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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.
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Objective To study the pathological features of argyrophilic grains in normal aging brain, AD, PD and progressive superior nuclear paralysis patients. Methods Brain tissue samples taken from 5 AD, 3 PD, 2 progressive superior nuclear paralysis patients with complete clinico-pathological data and 4 normal aging brain subjects were stained with HE, Luxol fast blue and Gallyas-Braak silver respectively. Aβ, tau, α-synuclein and P62 antibodies were detected by microscopy with immunohistochemical staining. The pathological features of argyrophilic grains were recorded. Results The Gallyas-Braak silver staining showed argyrophilic grain structure in 4 out of the 14 amygdaloid nucleus tissue samples (2 from AD patients, 1 from progressive superior nuclear paralysis patients and 1 from normal aging brain patients) with a positive rate of 28.6％. The immunohistochemical staining showed positive tau and P62 antibodies. Conclusion Argyrophilic grain lesion is not uncommon in aging-related neurodegenerative diseases such as normal aging brain, AD and progressive superior nuclear paralysis and can thus produce its superposition effect on the clinical symptoms of cognitive impairment in AD and progressive superior nuclear paralysis patients.
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Objective To evaluate the effectiveness of quantitative midbrain measurements in differentiating progressive supranuclear palsy (PSP) from multiple system atrophy (MSA) and Parkinson's disease (PD).Methods Quantitative midbrain measurements,including midbrain width (MW),midbrain to pons ratio (M/P) and magnetic resonance parkinsonism index (MRPI),were performed in patients with parkinsonism who were diagnosed in the Movement Disorder Clinic of Peking Union Medical College Hospital during the period of January to September 2017.A cross-sectional study was conducted in the series to evaluate the effectiveness of these quantitative measurements.Results Ten PSP-RS,15 MSA-P and 49 PD patients were included in this study.The values of MW,M/P and MRPI in PSP-RS patients were (8.21 ± 1.30) mm,0.49 ±0.06 and 15.26 ±4.53,respectively,with statistically significant difference compared toMSA-P ((10.24±0.77) mm,0.65 ±0.09,7.75 ±2.71) and PD patients ((10.53 ±0.93) mm,0.62±0.06,9.86 ±2.46;F=24.27,18.37,21.47,all P＜0.01).After adjusting for age,disease course and gender,analysis of covariance suggested significant correlation between the quantitative midbrain measurements and diagnosis of these diseases.Receiver operating characteristic curve indicated that MW ≤9.4 mm,M/P≤0.57 and MRPI ＞ 10.77 showed ideal sensitivity and specificity (90.0％ and 92.1％,80.0％ and 93.7％,100.0％ and 82.5％) in differentiating PSP-RS from MSA-P and PD.Conclusion Quantitative measurement of midbrain atrophy is useful in differentiating PSP-RS from MSA-P and PD.
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Abstract Parkinson's disease is one of the most common neurodegenerative diseases. Clinically, it is characterized by motor symptoms. Parkinson's disease should be differentiated from atypical parkinsonism conditions. Conventional magnetic resonance imaging is the primary imaging method employed in order to facilitate the differential diagnosis, and its role has grown after the development of advanced techniques such as diffusion-weighted imaging. The purpose of this article was to review the role of magnetic resonance imaging in Parkinson's disease and in the differential diagnosis with atypical parkinsonism, emphasizing the diffusion technique.
Resumo A doença de Parkinson é uma das doenças neurodegenerativas mais comuns. Clinicamente, é caracterizada por sintomas motores, devendo ser diferenciada com outras doenças que cursam com parkinsonismo, definidas como parkinsonismo atípico. A ressonância magnética convencional é o principal método de imagem para auxiliar este diagnóstico diferencial e, atualmente, seu papel tem crescido com as técnicas avançadas, como a difusão. O objetivo deste artigo é revisar o papel da ressonância magnética na doença de Parkinson e no diagnóstico diferencial com parkinsonismo atípico, com ênfase na técnica de difusão.
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Las caídas en los adultos mayores son comunes, peligrosas y frecuentemente incapacitantes. Con frecuenciason multifactoriales y requieren un amplio abordaje diagnóstico. Las demencias, la enfermedad de Parkinsonidiopática (EP), la enfermedad cerebrovascular y las neuropatías se encuentran entre las condiciones neurológicasque con mayor frecuencia se asocian a caídas. Sin embargo, cuando estas se presentan con recurrenciaen el contexto de un síndrome parkinsoniano, particularmente al inicio de la enfermedad, deben sugerir eldiagnóstico de una parálisis supranuclear progresiva (PSP). Esta entidad es de diagnóstico esencialmente clínico;sin embargo, dado su pronóstico sombrío, se realizan imágenes por resonancia magnética (IRM) de cerebro atodos los pacientes para excluir otros diagnósticos alternativos y buscar hallazgos característicos de PSP. Estosson, principalmente, atrofia del mesencéfalo, borramiento de la sustancia nigra, atrofia del putamen y atrofiacon aumento de la señal del globo pálido.Se presenta a continuación el caso de un paciente adulto mayor que presentaba demencia y parkinsonismorefractario a levodopa, con caídas frecuentes, en quien la realización de una resonancia magnética sirvió paraaclarar el diagnóstico al evidenciar los hallazgos típicos de PSP. Además, se hace una breve revisión de losprincipales hallazgos con imágenes, de demencias parkinsonianas, y la prevención de caídas en estos pacientes.
Falls in the elderly are common, dangerous and often disabling. They are often multifactorial and require extensive diagnostic approach. Dementia, Idiopathic Parkinson's Disease, cerebrovascular disease and neuropathies are among the most frequent neurological condictions associated with falls. However, if a patient has had early instability and recurrent falls associated with a Parkinsonian syndrome, particularly at the beginning of the disease, it should suggest the diagnosis of Progressive Supranuclear Palsy (PSP). PSP is essentially a clinical diagnosis, however, given the bleak prognosis, Magnetic Resonance Imaging (MRI) of the brain is performed in all patients to exclude alternative diagnoses and to look for supporting features of PSP. These include signal increase and atrophy of the midbrain, thinning or smudging of the substantia nigra, atrophy of the putamen, atrophy and signal increase of globus pallidus.We present the case of an adult patient who had dementia and parkinsonism refractory to levodopa with frequent falls in whom typical findings of PSP were documented in MRI. In addition, a brief review of the main findings in Parkinsonian Dementias images and fall prevention in these patients.
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Humanos , Trastornos Parkinsonianos , Parálisis Supranuclear ProgresivaRESUMEN
We report a patient with progressive supranuclear palsy (PSP) with his serial photographs before the onset of ocular symptoms and after the onset with two year intervals. These photographs show his progressive eyeball deviations toward complete exotropia. There were no effective voluntary eyeball movements, Bell's phenomenon, doll's eye movements, and vestibulo-ocular reflexes. These signs indicate the involvement of the oculomotor nuclear complex by the disease. We suggest that PSP may cause not only 'supranuclear' but also 'nuclear' complete ophthalmoplegia with exodeviation of the eyes.
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Anciano , Humanos , Masculino , Exotropía/diagnóstico , Movimientos Oculares , Imagen por Resonancia Magnética , Oftalmoplejía/diagnóstico , Parálisis Supranuclear Progresiva/diagnóstico , Factores de Tiempo , Visión BinocularRESUMEN
Objective To investigate the MRI abnormalities and feature of FDG metabolism in the brain of patients with progressive supranuclear palsy.Methods Eleven patients with PSP and 178 patients with PD were prospectively studied with mid-sagittal plain MRI;five patients with PSP and 48 patients with PD were prospectively studied with 18F-FDG PET.Results The atrophy of the midbrain tegmentum and hummingbird sign were demonstrated in all of the PSP patients studied,but were not observed in PD patients.The area of the midbrain on mid-sagittal plain MRI in the patients with PSP was significantly smaller than that in those with PD.The patients with PSP had more prominent decrease of glucose metabolism symmetrically in frontal cortices and lentiform compared with PD.The lentiform in PD showed significant glucose hypermetabolism compared with thalamus.The regional glucose metabolism of PSP was symmetrical while that of PD was asymmetrical in lentiform and thalamus.Conclusion The assessment of the mid-sagittal plain MRI and 18F-FDG PET images may be a useful adjunct to a clinical examination when making a differential diagnosis of PSP with PD.