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进行性核上性麻痹为神经变性疾病,早期症状不典型,需与帕金森病、橄榄脑桥小脑萎缩、阿尔茨海默病、皮质基底节变性等疾病相鉴别。其临床表现包括垂直性核上性眼肌麻痹、帕金森综合征、轴性肌张力增高、姿势不稳、假性延髓性麻痹以及认知功能障碍等。影像学检查对诊断有重要作用。文中就进行性核上性麻痹的发病机制、临床表现、影像学特点、诊断标准等研究进展予以介绍。 相似文献
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Purpose of review
Progressive supranuclear palsy (PSP) is a 4R tau neuropathologic entity. While historically defined by the presence of a vertical supranuclear gaze palsy and falls in the first symptomatic year, clinicopathologic studies identify alternate presenting phenotypes. This article reviews the new PSP diagnostic criteria, diagnostic approaches, and treatment strategies.Recent findings
The 2017 International Parkinson and Movement Disorder Society PSP criteria outline 14 core clinical features and 4 clinical clues that combine to diagnose one of eight PSP phenotypes with probable, possible, or suggestive certainty. Evidence supports the use of select imaging approaches in the classic PSP-Richardson syndrome phenotype. Recent trials of putative disease-modifying agents showed no benefit.Summary
The new PSP diagnostic criteria incorporating the range of presenting phenotypes have important implications for diagnosis and research. More work is needed to understand how diagnostic evaluations inform phenotype assessment and identify expected progression. Current treatment is symptomatic, but tau-based therapeutics are in active clinical trials.8.
Ling Li MD Feng-Tao Liu MD PhD Ming Li PhD Jia-Ying Lu MD Yi-Min Sun MD PhD Xiaoniu Liang PhD Weiqi Bao MD PhD Qi-Si Chen MD Xin-Yi Li MD Xin-Yue Zhou MD Yihui Guan MD PhD Jian-Jun Wu MD PhD Tzu-Chen Yen MD PhD Ming-Kuei Jang PhD Jian-Feng Luo PhD Jian Wang MD PhD Chuantao Zuo MD PhD the Progressive Supranuclear Palsy Neuroimage Initiative 《Movement disorders》2021,36(10):2314-2323
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B. Borroni S. Goldwurm C. Cerini M. Cosseddu N. Meucci C. Mariani G. Pezzoli A. Padovani 《European journal of neurology》2011,18(1):195-197
Background: Studies on familial aggregation might be of help to evaluate whether the genetic background has a key role in Progressive Supranuclar Palsy (PSP) and Corticobasal Syndrome (CBS). Only a few studies are available. Objective: To evaluate the prevalence of positive family history (FH) in PSP and CBS in a large sample of patients. Methods: Two hundred and thirty patients and 110 controls entered the study. Patients underwent an extensive clinical, neurological and neuropsychological assessment as well as a structural brain imaging study. A clinical follow‐up further confirmed the diagnosis. Familial aggregation was carefully recorded by a standardised questionnaire. Results: One hundred and twenty‐nine PSP (age at onset = 66.6 ± 7.3, female = 46.1%) and 101 CBS (age at onset = 62.8 ± 8.9, female = 41.6%) were consecutively enrolled. Positive FH was found in 31.8% of PSP (n = 41) and in 31.7% of CBS (n = 32). Familial aggregation was lower in the age‐matched control group compared to patient group (21.8%, P = 0.05). Patients with PSP had higher positive FH for Parkinsonism (63.4%) when compared to FH for dementia (36.6%). In CBS, FH was equally distributed between Parkinsonism (53.1%) and dementia (46.9%). In addition, FH was not associated with age at disease onset in PSP (FH+ versus FH−, 67.0 ± 7.3 vs. 66.7 ± 7.1, P = 0.788) and in CBS (62.6 ± 7.9 vs. 62.9 ± 9.5, P= 0.877). Conclusions: These results argue for familial aggregation in PSP and CBS, further underlying the importance of genetic background in these disorders. Further studies on possible genetic modulators or genetic epistasis contributing to PSP and CBS development are warranted. 相似文献
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Marie-Therese Grötsch Gesine Respondek MD Carlo Colosimo MD FEAN Yaroslau Compta MD Jean Christophe Corvol MD PhD Joaquim Ferreira MD Meret Koroni Huber MD Martin Klietz MD Lea F.M. Krey MD Johannes Levin MD Milica Jecmenica-Lukic MD Daniel Macías-García MD Wassilios G. Meissner MD PhD Pablo Mir MD Huw Morris MD Christer Nilsson MD James B. Rowe MD PhD Klaus Seppi MD Maria Stamelou MD PhD John C. van Swieten MD Gregor Wenning MD PhD Teodoro Del Ser MD Lawrence I. Golbe MD Günter U. Höglinger MD for the Describe PSP Study Group the ProPSP Study Group and the Movement Disorder Society–Endorsed PSP Study Group 《Movement disorders》2021,36(5):1203-1215
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The discovery of mirror neurons in macaque has led to a resurrection of motor theories of speech perception. Although the majority of lesion and functional imaging studies have associated perception with the temporal lobes, it has also been proposed that the ‘human mirror system’, which prominently includes Broca's area, is the neurophysiological substrate of speech perception. Although numerous studies have demonstrated a tight link between sensory and motor speech processes, few have directly assessed the critical prediction of mirror neuron theories of speech perception, namely that damage to the human mirror system should cause severe deficits in speech perception. The present study measured speech perception abilities of patients with lesions involving motor regions in the left posterior frontal lobe and/or inferior parietal lobule (i.e., the proposed human ‘mirror system’). Performance was at or near ceiling in patients with fronto-parietal lesions. It is only when the lesion encroaches on auditory regions in the temporal lobe that perceptual deficits are evident. This suggests that ‘mirror system’ damage does not disrupt speech perception, but rather that auditory systems are the primary substrate for speech perception. 相似文献
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Rosalie M. Grijalva BS Nha Trang Thu Pham BS Qiao Huang PhD Peter R. Martin MS Farwa Ali MD Heather M. Clark PhD Joseph R. Duffy PhD Rene L. Utianski PhD Hugo Botha MD Mary M. Machulda PhD Stephen D. Weigand MS J. Eric Ahlskog PhD MD Dennis W. Dickson MD Keith A. Josephs MD MST MSc Jennifer L. Whitwell PhD 《Movement disorders》2022,37(4):702-712
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Farwa Ali Peter R. Martin Hugo Botha J. Eric Ahlskog James H. Bower Joseph Y. Masumoto Demetrius Maraganore Anhar Hassan Scott Eggers Bradley F. Boeve David S. Knopman Daniel Drubach Ronald C. Petersen Erika Driver Dunkley Jay van Gerpen Ryan Uitti Jennifer L. Whitwell Dennis W. Dickson Keith A. Josephs 《Movement disorders》2019,34(8):1144-1153
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Progressive supranuclear palsy (PSP) is a sporadic and progressive neurodegenerative disease, most often leading to a symmetric,
akinetic-rigid syndrome with prominent postural instability, vertical supranuclear gaze palsy, and cognitive decline. It belongs
to the family of tauopathies and involves both cortical and subcortical structures. There is evidence from laboratory as well
as in vivo studies suggesting that mitochondrial energy metabolism is impaired in PSP. Furthermore, several findings suggest
that a failure in mitochondrial energy production might act as an upstream event in the chain of pathological events leading
to the aggregation of tau and neuronal cell death. Agents targeting mitochondrial dysfunction have already shown a positive
effect in a phase II study; however, further studies to verify these results need to be conducted. This review will focus
on the pathophysiological concept of mitochondrial dysfunction in PSP and its possible role as a therapeutic target. 相似文献