Resolution of Wing-Beating Tremor and Magnetic Resonance Imaging Lesions in Wilson's Disease Following Penicillamine.

Background: The wing-beating tremor, characteristic of Wilson's disease (WD), is a disabling symptom that can be resistant to anti-copper and anti-tremor medications. Phenomenology Shown: This video illustrates severe bilateral wing-beating tremor, moderate head and lower limb tremors, mild cervical dystonia, and subtle cerebellar ataxia, with nearly resolution after penicillamine treatment. Educational Value: This case highlights a typical aspect of WD, emphasizing the importance of early detection and treatment, and its correlation with MRI findings. Highlights: This case highlights the typical wing-beating tremor in Wilson's disease and its correlation with the involvement of the dentato-rubro-thalamic pathway. The early diagnosis and initiation of treatment with penicillamine resulted in an excellent clinical and radiological response.

Dopa-responsive dystonia and paroxysmal dystonic attacks associated with ATP1A3 gene variant.

An 18-year-old man had episodes of severe generalised dystonia, from aged 7 months and becoming progressively more frequent. He also had gradually developed interictal limb dystonia. He was initially diagnosed with paroxysmal kinesigenic dyskinesia but he did not improve with several medications. A levodopa trial led to levodopa-induced dyskinetic movements. However, a lower titration of 25 mg of levodopa two times per day substantially improved his motor features and quality of life. Laboratory investigations and MR scans of the brain were unremarkable. Whole-exome sequencing identified a pathogenic variant in the ATP1A3 gene. The ATP1A3-spectrum disorders include non-classical phenotypes such as paroxysmal dystonic attacks. A response to dopamine response is unusual in these disorders. This case highlights the importance of levodopa trials in early-onset dystonia cases.

X-Linked Levodopa-Responsive Parkinsonism-Epilepsy Syndrome: A Novel PGK1 Mutation and Literature Review.

BACKGROUND: Genetic underpinnings in Parkinson's disease (PD) and parkinsonian syndromes are challenging, and recent discoveries regarding their genetic pathways have led to potential gene-specific treatment trials. CASES: We report 3 X-linked levodopa (l-dopa)-responsive parkinsonism-epilepsy syndrome cases due to a hemizygous variant in the phosphoglycerate kinase 1 (PGK1) gene. The likely pathogenic variant NM_000291.4 (PGK1):c.950G > A;p.(Gly317Asp) was identified in a hemizygous state. LITERATURE REVIEW: Only 8 previous cases have linked this phenotype to PGK1, a gene more commonly associated with hemolytic anemia and myopathy. The unusual association of epilepsy, psychiatric symptoms, action tremor, limb dystonia, cognitive symptoms, and l-dopa-responsive parkinsonism must draw attention to PGK1 mutations, especially because this gene is absent from most commercial hereditary parkinsonism panels. CONCLUSIONS: This report aims to shed light on an overlooked gene that causes hereditary parkinsonian syndromes. Further research regarding genetic pathways in PD may provide a better understanding of its pathophysiology and open possibilities for new disease-modifying trials, such as SNCA, LRRK2, PRKN, PINK1, and DJ-1 genes.

Probable 4-Repeat Tauopathy Criteria Predict Brain Amyloid Negativity, Distinct Clinical Features, and FDG-PET/MRI Neurodegeneneration Patterns in Corticobasal Syndrome.

BACKGROUND: Corticobasal syndrome (CBS) is associated with diverse underlying pathologies, including the four-repeat (4R)-tauopathies. The Movement Disorders Society (MDS) criteria for progressive supranuclear palsy (PSP) proposed the novel category "probable 4R-tauopathy" to address the phenotypic overlap between PSP and corticobasal degeneration (CBD). OBJECTIVES: To investigate the clinical ability of the MDS-PSP criteria for probable 4R-tauopathy in predicting a negative amyloid-PET in CBS. Additionally, this study aims to explore CBS patients classified as 4R-tauopathy concerning their clinical features and neuroimaging degeneration patterns. METHODS: Thirty-two patients with probable CBS were prospectively evaluated and split into those who fulfilled or did not fulfill the 4R-tauopathy criteria (CBS-4RT+ vs. CBS-4RT-). All patients underwent positron emission tomographies (PET) with [18 F]fluorodeoxyglucose and [11 C]Pittsburgh Compound-B (PIB) on a hybrid PET-MRI scanner to perform multimodal quantitative comparisons with a control group. RESULTS: Eleven patients were clinically classified as CBS-4RT+, and only one had a positive PIB-PET. The CBS-4RT+ classification had 92% specificity, 52% sensitivity, and 69% accuracy in predicting a negative PIB-PET. The CBS-4RT+ group presented with dysarthria and perseveration more often than the CBS-4RT- group. Moreover, the CBS-4RT+ group showed a prominent frontal hypometabolism extending to the supplementary motor area and striatum, and brain atrophy at the anterior cingulate and bilateral striata. CONCLUSIONS: The 4R-tauopathy criteria were highly specific in predicting a negative amyloid-PET in CBS. Patients classified as 4R-tauopathy presented distinct clinical aspects, as well as brain metabolism and atrophy patterns previously associated with tauopathies.

Tic Status in Tourette Syndrome Due to Depletion of the Deep Brain Stimulation Battery.

In this case report, a patient with Tourette syndrome was admitted to the emergency department with nonrhythmic, continuous, generalized hyperkinetic movements associated with muscle contractions in the trunk, neck, and upper and lower limbs caused by depletion of the deep brain stimulation battery.

The Discourse Profile in Corticobasal Syndrome: A Comprehensive Clinical and Biomarker Approach.

The aim of this study was to characterize the oral discourse of CBS patients and to verify whether measures obtained during a semi-spontaneous speech production could differentiate CBS patients from controls. A second goal was to compare the performance of patients with CBS probably due to Alzheimer's disease (CBS-AD) pathology and CBS not related to AD (CBS-non-AD) in the same measures, based on the brain metabolic status (FDG-PET) and in the presence of amyloid deposition (amyloid-PET). Results showed that CBS patients were significantly different from controls in speech rate, lexical level, informativeness, and syntactic complexity. Discursive measures did not differentiate CBS-AD from CBS-non-AD. However, CBS-AD displayed more lexical-semantic impairments than controls, a profile that is frequently reported in patients with clinical AD and the logopenic variant of primary progressive aphasia (lvPPA). CBS-non-AD presented mainly with impairments related to motor speech disorders and syntactic complexity, as seen in the non-fluent variant of PPA.

Diagnosis and management of Parkinson's disease dementia and dementia with Lewy bodies: recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology.

Parkinson's disease dementia (PDD) and dementia with Lewy bodies (DLB) represent the second most common type of degenerative dementia in patients aged 65 years and older, leading to progressive cognitive dysfunction and impaired quality of life. This study aims to provide a consensus based on a systematic Brazilian literature review and a comprehensive international review concerning PDD and DLB. Moreover, we sought to report on and give recommendations about the best diagnostic approaches focusing on primary and secondary care. Based on the available data, we recommend clinicians to apply at least one brief global cognitive instrument to assess PDD, such as the Mini-Mental State Examination and preferably the Montreal Cognitive Assessment and the Addenbrooke's Cognitive Examination-Revised. Validated instruments to accurately assess functional abilities in Brazilian PD patients are still incipient. Further studies should focus on biomarkers with Brazilian cohorts.

A demência da doença de Parkinson (DDP) e a demência com corpos de Lewy (DCL) representam a segunda causa mais comum de demência neurodegenerativa em pessoas com mais de 65 anos, ocasionando progressivo declínio cognitivo e comprometimento da qualidade de vida. O presente estudo tem como objetivo prover um consenso de especialistas sobre a DDP e DCL, baseado em revisão sistemática da literatura brasileira e revisão não-sistemática de literatura internacional. Ademais, tal estudo visa promover informação e conceder recomendações sobre abordagem diagnóstica, com foco nos níveis de atenção primária e secundária em saúde. Com base nos dados disponíveis, recomendamos que os profissionais realizem pelo menos um breve instrumento cognitivo global, como o Mini-Exame do Estado Mental, contudo de preferência optem pela Avaliação Cognitiva de Montreal e o Exame Cognitivo de Addenbrooke-Revisado. Observa-se uma carência de instrumentos validados para a avaliação precisa das habilidades funcionais em pacientes brasileiros com DDP e DCL. Além disso, mais estudos focando em biomarcadores com coortes brasileiras também são necessários.

Diagnóstico e manejo da demência da doença de Parkinson e demência com corpos de Lewy: recomendações do Departamento Científico de Neurologia Cognitiva e do Envelhecimento da Academia Brasileira de Neurologia / Diagnosis and management of Parkinson's disease dementia and dementia with Lewy bodies: recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology

RESUMO A demência da doença de Parkinson (DDP) e a demência com corpos de Lewy (DCL) representam a segunda causa mais comum de demência neurodegenerativa em pessoas com mais de 65 anos, ocasionando progressivo declínio cognitivo e comprometimento da qualidade de vida. O presente estudo tem como objetivo prover um consenso de especialistas sobre a DDP e DCL, baseado em revisão sistemática da literatura brasileira e revisão não-sistemática de literatura internacional. Ademais, tal estudo visa promover informação e conceder recomendações sobre abordagem diagnóstica, com foco nos níveis de atenção primária e secundária em saúde. Com base nos dados disponíveis, recomendamos que os profissionais realizem pelo menos um breve instrumento cognitivo global, como o Mini-Exame do Estado Mental, contudo de preferência optem pela Avaliação Cognitiva de Montreal e o Exame Cognitivo de Addenbrooke-Revisado. Observa-se uma carência de instrumentos validados para a avaliação precisa das habilidades funcionais em pacientes brasileiros com DDP e DCL. Além disso, mais estudos focando em biomarcadores com coortes brasileiras também são necessários.

ABSTRACT Parkinson's disease dementia (PDD) and dementia with Lewy bodies (DLB) represent the second most common type of degenerative dementia in patients aged 65 years and older, leading to progressive cognitive dysfunction and impaired quality of life. This study aims to provide a consensus based on a systematic Brazilian literature review and a comprehensive international review concerning PDD and DLB. Moreover, we sought to report on and give recommendations about the best diagnostic approaches focusing on primary and secondary care. Based on the available data, we recommend clinicians to apply at least one brief global cognitive instrument to assess PDD, such as the Mini-Mental State Examination and preferably the Montreal Cognitive Assessment and the Addenbrooke's Cognitive Examination-Revised. Validated instruments to accurately assess functional abilities in Brazilian PD patients are still incipient. Further studies should focus on biomarkers with Brazilian cohorts.

Magnetic resonance and dopamine transporter imaging for the diagnosis of Parkinson´s disease: a narrative review.

BACKGROUND: the diagnosis of Parkinson's disease (PD) can be challenging, especially in the early stages, albeit its updated and validated clinical criteria. Recent developments on neuroimaging in PD, altogether with its consolidated role of excluding secondary and other neurodegenerative causes of parkinsonism, provide more confidence in the diagnosis across the different stages of the disease. This review highlights current knowledge and major recent advances in magnetic resonance and dopamine transporter imaging in aiding PD diagnosis. OBJECTIVE: This study aims to review current knowledge about the role of magnetic resonance imaging and neuroimaging of the dopamine transporter in diagnosing Parkinson's disease. METHODS: We performed a non-systematic literature review through the PubMed database, using the keywords "Parkinson", "magnetic resonance imaging", "diffusion tensor", "diffusion-weighted", "neuromelanin", "nigrosome-1", "single-photon emission computed tomography", "dopamine transporter imaging". The search was restricted to articles written in English, published between January 2010 and February 2022. RESULTS: The diagnosis of Parkinson's disease remains a clinical diagnosis. However, new neuroimaging biomarkers hold promise for increased diagnostic accuracy, especially in earlier stages of the disease. CONCLUSION: Future validation of new imaging biomarkers bring the expectation of an increased neuroimaging role in the diagnosis of PD in the following years.

Progressive supranuclear palsy and corticobasal degeneration: novel clinical concepts and advances in biomarkers.

BACKGROUND: Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are sporadic adult-onset primary tauopathies clinically classified among the atypical parkinsonian syndromes. They are intrinsically related with regard to their clinical features, pathology, biochemistry, and genetic risk factors. OBJECTIVES: This review highlights the current knowledge on PSP and CBD, focusing on evolving clinical concepts, new diagnostic criteria, and advances in biomarkers. METHODS: We performed a non-systematic literature review through the PubMed database. The search was restricted to articles written in English, published from 1964 to date. RESULTS: Clinicopathologic and in vivo biomarkers studies have broadened PSP and CBD clinical phenotypes. They are now recognized as a range of motor and behavioral syndromes associated with underlying 4R-tauopathy neuropathology. The Movement Disorders Society PSP diagnostic criteria included clinical variants apart from the classical description, increasing diagnostic sensitivity. Meanwhile, imaging biomarkers have explored the complexity of symptoms and pathological processes related to corticobasal syndrome and CBD. CONCLUSIONS: In recent years, several prospective or clinicopathologic studies have assessed clinical, radiological, and fluid biomarkers that have helped us gain a better understanding of the complexity of the 4R-tauopathies, mainly PSP and CBD.

Translation, cross-cultural adaptation, and validity of the Brazilian version of the Cognitive Function Instrument.

Subjective cognitive decline (SCD) is defined as a self-perception of a progressive cognitive impairment, which is not detected objectively through neuropsychological tests. The Alzheimer's Disease Cooperative Study developed the Cognitive Function Instrument (CFI) to evaluate individuals with SCD. The CFI consists of two versions, namely, a self-report and a partner report. Objective: This study aimed to translate CFI into Brazilian Portuguese, perform a cross-cultural adaptation, and validate the Brazilian version. Methods: The translation and transcultural adaptation process consisted of six stages, and the preliminary version was answered by a sample of individuals recruited among the patients' caregivers from a cognitive neurology outpatient clinic. Finally, the final Brazilian version of the CFI was applied to a sample of nondemented older adults to validate the instrument, which was divided into with and without SCD, according to the answer "yes" for the question: "Do you feel like your memory is becoming worse?". Results: The final version of CFI showed a high level of acceptability as an assessment tool in nondemented older adults. Participants with SCD had higher scores in the CFI self-report compared with those without complaints. In the receiver operating characteristic curve analysis, the area under the curve of the CFI self-report was 0.865 (95% confidence interval 0.779-0.951), and the cutoff score of 2.0 was the one that best distinguished the SCD group from the control group, with a sensitivity of 73.3% and a specificity of 81.5%. Conclusions: CFI proved to be an instrument with good accuracy and easy applicability to identify older adults with SCD.

O declínio cognitivo subjetivo (DCS) é definido como uma autopercepção de um comprometimento cognitivo progressivo, não detectado objetivamente por meio de testes neuropsicológicos. O Alzheimer's Disease Cooperative Study desenvolveu o instrumento de função cognitiva (IFC) para avaliar indivíduos com DCS. O IFC existe em duas versões, uma do paciente e outra do acompanhante. Objetivo: O objetivo deste estudo foi traduzir para o português brasileiro, fazer uma adaptação transcultural e validar a versão brasileira do IFC. Métodos: O processo de tradução e adaptação transcultural consistiu em seis etapas, e a versão preliminar foi respondida por uma amostra de voluntários recrutados entre os cuidadores de pacientes de um ambulatório de Neurologia Cognitiva. Por fim, a versão brasileira final do IFC foi aplicada a idosos sem demência, que foram divididos naqueles com e sem DCS de acordo com a resposta "sim" à questão: "Você sente que a sua memória está piorando?". Resultados: A versão final do IFC mostrou alto nível de aceitabilidade como ferramenta de avaliação em idosos sem demência. Os participantes com DCS tiveram pontuações mais altas na versão do paciente em comparação com aqueles sem queixas. Nas análises da curva característica de operação do receptor (ROC), a área sobre a curva da versão do paciente foi de 0,865 (intervalo de confiança [IC95%] 0,779­0,951) e a pontuação de corte de 2,0 foi a que melhor distinguiu o grupo com DCS dos controles, com sensibilidade de 73,3% e especificidade de 81,5%. Conclusões: O IFC mostrou-se um instrumento de boa acurácia e de fácil aplicabilidade para identificar idosos com DCS.

Progressive supranuclear palsy and corticobasal degeneration: novel clinical concepts and advances in biomarkers / Paralisia supranuclear progressiva e degeneração corticobasal: novos conceitos clínicos e avanços em biomarcadores

ABSTRACT Background: Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are sporadic adult-onset primary tauopathies clinically classified among the atypical parkinsonian syndromes. They are intrinsically related with regard to their clinical features, pathology, biochemistry, and genetic risk factors. Objectives: This review highlights the current knowledge on PSP and CBD, focusing on evolving clinical concepts, new diagnostic criteria, and advances in biomarkers. Methods: We performed a non-systematic literature review through the PubMed database. The search was restricted to articles written in English, published from 1964 to date. Results: Clinicopathologic and in vivo biomarkers studies have broadened PSP and CBD clinical phenotypes. They are now recognized as a range of motor and behavioral syndromes associated with underlying 4R-tauopathy neuropathology. The Movement Disorders Society PSP diagnostic criteria included clinical variants apart from the classical description, increasing diagnostic sensitivity. Meanwhile, imaging biomarkers have explored the complexity of symptoms and pathological processes related to corticobasal syndrome and CBD. Conclusions: In recent years, several prospective or clinicopathologic studies have assessed clinical, radiological, and fluid biomarkers that have helped us gain a better understanding of the complexity of the 4R-tauopathies, mainly PSP and CBD.

RESUMO Antecedentes: A paralisia supranuclear progressiva (PSP) e a degeneração corticobasal (DCB) são taupatias esporádicas primárias clinicamente classificadas no grupo das síndromes parkinsonianas atípicas. Ambas estão intrinsecamente relacionadas no que concerne aos aspectos clínicos, patológicos, bioquímicos e genéticos. Objetivos: Abordar os avanços recentes no conhecimento da PSP e DCB, focando na evolução dos conceitos clínicos, critérios diagnósticos, e avanços em biomarcadores. Métodos: Trata-se de uma revisão não-sistemática através da base de dados PubMed. Foram revisados artigos escritos em língua inglesa, publicados desde 1964 até a presente data. Resultados: Estudos com biomarcadores e análises postmortem ampliaram os fenótipos conhecidos da PSP e DCB. Tais doenças englobam um espectro amplo de síndromes motoras e cognitivas, associadas a neuropatologia tau com isoforma predominante 4R. O atual critério para PSP estabeleceu novas variantes clínicas para além da descrição clássica, aumentando a sensibilidade diagnóstica. Concomitantemente, biomarcadores in vivo exploraram a complexidade dos sintomas e processos patológicos relacionados à síndrome e degeneração corticobasal. Conclusões: Recentemente, alguns estudos prospectivos e clinicopatológicos investigaram aspectos clínicos, radiológicos e de biofluidos, que ajudaram a melhor compreender a heterogeneidade e complexidade clínica das taupatias 4R, sobretudo PSP e DCB.

Magnetic resonance and dopamine transporter imaging for the diagnosis of Parkinson´s disease: a narrative review / Ressonância magnética e neuroimagem do transportador de dopamina no diagnóstico da doença de Parkinson: uma revisão narrativa

ABSTRACT Background: the diagnosis of Parkinson's disease (PD) can be challenging, especially in the early stages, albeit its updated and validated clinical criteria. Recent developments on neuroimaging in PD, altogether with its consolidated role of excluding secondary and other neurodegenerative causes of parkinsonism, provide more confidence in the diagnosis across the different stages of the disease. This review highlights current knowledge and major recent advances in magnetic resonance and dopamine transporter imaging in aiding PD diagnosis. Objective: This study aims to review current knowledge about the role of magnetic resonance imaging and neuroimaging of the dopamine transporter in diagnosing Parkinson's disease. Methods: We performed a non-systematic literature review through the PubMed database, using the keywords "Parkinson", "magnetic resonance imaging", "diffusion tensor", "diffusion-weighted", "neuromelanin", "nigrosome-1", "single-photon emission computed tomography", "dopamine transporter imaging". The search was restricted to articles written in English, published between January 2010 and February 2022. Results: The diagnosis of Parkinson's disease remains a clinical diagnosis. However, new neuroimaging biomarkers hold promise for increased diagnostic accuracy, especially in earlier stages of the disease. Conclusion: Future validation of new imaging biomarkers bring the expectation of an increased neuroimaging role in the diagnosis of PD in the following years.

RESUMO Antecedentes: O diagnóstico da doença de Parkinson (DP) pode ser desafiador, principalmente nas fases iniciais da doença, embora tenha critérios clínicos atualizados e validados. Os avanços recentes em neuroimagem na DP, além do seu papel já consolidado de excluir causas secundárias e outras causas neurodegenerativas de parkinsonismo, tem contribuído para uma maior confiabilidade no diagnóstico em diferentes estágios da doença. Nesta revisão, nós destacamos os principais avanços de ressonância magnética e imagem do transportador de dopamina em auxiliar o diagnóstico de DP. Objetivo: realizar uma revisão acerca do conhecimento atual sobre o papel da ressonância magnética e imagem do transportador de dopamina no diagnóstico de doença de Parkinson. Método: Realizamos uma revisão não sistemática da literatura através da base de dados PubMed, utilizando as palavras-chave "Parkinson", "magnetic resonance imaging", "diffusion tensor", "diffusion-weighted", "neuromelanin", "nigrosome-1", "single-photon emission computed tomography", "dopamine transporter imaging". A busca foi restrita a artigos escritos em inglês, publicados entre janeiro de 2010 e fevereiro de 2022. Resultados: O diagnóstico de doença de Parkinson continua sendo um diagnóstico clínico, contudo, novos biomarcadores de neuroimagem são promissores para o aumento da acurácia diagnóstica, especialmente em fases mais precoces da doença. Conclusão: A validação futura de novos biomarcadores de imagem traz a expectativa de um maior papel da neuroimagem no diagnóstico de doença de Parkinson nos próximos anos.

Metabolic and Structural Signatures of Speech and Language Impairment in Corticobasal Syndrome: A Multimodal PET/MRI Study.

Introduction: Corticobasal syndrome (CBS) is a progressive neurological disorder related to multiple underlying pathologies, including four-repeat tauopathies, such as corticobasal degeneration and progressive supranuclear palsy, and Alzheimer's disease (AD). Speech and language are commonly impaired, encompassing a broad spectrum of deficits. We aimed to investigate CBS speech and language impairment patterns in light of a multimodal imaging approach. Materials and Methods: Thirty-one patients with probable CBS were prospectively evaluated concerning their speech-language, cognitive, and motor profiles. They underwent positron emission tomography with [18F]fluorodeoxyglucose (FDG-PET) and [11C]Pittsburgh Compound-B (PIB-PET) on a hybrid PET-MRI machine to assess their amyloid status. PIB-PET images were classified based on visual and semi-quantitative analyses. Quantitative group analyses were performed on FDG-PET data, and atrophy patterns on MRI were investigated using voxel-based morphometry (VBM). Thirty healthy participants were recruited as imaging controls. Results: Aphasia was the second most prominent cognitive impairment, presented in 67.7% of the cases, following apraxia (96.8%). We identified a wide linguistic profile, ranging from nonfluent variant-primary progressive aphasia to lexical-semantic deficits, mostly with impaired verbal fluency. PIB-PET was classified as negative (CBS-A- group) in 18/31 (58%) and positive (CBS-A+ group) in 13/31 (42%) patients. The frequency of dysarthria was significantly higher in the CBS-A- group than in the CBS-A+ group (55.6 vs. 7.7%, p = 0.008). CBS patients with dysarthria had a left-sided hypometabolism at frontal regions, with a major cluster at the left inferior frontal gyrus and premotor cortex. They showed brain atrophy mainly at the opercular frontal gyrus and putamen. There was a positive correlation between [18F]FDG uptake and semantic verbal fluency at the left inferior (p = 0.006, R 2 = 0.2326), middle (0.0054, R 2 = 0.2376), and superior temporal gyri (p = 0.0066, R 2 = 0.2276). Relative to the phonemic verbal fluency, we found a positive correlation at the left frontal opercular gyrus (p = 0.0003, R 2 = 0.3685), the inferior (p = 0.0004, R 2 = 0.3537), and the middle temporal gyri (p = 0.0001, R 2 = 0.3993). Discussion: In the spectrum of language impairment profile, dysarthria might be helpful to distinguish CBS patients not related to AD. Metabolic and structural signatures depicted from this feature provide further insights into the motor speech production network and are also helpful to differentiate CBS variants.

Language in corticobasal syndrome: a systematic review.

Language is commonly impacted in corticobasal syndrome (CBS). However, the profile and type of language assessment in CBS are poorly studied. OBJECTIVE: To identify language impairments in CBS. METHODS: A search was performed in the Medline/PubMed database, according to the PRISMA criteria, using the keywords "corticobasal syndrome" OR "corticobasal degeneration" AND "language". Articles on CBS covering language assessment that were written in English were included, with no constraints on the publication date. RESULTS: A total of 259 articles were found and 35 were analyzed, consisting of 531 participants. Twenty-eight studies showed heterogeneous language deficits and seven mentioned nonfluent primary progressive aphasia. The most used tests were the Western Aphasia Battery (8 studies) and the Boston Naming Test (8 studies). CONCLUSION: It was not possible to identify a unique linguistic profile in CBS.

A linguagem encontra-se comumente alterada na síndrome corticobasal (SCB). No entanto, o perfil e a forma de avaliação da linguagem na SCB são pouco estudados. OBJETIVO: identificar as alterações de linguagem na SCB. MÉTODO: Realizou-se uma busca na base de dados Medline/PubMed, com as palavras-chave "síndrome corticobasal" OU "degeneração corticobasal" E "linguagem". Artigos sobre SCB envolvendo avaliação de linguagem, escritos em inglês, foram incluídos, sem restrição de data de publicação. RESULTADOS: Foram encontrados 259 artigos, e 35 estudos foram analisados, abrangendo 531 sujeitos. Um total de 28 estudos mostraram déficits heterogêneos de linguagem, e sete mencionaram afasia progressiva primária não-fluente. Os testes mais utilizados foram Western Aphasia Battery (8 estudos) e o Teste de Nomeação de Boston (8 estudos). CONCLUSÃO: Não foi possível identificar um perfil linguístico único em pacientes com SCB.

Acute chorea: case series from the emergency room of a Brazilian tertiary-level center.

BACKGROUND: Chorea is a movement disorder characterized by random, brief and migratory involuntary muscle contractions. It is defined as acute when present within hours to days. Three main causes for this scenario have emerged as most likely: vascular, toxic-metabolic and inflammatory. OBJECTIVES: To identify the prevalence of the main etiologies and major clinical findings of acute chorea in the emergency room of a tertiary-level referral center; and to suggest an approach for guiding the diagnostic workup and clinical management. METHODS: We retrospectively reviewed the clinical aspects and neuroimaging data of 10 patients presenting with acute chorea at the neurological emergency room of our hospital from 2015 to 2019. RESULTS: Stroke was the most common etiology (50% of the cases). All of them were ischemic. It was noteworthy that only one case demonstrated the classical ischemic topographic lesion at the contralateral subthalamic nuclei. Regarding nonvascular etiologies, nonketotic hyperglycemia was the major cause, followed by drug-related chorea. One patient showed inflammatory etiology, which was probably Sydenham chorea reactivation. CONCLUSION: Acute chorea is an uncommon and challenging problem at the emergency room, often associated with potentially treatable causes. We suggest that use of the acronym DANCE (Diagnosis of chorea, Acute stroke protocol, Normal glucose levels, Check neuroimaging, Exposure to drugs) could form a potential initial approach in the evaluation, in order to emphasize causes that require prompt proper management (e.g. thrombolysis).