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Background Memory complaints are frequent in older adults and are associated with higher risk of cognitive decline.

Background Telemedicine allows Parkinson disease (PD) patients to overcome physical barriers to access health care services and increases accessibility for people with mobility impairments. Objective To investigate the feasibility indicators of a telehealth intervention for PD patients, including patient recruitment, attendance, technical issues, satisfaction, and benefits on levels of physical activity and sleep. Methods We conducted a single-center, single-arm study of telehealth video consultations using WhatsApp (Meta Platforms, Inc., Menlo Park, CA, USA). Also, we collected the feasibility indicators as the primary endpoints. All the patients in the study were previously evaluated in person by the same team. Results Patient recruitment, attendance, and technical issues rates were 61.3%, 90.5%, and 13.3%, respectively, with good scores of patient acceptance and satisfaction with the study intervention. The telehealth intervention improved physical activity, including the number of walks for at least 10 continuous minutes (p = 0.009) and the number of moderate-intensity activities lasting at least 10 continuous minutes (p = 0.001). The Pittsburgh sleep quality index (PSQI) scores also improved for one of its components: perceived sleep duration (p

Background Coronavirus 2 severe acute respiratory syndrome (SARS-CoV-2), generically called COVID-2019, classified as a pandemic by the World Health Organization, has made health practices around the world face unique challenges. Since then, physical distancing and measures such as confinement have been adopted by different governments to control human-to-human transmission. This distance affected the treatment of individuals with progressive diseases such as neuromuscular diseases (NMDs). Objective To identify how patients with NMDs performed the therapeutic routine during social distancing and confinement resulting from the COVID-19 pandemic. Methods Application of a questionnaire prepared in the Google forms application, whose link for access and participation was sent by email or WhatssApp for family members and/or individuals with DNMs to respond. The questionnaire consisted of multiple-choice questions, divided into the following sections: personal data, treatments performed before and during the pandemic, activities performed during confinement, and characterization of motor function in activities of daily living comprising the period between September and October 2020. Results We observed a significant reduction in medical appointments for patients with NMDs. On the other hand, the results showed that most patients underwent motor and/or respiratory physiotherapy in person or by telemonitoring. The study participants reported spending more time playing indoors, and all pointed out motor changes during social distancing. Conclusion There were changes in the therapeutic routine of patients with NMDs during the period of social distancing due to COVID-19.

ABSTRACT Background: Brazil has a top position regarding scientific production on noninvasive neuromodulation worldwide. Knowledge of scientometric phenomena involving Brazilian researchers who produce science on this theme may aid confidence in Brazilian clinical and research professionals. Objective: To investigate the scenario of research on the theme of noninvasive neuromodulation in Brazil. Methods: This was a scientometric study for mapping scientific production on this subject involving network phenomena, the professions of researchers, institutional affiliation, main research unit, total number of scientific articles on noninvasive neuromodulation published in journals, research sub-area and year of obtaining the PhD title. Public data from Lattes Platform curricula vitae and from VOSViewer© were used. Results: A total of 54 Brazilian researchers were identified, of whom 16 are research productivity fellows. Most of them are linked to institutions in southeastern Brazil, involving the professions of biology, biochemistry, physical education, physiotherapy, speech therapy, gerontology, medicine and psychology, with 1175 articles published in journals. These studies involve experimental animal and human models to account for mechanisms, observational studies, case reports, randomized clinical trials, systematic reviews, meta-analyses, product and process development, computer modeling and guidelines. Conclusions: Brazil occupies a prominent place in the world scenario of research on noninvasive neuromodulation, which is used by different professions for treatment of brain dysfunctions, with a trend towards expansion to other fields.

ABSTRACT Background: Alzheimer's disease (AD) is the leading cause of dementia worldwide. Despite alarming evidence on dementia prevalence, the condition is still underdiagnosed by general practitioners (GPs) in primary care. Early detection of the disease is beneficial for patients and relatives, who should be provided comprehensive guidance on dealing with dementia complications, covering medical, family and social aspects, thereby providing an opportunity to plan for the future. Objective: The objective of this study was to assess the knowledge of and attitudes toward dementia held by GPs from a city in the interior of São Paulo State, Brazil. Methods: A non-randomized intervention study was conducted involving six lectures about dementia. Before and after the intervention, the participating physicians completed two quizzes about knowledge of and attitudes towards dementia. The study was carried out in the primary care services of the town and a total of 34 GPs participated in the study. Results: The mean age of the sample was 33.9 (±10.2) years and the majority (76.5%) of the sample had not undertaken medical residency training. The mean number of correct answers on the Knowledge Quiz about dementia before and after the training intervention was 59.6 and 71.2% (p

ABSTRACT Background: Central neuropathic pain (CNP) is often refractory to available therapeutic strategies and there are few evidence-based treatment options. Many patients with neuropathic pain are not diagnosed or treated properly. Thus, consensus-based recommendations, adapted to the available drugs in the country, are necessary to guide clinical decisions. Objective: To develop recommendations for the treatment of CNP in Brazil. Methods: Systematic review, meta-analysis, and specialists opinions considering efficacy, adverse events profile, cost, and drug availability in public health. Results: Forty-four studies on CNP treatment were found, 20 were included in the qualitative analysis, and 15 in the quantitative analysis. Medications were classified as first-, second-, and third-line treatment based on systematic review, meta-analysis, and expert opinion. As first-line treatment, gabapentin, duloxetine, and tricyclic antidepressants were included. As second-line, venlafaxine, pregabalin for CND secondary to spinal cord injury, lamotrigine for CNP after stroke, and, in association with first-line drugs, weak opioids, in particular tramadol. For refractory patients, strong opioids (methadone and oxycodone), cannabidiol/delta-9-tetrahydrocannabinol, were classified as third-line of treatment, in combination with first or second-line drugs and, for central nervous system (CNS) in multiple sclerosis, dronabinol. Conclusions: Studies that address the treatment of CNS are scarce and heterogeneous, and a significant part of the recommendations is based on experts opinions. The CNP approach must be individualized, taking into account the availability of medication, the profile of adverse effects, including addiction risk, and patients' comorbidities.

Abstract Background: Physical therapy has positive results in people with tropical spastic paraparesis (TSP). However, mobility and distance from rehabilitation centers limit the participation in outpatient programs. Objective: To evaluate the impact of a home exercise program on the posture and functional mobility of people with TSP. Methods: A randomized controlled trial comparing three groups of people who performed guided exercises from a guidebook for six months: supervised (SG), unsupervised (WG), and control (CG). Primary outcomes: postural angles (SAPO®) and functional mobility (TUG). Secondary outcomes: gait parameters (CVMob®). Results: The protocol described in the guidebook improved postural angles and functional mobility. There were also positive gait parameter effects (p

Objective This study aims to present the Selective Reminding Test(SRT) and Word List Generation (WLG) adaptation to the Portuguese population, within the validation of the Brief Repeatable Battery of Neuropsychological Tests (BRBN-T)for multiple sclerosis (MS) patients.Method 66 healthy participants (54.5% female) recruited from the community volunteered to participate in this study.Results A combination of procedures from Classical Test Theory (CTT) and Item Response Theory (ITR) were applied to item analysis and selection. For each SRT list, 12 words were selected and 3 letters were chosen for WLG to constitute the final versions of these tests for the Portuguese population.Conclusion The combination of CTT and ITR maximized the decision making process in the adaptation of the SRT and WLG to a different culture and language (Portuguese). The relevance of this study lies on the production of reliable standardized neuropsychological tests, so that they can be used to facilitate a more rigorous monitoring of the evolution of MS, as well as any therapeutic effects and cognitive rehabilitation.

Objective The study aims to investigate the presence of pain amongst multiple sclerosis (MS) patients. Method One hundred MS patients responded to questionnaires evaluating neuropathic and nociceptive pain, depression and anxiety. Statistical analysis was performed using the Mann–Whitney U, Chi-Square and two-tailed Fisher’s exact tests and multivariate logistic regression. Results Women had a statistically higher prevalence of pain (p = 0.037), and chances of having pain after the age of 50 reduced. Women with pain had a statistically significant lower number of relapses (p = 0.003), restricting analysis to those patients with more than one relapse. After the second relapse, each relapse reduced the chance of having pain by 46%. Presence of pain was independent of Expanded Disability Status Scale (EDSS) anxiety, and depression. Conclusion Our findings suggest a strong inverse association between relapses and pain indicating a possible protective role of focal inflammation in the control of pain.

São relatados quatro casos de cistos perineurais sacrais, conhecidos por cistos de Tarlov, e é feita revisão da literatura. A partir de amostra de 88 pacientes com queixas de radiculopatia em membros inferiores, e/ ou dor lombar ou sacral que foram submetidos à ressonância magnética de coluna lombossacra quatro (4.5%) tiveram o diagnóstico de cisto de Tarlov. O diagnóstico foi feito através de ressonância magnética de coluna vertebral lombossacra. Os quatro casos foram submetidos a laminectomia sacral. Todos evoluíram assintomáticos sem défices motores ou sensitivos. Os cistos de Tarlov podem apresentar manifestações clínicas semelhantes às provocadas por hérnia discal lombossacra embora, nem sempre sejam citados nos diagnósticos diferenciais. O tratamento clínico é basicamente fisioterápico. O tratamento cirúrgico visa sanar o efeito compressivo em estruturas ósseas e/ ou nervosas através de punção do cisto ou retirada dele através de laminectomia sacral.

The 50th anniversary of the Brazilian Academy Neurology (BAN) is being celebrated. BAN was founded on May 5th, in 1962. The BAN initial leaders and members are treated with reverence, as well as the honorable place where its foundation took place: the Institute of Neurology, first in Brazil, at the backyards of the ancient National Hospice for the Insane, which is an important and fruitful birthplace of the Brazilian Neurology.

The physiopathology of symptoms and signs in multiple sclerosis (MS) is a less divulged topic albeit its importance in the patients' management. OBJECTIVE: It was to summarize the main biophysical and biochemical mechanisms which produce the clinical manifestations in MS. RESULTS: The mechanisms underpinning neurological deficits are described in the relapsing and in the progressive phases, stressing inflammatory and neurodegenerative components, especially demyelination, axonal damage and conduction impairment. Transient worsening based in Uhthoff's phenomenon, mechanisms producing positive symptoms, as paraesthesias and Lhermitte sign due to axonal hiperexcitability and ephaptic interactions, and development of cortical symptoms will also be addressed. The variety of processes leading to neural repair and functional recovery in the remitting phase is focused, as remyelination and adaptive changes due to neural plasticity. CONCLUSION: The awareness of mechanisms producing symptoms in MS emphasises the role of symptomatic and rehabilitation therapies in the improvement of patients' well-being.

OBJECTIVE: To determine the predictive value of social support on health related quality of life (HRQoL) in multiple sclerosis (MS) patients. METHODS: The sample is composed by 150 MS consecutive patients. We used the Medical Outcomes Study Social Support Survey to assess social support and the Health Status Questionnaire to assess HRQoL. For inferential analysis, we used the Multiple Linear Regression with stepwise selection of variables. RESULTS: The age, basic education, psychological support and disability explains 41.6% of the variance in physical function, 29.4% in physical performance and 30.6% in emotional performance. Age and psychological support explains 23.1% of the variance in physical function and 29.4% in vitality. CONCLUSION: This study demonstrated that social support is a predictor with a significant effect on HRQoL in MS.

OBJECTIVE: Demonstrate brain perfusion changes due to neuronal activation after functional electrical stimulation (FES). METHOD: It was studied 14 patients with hemiplegia who were submitted to a program with FES during fourteen weeks. Brain perfusion SPECT was performed before and after FES therapy. These patients were further separated into 2 groups according to the hemiplegia cause: cranial trauma and major vascular insults. All SPECT images were analyzed using SPM. RESULTS: There was a significant statistical difference between the two groups related to patient's ages and extent of hypoperfusion in the SPECT. Patients with cranial trauma had a reduction in the hypoperfused area and patients with major vascular insult had an increase in the hypoperfused area after FES therapy. CONCLUSION: FES therapy can result in brain perfusion improvement in patients with brain lesions due to cranial trauma but probably not in patients with major vascular insults with large infarct area.

Interferon beta (IFNβ) and glatiramer acetate (GA) were the first immunomodulators approved to the treatment of relapsing-remitting multiple sclerosis (MS) and clinically isolated syndromes. Despite the enlargement of the therapeutic armamentarium, IFNβ and GA remain the most widely drugs and the therapeutic mainstay of MS. OBJECTIVE: To review the mechanisms of action of IFNβ and GA and main clinical results in MS. RESULTS: IFNβ modulates T and B-cell activity and has effects on the blood-brain barrier. The well proved mechanism of GA is an immune deviation by inducing expression of anti-inflammatory cytokines. Some authors favor the neuroprotective role of both molecules. Clinical trials showed a 30% reduction on the annualized relapse rate and of T2 lesions on magnetic resonance. CONCLUSION: Although the precise mechanisms how IFNβ and GA achieve their therapeutics effects remain unclear, these drugs have recognized beneficial effects and possess good safety and tolerability profiles. The large clinical experience in treating MS patients with these drugs along almost two decades deserves to be emphasized, at a time where the appearance of drugs with more selective mechanisms of action, but potentially less safer, pave the way to a better selection of the most appropriate individualized treatment.

OBJECTIVE: To compare the accuracy of SPM and visual analysis of brain SPECT in patients with mesial temporal lobe epilepsy (MTLE). METHOD: Interictal and ictal SPECTs of 22 patients with MTLE were performed. Visual analysis were performed in interictal (VISUAL(inter)) and ictal (VISUAL(ictal/inter)) studies. SPM analysis consisted of comparing interictal (SPM(inter)) and ictal SPECTs (SPM(ictal)) of each patient to control group and by comparing perfusion of temporal lobes in ictal and interictal studies among themselves (SPM(ictal/inter)). RESULTS: For detection of the epileptogenic focus, the sensitivities were as follows: VISUAL(inter)=68%; VISUAL(ictal/inter)=100%; SPM(inter)=45%; SPM(ictal)=64% and SPM(ictal/inter)=77%. SPM was able to detect more areas of hyperperfusion and hypoperfusion. CONCLUSION: SPM did not improve the sensitivity to detect epileptogenic focus. However, SPM detected different regions of hypoperfusion and hyperperfusion and is therefore a helpful tool for better understand pathophysiology of seizures in MTLE.OBJETIVO: Comparar a acurácia do SPM com a análise visual na detecção do foco epileptogênico e alterações perfusionais à distância no SPECT cerebral. MÉTODO: Foram realizados os SPECTs ictal e interictal de 22 pacientes com epilepsia de lobo temporal mesial (ELTM). A análise visual foi realizada nos estudos interictal (VISUAL(inter)) e ictal (VISUAL(ictal/inter)). Na análise com SPM foi comparado o estudo interictal (SPM(inter)) e ictal (SPM(ictal)) de cada paciente com o grupo controle e comparou-se a perfusão dos lobos temporais entre os estudos ictal e interictal (SPM(ictal/inter)). RESULTADOS: Para a detecção do foco epileptogênico, as sensibilidades foram as seguintes: VISUAL(inter)=68%; VISUAL(ictal/inter)=100%; SPM(inter)=45%; SPM(ictal)=64% and SPM(ictal/inter)=77%. O SPM foi capaz de detectar mais áreas de hiperperfusão e hipoperfusão. CONCLUSÃO: O SPM não aumentou a sensibilidade na detecção do foco epileptogênico. Entretanto, o SPM detectou diferentes regiões de hipoperfusão e hiperperfusão e portanto, ele pode ser uma ferramenta de ajuda para se melhor entender a patofisiologia das crises na ELTM.

Brain SPECT imaging (BSI) with statistical parametric mapping (SPM) offers a clear and accurate vision of perfusion changes. OBJECTIVE: To study brain perfusion abnormalities in adolescents' dependent of multiple drugs. METHOD: Sixteen male patients (15.1±2.1 years) were submitted to 99mTc-HMPAO BSI with SPM. RESULTS: Cortical hypoperfusion occurred in 7/16 patients (44%). There was a significant inverse correlation between the number of hypoperfused regions and the patient's age (p= -0.6737; p=0.004) and with the age when the drug dependence began (p= -0.5616; p=0.023). There was also a tendency towards an inverse correlation between regions of hypoperfusion and the duration of the drug dependence. CONCLUSION: BSI with SPM can help detect hypoperfusion in adolescents dependent on multiple drugs. The younger the patients, the more regions of hypoperfusion are noted. Probably, the neuronal plasticity has an important role in this phenomenon because the highest neural activity occurs in childhood.SPECT cerebral (SC) com statistical parametric mapping (SPM) oferece uma visão clara e acurada de alterações perfusionais. OBJETIVO: Avaliar anormalidades perfusionais cerebrais em adolescentes usuários de múltiplas drogas. MÉTODO: Dezesseis pacientes masculinos (15,1±2,1 anos) foram submetidos a SC com SPM utilizando-se HMPAO-99mTc. RESULTADOS: Hipoperfusão cortical ocorreu em 7/16 pacientes (44%). Houve uma significativa correlação inversa entre o número de áreas hipoperfundidas e a idade dos pacientes (p= -0,6737; p=0,004) e com a idade quando iniciaram o abuso das drogas (p= -0,5616; p=0,023). Também houve uma tendência para uma correlação inversa entre as áreas de hipoperfusão e a duração do abuso de drogas. CONCLUSÃO: SC com SPM pode auxiliar na detecção de hipoperfusão cortical em adolescentes dependentes de múltiplas drogas. Quanto menor a idade, mais áreas de hipoperfusão são identificadas. Provavelmente a plasticidade neuronal tem importante papel neste fenômeno, pois a maior atividade neuronal ocorre na infância.

BACKGROUND: Clinical and experimental evidence suggests that chronic obstructive pulmonary disease (COPD) is associated with a variety of mental symptoms that range from cognitive slowing to mental confusion and dementia. PURPOSE: To test the hypothesis that COPD leads to cognitive impairment in the absence of acute confusion or dementia. METHOD: The global cognitive status of 30 patients with COPD without dementia or acute confusion and 34 controls was assessed with a Brazilian version of the Mini-Mental State Exam (MMSE). RESULTS: The MMSE scores were significantly lower in the patient group and inversely related to the severity of COPD. This finding could not be attributed to age, education, gender, daytime sleepiness, hypoxemia, chronic tobacco use, or associated diseases such as diabetes, depression, high blood pressure or alcoholism. CONCLUSION: These results suggest the existence of a subclinical encephalopathy of COPD characterized by a subtle impairment of global cognitive ability.CONTEXTO: Evidências clínicas e experimentais sugerem que a doença pulmonar obstrutiva crônica (DPOC) se associa a sintomas neurocomportamentais que variam da lentidão cognitiva à confusão mental e à demência. PROPÓSITO: Testar a hipótese de que a DPOC pode comprometer a cognição na ausência de estado confusional agudo ou de demência. MÉTODO: O estado cognitivo global de 30 pacientes com DPOC sem demência e sem confusão mental aguda e o de 34 controles foi examinado com a versão brasileira do Mini-Exame do Estado Mental (MEEM). RESULTADOS: As pontuações no meem mostraram-se significativamente mais baixas nos pacientes, e inversamente relacionadas à gravidade da DPOC. Este achado não pôde ser atribuído a diferenças de idade, escolaridade, sonolência diurna, hypoxemia, tabagismo crônico, ou a doenças associadas como diabetes, depressão, hipertensão arterial ou alcoolismo. CONCLUSÃO: Estes resultados sugerem a existência de uma encefalopatia subclínica da DPOC caracterizada por comprometimento sutil da capacidade cognitiva global.

OBJECTIVE: To search for right/left asymmetries in the dendritic trees of the neuronal populations and in the cell-free layer volumes of the human hipoccampal formation. METHOD: In necropsic material obtained from six male individuals we performed a quantitative Golgi study of the dendritic trees of dentate granules, CA3 and CA1 pyramidal neurons and a volumetric analysis of dentate gyrus molecular layer, strata oriens plus alveus and strata lacunosum-moleculare plus radiatum of CA3 and CA1 fields. RESULTS: We found inter-hemispheric asymmetries in the dendrites trees of all neurons, reaching the significant level in the number of granule cells dendritic segments (higher in the left than in the right hemisphere), dendritic branching density of CA3 pyramidal cells and mean dendritic length of CA1 apical terminal segments (higher in the right than in the opposite side). No volumetric differences were observed. CONCLUSION: This study points to different anatomical patterns of connectivity in the hippocampal formations of both hemispheres which may underlie functional asymmetries.OBJETIVO: Pesquisar a existência de assimetrias direita/esquerda nas arborizações dendríticas neuronais e nos volumes das camadas não celulares da formação do hipocampo humano. MÉTODO: Efectuamos estudo quantitativo Golgi das arborizações dendríticas dos grânulos da fascia denteada e das células piramidais de CA3 e CA1, e uma análise estereológica dos volumes da camada molecular da fascia denteada, do strata oriens + alveus e do strata lacunosum-moleculare + radiatum de CA3 e de CA1 em material necrópsico colhido em 6 indivíduos do sexo masculino. RESULTADOS: Encontrámos assimetrias inter-hemisféricas nas arborizações dendríticas de todos os neurónios, significativas no número de segmentos dendríticos das células granulares (maior à esquerda do que à direita) na densidade de ramificação dendrítica das pirâmides de CA3 e no comprimento dendrítico médio dos segmentos apicais terminais das pirâmides de CA1 (maiores à direita do que à esquerda). Não encontramos diferenças volumétricas. CONCLUSÃO: Estes resultados alertam para diferentes padrões anatómicos de conectividade nas formações do hipocampo de ambos os hemisférios que podem fundamentar assimetrias funcionais.

OBJECTIVE: To characterize neurological involvement in juvenile systemic lupus erythe-matosus. METHOD: The charts of all patients with the diagnosis of systemic lupus erythematosus before the age of 16 years, followed at the Rheumatology Unit of Pequeno Príncipe Hospital, from January 1992 to January 2006, were retrospectively reviewed, highlighting neuropsychiatric aspects. RESULTS: Forty-seven patients were included. Neuropsychiatric syndromes were found 29 (61.7%): seizures (17 / 36.2%), intractable headache (7 / 14.9%), mood disorders (5 / 10.6%), cerebrovascular disease (4 / 8.5%), acute confusional state (3 / 6.4%), aseptic meningitis (3 / 6.4%), psychosis (3 / 6.4%), chorea (3 / 6.4%), Guillain-Barré syndrome (2 / 4.3%) and cranial neuropathy (1 / 2.1%). Morbidity indexes (SLEDAI and SLICC) were higher among patients with neuropsychiatric manifestations (pOBJETIVO: Caracterizar o comprometimento neurológico no lupus eritematoso sistêmico juvenil. MÉTODO: Os prontuários dos pacientes com o diagnóstico de lupus eritematoso sistêmico antes dos 16 anos de idade, em acompanhamento na Unidade de Reumatologia do Hospital Pequeno Príncipe, de janeiro de 1992 a janeiro de 2006, foram revisados retrospectivamente enfatizando aspectos neuropsiquiátricos. RESULTADOS: Quarenta e sete pacientes foram incluídos. Síndromes neuropsiquiátricas foram encontradas em 29 (61,7%): crises convulsivas (17 / 36,2%), cefaléia intratável (7 / 14,9%), distúrbios do humor (5 / 10,6%), doença cerebrovascular (4 / 8,5%), estado confusional agudo (3 / 6,4%), meningite asséptica (3 / 6,4%), psicose (3 / 6,4%), coréia (3 / 6,4%), síndrome de Guillain-Barré (2 / 4,3%) e neuropatia craniana (1 / 2,1%). Índices de morbidade (SEDAI e SLICC) foram maiores em pacientes com manifestações neuropsiquiátricas (p

As manifestações das doenças mitocondriais são variadas, acometendo, mais freqüentemente, órgãos com alto metabolismo aeróbico em que são mais abundantes, como, por exemplo, o sistema nervoso. O início dos sintomas em geral é observado na infância havendo relatos de início na idade adulta. Apresentamos caso atípico de doença mitocondrial associada à deleção do DNA mitocondrial em um homem de 39 anos com sintomas psiquiátricos configuraram quadro clínico inicial e somente 12 anos após o início dos sintomas surgiram alterações neurológicas. O diagnóstico da doença mitocondrial foi confirmado por biópsia de músculo sendo documentada deleção do DNA mitocondrial.The manifestations of mitochondrial disease are variable, affecting more frequently the organs with high aerobic metabolism in which they are more abundant, for example the nervous system. The beginning of symptoms in general is observed at chilhood, but some patients presented on adult age. We present an atypical case associated with mitochondrial DNA deletion. A 39-years-old man with psychiatric symptoms that configured initial clinical picture and only after 12 years of the beginning of symptoms neurological alterations became noticeable. The diagnosis of mitochondrial illness was confirmed by muscle biopsy being documented mitochondrial DNA deletion.

Intermittent photic stimulation (IPS) is an important functional test, which can induce the photic driving in the electroencephalogram (EEG). It is capable of enhancing latent oscillation’s manifestations not present in the resting EEG. However, for adequate quantitative evaluation of the photic driving, these changes should be assessed on a statistical basis. With this aim, the sampling distribution of spectral F test was investigated. On this basis, confidence limits of the SFT-estimate could be obtained for different practical situations, in which the signal-to-noise ratio and the number of epochs used in the estimation may vary. The technique was applied to the EEG of 10 normal subjects during IPS, and allowed detecting responses not only at the fundamental IPS frequency but also at higher harmonics. It also permitted to assess the strength of the photic driving responses and to compare them in different derivations and in different subjects.A fotoestimulação intermitente (FEI) é um importante teste functional, que pode induzir o fotorecrutamento no eletroencefalograma (EEG), sendo capaz de realçar manifestações latentes de oscilações não observadas no EEG de repouso. Entretanto, para uma análise quantitativa adequada do fotorecrutamento, tais alterações devem ser avaliadas com base estatística. Assim, a distribuição de probabilidade do teste-F espectral (TFE) foi investigada. Neste sentido, limites de confiança para a estimativa do TFE puderam ser obtidos para diferentes situações práticas, nas quais a razão sinal-ruído e o número de épocas usadas na estimação podem variar. A técnica foi aplicada ao EEG de 10 sujeitos normais durante FEI, e permitiu a detecção de respostas não somente na freqüência fundamental da FEI como também em seus harmônicos. Além disso, permitiu avaliar o grau de fotorecrutamento entre derivações distintas e entre diferentes sujeitos.

OBJECTIVE: To investigate the pattern of perfusion abnormalities in ictal and interictal brain perfusion SPECT images (BSI) from patients with temporal lobe epilepsy (TLE). METHOD: It was acquired interictal and ictal BSI from 24 patients with refractory TLE. BSIs were analyzed by visual inspection and statistical parametric mapping (SPM2). Statistical analysis compared the patients group to a control group of 50 volunteers. The images from patients with left-TLE were left-right flipped. RESULTS: It was not observed significant perfusional differences in interictal scans with SPM. Ictal BSI in SPM analysis revealed hyperperfusion within ipsilateral temporal lobe (epileptogenic focus) and also contralateral parieto-occipital region, ipsilateral posterior cingulate gyrus, occipital lobes and ipsilateral basal ganglia. Ictal BSI also showed areas of hypoperfusion. CONCLUSION: In a group analysis of ictal BSI of patients with TLE, voxel-wise analysis detects a network of distant regions of perfusional alteration which may play active role in seizure genesis and propagation.OBJETIVO: Investigar o padrão de anormalidades perfusionais no SPECT de perfusão cerebral (SPC) ictal e interictal na epilepsia de lobo temporal (ELT). MÉTODO: Foram realizados SPCs ictal e interictal de 24 pacientes com ELT que foram analisados visualmente e com o statistical parametric mapping (SPM2). A análise estatística comparou o grupo de pacientes versus um grupo controle de 50 voluntários. RESULTADOS: Na análise do SPM não foram observadas diferenças significativas no grupo de SPC interictal. No grupo de SPC ictal o SPM revelou hiperperfusão no lobo temporal ipsilateral (foco epileptogênico) e também na região parieto-occipital contralateral, porção posterior do cíngulo ipsilateral, lobos occipitais e núcleos da base ipsilateral. O SPC ictal também mostrou áreas de hipoperfusão. CONCLUSÃO: Em uma análise de grupo do SPC ictal de pacientes com ELT, a análise baseada em voxel detecta uma rede de alteração perfusional em regiões distantes que pode ter uma função ativa na origem e propagação das crises.

OBJECTIVE: To analyze the prevalence, severity and functional interference of movement disorders (MD) secondary to chronic use of cyclosporine A (CsA). METHOD: We conducted a cross-sectional study of 60 patients (58.3% male) with mean age 23.1 (3-75) years, followed at the Bone Marrow Transplantation Service of the Hospital de Clínicas of the Federal University of Paraná, Brazil, taking CsA for at least six months. Our protocol included clinical data, assessment of functional interference of symptoms and neurological examination including observation and grading of MD. RESULTS: Eight (13.3%) subjects reported the presence of tremor at the moment of interview and 29 (48.3%) recalled this symptom at some point during treatment. Neurological examination identified 14 (23.3%) subjects with MD: upper limb symmetric action tremor in 13 (21.6%) and parkinsonism (rigidity and bradykinesia) in 1 (1.7%). No other MD was detected. The mean scores indicated mild clinical signs in all cases. Symptoms were considered subjectively mild with no functional interference. CONCLUSION: Almost one quarter of patients using CsA chronically presented MD, almost always mild and transitory action tremor, with minimal interference on daily living activities, not requiring any form of intervention in the majority of cases.OBJETIVO: Analisar a prevalência, gravidade e interferência funcional de transtornos do movimento (TM) secundários ao uso crônico de ciclosporina A (CsA). MÉTODO: Realizamos um estudo transversal em 60 pacientes (58.3% do sexo masculino) com idade média de 23.1 (3-75) anos, acompanhados pelo Serviço de Transplante de Medula Óssea do Hospital de Clínicas da Universidade Federal do Paraná, usando CsA por pelo menos seis meses. A avaliação incluiu dados clínicos, interferência funcional de possíveis sintomas e exame neurológico incluindo observação e graduação de TM. RESULTADOS: Oito (13.3%) entrevistados relataram tremor no momento da entrevista e 29 (48.3%) em alguma fase do tratamento. O exame neurológico identificou 14 (23.3%) pacientes com TM: 13 (21.6%) tremor de ação simétrico de membros superiores e em 1 (1.7%) parkinsonismo (rigidez e bradicinesia). Nenhum outro TM foi detectado. Os escores médios indicaram quadros leves em todos os casos. Os sintomas foram também considerados subjetivamente leves e sem interferência funcional. CONCLUSÃO: Quase um quarto dos pacientes usando CsA cronicamente apresenta TM, quase sempre tremor de ação leve e transitório, interferindo pouco funcionalmente, não requerendo intervenção na maioria dos casos.

OBJECTIVE: To compare magnetic resonance proton spectroscopic with clinical data and to propose a spectroscopic staging of Alzheimer's disease (AD). METHOD: Subjects (n=46), normals (12) and with AD (34), paired to age (CDR0-CDR3); AD diagnosis according to DSM-IV/NINCDS-ADRDA criteria; ¹H-MRS with Signa Horizon LX-GE, 1.5T; single voxel at hippocampal region/HCR and posterior cingulate area/PCA. RESULTS: Statistically significant decrease (pOBJETIVO: Comparar dados de espectroscopia de prótons por ressonância magnética com clínicos e propor um estadiamento espectroscópico da doença de Alzheimer (DA). MÉTODO: Sujeitos (n=46), normais (12) e com DA (34), emparelhados por idade (CDR0-CDR3); diagnóstico de DA de acordo com os critérios DSM-IV/NINCDS-ADRDA; ¹H-MRS com Signa Horizon LX-GE, 1.5T; voxel único em região hipocampal/RHC e área posterior do cíngulo/APC. RESULTADOS: Redução estatisticamente significativa (p

We assessed the frequency of cerebrospinal fluid (CSF) restricted oligoclonal IgG bands (IgG-OCB) in Portuguese multiple sclerosis (MS) patients and its relationship with outcome. Paired CSF/serum samples of 406 patients with neurological disorders were submitted to isoelectric focusing with immunodetection of IgG. Ninety-two patients had definite MS; non-MS cases were assembled in groups inflammatory/infectious diseases (ID, n=141) and other/controls (OD, n=173). We found in the MS group: mean duration, 38.9 months; clinically isolated syndromes, 24%; relapsing/remitting course (RR), 65%; in RR patients the mean EDSS was 2.1 and the mean index of progression was 0.31. Positive patterns significantly predominated in MS (82.6%; ID, 40.4%; OD, 3.5%). The sensitivity and the specificity of positive IgG-OCB for MS diagnosis was 82.6% and 79.9%, respectively. The sole statistically significant difference in the MS group was the lower progression index observed in negative cases. We conclude that the frequency of positive IgG-OCB patterns in our MS patients fits most values reported in the literature, and that negative results indicate benign disease.Analisamos a frequência de bandas oligoclonais (BOC) restritas ao líquido céfalo-raquidiano (LCR) em doentes portugueses com esclerose múltipla (EM) e sua relação com a clínica. Determinaram-se por focagem isoeléctrica e imunodetecção as BOC da IgG em pares de amostras LCR/soro de 406 doentes com diversas patologias neurológicas: 92 tinham EM definitiva; os casos "não-EM" agruparam-se em doenças inflamatórias/infecciosas (ID; n=141) e outras/controles (OD; n=173). O grupo EM apresentava duração média: 38,9 meses; síndromes clinicamente isolados (CIS), 24%; formas surto/remissão (RR), 65%, nas quais se encontrou EDSS e índice de progressão médios de 2,1 e 0,31, respectivamente. O perfil positivo predominava significativamente na EM (82,6%; ID, 40,4%; OD, 3,5%), cuja sensibilidade e especificidade neste diagnóstico foi 82% e 79,9%, respectivamente. A única diferença estatisticamente significativa no grupo EM foi o menor índice de progressão nos casos negativos. Em conclusão, a frequência de BOC positivas nos nossos doentes EM enquadrou-se nos valores da literatura, e a sua negatividade indicou evolução benigna.

O mapeamento com estimulação direta do córtex cerebral foi utilizado quando o tumor estava próximo ou infiltrava o lobo central. OBJETIVO: Avaliar interferências na técnica de estimulação eletrica direta do córtex e substância branca, sob anestesia geral, durante cirurgia para tumor cerebral relacionado ao lobo central. MÉTODO: Foram estudados 42 pacientes operados de junho de 2000 a junho de 2003. Os fatores que modificaram a intensidade da estimulação necessaria para localizar a área motora durante a cirurgia foram estudados. RESULTADOS: A intensidade necessária do estimulo foi maior entre os pacientes com déficit motor antes da cirurgia (p=0,425), edema na ressonância magnetica (p=0,468) e anestesia com proporfol contínuo (p=0,001). CONCLUSÃO: O mapeamento funcional do lobo central durante a cirurgia foi prejudicado pelo deficit motor acentuado, edema cerebral e anestesia com propofol contínuo.Brain mapping with direct electrical stimulation is usefull when the tumor is located near or has infiltrated the central lobe. OBJETIVE: To analize the surgical findings with direct electrical stimulation of the cortex and white matter under general anesthesia during surgery for brain tumors related to the central lobe. METHOD: We studied 42 patients operated on from June 2000 to June 2003. We analyzed surgical findings and details of brain mapping. RESULTS: The mean value of the intensity of the stimulus was greater among those who presented motor deficit prior to surgery (p = 0.0425) and edema on MRI (p= 0.0468) or during anesthesia with continuous propofol (p=0.001). CONCLUSION: The functional mapping of the central lobe may be influenced by severe motor deficit, edema on MRI and propofol's anesthesia.

It is reported the Brazilian Neurology birth (1912), that has as the hallmark its first Neurology Cathedra of Rio de Janeiro, and the links between Neurology and Psychiatry, besides the main medical protagonists at that time in Rio de Janeiro: João Carlos Teixeira Brandão (1854-1921), first professor of the cathedra of Clinical Psychiatry and Nervous Diseases (1883-1921); Juliano Moreira (1873-1933), the founder of the Brazilian scientific Psychiatry and director of the Hospício Nacional de Alienados (National Hospice for the Insane) (1903-1930); Antônio Austregésilo Rodrigues de Lima (1876-1960), first professor of the cathedra of Neurology, considered the father of the Brazilian Neurology. Aloysio de Castro (1881-1959) was a great Brazilian neurosemiologist at that time. Austregésilo practiced both disciplines, Neurology and Psychiatry, and like Jean-Martin-Charcot, he was very interested in a typically psychiatric disorder, the hysteria. It is also considered in this paper the first Brazilian authors of Neurology and/or Psychiatric texts and the places where Neurology was initially developed by the main founders: Hospício Nacional de Alienados, Santa Casa de Misericórdia do Rio de Janeiro and Policlínica Geral do Rio de Janeiro.É relatado o nascimento da Neurologia brasileira (1912), que tem como marco principal a primeira Cátedra de Neurologia do Rio de Janeiro, bem como as ligações entre Neurologia e Psiquiatria, além dos principais protagonistas médicos da época naquela cidade: João Carlos Teixeira Brandão (1854-1921), primeiro professor da cátedra da Clínica psiquiátrica e de moléstias nervosas (1883-1921); Juliano Moreira (1873-1933), fundador da Psiquiatria científica brasileira e diretor do Hospício Nacional dos Alienados (1903-1930); Antônio Austregésilo Rodrigues de Lima (1876-1960), primeiro catedrático de Neurologia, considerado o pai da Neurologia brasileira. Aloysio de Castro (1881-1959) foi um grande neurosemiologista brasileiro da época. Austregésilo praticava tanto a Neurologia quanto a Psiquiatria e, como Jean Martin-Charcot, estava muito interessado em um típico transtorno psiquiátrico, a histeria. Consideram-se também os primeiros autores brasileiros de textos de Neurologia e/ou Psiquiatria e os locais onde a Neurologia foi inicialmente desenvolvida pelos fundadores principais: Hospício Nacional dos Alienados, Santa Casa de Misericórdia do Rio de Janeiro e Policlínica Geral do Rio de Janeiro.

O tremor essencial (TE) é o distúrbio do movimento mais frequente. Entre os tratamentos de primeira escolha está o uso de beta-bloqueadores. O objetivo deste trabalho é relatar os resultados do uso de propranolol de ação prolongada (PAP) em 40 pacientes com TE e virgens de tratamentos anteriores. MÉTODO: 40 pacientes com TE foram submetidos a um protocolo de avaliação pré-estabelecido em que constavam escalas de classificação para o tremor e escalas de avaliação da severidade do tremor. Todos os pacientes foram submetidos a avaliação inicial e após 1 mês de tratamento. RESULTADOS: com relação ao tipo de tremor, 36 pacientes (90% do total) tinham o tipo 2; os tipos 3 e 4 ocorreram em dois pacientes cada (10% do total). Houve história familiar de tremor em 25 casos (62,5%). A média de idade dos pacientes foi 43,1 anos e a média de idade de início dos sintomas foi 27,4 anos. Dos 40 indivíduos avaliados, 33 ou 82,5% apresentaram algum grau de melhora com PAP; em 52,5 % a melhora foi considerada ótima ou boa. CONCLUSÃO: o PAP mostrou ser uma medicação adequada para o tratamento do TE nesta amostra de 40 pacientes avaliados.

Oxidative stress causes metabolic and structural abnormalities during reperfusion. In an animal model of electrophysiological evaluation of cerebral ischemia-reperfusion, alpha-lipoic acid effect on the oxidative stress was studied by mean absolute amplitude of EEG spectra evaluation. The left carotideal infusion of 3.03 mM alpha-lipoic acid in Wistar rats after cerebral ischemia and reperfusion caused initial reduction and partial final recuperation of the various EEG spectral frequency mean absolute amplitudes (p

Charcot contribuiu significativamente no século XIX na descrição de várias enfermidades neurológicas, em particular na área dos distúrbios do movimento. Charcot contribuiu de forma exponencial na descrição clínica minuciosa da doença de Parkinson, além de introduzir o primeiro tratamento farmacológico. Na área das hipercinesias realizou estudos sobre a síndrome de Tourette, o diagnóstico diferencial dos tremores, das coréias e o estudo inicial sobre startle. Marsden, recentemente falecido, destacou-se no século XX com inúmeras publicações na área dos movimentos anormais.São contribuições seminais os estudos sobre a doença de Parkinson, distonias, mioclonias , tremor essencial, a descrição das síndromes " Painful Legs Moving Toes ", "Gait Ignition Failure" e o "Tremor Primário da Escrita". As contribuições de Charcot no século XIX e de Marsden no século XX na área dos distúrbios do movimento permitem concluir que ambos foram as figuras mais representativas desta área nos últimos dois séculos.

This study evaluated two cases of Apert's syndrome, through phonological, cognitive, and neuropsychological instruments and correlated the results to complementary exams. In short, this study reveals the necessity of application of neuropsychological, cognitive and phonological evaluation and correlation of the results with complementary testings because significant differences can be present in the Apert's syndrome.

OBJECTIVE: To describe the relationship between Professor Charcot and Brazil. BACKGROUND: During the XIX century, French Neurology and its most prominent figure, Professor Charcot, dominated the area of nervous system diseases in the world. METHOD: We have reviewed some of the main publications about Charcot's life, the biography of Dom Pedro II, Emperor of Brazil and the development of Neurology in Brazil. RESULTS: Among the most important patients in Charcot's practice was the Emperor of Brazil. Dom Pedro II became a close friend of Charcot and he was a distinguished guest at Charcot's house, particularly at Tuesday soirées on boulevard St. Germain. In 1887, during the visit of Dom Pedro II to France, Charcot evaluated him and made the diagnosis of surmenage. In 1889, Dom Pedro II was deposed and went to Paris, where he lived until his death in 1891. Charcot signed the death certificate and gave the diagnosis of pneumonitis. Charcot had a passionate affection for animals, a feeling shared by Dom Pedro II. Dom Pedro II was affiliated to the French Society for the Protection of Animals. It is conceivable that Charcot's little monkey, from South America, was given to him by Dom Pedro II. The Brazilian Neurological School was founded by Professor A . Austregésilo in 1911, in Rio de Janeiro. At the time, of Charcot's death in 1893, his influence was still very important in the whole world. He and his pupils played a major role in the development of Brazilian Neurology. CONCLUSION: Professor Charcot had a close relationship with the Emperor of Brazil, Dom Pedro II. He was his private physician and they were close friends. The neurological school, created by professor Charcot, contributed significantly, albeit in an indirect way, to the development of Brazilian Neurology, starting in 1911, in Rio de Janeiro, by Professor A . Austregésilo.

OBJECTIVE: To evaluate the efficacy and safety of bilateral pallidotomies in five patients with generalized dystonia. BACKGROUND: Generalized dystonias are frequently a therapeutic challenge, with poor responses to pharmacological treatment. GPi (globus pallidus internus) pallidotomies for Parkinson's disease ameliorate all kinds of dyskinesias/dystonia, and recent studies reported a marked improvement of refractory dystonias with this procedure. METHODS: Five patients with generalized dystonias refractory to medical treatment were selected; one posttraumatic and four idiopathic. The decision to perform bilateral procedures was based on the predominant axial involvement in these patients. Dystonia severity was assessed with the Burke-Fahn-Marsden Dystonia Scale (BFM). Simultaneous procedures were performed in all but one patient, who had a staged procedure. They were reevaluated with the same scale (BFM) by an unblinded rater at 1, 2, 3, 30, 60, 90, 120 and 180 days post-operatively. RESULTS: The four patients with idiopathic dystonia showed a progressive improvement up to three months; the patient with posttraumatic dystonia relapsed at three months. One patient had a marked improvement, being able to discontinue all the medications. A mean decrease in the BFM scores of 52,58% was noted. One patient had a trans-operative motor seizure followed by a transient hemiparesis secondary to rack hemorrhage; other was lethargic up to three days after the procedure. CONCLUSIONS: Our results show that bilateral GPi pallidotomies may be a safe and effective approach to medically refractory generalized dystonias; it can also be speculated that the posttraumatic subgroup may not benefit with this procedure.As distonias generalizadas são freqüentemente um desafio terapêutico, com pobres respostas aos tratamentos farmacológicos. As cirurgias estereotáxicas, como a palidotomia, têm sido utilizadas com êxito no tratamento da doença de Parkinson e estudos recentes relatam importante melhora das distonias generalizadas, refratárias ao tratamento farmacológico, com a palidotomia bilateral. O objetivo dos autores foi avaliar a eficácia e segurança da palidotomia bilateral em cinco pacientes com distonia generalizada. Foram selecionados cinco pacientes com distonia generalizada, predominante axial, refratários ao tratamento farmacológico (quatro idiopáticas e uma pós-traumática). A severidade da distonia foi avaliada através da escala de Burke-Fahn-Marsden (BFM), no 1º, 2º, e 3º dia após a cirurgia e nos dias 30, 60, 90, 120 e 180 do pós-operatório. Quatro pacientes com distonia idiopática tiveram uma progressiva melhora dentro de 3 meses após a cirurgia e o paciente com distonia pós-traumática teve uma piora da distonia após 3 meses da cirurgia. Um dos pacientes teve uma melhora acentuada do quadro de distonia, ficando livre das medicações. Em média ocorreu redução de 52,58 % dos escores da escala de BFM. Um dos pacientes teve crise convulsiva no trans-operatório, seguida por hemiparesia transitória secundária a hemorragia cerebral e um outro paciente ficou letárgico nos três dias após o procedimento cirúrgico, com melhora posterior do nível de consciência . Os nossos resultados mostraram que a palidotomia bilateral pode ser uma abordagem segura e efetiva para o tratamento das distonias generalizadas refratárias ao tratamento clínico. Pode-se especular que o sub-grupo de distonias generalizadas pós-traumáticas podem não se beneficiar da palidotomia bilateral.

A distonia laríngea (disfonia espasmódica) é distúrbio do movimento caracterizado por contrações involuntárias da musculatura laríngea envolvida no processo de vocalização. A utilização da toxina botulínica no tratamento da distonia laríngea trouxe consideráveis benefícios clínicos. Descrevemos os resultados preliminares do uso terapêutico da toxina botulínica no tratamento da distonia laríngea em 12 pacientes. Após investigação clínica, os pacientes foram submetidos a videolaringoestroboscopia para confirmação diagnóstica e as injeções de toxina botulínica foram realizadas através de punção da membrana cricotireóidea em direção ao músculo tireoaritenóideo, com uso de eletromiografia. A maioria dos pacientes submetidos ao tratamento com toxina botulínica apresentou melhora significativa da distonia laríngea (83% dos casos), com duração média do efeito de quatro meses, sem efeitos colaterais significativos.Laryngeal dystonia (spasmodic dysphonia) is a movement disorder characterized by involuntary contractions of laryngeal muscles involved with vocalization. The introduction of botulinum toxin in the treatment of laryngeal dystonia had a major clinical impact due to the striking improvement of symptoms. We report the preliminary results of therapeutical use of botulinum toxin in the treatment of twelve patients with laryngeal dystonia. After an extensive clinical evaluation, the patients underwent a videostroboscopic exam for diagnostic confirmation. Botulinum toxin was injected in the cricothyreoid membrane, directed towards the thyreoaritenoid muscle, with the aid of eletromyography needles. Most of patients who underwent botulinum toxin injection had a significant improvement of their symptoms (83%), with effects lasting for four months in average and without important side effects.

Procedimentos cirúrgicos alternativos para o tratamento de aneurismas "não clipáveis" da carótida intracavernosa incluem ligadura do vaso ou exclusão do segmento que origina a lesão. Essas técnicas estão associadas a riscos de complicações isquêmicas, mesmo em pacientes com sistema de colaterais funcionante. Portanto, um tratamento adequado requer revascularização encefálica para manter o fluxo sanguíneo nos territórios envolvidos. Relatamos o caso de uma paciente de 47 anos com sintomas e sinais isquêmicos embólicos e paralisia do III nervo craniano causados por aneurisma gigante, parcialmente trombosado, do segmento cavernoso da carótida interna. A paciente foi submetida a ponte anastomótica entre a carótida externa cervical e a carótida interna supraclinoidea utilizando enxerto de veia safena, seguido de exclusão do segmento vascular contendo o aneurisma, com bom resultado e sem complicações.

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We describe two cases of palatal myoclonus (PM), one essential and another secondary to a stroke. Case 1: a 64 years old female who developed clicking sounds in both ears after a stroke and three years later on noticed a progressive involuntary movement of the throat associated with rhythmic contractions of the soft palate, muscles of tongue and throat. MRI showed an ischemic area in brainstem. The patient had a partial response to the use of sumatriptan 6 mg subcutaneously. Case 2: a 66 years old female who began with ear clicking at left ear that worsed slowly associated with tinnitus and arrhythmic movements of soft palate and an audible click at left ear. Brain MRI was normal; audiometry showed bilateral neurosensory loss. She was prescribed clonazepan 1 mg daily with complete recovery. Primary and secondary palatal myoclonus share the same clinical features but probably have different pathophysiological underlying mechanisms.

Relatamos dois casos de doença de Lafora que apresentaram distúrbios do movimento, ataxia cerebelar, disartria e fenômeno do "susto exagerado", como manifestações clínicas iniciais. Estes sintomas precederam as convulsões, mioclonias e a demência progressiva. O diagnóstico foi confirmado pela identificação de corpos de inclusão, PAS positivo, na biópsia de pele de ambos os casos. Os pacientes relatados apresentam uma progressão lenta da doença, o que é incomum, com longa sobrevida. A doença de Lafora deve sempre ser incluída entre as causas de ataxia lentamente progressiva associada com epilepsia.

Afasia global geralmente é acompanhada por hemiparesia direita devido à extensão da lesão subjacente. Recentemente têm sido registrados na literatura casos em que tal síndrome ou não se acompanha do déficit motor ou este é apenas transitório, sendo esta condição conhecida como afasia global sem hemiparesia (AGSH). Relatamos caso de AGSH devido a infarto cerebral embólico cardiogênico, corroborando a tese de que esta condição pode ter valor preditivo para o diagnóstico de infartos embólicos.Symptoms and signs of a stroke indicate which areas of the brain are affected and may also suggest the pathophysiology. We report herein a case of global aphasia without hemiparesis due to embolic infarct. Our case suggests that this situation may be an important sign for embolic cerebral infarction, as reported in literature.

A carência da vitamina B12 em muitas situações manifesta-se também por alterações neuropsiquiátricas, sendo a mais comum a degeneração combinada subaguda da medula espinhal. No sistema nervoso central a repercussão maior é na mielina, sendo a degeneração esponjosa e a desmielinização difusa das colunas lateral e posterior da medula as mais típicas alterações patológicas. O mesmo ocorre nos hemisférios cerebrais, sendo que anormalidades nas imagens por ressonância magnética são esperadas. No entanto muito pouco tem sido relatado e a carência da cobalamina não figura habitualmente na lista de diagnósticos diferenciais de lesões desmielinizantes. Relatamos caso de anemia perniciosa com manifestações neurológicas em que a ressonância magnética mostrou alterações compatíveis com desmielinização do feixe piramidal, consequentes, possivelmente, à carência da vitamina B12. Discutimos os achados neuropatológicos da hipovitaminose. Sugerimos que a degeneração combinada subaguda da medula deve fazer parte do diagnóstico diferencial radiológico das lesões desmielinizantes no sistema nervoso central.

Este estudo visa descrever aspectos clínicos e respostas terapêuticas de 135 pacientes com distonia. Quanto à classificação, 54% apresentava distonia focal, 17,8% segmentar, 8,1% hemidistonia, 1,5% multifocal e 18,6% generalizada. Vinte e seis por cento apresentavam distonia secundária; e 5,9% tinham história familiar. O tratamento das distonias idiopáticas divide-se em específico e sintomático, podendo ser local, com toxina botulínica; ou sistêmico, com drogas orais. As drogas utilizadas foram anticolinérgicos e benzodiazepínicos, com resposta pobre em formas generalizadas. A toxina botulínica foi utilizada em 54 pacientes com distonia focal ou segmentar. Na distonia cervical o início do efeito (IE) ocorreu em oito dias; obtendo-se efeito máximo (EM) em 25,2 dias, e duração média do efeito (DME) de 76,8 dias. Na síndrome de Meige e blefaroespasmo obtivemos resultados encorajadores: IE=4,5dias; EM=17,6dias; DME=87,6dias. Conclui-se que a toxina botulínica A é a primeira escolha para distonias focais e segmentares, enquanto formas generalizadas apresentam resposta pobre às drogas utilizadas.This study aims to describe the clinical patterns and therapeutic responses in 135 patients with dystonia. According to the classification, 54% were focal; 17.8% were segmental; 8.1% hemidistonia; 18.6% generalized and 1.5% were multifocal. There was a positive familial history in 5.9% of the cases. The treatment of the idiopathic dystonias is divided in: specific and symptomatic, and it can be local with botulinum toxin, or systemic with oral drugs. The most common drugs used in the treatment were anticholinergics and benzodiazepines, with poor responses in the generalized forms. Botulinum toxin A was the first line treatment for focal and segmental forms of dystonia. Meanwhile, the generalized forms of dystonia show poor response to the therapies utilized.

Relatamos um caso de obstrução bilateral na origem das artérias carótidas internas, apresentando como sinais/sintomas associados hemiparesia e hipoestesia superficial e profunda à direita, associada a sífilis meningovascular em paciente com SIDA. Tomografia de crânio apresentou pequenas lesões hipodensas, com predomínio à esquerda, e arteriografia evidenciou oclusão bilateral das artérias carótidas. A associação entre lues e SIDA não é infrequente, porém o quadro oligossintomático do paciente, provavelmente devido a oclusão arterial lenta e gradual chama a atenção.

O Professor Antonio Austregésilo foi o pioneiro da neurologia brasileira, criando a primeira escola neurológica no Rio de Janeiro em 1912. Ele foi também o primeiro a estudar os distúrbios do movimento no Brasil, tendo publicado vários artigos nesta área, particularmente nas consagradas revistas "Revue Neurologique" e "L'Encephale", incluindo a descrição de um sinal sucedâneo de Babinski e a primeira descrição mundial de distonia pós-traumática.

A doença de moyamoya é anormalidade cerebrovascular crônica e progressiva identificada através das características angiográficas; estão presentes no quadro clínico episódios isquêmicos transitórios, cefaléia, crises convulsivas, hemiparesia, que podem desaparecer após tratamento cirúrgico. Nós descrevemos o caso de uma menina com características clássicas da doença, comparando-o em dois momentos, antes e depois da cirurgia, através de avaliações neurológicas, neuropsicológicas, e exames complementares.

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The main objective of this retrospective review of clinical and cerebrospinal fluid (CSF) data from 41 patients with intracranial tumors diagnosed between 1975 and 1989, is to report the role that the finding of neoplastic cells in CSF plays, specially when cerebral CT-scanning and MRI were not currently done. Another objective is to study the CSF proteic abnormalities in cerebral tumors. CSF cell count, cytomorphologic pictures obtained after sedimentation and protein findings are described. Tumor cells were seen in 12 cases (29%): medulloblastomas - 6, meningeal carcinomatosis - 3, multiforme glioblastoma - 1, ependymoma -1, cerebral metastasis -1; in two cases it was an unexpected finding. We noticed that tumoral localization next to the ventricles favoured cell exfoliation. Although pleocytosis was rare and uncorrelated with the presence of neoplastic cells, pathological cytomorphologic pictures appeared in most of the cases including all "positive" ones. Our results stress that the appearance of neoplastic cells in CSF remains helpful specially when it is an unexpected finding.