Characteristics of clinical and neuroimage findings in patients with corticobasal syndrome / 中华神经科杂志

Objective:To investigate the clinical, neuropsychological, and neuroimage characteristics in patients with corticobasal syndrome (CBS), and to elucidate the exact diagnosis of CBS patients.Methods:Twelve CBS cases admitted to the Department of Neurology, Huashan Hosiptal,Fudan University from April 2019 to July 2021 were retrospectively enrolled in this study. Those data, including clinical features (demographic data and clinical characteristics of cortical dysfunction and movement disorder), neuropsychological assessment [Mini-Mental State Examination (MMSE) and Montreal Cognitive Assessment (MoCA) scales score], brain magnetic resonance imaging (MRI) and multi-mode positron emission tomography (PET)/CT, were collected and carefully reviewed. Exact diagnosis of these patients was given according to the disease diagnosis criteria.Results:Cortical dysfunction and asymmetrical movement disorders were found in all cases, with poor response to levodopa. Patients suffered from cognitive impairment (MMSE score 16.16±9.82, MoCA score 13.44±7.35). The cranial MRI demonstrated significant asymmetric atrophy of frontal and parietal lobes, especially in the pre- and post-central gyrus. Fluorodeoxyglucose PET of 12 patients showed asymmetric frontal lobe and basal ganglia (especially caudate and putamen) hypometabolism (obviously on the contralateral side of the affected limb). Tau PET was implemented in 11 patients and displayed that abnormal tau protein deposition was positive in the cortex and/or subcortex in all patients. Of the 4 cases, who completed amyloid PET, amyloid protein deposition was positive in the cortex of 2 patients. As a result, 6 patients were diagnosed as progressive supranuclear palsy, 1 patient was diagnosed as corticobasal degeneration, and 5 patients were diagnosed as Alzheimer′s disease.Conclusions:The etiology of CBS is heterogeneous. The combination of clinical manifestation, cranial MRI and multi-mode PET/CT helps the differential diagnosis of CBS.

Language in corticobasal syndrome: a systematic review / Linguagem na síndrome corticobasal: uma revisão sistemática

ABSTRACT. 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.

RESUMO. 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.

Pharmacological interventions in corticobasal degeneration: a review / Intervenções farmacológicas na degeneração corticobasal: uma revisão

ABSTRACT. Corticobasal degeneration (CBD) is a sporadic tauopathy that presents with a varied combination of motor, cognitive, and behavioral features, making its diagnosis difficult. CBD has high morbidity and poor prognosis, with no effective therapy at present. We searched the PubMed/MEDLINE database for articles published from 1990 to 2019, using the keywords "corticobasal degeneration" AND "treatment." The PRISMA method was adopted. Retrieved articles were characterized as having one of two methodological approaches: (1) studies aimed at primary tauopathy treatment and (2) symptomatic management. Review articles (based on CBD expert groups), case reports, case series, and pilot clinical trials were selected. Few attempts have been made to study drug options and drug efficacy in CBD systematically, and an effective treatment is not yet available. Treatment is symptomatic and based on similarity with other diseases due to the scarcity of studies specifically addressing CBD. CBD seems not to spark interest in more clinical trials for its low prevalence and reliability in clinical diagnosis.

RESUMO. A degeneração corticobasal (DCB) é uma tauopatia esporádica que se apresenta com uma combinação variada de características motoras, cognitivas e comportamentais, dificultando seu diagnóstico. O CBD tem alta morbidade e mau prognóstico, sem terapia efetiva no momento. Pesquisamos o banco de dados PubMed / MEDLINE para artigos publicados de 1990 a 2019, usando as palavras-chave "degeneração corticobasal" e "tratamento". O método PRISMA foi adotado. Os artigos recuperados foram caracterizados como tendo uma de duas abordagens metodológicas: (1) estudos voltados para o tratamento da tauopatia primária e (2) manejo sintomático. Artigos de revisão (baseados em grupos de especialistas em CBD), relatos de casos, séries de casos e ensaios clínicos piloto foram selecionados. Poucas tentativas foram feitas para estudar as opções de drogas e eficácia de drogas no CBD de forma sistemática, e um tratamento eficaz ainda não está disponível. O tratamento é sintomático e baseado na semelhança com outras doenças devido à escassez de estudos que abordem especificamente o CBD. O CBD parece não despertar o interesse em mais ensaios clínicos por sua baixa prevalência e confiabilidade no diagnóstico clínico.

Clinical and neuroimaging features in 6 patients with corticobasal syndrome / 中华内科杂志

The clinical and imaging data in 6 patients with corticobasal syndrome were retrospectively analyzed. Six patients presented asymmetric clinical symptoms, including 5 with cognitive impairment, 6 with emotional disorders, 2 with cortical sensory deficit, 5 with lalopathy, and 4 with apraxia. All patients developed limb dystonia and limb or trunk stiffness, 4 with tumble, 4 with bradykinesia, and 2 with tremor. Brain magnetic resonance imaging (MRI) showed that 4 patients had unilateral cerebral atrophy and 2 had mild atrophy of bilateral hippocampus. Localized low glucose metabolism in the unilateral cerebral lobe was seen in four patients by positron emission computed tomography (PET) examination, suggesting that PET is helpful for the diagnosis of corticobasal syndrome.

Extending the range of differential diagnosis of chronic traumatic encephalopathy of the boxer: Insights from a case report / Ampliando a gama de diagnósticos diferencias da encefalopatia traumática crônica do pugilista: um relato de caso ilustrativo

ABSTRACT Sports activities associated with repetitive cranial trauma have become a fad and are popular in gyms and even among children. It is important to consistently characterize the consequences of such sports activities in order to better advise society on the real risks to the central nervous system. We present the case of a former boxer reporting cognitive and behavioral symptoms that began six years after his retirement as a boxer, evolving progressively with parkinsonian and cerebellar features suggestive of probable chronic traumatic encephalopathy (CTE). Using our case as a paradigm, we extended the range of differential diagnosis of CTE, including corticobasal degeneration, multiple system atrophy, vitamin B12 deficiency, neurosyphilis, frontotemporal dementia and Alzheimer's disease.

RESUMO As atividades esportivas associadas ao trauma craniano repetitivo tornaram-se uma moda e são populares nas academias e entre as crianças. É importante fazer uma caracterização consistente das consequências de tais atividades esportivas, a fim de aconselhar melhor uma sociedade sobre os riscos reais para o sistema nervoso central. Apresentamos um antigo boxeador relatando sintomas cognitivos e comportamentais que começaram seis anos após sua aposentadoria como boxeador e evoluiu progressivamente com características parkinsonianas e cerebelares sugestivas de provável encefalopatia traumática crônica (ETC). Usando nosso caso como paradigma, ampliamos a gama de diagnóstico diferencial de ETC, incluindo degeneração corticobasal, atrofia de múltiplos sistemas, deficiência de vitamina B12, neurossífilis, demência frontotemporal e doença de Alzheimer.

Analysis on the clinical, neuroimaging and pathological characteristics in patients with corticobasal syndrome / 中华老年医学杂志

Objective To investigate the clinical,neuroimaging and pathological features of patients with corticobasal syndrome (CBS).Methods Three CBS patients underwent neuropsychological assessment,MRI scan,18F-FDG PET scan,and 11C-PiB PET scan,and the test results were analyzed.Results All three patients showed poor response to levodopa treatment and asymmetric dyskinesia including rigidity,tremor,dystonia and abnormalities in posture and gait.One patient showed apraxia and three patients presented with cognitive impairment.The crania MRI demonstrated mild cerebral atrophy which was slightly more severe in the contralateral side of the initially affected limb.The (18)F-FDG PET/CT scan revealed asymmetric decreased metabolism in the frontal,parietal,temporal,and insular lobe,as well as in basal ganglia.The results of (11)C-PiB PET showed that amyloid beta-peptide (A Beta) deposition in the cortex was positive in one patient,and negative in the other two patients.Conclusions Corticobasal syndrome is characterized by asymmetric dyskinesia and cognitive impairment,and often associated with apraxia,cortical sensory deficits,and alien limb phenomena.The MRI and FDG PET are helpful for CBS diagnosis,and the PiB PET facilitates the pathological diagnosis.

Corticobasal syndrome: a diagnostic conundrum / O enigmático diagnóstico da síndrome Corticobasal

ABSTRACT Corticobasal syndrome (CBS) is an atypical parkinsonian syndrome of great interest to movement disorder specialists and behavioral neurologists. Although originally considered a primary motor disorder, it is now also recognized as a cognitive disorder, usually presenting cognitive deficits before the onset of motor symptoms. The term CBS denotes the clinical phenotype and is associated with a heterogeneous spectrum of pathologies. Given that disease-modifying agents are targeting the pathologic process, new diagnostic methods and biomarkers are being developed to predict the underlying pathology. The heterogeneity of this syndrome in terms of clinical, radiological, neuropsychological and pathological aspects poses the main challenge for evaluation.

RESUMO A síndrome corticobasal é classificada dentro do grupo das síndromes parkinsonianas atípicas, e atualmente desperta interesse em neurologistas especialistas em distúrbios do movimento e neurologia cognitiva e comportamental. Inicialmente considerada como uma síndrome tipicamente motora, hoje se reconhece a importância dos achados cognitivos na apresentação, podendo ocorrer mesmo na ausência de alterações motoras. Tal designação refere-se à síndrome clínica e é associada a várias patologias subjacentes. Tendo em vista que drogas modificadoras da doença estão focando na patologia de base, novos métodos diagnósticos de imagem e outros biomarcadores estão sendo desenvolvidos para predizer o processo patológico específico antemortem. A heterogeneidade clínica e patológica desta entidade, portanto, é o maior desafio a ser desvendado.

Corticobasal Syndrome Initially Presenting with Apraxia of Speech

Apraxia of speech is a disorder of speech programming and planning. We report a 64-year-old woman presenting with progressive apraxia of speech that evolved into right-side-dominant akinesia-rigidity syndrome over a 7-year period. Her motor symptoms were highly consistent with corticobasal syndrome, and neuroimaging revealed atrophy and hypometabolism in the left premotor and supplementary motor areas, which are relevant to apraxia of speech. This case illustrates that apraxia of speech may be an initial manifestation of corticobasal syndrome.

Clinical Approach to Progressive Supranuclear Palsy

Sixty years ago, Steele, Richardson and Olszewski designated progressive supranuclear palsy (PSP) as a new clinicopathological entity in their seminal paper. Since then, in addition to the classic Richardson's syndrome (RS), different clinical phenotypic presentations have been linked with this four-repeat tauopathy. The clinical heterogeneity is associated with variability of regional distribution and severity of abnormal tau accumulation and neuronal loss. In PSP subtypes, the presence of certain clinical pointers may be useful for antemortem prediction of the underlying PSP-tau pathology. Midbrain atrophy on conventional MRI correlates with the clinical phenotype of RS but is not predictive of PSP pathology. Cerebrospinal fluid biomarkers and tau ligand positron emission tomography are promising biomarkers of PSP. A multidisciplinary approach to meet the patients' complex needs is the current core treatment strategy for this devastating disorder.