloride (0.013mg.kg-1 BW). Earphones were inserted and rarefaction clicks at 90 dB and noise masking at 40 dB were used. After performing the test, the latencies of waves (I, II, III, IV, and V) and interpeaks(I-III, III-V, and I-V) were identified. The mean latencies of the waves were as follows: wave I, 2.4 ms; wave II, 2.24 ms; wave III, 3.61ms; wave IV, 4.61ms; and wave V, 5.49ms. The mean latencies of the interpeaks were as follows: I-III, 1.37ms; III-V, 1.88ms; and I-V, 3.26ms. This is the first study using BAEPs in horses in Brazil, and the observed latencies will be used as normative data for the interpretation of tests performed on horses with changes related to auditory system or neurologic abnormalities.

Objetivo Investigar a relação entre o Alberta Program Early CT Score (ASPECTS) e a Scandinavian Stroke Scale (SSS) para avaliação da incapacidade neurológica de pacientes na fase aguda do acidente vascular cerebral (AVC). Método 59 pacientes com diagnóstico de primeiro AVC foram avaliados. O ASPECTS foi avaliado por 2 neurologistas na admissão e por outro neurologista após 48 horas. O National Institute of Health Stroke Scale (NIHSS) e SSS foram aplicadas para determinar a gravidade do AVC. As correlação e concordâncias foram analisadas estatisticamente pelos testes de Spearman e Kappa. Resultados ASPECTS foi correlacionado com o NIHSS na admissão (r = -0,52; p < 0,001) e SSS (r = 0,50; p < 0,001). O ASPECTS e os itens do SSS que mais se relacionaram foram força do braço (r = 0,52; p < 0,001), da mão (r = 0,49; p < 0,001) e fala (r = 0,51; p < 0,001). A pontuação da SSS de 25,5 mostrou sensibilidade (68%) e especificidade (72%) quando associado ao ASPECTS ≤ 7. Conclusão A SSS pode predizer pior incapacidade neurológica quando associado a baixos valores do ASPECTS.

Objective To investigate the correlation between the Alberta Program Early CT Score (ASPECTS) and the Scandinavian Stroke Scale (SSS) for the evaluation of neurological impairment in patients with acute stroke. Method 59 patients with a first acute ischemic stroke were evaluated. The ASPECTS were evaluated by 2 neurologists at admission and by another neurologist after 48 hours. The NIHSS and SSS was applied to determinate stroke severity. Correlations and agreements were analysed statistically by Spearman and Kappa tests. Results ASPECTS was correlated with National Institute of Health Stroke Scale (NIHSS) at admission (r = -0.52; p < 0.001) and SSS (r = 0.50; p < 0.001). The ASPECTS and SSS items were most correlated with arm (r = 0.52; p < 0.001) and hand (r = 0.49; p < 0.001) motor power, and speech (r = 0.51; p < 0.001). The SSS of 25.5 shows sensitivity (68%) and specificity (72%) when associated with ASPECTS ≤ 7. Conclusion The SSS can predict worst neurological impairment when associated with lower values of ASPECTS.

Objetivo O objetivo do estudo foi avaliar a relação entre a negligência espacial unilateral (NEU) e hemoglobina (Hb) na fase aguda do acidente vascular cerebral (AVC). Método Foi realizado estudo transversal em pacientes com AVC de hemisfério direito dentro das primeiras 48 horas do ictus. Variáveis independentes: nível de Hb (mg/dL); Desfecho: NEU; Fatores potenciais de confundimento: Idade, National Institutes of Health Stroke Scale (NIHSS) e glicemia (mg/dL); A caracterização das variáveis foram obtidas por meio de prontuários eletrônicos, Hb e glicemia por exames laboratoriais, e NEU por meio do Line Cancellation (LCT), Star Cancelation (SCT), e Line Bisection Tasks (LBT). Resultados 40 indivíduos foram avaliados e foi observado que quanto maior os níveis de Hb, melhor o desempenho nos testes de NEU, demonstrando correlação negativa entre eles. Conclusão Níveis baixos de hemoglobina podem indicar pior desempenho nos testes de NEU na fase aguda do AVC.

Objective The objective of this study was to evaluate the relationship between unilateral spatial neglect (USN) and haemoglobin (Hb) level in acute phase of stroke. Method Cross-sectional study was performed after right hemisphere ischemic stroke. Independent variable: Hb level (mg/dL); Outcome: USN; Potential confounding factors: Age, National Institutes of Health Stroke Scale (NIHSS), and glycaemia (mg/dL); Characterization variables were obtained from electronic medical records, Hb, mean corpuscular volume (MCV) and glycaemia by laboratory exams, and USN by cancellation and bisection tasks. The relationship between Hb and USN was assessed by Spearman correlation and linear regression model. Results 40 individuals were evaluated; it was observed that the higher the Hb level, the better the USN test performance, with the two being negatively correlated. There was no significant correlation between VCM level and USN performance. Conclusion Low hemoglobin levels may indicate a worse performance in USN cancellation and bissection tests in acute phase of stroke.

O potencial evocado auditivo de tronco encefálico (PEATE) tem sido amplamente utilizado com diferentes finalidades na prática veterinária e é comumente usado para identificar a surdez hereditária e a presbiacusia. No presente estudo, 43 cães da raça Boxer foram pelo teste PEATE. Foi diagnosticada surdez em 3 cães (2 bilaterais e 1 unilateral), e 40 Boxers foram usados no estudo normativo incluindo avaliação da influência da idade no PEATE. Os animais foram divididos de acordo com a idade em 2 grupos de 20 Boxers cada. A idade media foi 4,54 anos (variação de 1-8) no grupo I e 9,83 anos (variação de 8,5-12) no grupo II. A latência media das ondas I, III e V foram: 1,14 (±0,07); 2,64 (±0,11) e 3,48 (±0,10) ms no grupo I; e 1,20 (±0,12); 2,73 (±0,15) e 3,58 (±0,22) ms no grupo II, respectivamente. A latência media para os intervalos I-III, III-V e I-V foram 1,50 (±0,15); 0,84 (±0,15) e 2,34 (±0,11) ms no grupo I; e 1,53 (±0,16); 0,85 (±0,15) e 2,38 (±0,19) ms no grupo II, respectivamente. As latências das ondas I e III foram estatisticamente diferentes entre os grupos I e II. Não foram observadas diferenças estatísticas entre os dois grupos para os intervalos I-III, III-V e I-V. De acordo com a revisão realizada, este é o primeiro estudo normativo de BAEP realizado em cães da raça Boxer.

Brainstem auditory-evoked potential (BAEP) has been widely used for different purposes in veterinary practice and is commonly used to identify inherited deafness and presbycusis. In this study, 43 Boxer dogs were evaluated using the BAEP. Deafness was diagnosed in 3 dogs (2 bilateral and 1 unilateral) allowing the remaining 40 Boxers to be included for normative data analysis including an evaluation on the influence of age on the BAEP. The animals were divided into 2 groups of 20 Boxers each based on age. The mean age was 4.54 years (range, 1-8) in group I, and 9.83 years (range, 8.5-12) in group II. The mean latency for I, III, and V waves were 1.14 (±0.07), 2.64 (±0.11), and 3.48 (±0.10) ms in group I, and 1.20 (±0.12), 2.73 (±0.15), and 3.58 (±0.22) ms in group II, respectively. The mean inter-peak latencies for the I-III, III-V and I-V intervals were 1.50 (±0.15), 0.84 (±0.15), and 2.34 (±0.11) ms in group I, and 1.53 (±0.16), 0.85 (±0.15), and 2.38 (±0.19) ms in group II, respectively. Latencies of waves I and III were significant different between group I and II. For the I-III, III-V and I-V intervals, no significant differences were observed between the 2 groups. As far as we know, this is the first normative study of BAEP obtained from Boxer dogs.

O potencial evocado auditivo de tronco encefálico (Brainstem Auditory Evoked Potential - BAEP) avalia a atividade elétrica do sistema auditivo, desde a cóclea até o tronco encefálico, permitindo diagnóstico de surdez em cães. Como alguns animais não permitem a obtenção do BAEP sem contenção química, o objetivo deste trabalho foi analisar a influência da sedação com morfina e acepromazinano BAEP de 16 cães com audição normal. Os potenciais foram obtidos antes e durante a sedação com administração intramuscular de morfina (0,5mgkg-1) e acepromazina (0,05mgkg-1). O protocolo de sedação utilizado permitiu contenção efetiva e segurança dos animais. A sedação causou prolongamento nas latências das ondas II, III e intervalos I-III e I-V, mas não dificultou as suas identificações. O uso deste protocolo de sedação não interferiu com a interpretação do potencial evocado e pode ser útil em cães. Pelo que se pode observar na literatura compilada, este é o primeiro estudo avaliando a influência da sedação no BAEP em cães no Brasil.

Brainstem auditory evoked potential (BAEP) reflects the electrical activity along the auditory pathway, from the cochlea to the brainstem, and contributes for the diagnosis of deafness in dogs. BAEP recording may require chemical restraint in some cases, so this study was designed to analyze the impact of sedation with morphine and acepromazine on the BAEP recordings of 16 dogs with normal hearing. BAEPs were recorded before and during sedation with a combination of morphine (0.5mgkg-1) and acepromazine (0.05mgkg-1) given intramuscularly. The protocol employed allowed safe and effective animal restraint. Sedation increased the latency of waves II and III and intervals I-III and I-V but did not interfere with wave identification. and showed to be safe in the dogs tested. Based on the current literature this is the first study which assessed the impact of sedation on BAEPs in dogs in Brazil.

O glioma cordoide é um tumor cerebral raro, recentemente descrito, localizado na região do terceiro ventrículo e com características histológicas, imuno-histoquímicas e ultraestruturais peculiares. Este estudo ilustra um caso de glioma cordoide do terceiro ventrículo em uma paciente de 59 anos de idade.

Chordoid glioma is a recently described and rare brain tumor located in the third ventricular region, with distinctive histological, immunohistochemical and ultrastructural features. The present report describes a case of chordoid glioma of the third ventricle in a 59-year-old female patient.

O potencial auditivo evocado de tronco encefálico é um método eletrodiagnóstico não invasivo que permite avaliação objetiva do estado auditivo, da orelha média ao tronco encefálico, captando a atividade elétrica do sistema auditivo, gerada a partir de um estímulo sonoro específico. O uso desse teste não é difundido em animais no Brasil. Sendo assim, o objetivo do presente trabalho é relatar o diagnóstico de surdez bilateral em dois cães sem raça definida, com a utilização do potencial evocado auditivo de tronco encefálico.

The brainstem auditory evoked potential is a noninvasive electrodiagnostic test allowing an objective assessment of the hearing status, by capturing the electrical activity of the auditory system, from the middle ear to the brainstem, generated after a specific sound stimulus is performed. The use of this test is not common in animals in Brazil. Therefore, the objective of this study is to report the diagnosis of bilateral deafness in two mongrel dogs, using the brainstem auditory evoked potential.

A doença descrita no livro bíblico de Jó é controversa, e tem interessado a teólogos, psiquiatras e dermatologistas, há tempos. Neste trabalho os autores apontam para evidências do diagnóstico de insuficiência renal crônica com alterações neurológicas.

The disease described in the Bible' Book of Job is controversial and had been of interest of theologists, psychiatrists, and dermatologists for many years. We describe several signs and symptoms compatible with chronic renal failure with neurological alterations.

Há controvérsia na literatura sobre os dermátomos humanos. Estudamos dermátomos do membro inferior comparando sinais e sintomas com estudos eletromiográficos, de imagem e achados cirúrgicos. Analisando 60 pacientes, concluímos que o dermátomo L4 provavelmente está localizado na região medial da perna, o dermátomo L5 na região lateral da perna e dorso do pé, e o dermátomo S1 na nádega, região posterior da coxa e da perna e na região plantar. Este é o primeiro estudo que os dermátomos do membro inferior foram analisados através de dados clínicos, eletromiográficos, imagem e achados cirúrgicos.

There is substantial controversy in literature about human dermatomes. We studied L4, L5, and S1 inferior limb dermatomes by comparing clinical signs and symptoms with conduction studies, electromyographical data, neurosurgical findings, and imaging data from computerized tomography (CT) or magnetic resonance imaging (MRI). After analyzing 60 patients, we concluded that L4 is probably located in the medial aspect of the leg, L5 in the lateral aspect of the leg and foot dorsus, and S1 in the posterior aspect of the backside, tight, leg and plantar foot skin. This is the first time that these human dermatomes have been evaluated by combined analysis of clinical, electromyographical, neurosurgical, and imaging data.