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Phenotypic Spectrum of Progressive Supranuclear Palsy: Clinical Study and Apolipoprotein E Effect
Amina Nasri, Ikram Sghaier, Anis Neji, Alya Gharbi, Youssef Abida, Saloua Mrabet, Amina Gargouri, Mouna Ben Djebara, Imen Kacem, Riadh Gouider
J Mov Disord. 2024;17(2):158-170.   Published online January 30, 2024
DOI: https://doi.org/10.14802/jmd.23178
  • 1,899 View
  • 91 Download
  • 1 Web of Science
  • 1 Crossref
AbstractAbstract PDF
Objective
Progressive supranuclear palsy (PSP) is a rare neurodegenerative disorder encompassing several phenotypes with various motor and cognitive deficits. We aimed to study motor and cognitive characteristics across PSP phenotypes and to assess the influence of apolipoprotein E (APOE) gene variants on PSP phenotypic expression.
Methods
In this 20-year cross-sectional study, we retrospectively reviewed the charts of all patients classified as PSP patients and recategorized them according to phenotype using the Movement Disorder Society criteria (2017). Phenotypes were divided into three subgroups, Richardson’s syndrome (PSP-RS), PSP-cortical (PSP with predominant frontal presentation [PSP-F] + PSP with predominant speech/language disorder [PSP-SL] + PSP with predominant corticobasal syndrome [PSP-CBS]) and PSP-subcortical (PSP with predominant parkinsonism [PSP-P] + PSP with progressive gait freezing [PSP-PGF] + PSP with predominant postural instability [PSP-PI] + PSP with predominant ocular motor dysfunction [PSP-OM] + PSP with cerebellar ataxia [PSP-C] + PSP with primary lateral sclerosis [PSP-PLS]), based on clinical presentation during the first 3 years after symptom onset, which defines the early disease stage. Clinical and neuropsychological assessment data were collected. Genotyping of APOE was performed using restriction fragment length polymorphism polymerase chain reaction and verified by Sanger sequencing.
Results
We included 112 PSP patients comprising 10 phenotypes classified into 48 PSP-RS, 34 PSP-cortical (PSP-CBS, 17.6%; PSP-F, 9.4%; PSP-SL, 8.2%) and 30 PSP-subcortical (PSP-P, 11.6%; PSP-PI, 8%; PSP-OM, 2.7%; PSP-PGF, 1.8%; PSP-C, 1.8%; PSP-PLS, 0.9%) subgroups. PSP-RS patients were older at disease onset (p = 0.009) and had more akinetic-rigid and levodopa-resistant parkinsonism (p = 0.006), while PSP-cortical patients had more tremors and asymmetric and/or levodopa-responsive parkinsonism (p = 0.025). Cognitive domains were significantly less altered in the PSP-subcortical subgroup. Overall, PSP-APOEε4 carriers developed parkinsonism earlier (p = 0.038), had earlier oculomotor dysfunction (p = 0.052) and had more altered cognitive profiles. The APOEε4 allele was also associated with a younger age of parkinsonism onset in the PSP-RS phenotype group (p = 0.026).
Conclusion
This study demonstrated the wide phenotypic spectrum of PSP among Tunisians. Disease onset and akinetic-rigid and levodopa-resistant parkinsonism were the hallmarks of the PSP-RS phenotype, while milder cognitive impairment was characteristic of the PSP-subcortical subgroup. The APOEε4 allele was associated with earlier parkinsonism and oculomotor dysfunction and seemed to play a role in defining a more altered cognitive profile in PSP patients.

Citations

Citations to this article as recorded by  
  • Clinical and molecular predictors of survival among atypical parkinsonian syndromes in a North African tertiary referral center
    Ikram Sghaier, Amina Nasri, Amal Atrous, Youssef Abida, Alya Gharbi, Amira Souissi, Saloua Mrabet, Mouna Ben Djebara, Imen Kacem, Amina Gargouri-Berrechid, Riadh Gouider
    Journal of the Neurological Sciences.2024; 464: 123155.     CrossRef
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Potential Link Between Cognition and Motor Reserve in Patients With Parkinson’s Disease
Seok Jong Chung, Yae Ji Kim, Yun Joong Kim, Hye Sun Lee, Mijin Yun, Phil Hyu Lee, Yong Jeong, Young H. Sohn
J Mov Disord. 2022;15(3):249-257.   Published online September 7, 2022
DOI: https://doi.org/10.14802/jmd.22063
  • 3,726 View
  • 159 Download
  • 9 Web of Science
  • 9 Crossref
AbstractAbstract PDFSupplementary Material
Objective
To investigate whether there is a link between cognitive function and motor reserve (i.e., individual capacity to cope with nigrostriatal dopamine depletion) in patients with newly diagnosed Parkinson’s disease (PD).
Methods
A total of 163 patients with drug-naïve PD who underwent 18F-FP-CIT PET, brain MRI, and a detailed neuropsychological test were enrolled. We estimated individual motor reserve based on initial motor deficits and striatal dopamine depletion using a residual model. We performed correlation analyses between motor reserve estimates and cognitive composite scores. Diffusion connectometry analysis was performed to map the white matter fiber tracts, of which fractional anisotropy (FA) values were well correlated with motor reserve estimates. Additionally, Cox regression analysis was used to assess the effect of initial motor reserve on the risk of dementia conversion.
Results
The motor reserve estimate was positively correlated with the composite score of the verbal memory function domain (γ = 0.246) and with the years of education (γ = 0.251). Connectometry analysis showed that FA values in the left fornix were positively correlated with the motor reserve estimate, while no fiber tracts were negatively correlated with the motor reserve estimate. Cox regression analysis demonstrated that higher motor reserve estimates tended to be associated with a lower risk of dementia conversion (hazard ratio, 0.781; 95% confidence interval, 0.576–1.058).
Conclusion
The present study demonstrated that the motor reserve estimate was well correlated with verbal memory function and with white matter integrity in the left fornix, suggesting a possible link between cognition and motor reserve in patients with PD.

Citations

Citations to this article as recorded by  
  • Hippocampal Perfusion Affects Motor and Cognitive Functions in Parkinson Disease: An Early Phase 18F‐FP‐CIT Positron Emission Tomography Study
    Min Young Chun, Seok Jong Chung, Su Hong Kim, Chan Wook Park, Seong Ho Jeong, Hye Sun Lee, Phil Hyu Lee, Young H. Sohn, Yong Jeong, Yun Joong Kim
    Annals of Neurology.2024; 95(2): 388.     CrossRef
  • Imaging Procedure and Clinical Studies of [18F]FP-CIT PET
    Changhwan Sung, Seung Jun Oh, Jae Seung Kim
    Nuclear Medicine and Molecular Imaging.2024; 58(4): 185.     CrossRef
  • Influence of cognitive reserve on cognitive and motor function in α-synucleinopathies: A systematic review and multilevel meta-analysis
    Isaac Saywell, Lauren Foreman, Brittany Child, Alexander L. Phillips-Hughes, Lyndsey Collins-Praino, Irina Baetu
    Neuroscience & Biobehavioral Reviews.2024; 161: 105672.     CrossRef
  • Structural underpinnings and long-term effects of resilience in Parkinson’s disease
    Verena Dzialas, Merle C. Hoenig, Stéphane Prange, Gérard N. Bischof, Alexander Drzezga, Thilo van Eimeren
    npj Parkinson's Disease.2024;[Epub]     CrossRef
  • Considering the response in addition to the challenge – a narrative review in appraisal of a motor reserve framework
    Daniel Zeller, Shawn Hiew, Thorsten Odorfer, Carine Nguemeni
    Aging.2024; 16(6): 5772.     CrossRef
  • Defining the concept of reserve in the motor domain: a systematic review
    Andreina Giustiniani, Angelo Quartarone
    Frontiers in Neuroscience.2024;[Epub]     CrossRef
  • The association of motor reserve and clinical progression in Parkinson’s disease
    Xueqin Bai, Shiwei Zhang, Qiuyue Li, Tao Guo, Xiaojun Guan, Andan Qian, Shuangli Chen, Ronghui Zhou, Yitong Cheng, Haoxin Chen, Zhaoke Gou, Chenglong Xie, Zhen Wang, Minming Zhang, Xiangwu Zheng, Meihao Wang
    NeuroImage: Clinical.2024; 44: 103704.     CrossRef
  • Occipital hypoperfusion and motor reserve in Parkinson’s disease: an early-phase 18F-FP-CIT PET study
    Yeo Jun Yoon, Su Hong Kim, Seong Ho Jeong, Chan Wook Park, Hye Sun Lee, Phil Hyu Lee, Yun Joong Kim, Young H. Sohn, Yong Jeong, Seok Jong Chung
    npj Parkinson's Disease.2024;[Epub]     CrossRef
  • Extra-Basal Ganglia Brain Structures Are Related to Motor Reserve in Parkinson’s Disease
    Jinyoung Youn, Ji Hye Won, Mansu Kim, Junmo Kwon, Seung Hwan Moon, Minkyeong Kim, Jong Hyun Ahn, Jun Kyu Mun, Hyunjin Park, Jin Whan Cho
    Journal of Parkinson's Disease.2023; 13(1): 39.     CrossRef
Association between Olfactory Deficit and Motor and Cognitive Function in Parkinson’s Disease
Han Soo Yoo, Seok Jong Chung, Yang Hyun Lee, Byoung Seok Ye, Young H. Sohn, Phil Hyu Lee
J Mov Disord. 2020;13(2):133-141.   Published online April 6, 2020
DOI: https://doi.org/10.14802/jmd.19082
  • 11,168 View
  • 288 Download
  • 24 Web of Science
  • 24 Crossref
AbstractAbstract PDFSupplementary Material
Objective
To investigate whether baseline olfactory dysfunction in Parkinson’s disease (PD) patients is associated with baseline and longitudinal motor and cognitive function.
Methods
We recruited 228 drug-naïve PD patients who were followed for a mean of 6 years. Patients underwent the Cross-Cultural Smell Identification Test (CCSIT), a neuropsychological test, and N-(3-[18F]fluoropropyl)-2β-carbomethoxy-3β-(4-iodophenyl) nortropane positron emission tomography within 6 months of the baseline evaluation. Olfactory dysfunction was categorized as normosmia (CCSIT score ≥ 9), hyposmia (CCSIT score 5–8), and anosmia (CCSIT score ≤ 4). During the follow-up period, we investigated changes in the levodopa-equivalent dose (LED) and the occurrence of wearing-off, levodopa-induced dyskinesia, and dementia.
Results
Among the PD patients, 80.7% were hyposmic at the time of diagnosis, and 26.1% were anosmic. Baseline olfactory dysfunction was not associated with either initial parkinsonian motor symptoms or with the longitudinal LED increment and motor complications. Meanwhile, the anosmic group had lower baseline scores on the Korea version of the Boston Naming Test and Stroop color reading test than the normosmic and hyposmic groups. The anosmic group exhibited a higher rate of conversion to dementia than the normosmic [adjusted hazard ratio (HR) 3.99, 95% confidence interval (CI) 1.08–14.72] and hyposmic (adjusted HR 2.48, 95% CI 1.15–5.32) PD groups, regardless of baseline motor deficits and cognitive status.
Conclusion
Baseline olfactory dysfunction was not associated with motor deficits and complications, but it was associated with cognitive dysfunction and prognosis, suggesting that severe olfactory impairment may reflect early cortical involvement, probably in the frontotemporal region, and rapid spreading of Lewy body pathology.

Citations

Citations to this article as recorded by  
  • Correlation of olfactory function factors with cardiac sympathetic denervation in Parkinson’s disease
    Dong-Woo Ryu, Sang-Won Yoo, Ko-Eun Choi, Yoon-Sang Oh, Joong-Seok Kim
    Journal of Neurology.2024; 271(3): 1397.     CrossRef
  • Estimating motor progression trajectory pursuant to temporal dynamic status of cardiac denervation in Parkinson’s disease
    Sang-Won Yoo, Dong-Woo Ryu, Yoon-Sang Oh, Seunggyun Ha, Chul Hyoung Lyoo, Yuna Kim, Ji-Yeon Yoo, Joong-Seok Kim
    Journal of Neurology.2024; 271(4): 2019.     CrossRef
  • Parkinson’s disease with hyposmia and dysautonomia: does it represent a distinct subtype?
    So Hoon Yoon, Dae Hyuk You, Han Kyu Na, Sungwoo Kang, Kyoungwon Baik, Mincheol Park, Chul Hyoung Lyoo, Young H. Sohn, Phil Hyu Lee
    Journal of Neurology.2024; 271(8): 5064.     CrossRef
  • Unraveling olfactory subtypes in Parkinson’s disease and their effect on the natural history of the disease
    Sang-Won Yoo, Dong-Woo Ryu, Yoonsang Oh, Seunggyun Ha, Chul Hyoung Lyoo, Joong-Seok Kim
    Journal of Neurology.2024; 271(9): 6102.     CrossRef
  • Determination of objective taste perception among Iranian medical sciences students during COVID‑19 pandemic in Yazd, Eastern Iran: a case-control pilot study
    Samira Hajimaghsoodi, Elham Paydar, Fatemeh Owlia
    BMC Infectious Diseases.2024;[Epub]     CrossRef
  • Olfactory Dysfunction in Parkinson’s Disease, Its Functional and Neuroanatomical Correlates
    Gabriel Torres-Pasillas, Donají Chi-Castañeda, Porfirio Carrillo-Castilla, Gerardo Marín, María Elena Hernández-Aguilar, Gonzalo Emiliano Aranda-Abreu, Jorge Manzo, Luis I. García
    NeuroSci.2023; 4(2): 134.     CrossRef
  • Impact of deep brain stimulation (DBS) on olfaction in Parkinson's disease: Clinical features and functional hypotheses
    G. Brand, C. Bontempi, L. Jacquot
    Revue Neurologique.2023; 179(9): 947.     CrossRef
  • Sequential change in olfaction and (non) motor symptoms: the difference between anosmia and non-anosmia in Parkinson’s disease
    Ting-Chun Fang, Yu-Shan Tsai, Ming-Hong Chang
    Frontiers in Aging Neuroscience.2023;[Epub]     CrossRef
  • Traumatic brain injury-induced inflammatory changes in the olfactory bulb disrupt neuronal networks leading to olfactory dysfunction
    Xiang Liu, Zhuofan Lei, Dylan Gilhooly, Junyun He, Yun Li, Rodney M. Ritzel, Hui Li, Long-Jun Wu, Shaolin Liu, Junfang Wu
    Brain, Behavior, and Immunity.2023; 114: 22.     CrossRef
  • Serum Biomarkers of Olfactory Identification Deficits in Patients with Parkinson’s Disease
    Fu-Jia Li, Yang-Dan-Yu Li, Xu Liu, Jie Zu, Wei Zhang, Qi-Hua Xiao, Xue-Bin Niu, Li Du, Chen-Chen Cui, Ru-Yu Zhang, Xiao-Qing He, Gui-Yun Cui, Chuan-Ying Xu, Dominic B. Fee
    Acta Neurologica Scandinavica.2023; 2023: 1.     CrossRef
  • UPSIT subitems may predict motor progression in Parkinson’s disease
    Yu-Hsuan Lin, Ting-Chun Fang, Hsin-Bei Lei, Shih-Chi Chiu, Ming-Hong Chang, Yi-Jen Guo
    Frontiers in Neurology.2023;[Epub]     CrossRef
  • Olfactory dysfunction is associated with motor function only in tremor-dominant Parkinson’s disease
    Fardin Nabizadeh, Kasra Pirahesh, Elham Khalili
    Neurological Sciences.2022; 43(7): 4193.     CrossRef
  • Novel diagnostic tools for identifying cognitive impairment using olfactory-stimulated functional near-infrared spectroscopy: patient-level, single-group, diagnostic trial
    Jaewon Kim, Dong Keon Yon, Kyu Yeong Choi, Jang Jae Lee, Namwoo Kim, Kun Ho Lee, Jae Gwan Kim
    Alzheimer's Research & Therapy.2022;[Epub]     CrossRef
  • The Role of Olfactory System in the Etiogenesis of Parkinson’s Diseases: An Overview
    Jiju Narayanan Avanipully, Dithu Thekkekkara, Sahyadri M, Vipan K. Parihar, Santhepete Nanjundaiah Manjula
    Journal of Pharmacology and Pharmacotherapeutics.2022; 13(1): 31.     CrossRef
  • International consensus statement on allergy and rhinology: Olfaction
    Zara M. Patel, Eric H. Holbrook, Justin H. Turner, Nithin D. Adappa, Mark W. Albers, Aytug Altundag, Simone Appenzeller, Richard M. Costanzo, Ilona Croy, Greg E. Davis, Puya Dehgani‐Mobaraki, Richard L. Doty, Valerie B. Duffy, Bradley J. Goldstein, David
    International Forum of Allergy & Rhinology.2022; 12(4): 327.     CrossRef
  • Does Olfactory Dysfunction Correlate with Disease Progression in Parkinson’s Disease? A Systematic Review of the Current Literature
    Tommaso Ercoli, Carla Masala, Gianluca Cadeddu, Marcello Mario Mascia, Gianni Orofino, Angelo Fabio Gigante, Paolo Solla, Giovanni Defazio, Lorenzo Rocchi
    Brain Sciences.2022; 12(5): 513.     CrossRef
  • Olfactory dysfunction and striatal dopamine transporter binding in motor subtypes of Parkinson’s disease
    Fardin Nabizadeh, Fatemeh Sodeifian, Kasra Pirahesh
    Neurological Sciences.2022; 43(8): 4745.     CrossRef
  • Olfaction and Executive Cognitive Performance: A Systematic Review
    Vasudeva Murthy Challakere Ramaswamy, Peter William Schofield
    Frontiers in Psychology.2022;[Epub]     CrossRef
  • Nasal and Parotid Blood Pool Activity Is Significantly Correlated with Metabolic Syndrome Components and Sleep Apnea
    William T. Phillips, Nasser J. Issa, Shereef B. Elhalwagi, Hilda T. Draeger, Joyce G. Schwartz, Jonathan A. Gelfond
    Metabolic Syndrome and Related Disorders.2022; 20(7): 395.     CrossRef
  • Chronic neuropsychiatric sequelae of SARS‐CoV‐2: Protocol and methods from the Alzheimer's Association Global Consortium
    Gabriel A. de Erausquin, Heather Snyder, Traolach S. Brugha, Sudha Seshadri, Maria Carrillo, Rajesh Sagar, Yueqin Huang, Charles Newton, Carmela Tartaglia, Charlotte Teunissen, Krister Håkanson, Rufus Akinyemi, Kameshwar Prasad, Giovanni D'Avossa, Gabriel
    Alzheimer's & Dementia: Translational Research & Clinical Interventions.2022;[Epub]     CrossRef
  • Machine learning-based prediction of cognitive outcomes in de novo Parkinson’s disease
    Joshua Harvey, Rick A. Reijnders, Rachel Cavill, Annelien Duits, Sebastian Köhler, Lars Eijssen, Bart P. F. Rutten, Gemma Shireby, Ali Torkamani, Byron Creese, Albert F. G. Leentjens, Katie Lunnon, Ehsan Pishva
    npj Parkinson's Disease.2022;[Epub]     CrossRef
  • Impact of Subthalamic Deep Brain Stimulation on Hyposmia in Patients With Parkinson's Disease Is Influenced by Constipation and Dysbiosis of Microbiota
    Chao Li, Ying Hou, Xu Wang, Yue-xuan Li, Feng Li, Chao Zhang, Wei-guo Li
    Frontiers in Neurology.2021;[Epub]     CrossRef
  • Hyposmia may predict development of freezing of gait in Parkinson’s disease
    Jae Jung Lee, Jin Yong Hong, Jong Sam Baik
    Journal of Neural Transmission.2021; 128(6): 763.     CrossRef
  • Clinical and Dopamine Depletion Patterns in Hyposmia- and Dysautonomia-Dominant Parkinson’s Disease
    Han Soo Yoo, Sangwon Lee, Seong Ho Jeong, Byoung Seok Ye, Young H. Sohn, Mijin Yun, Phil Hyu Lee
    Journal of Parkinson's Disease.2021; 11(4): 1703.     CrossRef
Article image
Cognition, Olfaction and Uric Acid in Early de novo Parkinson’s Disease
Hwa Reung Lee, Joong Hyun Park, Sang Won Han, Jong Sam Baik
J Mov Disord. 2018;11(3):139-144.   Published online September 30, 2018
DOI: https://doi.org/10.14802/jmd.18037
  • 7,291 View
  • 154 Download
  • 8 Web of Science
  • 7 Crossref
AbstractAbstract PDF
Objective
Cognitive impairment is one of the nonmotor symptoms in Parkinson’s disease (PD), and olfactory dysfunction is used as a marker to detect premotor stages of PD. Serum uric acid (sUA) levels have been found to be a risk factor for PD. Our objective in this study was to examine whether sUA levels are associated with cognitive changes and olfactory dysfunction in early de novo PD patients.
Methods
The study participants included 196 de novo PD patients. We assessed cognitive function by the Korean versions of the Mini-Mental State Examination and the Montreal Cognitive Assessment and assessed olfactory function by the Korean version of the Sniffin’ Sticks test.
Results
The mean sUA level was 4.7 mg/dL and was significantly lower in women than in men. Cognitive scores were lower in women, suggesting that sUA levels were related to cognitive function. The olfactory functions were not related to sUA level but were clearly associated with cognitive scores. Olfactory threshold, odor discrimination, and odor identification were all significantly related to cognitive scores.
Conclusion
We conclude that lower sUA levels were associated with cognitive impairment, not olfactory dysfunction, in de novo PD patients. This finding suggests that UA is neuroprotective as an antioxidant in the cognitive function of PD patients.

Citations

Citations to this article as recorded by  
  • Relationships between Serum Lipid, Uric Acid Levels and Mild Cognitive Impairment in Parkinson's Disease and Multiple System Atrophy
    Xiaoqiao Ren, Pan Wang, Hao Wu, Shuai Liu, Jinhong Zhang, Xiyu Li, Yong Ji, Zhihong Shi
    Journal of Integrative Neuroscience.2024;[Epub]     CrossRef
  • Serum uric acid and Parkinson's disease: A systematic review and meta‐analysis
    Mohammad Balabandian, Sarvenaz Salahi, Behnaz Mahmoudvand, Mahla Esmaeilzadeh, Seyedeh Melika Hashemi, Fardin Nabizadeh
    Neurology and Clinical Neuroscience.2023; 11(6): 299.     CrossRef
  • International consensus statement on allergy and rhinology: Olfaction
    Zara M. Patel, Eric H. Holbrook, Justin H. Turner, Nithin D. Adappa, Mark W. Albers, Aytug Altundag, Simone Appenzeller, Richard M. Costanzo, Ilona Croy, Greg E. Davis, Puya Dehgani‐Mobaraki, Richard L. Doty, Valerie B. Duffy, Bradley J. Goldstein, David
    International Forum of Allergy & Rhinology.2022; 12(4): 327.     CrossRef
  • Association of serum uric acid and non-motor symptoms in Parkinson's disease
    Olanike A. Odeniyi, Oluwadamilola O. Ojo, Ifedayo Adeola Odeniyi, Njideka Ulunma Okubadejo
    Journal of Clinical Sciences.2022; 19(3): 104.     CrossRef
  • A postmortem study suggests a revision of the dual-hit hypothesis of Parkinson’s disease
    Per Borghammer, Mie Kristine Just, Jacob Horsager, Casper Skjærbæk, Anna Raunio, Eloise H. Kok, Sara Savola, Shigeo Murayama, Yuko Saito, Liisa Myllykangas, Nathalie Van Den Berge
    npj Parkinson's Disease.2022;[Epub]     CrossRef
  • What substance P might tell us about the prognosis and mechanism of Parkinson's disease?
    Paola Tirassa, Tommaso Schirinzi, Marcello Raspa, Massimo Ralli, Antonio Greco, Antonella Polimeni, Roberta Possenti, Nicola Biagio Mercuri, Cinzia Severini
    Neuroscience & Biobehavioral Reviews.2021; 131: 899.     CrossRef
  • Brain-First versus Gut-First Parkinson’s Disease: A Hypothesis
    Per Borghammer, Nathalie Van Den Berge, Teus van Laar
    Journal of Parkinson's Disease.2019; 9(s2): S281.     CrossRef
Prospective Characterization of Cognitive Function in Typical and ‘Brainstem Predominant’Progressive Supranuclear Palsy Phenotypes
Young-Eun C Lee, David R Williams, Jacqueline F I Anderson
J Mov Disord. 2018;11(2):72-77.   Published online May 30, 2018
DOI: https://doi.org/10.14802/jmd.17067
  • 8,874 View
  • 131 Download
  • 7 Web of Science
  • 7 Crossref
AbstractAbstract PDF
Objective
Clinicopathological studies over the last decade have broadened the clinical spectrum of progressive supranuclear palsy (PSP) to include several distinct clinical syndromes. We examined the cognitive profiles of patients with PSP-Richardson’s syndrome (PSP-RS) and two atypical ‘brainstem predominant’ PSP phenotypes (PSP-parkinsonism, PSP-P; and PSP-pure akinesia with gait freezing, PSP-PAGF) using a comprehensive neuropsychological battery.
Methods
Fourteen patients diagnosed as PSP-RS, three patients with PSP-P and four patients with PSP-PAGF were assessed using a comprehensive battery of neuropsychological tests.
Results
The typical PSP-RS subgroup demonstrated greater impairments in processing speed [t(19) = -4.10, p = 0.001 (d =1.66)] and executive function [t(19) = -2.63, p = 0.02 (d = 1.20)] compared to the ‘brainstem predominant’ PSP phenotype.
Conclusion
This is the first prospective study to demonstrate that PSP-RS and ‘brainstem predominant’ PSP phenotypes can be differentiated on cognitive grounds. These differences correspond with variations in pathological profiles reported in the literature.

Citations

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  • Pathomechanisms of cognitive impairment in progressive supranuclear palsy
    Kurt A. Jellinger
    Journal of Neural Transmission.2023; 130(4): 481.     CrossRef
  • Differential Diagnosis of Rare Subtypes of Progressive Supranuclear Palsy and PSP-Like Syndromes—Infrequent Manifestations of the Most Common Form of Atypical Parkinsonism
    Patrycja Krzosek, Natalia Madetko, Anna Migda, Bartosz Migda, Dominika Jaguś, Piotr Alster
    Frontiers in Aging Neuroscience.2022;[Epub]     CrossRef
  • Clinical Spectrum of Tauopathies
    Nahid Olfati, Ali Shoeibi, Irene Litvan
    Frontiers in Neurology.2022;[Epub]     CrossRef
  • Neuropsychological assessment could distinguish among different clinical phenotypes of progressive supranuclear palsy: A Machine Learning approach
    Maria Grazia Vaccaro, Alessia Sarica, Andrea Quattrone, Carmelina Chiriaco, Maria Salsone, Maurizio Morelli, Aldo Quattrone
    Journal of Neuropsychology.2021; 15(3): 301.     CrossRef
  • “Parkinson’s disease” on the way to progressive supranuclear palsy: a review on PSP-parkinsonism
    Ján Necpál, Miroslav Borsek, Bibiána Jeleňová
    Neurological Sciences.2021; 42(12): 4927.     CrossRef
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    Theodore P. Parthimos, Kleopatra H. Schulpis
    Clinical Gerontologist.2020; 43(2): 155.     CrossRef
  • Progressive Supranuclear Palsy—Parkinsonism Predominant (PSP-P)—A Clinical Challenge at the Boundaries of PSP and Parkinson's Disease (PD)
    Piotr Alster, Natalia Madetko, Dariusz Koziorowski, Andrzej Friedman
    Frontiers in Neurology.2020;[Epub]     CrossRef
Article image
Cognition and Visit-to-Visit Variability of Blood Pressure and Heart Rate in De Novo Patients with Parkinson’s Disease
Kyum-Yil Kwon, Seon Jong Pyo, Hye Mi Lee, Woo-Keun Seo, Seong-Beom Koh
J Mov Disord. 2016;9(3):144-151.   Published online September 21, 2016
DOI: https://doi.org/10.14802/jmd.16012
  • 14,142 View
  • 128 Download
  • 8 Web of Science
  • 8 Crossref
AbstractAbstract PDFSupplementary Material
Objective
We sought to identify whether the characteristics of long-term visit-to-visit blood pressure (BP) and heart rate (HR) are related to baseline cognitive profiles in, Parkinson’s disease (PD).
Methods
We selected drug-naïve PD patients who visited our hospital at least 10 times with a baseline assessment of the Seoul neuropsychological battery. BP and HR were measured at each visit, and the variability of the systolic BP/diastolic BP (DBP) and HR was derived from the parameters of serial 10 office visits. Mild cognitive impairment (MCI) in PD patients was determined according to the proposed criteria with a cut-off value of z-score ≤ -2.
Results
Forty-seven patients with PD (mean follow-up duration = 22.3 months) were enrolled in the study. Compared with non-MCI PD patients, MCI PD patients revealed a significant increase in HR and/or variability in DBP.
Conclusion
This exploratory study showed that baseline cognition in drug-naïve PD patients might be related to the visit-to-visit variability of DBP and/or HR.

Citations

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  • Association between the blood pressure variability and cognitive decline in Parkinson's disease
    Yi Xiao, Tianmi Yang, Lingyu Zhang, Qianqian Wei, Ruwei Ou, Yanbing Hou, Kuncheng Liu, Junyu Lin, Qirui Jiang, Huifang Shang
    Brain and Behavior.2023;[Epub]     CrossRef
  • Cardiovascular autonomic dysfunction is associated with executive dysfunction and poorer quality of life in progressive supranuclear palsy-Richardson’s syndrome
    Peng Liu, Yueting Chen, Bo Wang, Sheng Wu, Leilei Zeng, Zhidong Cen, Dehao Yang, Haotian Wang, Xinhui Chen, Lebo Wang, Zhiyuan Ouyang, Wei Luo
    Journal of Clinical Neuroscience.2022; 96: 147.     CrossRef
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    Nur Fazidah Asmuje, Sumaiyah Mat, Phyo Kyaw Myint, Maw Pin Tan
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    Kyum-Yil Kwon, Suyeon Park, Rae On Kim, Eun Ji Lee, Mina Lee
    Scientific Reports.2022;[Epub]     CrossRef
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    Yerim Kim, Jae-Sung Lim, Mi Sun Oh, Kyung-Ho Yu, Ji Sung Lee, Jong-Ho Park, Yong-Jae Kim, Joung-Ho Rha, Yang-Ha Hwang, Sung Hyuk Heo, Seong Hwan Ahn, Ju-Hun Lee, Sun U. Kwon
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Review Article
Article image
Cerebrospinal Fluid Amyloid β1-42, Tau, and Alpha-Synuclein Predict the Heterogeneous Progression of Cognitive Dysfunction in Parkinson’s Disease
Ju-Hee Kang
J Mov Disord. 2016;9(2):89-96.   Published online May 25, 2016
DOI: https://doi.org/10.14802/jmd.16017
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AbstractAbstract PDF
Parkinson’s disease (PD) is a neurodegenerative disease with heterogeneous pathological and clinical features. Cognitive dysfunction, a frequent non-motor complication, is a risk factor for poor prognosis and shows inter-individual variation in its progression. Of the clinical studies performed to identify biomarkers of PD progression, the Parkinson’s Progression Markers Initiative (PPMI) study is the largest study that enrolled drug-naïve and very early stage PD patients. The baseline characteristics of the PPMI cohort were recently published. The diagnostic utility of cerebrospinal fluid (CSF) biomarkers, including alpha-synuclein (α-syn), total tau, phosphorylated tau at Thr181, and amyloid β1-42, was not satisfactory. However, the baseline data on CSF biomarkers in the PPMI study suggested that the measurement of the CSF biomarkers enables the prediction of future cognitive decline in PD patients, which was consistent with previous studies. To prove the hypothesis that the interaction between Alzheimer’s pathology and α-syn pathology is important to the progression of cognitive dysfunction in PD, longitudinal observational studies must be followed. In this review, the neuropathological nature of heterogeneous cognitive decline in PD is briefly discussed, followed by a summarized interpretation of baseline CSF biomarkers derived from the data in the PPMI study. The combination of clinical, biochemical, genetic and imaging biomarkers of PD constitutes a feasible strategy to predict the heterogeneous progression of PD.

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