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Volume 6(1); April 2013
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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.
  • 24,449 View
  • 238 Download
  • 25 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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  • Antidepressant medications in dementia: evidence and potential mechanisms of treatment-resistance
    Harry Costello, Jonathan P. Roiser, Robert Howard
    Psychological Medicine.2023; 53(3): 654.     CrossRef
  • 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
    Molecular Psychiatry.2022; 27(3): 1300.     CrossRef
  • Novel frameshift CTSF mutation causing kufs disease type B mimicking frontotemporal dementia-parkinsonism
    Murat Gultekin, Zeynep Tufekcioglu, Recep Baydemir
    Neurocase.2022; 28(1): 107.     CrossRef
  • 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
    Neurology.2022;[Epub]     CrossRef
  • Multi-Modal Synergistic 99mTc-TRODAT-1 SPECT and MRI for Evaluation of the Efficacy of Hyperbaric Oxygen Therapy in CO-Induced Delayed Parkinsonian and Non-Parkinsonian Syndromes
    Skye Hsin-Hsien Yeh, Chuang-Hsin Chiu, Hung-Wen Kao, Ching-Po Lin, Yu-Hus Lai, Wen-Sheng Huang
    Antioxidants.2022; 11(11): 2289.     CrossRef
  • Defective Lysosomal Lipid Catabolism as a Common Pathogenic Mechanism for Dementia
    Jun Yup Lee, Oana C. Marian, Anthony S. Don
    NeuroMolecular Medicine.2021; 23(1): 1.     CrossRef
  • Different miRNA Profiles in Plasma Derived Small and Large Extracellular Vesicles from Patients with Neurodegenerative Diseases
    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
    Katarzyna Gaweda-Walerych, Emilia Jadwiga Sitek, Ewa Narożańska, Emanuele Buratti
    Cells.2021; 10(12): 3389.     CrossRef
  • Joint contractures and acquired deforming hypertonia in older people: Which determinants?
    Patrick Dehail, Nathaly Gaudreault, Haodong Zhou, Véronique Cressot, Anne Martineau, Julie Kirouac-Laplante, Guy Trudel
    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 Reports
Ventricular Bigeminy after Subcutaneous Administration of Apomorphine in a Patient with Refractory Parkinson’s Disease: A Case Report
Anastasia N. Kaminioti, Georgios T. Nikitas, Apostolos K. Terlis, Athanasios G. Manolis, Thomas Thomaides, Aggeliki N. Panousopoulou
J Mov Disord. 2013;6(1):9-12.
  • 30,731 View
  • 62 Download
  • 7 Crossref
AbstractAbstract PDF

Apomorphine is a well established treatment for the management of sudden, unexpected and refractory levodopa-induced “off” states in fluctuating Parkinson’s disease either as bolus injections or as continuous infusions. Incidents of atrial fibrillation associated with the administration of the drug have been reported in the past but no incidents of ventricular arrhythmias. We report a case of ventricular bigeminy recorded in a female patient after the administration of apomorphine.


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  • Emerging Pharmacotherapies for Motor Symptoms in Parkinson’s Disease
    Mohammad Al Majali, Michael Sunnaa, Pratap Chand
    Journal of Geriatric Psychiatry and Neurology.2021; 34(4): 263.     CrossRef
  • Safety and Tolerability of Pharmacotherapies for Parkinson’s Disease in Geriatric Patients
    Martin Klietz, Stephan Greten, Florian Wegner, Günter U. Höglinger
    Drugs & Aging.2019; 36(6): 511.     CrossRef
  • Pharmacological Insights into the Use of Apomorphine in Parkinson’s Disease: Clinical Relevance
    Manon Auffret, Sophie Drapier, Marc Vérin
    Clinical Drug Investigation.2018; 38(4): 287.     CrossRef
  • Cardiac abnormalities in Parkinson’s disease and Parkinsonism
    Fulvio A. Scorza, Ana C. Fiorini, Carla A. Scorza, Josef Finsterer
    Journal of Clinical Neuroscience.2018; 53: 1.     CrossRef
  • Apomorphine en perfusion sous-cutanée continue dans la maladie de Parkinson : le point de vue pharmacologique
    M. Auffret, S. Drapier, M. Vérin, P. Sauleau
    Pratique Neurologique - FMC.2016; 7(1): 25.     CrossRef
  • Practical management of adverse events related to apomorphine therapy
    Roongroj Bhidayasiri, Pedro J. Garcia Ruiz, Tove Henriksen
    Parkinsonism & Related Disorders.2016;[Epub]     CrossRef
A Case of Isolated Middle Cerebral Artery Stenosis with Hemichorea and Moyamoya Pattern Collateralization
Seok Jong Chung, Hyung Seok Lee, Han Soo Yoo, Kyung Min Kim, Ki Jeong Lee, Jong-Soo Kim, Jae-Wook Lee, Jong Hun Kim, Jeong Hee Cho, Gyu Sik Kim, Jun Hong Lee, Sun-Ah Choi
J Mov Disord. 2013;6(1):13-16.
  • 11,550 View
  • 98 Download
  • 5 Crossref
AbstractAbstract PDF

Isolated middle cerebral artery (MCA) stenosis in young patients with no other medical condition may be a unique pathologic entity with a benign long-term course. Generally, moyamoya disease shows a progression of stenosis from internal cerebral artery (ICA) to other intracranial vessel. A 26-year-old woman was admitted for choreic movements of the right arm and leg. Brain magnetic resonance imaging showed no stroke. Conventional angiography revealed 48% stenosis of the left M1 without ICA stenosis. Single photon emission computed tomography revealed perfusion asymmetry after acetazolamide injection, suggesting decreased uptake in the left basal ganglia and the cerebral cortex. Her hemichorea was mildly decreased with risperidone. One year later, follow-up angiography showed complete occlusion of the left M1 with neovascularization suggestive of moyamoya disease. The patient underwent bypass surgery and her hemichorea disappeared. This may be an atypical presentation of moyamoya disease. The bypass surgery was an effective measure for restoring the vascular insufficiency and, resultantly, controlling her hemichorea.


Citations to this article as recorded by  
  • Hemichorea in patients with temporal lobe infarcts: Two case reports
    Xu-Dong Wang, Xing Li, Chun-Lian Pan
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  • Truncal dystonia with isolated middle cerebral artery ischemia: A case report of revascularization therapy for dystonia
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    Surgical Neurology International.2022; 13: 155.     CrossRef
  • Geriatric Case of Moyamoya Disease Presenting with Hemiballismus as an Initial Manifestation
    Koichiro SATO, Masaru IDEI, Kenichirou NOGAMI, Makoto KURESHIMA, Kaname HOKAO, Rina TORII, Takenori UOZUMI, Junkoh YAMAMOTO
    Surgery for Cerebral Stroke.2022; 50(6): 508.     CrossRef
  • Persistent Hemichorea as a Preceding Symptom of Cerebral Infarction Due to Middle Cerebral Artery Stenosis
    Yuki Ueta, Haruhisa Kato, Makiko Naito, Takeshi Taguchi, Hiroo Terashi, Hitoshi Aizawa
    Internal Medicine.2021; 60(23): 3805.     CrossRef
  • Clinical findings of a patient with hemiballism after superficial temporal artery-middle cerebral artery anastomosis for idiopathic middle cerebral artery stenosis
    Hideaki Shibata, Yuichi Hayashi, Nobuaki Yoshikura, Megumi Yamada, Akio Kimura, Takayoshi Shimohata
    Rinsho Shinkeigaku.2019; 59(12): 829.     CrossRef
Acute Hemichorea as an Unusual Presentation of Internal Carotid Artery Stenosis
Dong Wook Kim, Youngchai Ko, Sang Hyun Jang, Soo Jin Yoon, Gun-Sei Oh, Soo Joo Lee, Dong Joo Yun
J Mov Disord. 2013;6(1):17-20.
  • 16,414 View
  • 98 Download
  • 10 Crossref
AbstractAbstract PDF

Involuntary movement associated with deep watershed ischemic lesions has been rarely reported. A 67-year-old woman presented with acute hemichorea on the left side. Magnetic resonance imaging showed acute infarcts in the anterior border zone. On perfusion studies, impaired cerebral blood flow was observed on the subcortical region sparing the basal ganglia. Cerebral angiogram confirmed severe stenosis in the right internal carotid artery. Her hemichorea gradually improved along with normalization of perfusion after carotid artery stenting with angioplasty. We suggest that impaired cerebral blood flow in critical watershed territories may be an important contributing factor in hemichorea associated with carotid occlusive disease.


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    Frontiers in Neurology.2022;[Epub]     CrossRef
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    I. Ion, T. Parvu, G. Farouil, D. Sablot
    Revue Neurologique.2022; 178(8): 859.     CrossRef
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    Sintija Strautmane, Zanda Priede, Andrejs Millers
    Medicina.2022; 58(10): 1328.     CrossRef
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    Satoshi Hosoki, Takeshi Yoshimoto, Masafumi Ihara
    BMC Neurology.2021;[Epub]     CrossRef
  • Acute hemichorea with T1 ischemic hyperintensity associated with steno‐occlusive middle cerebral artery dissection
    Yong Chuan Chee, Beng Hooi Ong
    Neurology and Clinical Neuroscience.2020; 8(1): 44.     CrossRef
  • A case of hemichorea caused by right internal carotid artery stenosis
    Kazuki Muguruma, Atsuko Motoda, Takamichi Sugimoto, Takeshi Kitamura
    Rinsho Shinkeigaku.2019; 59(8): 509.     CrossRef
  • Movement Disorders Following Cerebrovascular Lesions: Etiology, Treatment Options and Prognosis
    Do-Young Kwon
    Journal of Movement Disorders.2016; 9(2): 63.     CrossRef
  • Hemichorea improvement following endarterectomy for internal carotid artery stenosis
    Kazuyuki Noda, Reo Ishimoto, Nobutaka Hattori, Yasuyuki Okuma, Takuji Yamamoto
    Journal of the Neurological Sciences.2016; 371: 45.     CrossRef
  • Hemichorea Resulting from Ischemic Stroke in the Ipsilateral Caudate Nucleus
    Seung-Keun Lee, Ji Sun Kim, Kyung-Bok Lee, Hakjae Roh, Moo-young Ahn
    Journal of Neurocritical Care.2015; 8(2): 109.     CrossRef
Thrombocytopenia Associated with Levodopa Treatment
Ku-Eun Lee, Hyun Seok Kang, Hyun-Jeung Yu, Sook Young Roh
J Mov Disord. 2013;6(1):21-22.
  • 54,562 View
  • 80 Download
  • 3 Crossref
AbstractAbstract PDF

There were few cases of thrombocytopenia associated with levodopa. Herein, we report a patient with Parkinson’s disease, who suffered thrombocytopenia related to long-term use of levodopa.


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  • A 70-year-old male exhibiting parkinsonism-hyperpyrexia syndrome during levodopa carbidopa intestinal gel (LCIG) treatment and suspected carbidopa allergy due to positive drug-induced lymphocyte stimulation test (DLST)
    Aya Kawanami, Masanobu Miyashita, Yuichi Miyagi, Kazuko Hasegawa
    Rinsho Shinkeigaku.2024; 64(1): 17.     CrossRef
  • High rates of blood transfusion associated with Parkinson’s disease
    Shane Shahrestani, Julian Gendreau, Ali R. Tafreshi, Nolan J. Brown, Khashayar Dashtipour
    Neurological Sciences.2022; 43(8): 4761.     CrossRef
  • Platelet abnormalities in patients with Parkinson's disease undergoing preoperative evaluation for deep brain stimulation
    Sheng-Che Chou, Chun-Hwei Tai, Sheng-Hong Tseng
    Scientific Reports.2022;[Epub]     CrossRef

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