Relevance of genetic testing in the gene-targeted trial era: the Rostock Parkinson's disease study.

Estimates of the spectrum and frequency of pathogenic variants in Parkinson's disease (PD) in different populations are currently limited and biased. Furthermore, although therapeutic modification of several genetic targets has reached the clinical trial stage, a major obstacle in conducting these trials is that PD patients are largely unaware of their genetic status and, therefore, cannot be recruited. Expanding the number of investigated PD-related genes and including genes related to disorders with overlapping clinical features in large, well-phenotyped PD patient groups is a prerequisite for capturing the full variant spectrum underlying PD and for stratifying and prioritizing patients for gene-targeted clinical trials. The Rostock Parkinson's disease (ROPAD) study is an observational clinical study aiming to determine the frequency and spectrum of genetic variants contributing to PD in a large international cohort. We investigated variants in 50 genes with either an established relevance for PD or possible phenotypic overlap in a group of 12 580 PD patients from 16 countries [62.3% male; 92.0% White; 27.0% positive family history (FH+), median age at onset (AAO) 59 years] using a next-generation sequencing panel. Altogether, in 1864 (14.8%) ROPAD participants (58.1% male; 91.0% White, 35.5% FH+, median AAO 55 years), a PD-relevant genetic test (PDGT) was positive based on GBA1 risk variants (10.4%) or pathogenic/likely pathogenic variants in LRRK2 (2.9%), PRKN (0.9%), SNCA (0.2%) or PINK1 (0.1%) or a combination of two genetic findings in two genes (∼0.2%). Of note, the adjusted positive PDGT fraction, i.e. the fraction of positive PDGTs per country weighted by the fraction of the population of the world that they represent, was 14.5%. Positive PDGTs were identified in 19.9% of patients with an AAO ≤ 50 years, in 19.5% of patients with FH+ and in 26.9% with an AAO ≤ 50 years and FH+. In comparison to the idiopathic PD group (6846 patients with benign variants), the positive PDGT group had a significantly lower AAO (4 years, P = 9 × 10-34). The probability of a positive PDGT decreased by 3% with every additional AAO year (P = 1 × 10-35). Female patients were 22% more likely to have a positive PDGT (P = 3 × 10-4), and for individuals with FH+ this likelihood was 55% higher (P = 1 × 10-14). About 0.8% of the ROPAD participants had positive genetic testing findings in parkinsonism-, dystonia/dyskinesia- or dementia-related genes. In the emerging era of gene-targeted PD clinical trials, our finding that ∼15% of patients harbour potentially actionable genetic variants offers an important prospect to affected individuals and their families and underlines the need for genetic testing in PD patients. Thus, the insights from the ROPAD study allow for data-driven, differential genetic counselling across the spectrum of different AAOs and family histories and promote a possible policy change in the application of genetic testing as a routine part of patient evaluation and care in PD.

Influence of unilateral eyelid spasms and botulinum toxin treatment on intraocular pressure measured by transpalpebral tonometer.

PURPOSE: Eyelid spasms might be associated with elevated intraocular pressure (IOP) in hemifacial spasm (HFS) patients. IOP assessment using a Goldmann applanation tonometer (GAT) is often compromised by eyelid spasms. This study aimed to assess the effect of HFS on IOP measurements using the transpalpebral tonometer Diaton® before and after treatment with botulinum toxin type A (BTX-A) and compared Diaton® and GAT measurements after treatment with BTX-A. METHODS: IOP measurements were obtained with Diaton® in 27 patients with moderate-to-severe HFS before and after treatment with BTX-A. After treatment, the IOP was also measured using GAT and the results were compared with the ones measured with a Diaton®. The patients underwent automated perimetry, OCT, and pachymetry for screening to glaucoma. RESULTS: Mean IOP with Diaton® was 11 ± 3.42 mmHg before treatment in the affected eye and 9 ± 2.98 mmHg in the contralateral eye. This difference was statistically significant (P = 0.012). However, after treatment with BTX-A, no interocular difference was found in IOP obtained with Diaton® (P = 0.204) or GAT (P = 0.971). Comparison between GAT and Diaton® measurements showed no significant differences after BTX-A treatment between the affected (P = 0.212) and contralateral eye (P = 0.971). CONCLUSIONS: A significant reduction in IOP measurements on the affected side of HFS patients was observed after treatment with BTX-A, demonstrating that eyelid spasms may increase the IOP. No significant difference was observed between Diaton® and GAT measurements after the application of BTX-A. No differences were found in automated perimetry, OCT, and CCT when comparing affected eyes with contralateral eyes.

Clinical and functional correlates of parkinsonism in a population-based sample of individuals aged 75 + : the Pietà study.

BACKGROUND: Parkinsonism is strongly associated with ageing, and many studies have suggested that parkinsonian signs may affect up to half of older adults and is associated with a wide range of adverse health outcomes. We compared clinical and functional characteristics of oldest-old community-dwelling individuals with parkinsonism (parkinsonian group [PG]) to individuals without parkinsonism (non-parkinsonian group [NPG]. METHODS: The Pietà study is a population-based study conducted in Caeté, southeast Brazil, involving 607 individuals aged 75 + years submitted to an extensive clinical evaluation. A subset of 65 PG individuals (61.5% women, median age of 82 years) was compared to 542 NPG individuals (64.8% women, median age of 80 years). RESULTS: PG individuals had significantly more functional impairment, clinical comorbidities (including number of falls, loss of bladder control and dysphagia) and major depression. Multivariate analysis revealed that older age, higher UPDRSm scores, lower category fluency test (animals/minute) and delayed recall memory scores were associated with PG. This group was also more cognitively impaired, with lower performance than NPG individuals in the Mini-Mental State Examination, category fluency test (animals/minute), clock drawing and in delayed recall (p < 0.001 for all tests). UPDRSm scores were the most contributing factor to cognition that independently explained variability in functionality of the entire sample. CONCLUSION: Individuals aged 75 + years with parkinsonism were significantly more clinically and functionally impaired in this population-based sample. Cognitive dysfunction explained most of the loss of functionality in these patients. UPDRS-m scores contributed independently to explain variability in functionality in the whole sample.

White matter hyperintensity presence, quantity, and location exhibits no association with motor and non-motor manifestations of PD.

INTRODUCTION: Nonspecific areas of brain white matter hyperintensity (WMH) are commonly found in the elderly. Some studies have shown that the presence, quantity, and location of WMHs may be associated with the development of cognitive and motor decline in patients with Parkinson's disease (PD), but the results remain controversial. This study aimed to evaluate the relationship of WMH to motor and non-motor symptoms, including dysautonomia and rapid eye movement sleep behavior disorder (RBD), in patients with PD. METHODS: Brain magnetic resonance images were acquired from 120 patients diagnosed with PD and analyzed for WMH classification and quantification. Motor symptoms were quantified using sub-scores of the Movement Disorder Society-Unified Parkinson Disease Rating Scale (MDS-UPDRS)-III. Dysautonomia was evaluated by autonomic reactivity tests, and polysomnography was used for the diagnosis of RBD. RESULTS: Age, total value of the MDS-UPDRS-III tremor sub-score, and the presence of dysautonomia were found to be linearly positively associated. Specifically, the duration of PD was positively associated with rigidity, bradykinesia, axial symptoms, prevalence of dysautonomia, and RBD sub-scores. However, in the multivariate analysis adjusted for variables of interest, no statistical significance was found for any of the models. CONCLUSION: The presence, quantity, and location of WMH were not associated with the analyzed motor and non-motor manifestations of PD.

Les démoniaques dans l'art: Charcot and the "hysterical saints".

Professor Jean-Martin Charcot was the founder of clinical neurology and one of the prominent researchers in the field of hysteria in the 19th century. His book Les démoniaques dans l'art is a representation of hysterical symptoms in religion and religious art. This paper aims to discuss Charcot's descriptions of hysteria in religion and his "hysterical saints".

Professor Jean-Martin Charcot foi o fundador da neurologia clínica e um dos pesquisadores mais proeminentes no campo da histeria durante o século XIX. Seu livro Les démoniaques dans l'art é uma representação dos sintomas histéricos na religião e arte religiosa. Esse artigo objetiva discutir as descrições de Charcot de histeria na religião e seus "santos histéricos".

Les démoniaques dans l'art: Charcot and the "hysterical saints" / Les démoniaques dans l'art: Charcot e os "santos histéricos"

Abstract Professor Jean-Martin Charcot was the founder of clinical neurology and one of the prominent researchers in the field of hysteria in the 19th century. His bookLes démoniaques dans l'art is a representation of hysterical symptoms in religion and religious art. This paper aims to discuss Charcot's descriptions of hysteria in religion and his "hysterical saints".

Resumo Professor Jean-Martin Charcot foi o fundador da neurologia clínica e um dos pesquisadores mais proeminentes no campo da histeria durante o século XIX. Seu livroLes démoniaques dans l'art é uma representação dos sintomas histéricos na religião e arte religiosa. Esse artigo objetiva discutir as descrições de Charcot de histeria na religião e seus "santos histéricos".

I'm looking through you: Mentalizing in frontotemporal dementia and progressive supranuclear palsy.

Mentalizing and emotion recognition are impaired in behavioral variant frontotemporal dementia (bvFTD). It is not clear whether these abilities are also disturbed in other conditions with prominent frontal lobe involvement, such as progressive supranuclear palsy (PSP). Our aim was to investigate social cognition (facial emotion recognition, recognition of social norms violation and mentalizing) in bvFTD and PSP. The neural basis of these functions in PSP and bvFTD groups, by analysis of structural neuroimaging, were also investigated. Twenty-three bvFTD patients, 21 PSP patients and 23 healthy controls were included. All participants underwent 3T brain MRI and a full cognitive exam including the short version of Social and Emotional Assessment (Mini-SEA), which is composed of a facial emotion recognition test (FERT) and the faux pas test. Two components of the faux pas test were distinguished: a score assessing the recognition of social norms violation and a score assessing mentalizing. Compared to controls, bvFTD and PSP patients had significantly reduced scores in all tests of social cognition but did not differ on these measures. PSP and bvFTD had cerebral atrophy in critical regions for social cognition processes, when compared to controls. The cortical correlates of emotion recognition partially overlapped in bvFTD and PSP, with correlations retrieved within the frontal medial cortex, cingulate, insula and limbic structures. PSP and bvFTD patients also displayed similar patterns of brain correlations for the composite score of social norms, with a significant cluster in anterior temporal lobes. Mentalizing scores were associated with frontal and temporal poles bilaterally, in both bvFTD and PSP. These findings support previous observations that PSP patients exhibit impairment in complex cognitive abilities, such as mentalizing. Moreover, these data extend previous findings showing that PSP and bvFTD share key clinical, cognitive and neuroimaging features.

Episodic memory in progressive supranuclear palsy: a neuropsychological and neuroimaging study.

BACKGROUND: Episodic memory impairment may occur in progressive supranuclear palsy (PSP). However, it remains uncertain whether this is due to executive dysfunction or to the involvement of brain areas responsible for memory. OBJECTIVES: To investigate the specific brain regions underlying episodic memory impairment in PSP. METHODS: Twenty-one patients with PSP and 20 healthy controls underwent the Figure Memory Test (FMT) from the Brief Cognitive Screening Battery and brain MRI. We explored correlations between gray matter volumes and memory scores in PSP patients, adjusting for age and performance on the Frontal Assessment Battery. RESULTS: PSP patients performed worse than controls (p < 0.001) on delayed recall in the FMT. Delayed recall scores correlated to bilateral hippocampal and parahippocampal volumes in PSP patients. CONCLUSIONS: Medial temporal structures may play a role in episodic memory impairment in PSP, suggesting that amnesia in PSP is not solely due to executive dysfunction.

Do Patients with Progressive Supranuclear Palsy Have Episodic Memory Impairment? A Systematic Review.

Background: Progressive supranuclear palsy (PSP) is the most common atypical parkinsonism and has executive dysfunction as a core feature. The magnitude of episodic memory disturbance in PSP is yet to be clarified. Objectives: To investigate how impaired is episodic memory in PSP compared to healthy controls and other neuropsychiatric disorders. Also, we sought to identify the brain correlates underlying these memory disturbances. Methods: We performed a systematic search on PubMed and Scopus, combining the terms "progressive supranuclear palsy" AND "memory". The search was limited to papers published in English, French, Portuguese or Spanish, with no chronological filters. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were followed. Results: The initial search returned 464 results. After extraction of duplicates, 356 records were screened, leading to inclusion of 38 studies. Most studies found that PSP patients had lower scores on episodic memory compared to healthy controls. In addition, the majority of studies suggest that PSP does not differ from Parkinson's disease and from atypical parkinsonism in terms of episodic memory performance. The same is seen for PSP and frontotemporal dementia. Conversely, episodic memory impairment seems to be greater in typical Alzheimer's disease compared to PSP. Neuroimaging findings indicate that striatofrontal structures may be involved in PSP episodic memory dysfunction, while no associations with mesial structures (including hippocampi) were found. Conclusions: Episodic memory is impaired in PSP. Whether this amnesia refers to executive dysfunction is still controversial. More studies are warranted to clarify the neural basis of memory impairment in PSP.

Guidelines for Parkinson's disease treatment: consensus from the Movement Disorders Scientific Department of the Brazilian Academy of Neurology - motor symptoms.

The treatment of Parkinson's disease (PD) is challenging, especially since it is considered highly individualized. The Brazilian Academy of Neurology has recognized the need to disseminate knowledge about the management of PD treatment, adapting the best evidence to the Brazilian reality. Thus, the main published treatment guidelines were reviewed based on the recommendations of group from the Movement Disorders Scientific Department of the Brazilian Academy of Neurology.

Guidelines for Parkinson's disease treatment: consensus from the Movement Disorders Scientific Department of the Brazilian Academy of Neurology - motor symptoms / Diretrizes para o tratamento da doença de Parkinson: consenso do Departamento Científico de Transtornos do Movimento da Academia Brasileira de Neurologia - sintomas motores

ABSTRACT The treatment of Parkinson's disease (PD) is challenging, especially since it is considered highly individualized. The Brazilian Academy of Neurology has recognized the need to disseminate knowledge about the management of PD treatment, adapting the best evidence to the Brazilian reality. Thus, the main published treatment guidelines were reviewed based on the recommendations of group from the Movement Disorders Scientific Department of the Brazilian Academy of Neurology.

Resumo O tratamento da doença de Parkinson (DP) constitui um desafio, especialmente por ser considerado muito individualizado. A Academia Brasileira de Neurologia (ABN) identificou a necessidade de disseminar o conhecimento sobre o manejo do tratamento da DP, adaptando as melhores evidências à realidade brasileira. Assim, foi realizada uma revisão sobre as principais orientações de tratamento publicadas, baseada nas recomendações elaboradas por um grupo de especialistas em transtornos do movimento do departamento científico da ABN.

Cross-cultural adaptation of the Activities of Daily Living Questionnaire-Brazil in Parkinson's disease.

BACKGROUND: Culturally adapted measures to assess the performance of activities of daily living (ADL) in individuals with Parkinson's disease (PD) are limited in Brazil. OBJECTIVE: To adapt the ADL Questionnaire to the Brazilian culture and to analyze its reproducibility in individuals with PD. METHODS: The ADL Questionnaire was translated and cross-culturally adapted to Brazilian Portuguese language. Reproducibility was analyzed using test-retest reliability and agreement values. The test-retest reliability of the individual items and total scores were calculated. The limits of agreement were verified using the Bland-Altman plot. The standard error of measurement (SEM) and the minimum detectable change (MDC) were calculated. Patients who were classified on a score of 1-4 on the modified Hoehn and Yahr scale were eligible. RESULTS: No divergence was identified between the original and the adapted version, which demonstrated adequate semantic and conceptual equivalence. The Bland-Altman plot showed no systematic changes in the mean test-retest scores. The intraclass correlation coefficient (ICC) was 0.98 (95% confidence interval [95%CI] 0.93-0.99), and all individual items showed good levels of reliability (>0.60). The SEM (SEM%) and MDC (MDC%) values were 3.0 (6.75%) and 8.2 (18.7%), respectively. These values are within the recommended values. CONCLUSIONS: The ADL-Brazil Questionnaire is a reliable instrument to be used for clinical and research purposes to assess self-perceptions of ADL performance in individuals with PD.

Cross-cultural adaptation of the activities of daily living questionnaire-Brazil in parkinson's disease / Adaptação transcultural do questionário de atividades de vida diária-Brasil para doença de parkinson

ABSTRACT Background: Culturally adapted measures to assess the performance of activities of daily living (ADL) in individuals with Parkinson's disease (PD) are limited in Brazil. Objective: To adapt the ADL Questionnaire to the Brazilian culture and to analyze its reproducibility in individuals with PD. Methods: The ADL Questionnaire was translated and cross-culturally adapted to Brazilian Portuguese language. Reproducibility was analyzed using test-retest reliability and agreement values. The test-retest reliability of the individual items and total scores were calculated. The limits of agreement were verified using the Bland-Altman plot. The standard error of measurement (SEM) and the minimum detectable change (MDC) were calculated. Patients who were classified on a score of 1-4 on the modified Hoehn and Yahr scale were eligible. Results: No divergence was identified between the original and the adapted version, which demonstrated adequate semantic and conceptual equivalence. The Bland-Altman plot showed no systematic changes in the mean test-retest scores. The intraclass correlation coefficient (ICC) was 0.98 (95% confidence interval [95%CI] 0.93-0.99), and all individual items showed good levels of reliability (>0.60). The SEM (SEM%) and MDC (MDC%) values were 3.0 (6.75%) and 8.2 (18.7%), respectively. These values are within the recommended values. Conclusions: The ADL-Brazil Questionnaire is a reliable instrument to be used for clinical and research purposes to assess self-perceptions of ADL performance in individuals with PD.

RESUMO Antecedentes: Medidas adaptadas transculturalmente para avaliar o desempenho nas atividades de vida diária (AVD) em indivíduos com doença de Parkinson (DP) são limitadas no Brasil. Objetivo: Adaptar transculturalmente o Questionário AVD e analisar sua reprodutibilidade em indivíduos com DP. Métodos: O Questionário AVD foi traduzido e adaptado transculturalmente para o português do Brasil. A reprodutibilidade foi analisada usando a confiabilidade teste-reteste e os valores de concordância. A confiabilidade dos itens individuais e as pontuações totais foram calculadas. Os limites de concordância foram verificados usando o gráfico Bland-Altman. O erro padrão da medida (EPM) e a diferença mínima detectável (DMD) foram calculadas. Pacientes classificados nos estágios 1-4 da escala de Hoehn e Yahr foram elegíveis. Resultados: Não foi identificada divergência entre a versão original e a versão adaptada, que demonstrou equivalência semântica e conceitual adequada. O gráfico Bland-Altman não mostrou mudanças sistemáticas nas pontuações médias do teste-reteste. O coeficiente de correlação intraclasse (CCI) foi de 0,98 (intervalo de confiança de 95% [IC95%] 0,93-0,99) e todos os itens individuais apresentaram bons níveis de confiabilidade (>0,60). Os valores do EPM (EPM%) e DMD (DMD%) foram 3,0 (6,75%) e 8,2 (18,7%), respectivamente. Esses valores estão em conformidade com os valores recomendados. Conclusões: O Questionário AVD-Brasil é um instrumento confiável para uso clínico e de pesquisa para avaliar a autopercepção do desempenho nas AVD em indivíduos com DP.

Movement Disorders in Brazil: the seminal contributions of Luiz Augusto Franco de Andrade and Egberto Reis Barbosa.

The major advances in the area of movement disorders in Brazil in recent years were driven by the work of Luiz Augusto Franco de Andrade and Egberto Reis Barbosa. This historical review describes the contributions made by these researchers, physicians, and educators to the development of this field in Brazil.

Cognitive impairment in Parkinson's disease: A clinical and pathophysiological overview.

Cognitive dysfunction in Parkinson's disease (PD) has received increasing attention, and, together with other non-motor symptoms, exert a significant functional impact in the daily lives of patients. This article aims to compile and briefly summarize selected published data about clinical features, cognitive evaluation, biomarkers, and pathophysiology of PD-related dementia (PDD). The literature search included articles indexed in the MEDLINE/PubMed database, published in English, over the last two decades. Despite significant progress on clinical criteria and cohort studies for PD-mild cognitive impairment (PD-MCI) and PDD, there are still knowledge gaps about its exact molecular and pathological basis. Here we overview the scientific literature on the role of functional circuits, neurotransmitter systems (monoaminergic and cholinergic), basal forebrain, and brainstem nuclei dysfunction in PD-MCI. Correlations between neuroimaging and cerebrospinal fluid (CSF) biomarkers, clinical outcomes, and pathological results are described to aid in uncovering the neurodegeneration pattern in PD-MCI and PDD.

How can neurophysiological studies help with movement disorders characterization in clinical practice? A review.

BACKGROUND: Neurophysiological studies are ancillary tools to better understand the features and nature of movement disorders. Electromyography (EMG), together with electroencephalography (EEG) and accelerometer, can be used to evaluate a hypo and hyperkinetic spectrum of movements. Specific techniques can be applied to better characterize the phenomenology, help distinguish functional from organic origin and assess the most probable site of the movement generator in the nervous system. OBJECTIVE: We intend to provide an update for clinicians on helpful neurophysiological tools to assess movement disorders in clinical practice. METHODS: Non-systematic review of the literature published up to June 2019. RESULTS: A diversity of protocols was found and described. These include EMG analyses to define dystonia, myoclonus, myokymia, myorhythmia, and painful legs moving toes pattern; EMG in combination with accelerometer to study tremor; and EEG-EMG to study myoclonus. Also, indirect measures of cortical and brainstem excitability help to describe and diagnose abnormal physiology in Parkinson's disease, atypical parkinsonism, dystonia, and myoclonus. CONCLUSION: These studies can be helpful for the diagnosis and are usually underutilized in neurological practice.

Prática de atividade física e fisioterapia em indivíduos com doença de Parkinson / The practice of physical activity and physical therapy in individuals with Parkinson's disease

Objetivo: Avaliar o perfil da prática de atividade física (AF) e de fisioterapia em indivíduos com doença de Parkinson (DP). Métodos: Pacientes foram recrutados a partir de dois centros de desordens de movimento de Belo Horizonte (Ambulatório Bias Fortes da Universidade Federal de Minas Gerais e Centro Metropolitano de Especialidades Médicas da Santa Casa de Belo Horizonte) entre fevereiro a dezembro de 2019. Resultados: Cento e oitenta e cinco indivíduos responderam um questionário para coleta de dados sociodemográficos, história médica e prática de AF e/ou fisioterapia. Ao comparar os indivíduos dos dois centros, houve diferença apenas em relação à frequência de comorbidades. A hipertensão arterial foi a comorbidade mais comum. Apenas 37,8% e 20,5% dos indivíduos relataram praticar AF ou fazer fisioterapia, respectivamente. A caminhada foi a atividade física mais comum. Pacientes sedentários tinham idade mais avançada, menor tempo de escolaridade, maior duração da DP, e eram mais acometidos por outras comorbidades quando comparados aos pacientes mais ativos. Conclusão: O presente estudo mostra a influência da idade, da escolaridade e do tempo de DP na adesão à prática de AF e fisioterapia. Iniciativas de saúde pública são necessárias para promover a mudança de comportamento e melhorar as oportunidades de AF entre os indivíduos com DP

Objective: The present study aimed to evaluate the profile of physical activity (PA) and physical therapy in individuals with Parkinson's disease (PD). Methods: Patients were recruited from two Outpatient Movement Disorder Clinics (Ambulatório Bias Fortes, Universidade Federal de Minas Gerais, and Centro Metropolitano de Especialidades Médicas, Santa Casa de Belo Horizonte) between February and December 2019. Results: One hundred and eighty-five individuals answered a questionnaire with sociodemographic profiles, medical conditions, and the practice of PA and physical therapy. When comparing individuals from the two centers, differences were only observed concerning the frequency of comorbidities. Arterial hypertension proved to be the most prevalent comorbidity. Only 37.8% and 20.5% of the individuals reported practicing PA or physical therapy, respectively. Walking was the most common physical activity. Sedentary patients were older, had lower levels of education, presented a longer duration of PD, and were more affected by other comorbidities, when compared to more physically active patients. Conclusion: The present study shows the influence of age, education, and PD time on adherence to the practice of PA and physical therapy. Public health initiatives are needed to promote behavioral change and improve PA opportunities among individuals with PD

How can neurophysiological studies help with movement disorders characterization in clinical practice? A review / Como os exames neurofisiológicos podem ajudar na caracterização dos transtornos do movimento na prática clínica? Uma revisão

ABSTRACT Background: Neurophysiological studies are ancillary tools to better understand the features and nature of movement disorders. Electromyography (EMG), together with electroencephalography (EEG) and accelerometer, can be used to evaluate a hypo and hyperkinetic spectrum of movements. Specific techniques can be applied to better characterize the phenomenology, help distinguish functional from organic origin and assess the most probable site of the movement generator in the nervous system. Objective: We intend to provide an update for clinicians on helpful neurophysiological tools to assess movement disorders in clinical practice. Methods: Non-systematic review of the literature published up to June 2019. Results: A diversity of protocols was found and described. These include EMG analyses to define dystonia, myoclonus, myokymia, myorhythmia, and painful legs moving toes pattern; EMG in combination with accelerometer to study tremor; and EEG-EMG to study myoclonus. Also, indirect measures of cortical and brainstem excitability help to describe and diagnose abnormal physiology in Parkinson's disease, atypical parkinsonism, dystonia, and myoclonus. Conclusion: These studies can be helpful for the diagnosis and are usually underutilized in neurological practice.

RESUMO Introdução: Os estudos neurofisiológicos são métodos auxiliares para compreender melhor as características e a natureza dos distúrbios do movimento. A eletromiografia (EMG), em associação com o eletroencefalograma (EEG) e o acelerômetro, podem ser utilizados para avaliar um espectro de movimentos hipo e hipercinéticos. Técnicas específicas podem ser aplicadas para melhor caracterizar a fenomenologia, ajudar a distinguir a origem psicogênica da orgânica e avaliar o local mais provável de geração do movimento no sistema nervoso. Objetivo: Pretendemos fornecer ao clínico uma atualização sobre ferramentas neurofisiológicas úteis para avaliar distúrbios do movimento na prática clínica. Métodos: Revisão não sistemática da literatura publicada até junho de 2019. Resultados: Uma diversidade de protocolos foi encontrada e descrita. Dentre eles, inclui-se o uso de EMG para a definição do padrão de distonia, mioclonia, mioquimia, miorritmia e painfull legs moving toes, além do uso de EMG em associação ao acelerômetro para avaliar tremor e, em associação ao EEG para avaliar mioclonia. Ademais, técnicas para medida indireta de excitabilidade cortical e do tronco encefálico ajudam a descrever e diagnosticar a fisiologia anormal da doença de Parkinson, parkinsonismo atípico, distonia e mioclonia. Conclusão: Esses estudos podem ser úteis para o diagnóstico e geralmente são subutilizados na prática neurológica.

Tribute to Professor Andrew J. Lees / Tributo ao Professor Andrew J. Lees

Abstract Professor Andrew John Lees, from the National Hospital for Neurology and Neurosurgery, a neurological hospital in Queen Square, London, UK, has contributed in a stupendous way to the development of the field of movement disorders in Brazil, with a constant and intense participation in numerous congresses and scientific meetings of this specialty since 1983.

Resumo O professor Andrew Lees, do National Hospital for Neurology and Neurosurgery, Queen Square, Londres, Reino Unido, tem contribuído de maneira estupenda para o desenvolvimento da área dos distúrbios do movimento no Brasil, com uma participação constante e intensa em inúmeros congressos e encontros científicos desta especialidade, desde o ano de 1983.

Tribute to Professor Andrew J. Lees.

Professor Andrew John Lees, from the National Hospital for Neurology and Neurosurgery, a neurological hospital in Queen Square, London, UK, has contributed in a stupendous way to the development of the field of movement disorders in Brazil, with a constant and intense participation in numerous congresses and scientific meetings of this specialty since 1983.