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Review Article
New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration
Hee Kyung Park, Sun J. Chung
J Mov Disord. 2013;6(1):1-8.
  • 24,867 View
  • 241 Download
  • 27 Crossref
AbstractAbstract PDF

Frontotemporal dementia (FTD) is the second most common type of presenile dementia. Three clinical prototypes have been defined; behavioral variant FTD, semantic dementia, and progressive nonfluent aphasia. Progressive supranuclear palsy, corticobasal degeneration, and motor neuron disease may possess clinical and pathological characteristics that overlap with FTD, and it is possible that they may all belong to the same clinicopathological spectrum. Frontotemporal lobar degeneration (FTLD) is a clinicopathological syndrome that encompasses a heterogenous group of neurodegenerative disorders. Owing to the advancement in the field of molecular genetics, diagnostic imaging, and pathology, FTLD has been the focus of great interest. Nevertheless, parkinsonism in FTLD has received relatively less attention. Parkinsonism is found in approximately 20–30% of patients in FTLD. Furthermore, parkinsonism can be seen in all FTLD subtypes, and some patients with familial and sporadic FTLD can present with prominent parkinsonism. Therefore, there is a need to understand parkinsonism in FTLD in order to obtain a better understanding of the disease. With regard to the clinical characteristics, the akinetic rigid type of parkinsonism has predominantly been described. Parkinsonism is frequently observed in familial FTD, more specifically, in FTD with parkinsonism linked to chromosome 17q (FTDP-17). The genes associated with parkinsonism are microtubule associated protein tau (MAPT), progranulin (GRN or PGRN), and chromosome 9 open reading frame 72 (C9ORF72) repeat expansion. The neural substrate of parkinsonism remains to be unveiled. Dopamine transporter (DAT) imaging revealed decreased uptake of DAT, and imaging findings indicated atrophic changes of the basal ganglia. Parkinsonism can be an important feature in FTLD and, therefore, increased attention is needed on the subject.


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  • Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors
    Luigi Fiondella, Priya Gami-Patel, Christian A. Blok, Annemieke J. M. Rozemuller, Jeroen J. M. Hoozemans, Yolande A. L. Pijnenburg, Marta Scarioni, Anke A. Dijkstra
    Acta Neuropathologica Communications.2023;[Epub]     CrossRef
  • Demencias degenerativas: ¿un dilema de síndromes o de enfermedades?
    A. Robles Bayón
    Neurología.2022; 37(6): 480.     CrossRef
  • Degenerative dementias: a question of syndrome or disease?
    A. Robles Bayón
    Neurología (English Edition).2022; 37(6): 480.     CrossRef
  • Deficient neurotransmitter systems and synaptic function in frontotemporal lobar degeneration—Insights into disease mechanisms and current therapeutic approaches
    Nadine Huber, Sonja Korhonen, Dorit Hoffmann, Stina Leskelä, Hannah Rostalski, Anne M. Remes, Paavo Honkakoski, Eino Solje, Annakaisa Haapasalo
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  • Dopamine Transporter Imaging for Frontotemporal Lobar Degeneration With Motor Neuron Disease
    Ryota Kobayashi, Shinobu Kawakatsu, Makoto Ohba, Daichi Morioka, Masafumi Kanoto, Koichi Otani
    Frontiers in Neuroscience.2022;[Epub]     CrossRef
  • Investigational therapeutics for the treatment of progressive supranuclear palsy
    David G Coughlin, Irene Litvan
    Expert Opinion on Investigational Drugs.2022; 31(8): 813.     CrossRef
  • Frequency and Longitudinal Course of Motor Signs in Genetic Frontotemporal Dementia
    Sonja Schönecker, Francisco J. Martinez-Murcia, Boris-Stephan Rauchmann, Nicolai Franzmeier, Catharina Prix, Elisabeth Wlasich, Sandra V. Loosli, Katja Bochmann, Juan-Manuel Gorriz Saez, Robert Laforce, Simon Ducharme, Maria Carmela Tartaglia, Elizabeth F
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    Skye Hsin-Hsien Yeh, Chuang-Hsin Chiu, Hung-Wen Kao, Ching-Po Lin, Yu-Hus Lai, Wen-Sheng Huang
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    Jun Yup Lee, Oana C. Marian, Anthony S. Don
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    Daisy Sproviero, Stella Gagliardi, Susanna Zucca, Maddalena Arigoni, Marta Giannini, Maria Garofalo, Martina Olivero, Michela Dell’Orco, Orietta Pansarasa, Stefano Bernuzzi, Micol Avenali, Matteo Cotta Ramusino, Luca Diamanti, Brigida Minafra, Giulia Peri
    International Journal of Molecular Sciences.2021; 22(5): 2737.     CrossRef
  • Parkin beyond Parkinson’s Disease—A Functional Meaning of Parkin Downregulation in TDP-43 Proteinopathies
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    Annals of Physical and Rehabilitation Medicine.2019; 62(6): 435.     CrossRef
  • Review: Clinical, genetic and neuroimaging features of frontotemporal dementia
    R. Convery, S. Mead, J. D. Rohrer
    Neuropathology and Applied Neurobiology.2019; 45(1): 6.     CrossRef
  • Granulin in Frontotemporal Lobar Degeneration: Molecular Mechanisms of the Disease
    Zemfira N. Karamysheva, Elena B. Tikhonova, Andrey L. Karamyshev
    Frontiers in Neuroscience.2019;[Epub]     CrossRef
  • The Whole Exome Sequencing Clarifies the Genotype- Phenotype Correlations in Patients with Early-Onset Dementia
    Yangqi Xu, Xiaoli Liu, Junyi Shen, Wotu Tian, Rong Fang, Binyin Li, Jianfang Ma, Li Cao, Shengdi Chen, Guanjun Li, Huidong Tang
    Aging and disease.2018; 9(4): 696.     CrossRef
  • Mouse models of frontotemporal dementia: A comparison of phenotypes with clinical symptomatology
    Rebekah M. Ahmed, Muireann Irish, Janet van Eersel, Arne Ittner, Yazi D. Ke, Alexander Volkerling, Julia van der Hoven, Kimi Tanaka, Tim Karl, Michael Kassiou, Jillian J. Kril, Olivier Piguet, Jürgen Götz, Matthew C. Kiernan, Glenda M. Halliday, John R. H
    Neuroscience & Biobehavioral Reviews.2017; 74: 126.     CrossRef
  • Translocator Protein-18 kDa (TSPO) Positron Emission Tomography (PET) Imaging and Its Clinical Impact in Neurodegenerative Diseases
    Anne-Claire Dupont, Bérenger Largeau, Maria Santiago Ribeiro, Denis Guilloteau, Claire Tronel, Nicolas Arlicot
    International Journal of Molecular Sciences.2017; 18(4): 785.     CrossRef
  • Intrinsic functional connectivity alterations in progressive supranuclear palsy: Differential effects in frontal cortex, motor, and midbrain networks
    Johannes Rosskopf, Martin Gorges, Hans‐Peter Müller, Dorothée Lulé, Ingo Uttner, Albert C. Ludolph, Elmar Pinkhardt, Freimut D. Juengling, Jan Kassubek
    Movement Disorders.2017; 32(7): 1006.     CrossRef
  • Phenotypic variability related to C9orf72 mutation in a large Sardinian kindred
    Gianluca Floris, Giuseppe Borghero, Francesca Di Stefano, Rosanna Melis, Roberta Puddu, Laura Fadda, Maria R. Murru, Daniela Corongiu, Stefania Cuccu, Stefania Tranquilli, Antonino Cannas, Maria G. Marrosu, Adriano Chiò, Francesco Marrosu
    Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration.2016; 17(3-4): 245.     CrossRef
  • Clinical features ofTBK1carriers compared withC9orf72,GRNand non-mutation carriers in a Belgian cohort
    Sara Van Mossevelde, Julie van der Zee, Ilse Gijselinck, Sebastiaan Engelborghs, Anne Sieben, Tim Van Langenhove, Jan De Bleecker, Jonathan Baets, Mathieu Vandenbulcke, Koen Van Laere, Sarah Ceyssens, Marleen Van den Broeck, Karin Peeters, Maria Mattheijs
    Brain.2016; 139(2): 452.     CrossRef
  • Characterization of Movement Disorder Phenomenology in Genetically Proven, Familial Frontotemporal Lobar Degeneration: A Systematic Review and Meta-Analysis
    Carmen Gasca-Salas, Mario Masellis, Edwin Khoo, Binit B. Shah, David Fisman, Anthony E. Lang, Galit Kleiner-Fisman, Patrick Lewis
    PLOS ONE.2016; 11(4): e0153852.     CrossRef
  • The clinical spectrum of sporadic and familial forms of frontotemporal dementia
    Ione O. C. Woollacott, Jonathan D. Rohrer
    Journal of Neurochemistry.2016; 138(S1): 6.     CrossRef
  • Genetics of Progressive Supranuclear Palsy
    Sun Young Im, Young Eun Kim, Yun Joong Kim
    Journal of Movement Disorders.2015; 8(3): 122.     CrossRef
Case Report
Levodopa-Induced Facial Dystonia in a Case of Progressive Supranuclear Palsy
Eun Joo Chung, Sang Jin Kim
J Mov Disord. 2012;5(1):28-32.
  • 17,275 View
  • 68 Download
  • 4 Crossref
AbstractAbstract PDF

Progressive supranuclear palsy (PSP) is frequently misdiagnosed as other Parkinsonism because of clinical heterogeneity of PSP. We present here a case of a 67-year-old male patient with frontotemporal dementia-like cognitive impairment including language difficulties and abnormal behaviors. He showed severe facial dystonia after the levodopa treatment. Herein, we describe an unusual case of a patient presenting with PSP which, we believe could contribute to our knowledge about atypical leveodopa-induced facial dystonia in PSP.


Citations to this article as recorded by  
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    Internal Medicine.2023; 62(11): 1671.     CrossRef
  • Dystonia in atypical parkinsonian disorders
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    Parkinsonism & Related Disorders.2019; 66: 25.     CrossRef
  • A Review of Treatment Options for Progressive Supranuclear Palsy
    Maria Stamelou, Günter Höglinger
    CNS Drugs.2016; 30(7): 629.     CrossRef

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