New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration
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 posse...
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doaj-3a1574e466e1424fa500c1ee89863b212020-11-24T22:42:41ZengKorean Movement Disorders SocietyJournal of Movement Disorders2005-940X2093-49392013-04-01611810.14802/jmd.130014New Perspective on Parkinsonism in Frontotemporal Lobar DegenerationHee Kyung ParkSun J. ChungFrontotemporal 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.http://e-jmd.org/upload/jmd-6-1-1-1.pdfFrontotemporal dementiaFrontotemporal lobar degenerationParkinsonism |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Hee Kyung Park Sun J. Chung |
spellingShingle |
Hee Kyung Park Sun J. Chung New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration Journal of Movement Disorders Frontotemporal dementia Frontotemporal lobar degeneration Parkinsonism |
author_facet |
Hee Kyung Park Sun J. Chung |
author_sort |
Hee Kyung Park |
title |
New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration |
title_short |
New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration |
title_full |
New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration |
title_fullStr |
New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration |
title_full_unstemmed |
New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration |
title_sort |
new perspective on parkinsonism in frontotemporal lobar degeneration |
publisher |
Korean Movement Disorders Society |
series |
Journal of Movement Disorders |
issn |
2005-940X 2093-4939 |
publishDate |
2013-04-01 |
description |
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. |
topic |
Frontotemporal dementia Frontotemporal lobar degeneration Parkinsonism |
url |
http://e-jmd.org/upload/jmd-6-1-1-1.pdf |
work_keys_str_mv |
AT heekyungpark newperspectiveonparkinsonisminfrontotemporallobardegeneration AT sunjchung newperspectiveonparkinsonisminfrontotemporallobardegeneration |
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