Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors

Abstract Movement disorders (MD) have been linked to degeneration of the substantia nigra (SN) in Parkinson’s disease and include bradykinesia, rigidity, and tremor. They are also present in frontotemporal dementia (FTD), where MD have been linked to frontotemporal lobar degeneration with tau pathol...
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Autor*in:	Fiondella, Luigi [verfasserIn] Gami-Patel, Priya Blok, Christian A. Rozemuller, Annemieke J. M. Hoozemans, Jeroen J. M. Pijnenburg, Yolande A. L. Scarioni, Marta Dijkstra, Anke A.

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2023

Schlagwörter:	Frontotemporal Dementia Frontotemporal Lobar Degeneration TDP-43 Substantia nigra Movement disorders Brain donors

Anmerkung:	© The Author(s) 2023

Übergeordnetes Werk:	Enthalten in: Acta Neuropathologica Communications - London : Biomed Central, 2013, 11(2023), 1 vom: 12. Apr.
Übergeordnetes Werk:	volume:11 ; year:2023 ; number:1 ; day:12 ; month:04

Links:	Volltext

DOI / URN:	10.1186/s40478-023-01560-7

Katalog-ID:	SPR050019600

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520			\|a Abstract Movement disorders (MD) have been linked to degeneration of the substantia nigra (SN) in Parkinson’s disease and include bradykinesia, rigidity, and tremor. They are also present in frontotemporal dementia (FTD), where MD have been linked to frontotemporal lobar degeneration with tau pathology (FTLD-tau). Although MD can also occur in FTLD with TDP-43 pathology (FTLD-TDP), the local pathology in the SN of FTLD-TDP patients with MD is currently unexplored. The aims of this study are to characterize the frequency and the nature of MD in a cohort of FTLD-TDP brain donors and to investigate the relationship between the presence of MD, the nigral neuronal loss, and the TDP-43 burden in the SN. From our cohort of FTLD-TDP patients (n = 53), we included 13 donors who presented with MD (FTLD-MD+), and nine age-sex matched donors without MD (FTLD-MD-) for whom the SN was available. In these donors, the TDP-43 burden and the neuronal density in the SN were assessed with ImageJ and Qupath software. The results were compared between the two groups using T-test. We found that the TDP-43 burden in the SN was higher in FTLD-MD+ (mean 3,43%, SD ± 2,7) compared to FTLD-MD- (mean 1,21%, SD ± 0,67) (p = 0,04), while no significant difference in nigral neuronal density was found between the groups (p = 0,09). 17% of FTLD-TDP patients developed MD, which present as symmetric akinetic-rigid parkinsonism or CBS. Given the absence of a significant nigral neuronal cell loss, TDP-43 induced neuronal dysfunction could be sufficient to cause MD.
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allfields	10.1186/s40478-023-01560-7 doi (DE-627)SPR050019600 (SPR)s40478-023-01560-7-e DE-627 ger DE-627 rakwb eng Fiondella, Luigi verfasserin (orcid)0000-0001-8170-8948 aut Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 Abstract Movement disorders (MD) have been linked to degeneration of the substantia nigra (SN) in Parkinson’s disease and include bradykinesia, rigidity, and tremor. They are also present in frontotemporal dementia (FTD), where MD have been linked to frontotemporal lobar degeneration with tau pathology (FTLD-tau). Although MD can also occur in FTLD with TDP-43 pathology (FTLD-TDP), the local pathology in the SN of FTLD-TDP patients with MD is currently unexplored. The aims of this study are to characterize the frequency and the nature of MD in a cohort of FTLD-TDP brain donors and to investigate the relationship between the presence of MD, the nigral neuronal loss, and the TDP-43 burden in the SN. From our cohort of FTLD-TDP patients (n = 53), we included 13 donors who presented with MD (FTLD-MD+), and nine age-sex matched donors without MD (FTLD-MD-) for whom the SN was available. In these donors, the TDP-43 burden and the neuronal density in the SN were assessed with ImageJ and Qupath software. The results were compared between the two groups using T-test. We found that the TDP-43 burden in the SN was higher in FTLD-MD+ (mean 3,43%, SD ± 2,7) compared to FTLD-MD- (mean 1,21%, SD ± 0,67) (p = 0,04), while no significant difference in nigral neuronal density was found between the groups (p = 0,09). 17% of FTLD-TDP patients developed MD, which present as symmetric akinetic-rigid parkinsonism or CBS. Given the absence of a significant nigral neuronal cell loss, TDP-43 induced neuronal dysfunction could be sufficient to cause MD. Frontotemporal Dementia (dpeaa)DE-He213 Frontotemporal Lobar Degeneration (dpeaa)DE-He213 TDP-43 (dpeaa)DE-He213 Substantia nigra (dpeaa)DE-He213 Movement disorders (dpeaa)DE-He213 Brain donors (dpeaa)DE-He213 Gami-Patel, Priya aut Blok, Christian A. aut Rozemuller, Annemieke J. M. aut Hoozemans, Jeroen J. M. aut Pijnenburg, Yolande A. L. aut Scarioni, Marta aut Dijkstra, Anke A. aut Enthalten in Acta Neuropathologica Communications London : Biomed Central, 2013 11(2023), 1 vom: 12. Apr. (DE-627)746066465 (DE-600)2715589-4 2051-5960 nnns volume:11 year:2023 number:1 day:12 month:04 https://dx.doi.org/10.1186/s40478-023-01560-7 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 11 2023 1 12 04
spelling	10.1186/s40478-023-01560-7 doi (DE-627)SPR050019600 (SPR)s40478-023-01560-7-e DE-627 ger DE-627 rakwb eng Fiondella, Luigi verfasserin (orcid)0000-0001-8170-8948 aut Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 Abstract Movement disorders (MD) have been linked to degeneration of the substantia nigra (SN) in Parkinson’s disease and include bradykinesia, rigidity, and tremor. They are also present in frontotemporal dementia (FTD), where MD have been linked to frontotemporal lobar degeneration with tau pathology (FTLD-tau). Although MD can also occur in FTLD with TDP-43 pathology (FTLD-TDP), the local pathology in the SN of FTLD-TDP patients with MD is currently unexplored. The aims of this study are to characterize the frequency and the nature of MD in a cohort of FTLD-TDP brain donors and to investigate the relationship between the presence of MD, the nigral neuronal loss, and the TDP-43 burden in the SN. From our cohort of FTLD-TDP patients (n = 53), we included 13 donors who presented with MD (FTLD-MD+), and nine age-sex matched donors without MD (FTLD-MD-) for whom the SN was available. In these donors, the TDP-43 burden and the neuronal density in the SN were assessed with ImageJ and Qupath software. The results were compared between the two groups using T-test. We found that the TDP-43 burden in the SN was higher in FTLD-MD+ (mean 3,43%, SD ± 2,7) compared to FTLD-MD- (mean 1,21%, SD ± 0,67) (p = 0,04), while no significant difference in nigral neuronal density was found between the groups (p = 0,09). 17% of FTLD-TDP patients developed MD, which present as symmetric akinetic-rigid parkinsonism or CBS. Given the absence of a significant nigral neuronal cell loss, TDP-43 induced neuronal dysfunction could be sufficient to cause MD. Frontotemporal Dementia (dpeaa)DE-He213 Frontotemporal Lobar Degeneration (dpeaa)DE-He213 TDP-43 (dpeaa)DE-He213 Substantia nigra (dpeaa)DE-He213 Movement disorders (dpeaa)DE-He213 Brain donors (dpeaa)DE-He213 Gami-Patel, Priya aut Blok, Christian A. aut Rozemuller, Annemieke J. M. aut Hoozemans, Jeroen J. M. aut Pijnenburg, Yolande A. L. aut Scarioni, Marta aut Dijkstra, Anke A. aut Enthalten in Acta Neuropathologica Communications London : Biomed Central, 2013 11(2023), 1 vom: 12. Apr. (DE-627)746066465 (DE-600)2715589-4 2051-5960 nnns volume:11 year:2023 number:1 day:12 month:04 https://dx.doi.org/10.1186/s40478-023-01560-7 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 11 2023 1 12 04
allfields_unstemmed	10.1186/s40478-023-01560-7 doi (DE-627)SPR050019600 (SPR)s40478-023-01560-7-e DE-627 ger DE-627 rakwb eng Fiondella, Luigi verfasserin (orcid)0000-0001-8170-8948 aut Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 Abstract Movement disorders (MD) have been linked to degeneration of the substantia nigra (SN) in Parkinson’s disease and include bradykinesia, rigidity, and tremor. They are also present in frontotemporal dementia (FTD), where MD have been linked to frontotemporal lobar degeneration with tau pathology (FTLD-tau). Although MD can also occur in FTLD with TDP-43 pathology (FTLD-TDP), the local pathology in the SN of FTLD-TDP patients with MD is currently unexplored. The aims of this study are to characterize the frequency and the nature of MD in a cohort of FTLD-TDP brain donors and to investigate the relationship between the presence of MD, the nigral neuronal loss, and the TDP-43 burden in the SN. From our cohort of FTLD-TDP patients (n = 53), we included 13 donors who presented with MD (FTLD-MD+), and nine age-sex matched donors without MD (FTLD-MD-) for whom the SN was available. In these donors, the TDP-43 burden and the neuronal density in the SN were assessed with ImageJ and Qupath software. The results were compared between the two groups using T-test. We found that the TDP-43 burden in the SN was higher in FTLD-MD+ (mean 3,43%, SD ± 2,7) compared to FTLD-MD- (mean 1,21%, SD ± 0,67) (p = 0,04), while no significant difference in nigral neuronal density was found between the groups (p = 0,09). 17% of FTLD-TDP patients developed MD, which present as symmetric akinetic-rigid parkinsonism or CBS. Given the absence of a significant nigral neuronal cell loss, TDP-43 induced neuronal dysfunction could be sufficient to cause MD. Frontotemporal Dementia (dpeaa)DE-He213 Frontotemporal Lobar Degeneration (dpeaa)DE-He213 TDP-43 (dpeaa)DE-He213 Substantia nigra (dpeaa)DE-He213 Movement disorders (dpeaa)DE-He213 Brain donors (dpeaa)DE-He213 Gami-Patel, Priya aut Blok, Christian A. aut Rozemuller, Annemieke J. M. aut Hoozemans, Jeroen J. M. aut Pijnenburg, Yolande A. L. aut Scarioni, Marta aut Dijkstra, Anke A. aut Enthalten in Acta Neuropathologica Communications London : Biomed Central, 2013 11(2023), 1 vom: 12. Apr. (DE-627)746066465 (DE-600)2715589-4 2051-5960 nnns volume:11 year:2023 number:1 day:12 month:04 https://dx.doi.org/10.1186/s40478-023-01560-7 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 11 2023 1 12 04
allfieldsGer	10.1186/s40478-023-01560-7 doi (DE-627)SPR050019600 (SPR)s40478-023-01560-7-e DE-627 ger DE-627 rakwb eng Fiondella, Luigi verfasserin (orcid)0000-0001-8170-8948 aut Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 Abstract Movement disorders (MD) have been linked to degeneration of the substantia nigra (SN) in Parkinson’s disease and include bradykinesia, rigidity, and tremor. They are also present in frontotemporal dementia (FTD), where MD have been linked to frontotemporal lobar degeneration with tau pathology (FTLD-tau). Although MD can also occur in FTLD with TDP-43 pathology (FTLD-TDP), the local pathology in the SN of FTLD-TDP patients with MD is currently unexplored. The aims of this study are to characterize the frequency and the nature of MD in a cohort of FTLD-TDP brain donors and to investigate the relationship between the presence of MD, the nigral neuronal loss, and the TDP-43 burden in the SN. From our cohort of FTLD-TDP patients (n = 53), we included 13 donors who presented with MD (FTLD-MD+), and nine age-sex matched donors without MD (FTLD-MD-) for whom the SN was available. In these donors, the TDP-43 burden and the neuronal density in the SN were assessed with ImageJ and Qupath software. The results were compared between the two groups using T-test. We found that the TDP-43 burden in the SN was higher in FTLD-MD+ (mean 3,43%, SD ± 2,7) compared to FTLD-MD- (mean 1,21%, SD ± 0,67) (p = 0,04), while no significant difference in nigral neuronal density was found between the groups (p = 0,09). 17% of FTLD-TDP patients developed MD, which present as symmetric akinetic-rigid parkinsonism or CBS. Given the absence of a significant nigral neuronal cell loss, TDP-43 induced neuronal dysfunction could be sufficient to cause MD. Frontotemporal Dementia (dpeaa)DE-He213 Frontotemporal Lobar Degeneration (dpeaa)DE-He213 TDP-43 (dpeaa)DE-He213 Substantia nigra (dpeaa)DE-He213 Movement disorders (dpeaa)DE-He213 Brain donors (dpeaa)DE-He213 Gami-Patel, Priya aut Blok, Christian A. aut Rozemuller, Annemieke J. M. aut Hoozemans, Jeroen J. M. aut Pijnenburg, Yolande A. L. aut Scarioni, Marta aut Dijkstra, Anke A. aut Enthalten in Acta Neuropathologica Communications London : Biomed Central, 2013 11(2023), 1 vom: 12. Apr. (DE-627)746066465 (DE-600)2715589-4 2051-5960 nnns volume:11 year:2023 number:1 day:12 month:04 https://dx.doi.org/10.1186/s40478-023-01560-7 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 11 2023 1 12 04
allfieldsSound	10.1186/s40478-023-01560-7 doi (DE-627)SPR050019600 (SPR)s40478-023-01560-7-e DE-627 ger DE-627 rakwb eng Fiondella, Luigi verfasserin (orcid)0000-0001-8170-8948 aut Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors 2023 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2023 Abstract Movement disorders (MD) have been linked to degeneration of the substantia nigra (SN) in Parkinson’s disease and include bradykinesia, rigidity, and tremor. They are also present in frontotemporal dementia (FTD), where MD have been linked to frontotemporal lobar degeneration with tau pathology (FTLD-tau). Although MD can also occur in FTLD with TDP-43 pathology (FTLD-TDP), the local pathology in the SN of FTLD-TDP patients with MD is currently unexplored. The aims of this study are to characterize the frequency and the nature of MD in a cohort of FTLD-TDP brain donors and to investigate the relationship between the presence of MD, the nigral neuronal loss, and the TDP-43 burden in the SN. From our cohort of FTLD-TDP patients (n = 53), we included 13 donors who presented with MD (FTLD-MD+), and nine age-sex matched donors without MD (FTLD-MD-) for whom the SN was available. In these donors, the TDP-43 burden and the neuronal density in the SN were assessed with ImageJ and Qupath software. The results were compared between the two groups using T-test. We found that the TDP-43 burden in the SN was higher in FTLD-MD+ (mean 3,43%, SD ± 2,7) compared to FTLD-MD- (mean 1,21%, SD ± 0,67) (p = 0,04), while no significant difference in nigral neuronal density was found between the groups (p = 0,09). 17% of FTLD-TDP patients developed MD, which present as symmetric akinetic-rigid parkinsonism or CBS. Given the absence of a significant nigral neuronal cell loss, TDP-43 induced neuronal dysfunction could be sufficient to cause MD. Frontotemporal Dementia (dpeaa)DE-He213 Frontotemporal Lobar Degeneration (dpeaa)DE-He213 TDP-43 (dpeaa)DE-He213 Substantia nigra (dpeaa)DE-He213 Movement disorders (dpeaa)DE-He213 Brain donors (dpeaa)DE-He213 Gami-Patel, Priya aut Blok, Christian A. aut Rozemuller, Annemieke J. M. aut Hoozemans, Jeroen J. M. aut Pijnenburg, Yolande A. L. aut Scarioni, Marta aut Dijkstra, Anke A. aut Enthalten in Acta Neuropathologica Communications London : Biomed Central, 2013 11(2023), 1 vom: 12. Apr. (DE-627)746066465 (DE-600)2715589-4 2051-5960 nnns volume:11 year:2023 number:1 day:12 month:04 https://dx.doi.org/10.1186/s40478-023-01560-7 kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2005 GBV_ILN_2009 GBV_ILN_2014 GBV_ILN_2055 GBV_ILN_2111 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 11 2023 1 12 04
language	English
source	Enthalten in Acta Neuropathologica Communications 11(2023), 1 vom: 12. Apr. volume:11 year:2023 number:1 day:12 month:04
sourceStr	Enthalten in Acta Neuropathologica Communications 11(2023), 1 vom: 12. Apr. volume:11 year:2023 number:1 day:12 month:04
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	Frontotemporal Dementia Frontotemporal Lobar Degeneration TDP-43 Substantia nigra Movement disorders Brain donors
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container_title	Acta Neuropathologica Communications
authorswithroles_txt_mv	Fiondella, Luigi @@aut@@ Gami-Patel, Priya @@aut@@ Blok, Christian A. @@aut@@ Rozemuller, Annemieke J. M. @@aut@@ Hoozemans, Jeroen J. M. @@aut@@ Pijnenburg, Yolande A. L. @@aut@@ Scarioni, Marta @@aut@@ Dijkstra, Anke A. @@aut@@
publishDateDaySort_date	2023-04-12T00:00:00Z
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author	Fiondella, Luigi
spellingShingle	Fiondella, Luigi misc Frontotemporal Dementia misc Frontotemporal Lobar Degeneration misc TDP-43 misc Substantia nigra misc Movement disorders misc Brain donors Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors
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illustrated	Not Illustrated
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topic_title	Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors Frontotemporal Dementia (dpeaa)DE-He213 Frontotemporal Lobar Degeneration (dpeaa)DE-He213 TDP-43 (dpeaa)DE-He213 Substantia nigra (dpeaa)DE-He213 Movement disorders (dpeaa)DE-He213 Brain donors (dpeaa)DE-He213
topic	misc Frontotemporal Dementia misc Frontotemporal Lobar Degeneration misc TDP-43 misc Substantia nigra misc Movement disorders misc Brain donors
topic_unstemmed	misc Frontotemporal Dementia misc Frontotemporal Lobar Degeneration misc TDP-43 misc Substantia nigra misc Movement disorders misc Brain donors
topic_browse	misc Frontotemporal Dementia misc Frontotemporal Lobar Degeneration misc TDP-43 misc Substantia nigra misc Movement disorders misc Brain donors
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title	Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors
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title_full	Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors
author_sort	Fiondella, Luigi
journal	Acta Neuropathologica Communications
journalStr	Acta Neuropathologica Communications
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author_browse	Fiondella, Luigi Gami-Patel, Priya Blok, Christian A. Rozemuller, Annemieke J. M. Hoozemans, Jeroen J. M. Pijnenburg, Yolande A. L. Scarioni, Marta Dijkstra, Anke A.
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author-letter	Fiondella, Luigi
doi_str_mv	10.1186/s40478-023-01560-7
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title_sort	movement disorders are linked to tdp-43 burden in the substantia nigra of ftld-tdp brain donors
title_auth	Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors
abstract	Abstract Movement disorders (MD) have been linked to degeneration of the substantia nigra (SN) in Parkinson’s disease and include bradykinesia, rigidity, and tremor. They are also present in frontotemporal dementia (FTD), where MD have been linked to frontotemporal lobar degeneration with tau pathology (FTLD-tau). Although MD can also occur in FTLD with TDP-43 pathology (FTLD-TDP), the local pathology in the SN of FTLD-TDP patients with MD is currently unexplored. The aims of this study are to characterize the frequency and the nature of MD in a cohort of FTLD-TDP brain donors and to investigate the relationship between the presence of MD, the nigral neuronal loss, and the TDP-43 burden in the SN. From our cohort of FTLD-TDP patients (n = 53), we included 13 donors who presented with MD (FTLD-MD+), and nine age-sex matched donors without MD (FTLD-MD-) for whom the SN was available. In these donors, the TDP-43 burden and the neuronal density in the SN were assessed with ImageJ and Qupath software. The results were compared between the two groups using T-test. We found that the TDP-43 burden in the SN was higher in FTLD-MD+ (mean 3,43%, SD ± 2,7) compared to FTLD-MD- (mean 1,21%, SD ± 0,67) (p = 0,04), while no significant difference in nigral neuronal density was found between the groups (p = 0,09). 17% of FTLD-TDP patients developed MD, which present as symmetric akinetic-rigid parkinsonism or CBS. Given the absence of a significant nigral neuronal cell loss, TDP-43 induced neuronal dysfunction could be sufficient to cause MD. © The Author(s) 2023
abstractGer	Abstract Movement disorders (MD) have been linked to degeneration of the substantia nigra (SN) in Parkinson’s disease and include bradykinesia, rigidity, and tremor. They are also present in frontotemporal dementia (FTD), where MD have been linked to frontotemporal lobar degeneration with tau pathology (FTLD-tau). Although MD can also occur in FTLD with TDP-43 pathology (FTLD-TDP), the local pathology in the SN of FTLD-TDP patients with MD is currently unexplored. The aims of this study are to characterize the frequency and the nature of MD in a cohort of FTLD-TDP brain donors and to investigate the relationship between the presence of MD, the nigral neuronal loss, and the TDP-43 burden in the SN. From our cohort of FTLD-TDP patients (n = 53), we included 13 donors who presented with MD (FTLD-MD+), and nine age-sex matched donors without MD (FTLD-MD-) for whom the SN was available. In these donors, the TDP-43 burden and the neuronal density in the SN were assessed with ImageJ and Qupath software. The results were compared between the two groups using T-test. We found that the TDP-43 burden in the SN was higher in FTLD-MD+ (mean 3,43%, SD ± 2,7) compared to FTLD-MD- (mean 1,21%, SD ± 0,67) (p = 0,04), while no significant difference in nigral neuronal density was found between the groups (p = 0,09). 17% of FTLD-TDP patients developed MD, which present as symmetric akinetic-rigid parkinsonism or CBS. Given the absence of a significant nigral neuronal cell loss, TDP-43 induced neuronal dysfunction could be sufficient to cause MD. © The Author(s) 2023
abstract_unstemmed	Abstract Movement disorders (MD) have been linked to degeneration of the substantia nigra (SN) in Parkinson’s disease and include bradykinesia, rigidity, and tremor. They are also present in frontotemporal dementia (FTD), where MD have been linked to frontotemporal lobar degeneration with tau pathology (FTLD-tau). Although MD can also occur in FTLD with TDP-43 pathology (FTLD-TDP), the local pathology in the SN of FTLD-TDP patients with MD is currently unexplored. The aims of this study are to characterize the frequency and the nature of MD in a cohort of FTLD-TDP brain donors and to investigate the relationship between the presence of MD, the nigral neuronal loss, and the TDP-43 burden in the SN. From our cohort of FTLD-TDP patients (n = 53), we included 13 donors who presented with MD (FTLD-MD+), and nine age-sex matched donors without MD (FTLD-MD-) for whom the SN was available. In these donors, the TDP-43 burden and the neuronal density in the SN were assessed with ImageJ and Qupath software. The results were compared between the two groups using T-test. We found that the TDP-43 burden in the SN was higher in FTLD-MD+ (mean 3,43%, SD ± 2,7) compared to FTLD-MD- (mean 1,21%, SD ± 0,67) (p = 0,04), while no significant difference in nigral neuronal density was found between the groups (p = 0,09). 17% of FTLD-TDP patients developed MD, which present as symmetric akinetic-rigid parkinsonism or CBS. Given the absence of a significant nigral neuronal cell loss, TDP-43 induced neuronal dysfunction could be sufficient to cause MD. © The Author(s) 2023
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title_short	Movement disorders are linked to TDP-43 burden in the substantia nigra of FTLD-TDP brain donors
url	https://dx.doi.org/10.1186/s40478-023-01560-7
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author2	Gami-Patel, Priya Blok, Christian A. Rozemuller, Annemieke J. M. Hoozemans, Jeroen J. M. Pijnenburg, Yolande A. L. Scarioni, Marta Dijkstra, Anke A.
author2Str	Gami-Patel, Priya Blok, Christian A. Rozemuller, Annemieke J. M. Hoozemans, Jeroen J. M. Pijnenburg, Yolande A. L. Scarioni, Marta Dijkstra, Anke A.
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up_date	2024-07-04T03:09:03.101Z
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