Dissociation of structural and functional integrities of the motor system in amyotrophic lateral sclerosis and behavioral-variant frontotemporal dementia.
Dissociation of structural and functional integrities of the motor system in amyotrophic lateral sclerosis and behavioral-variant frontotemporal dementia.
Background and PurposezzThis study investigated the structural and functional changes inthe motor system in amyotrophic lateral sclerosis (ALS; n=25) and behavioral-variant frontotemporal dementia (bvFTD; n=17) relative to healthy controls (n=37).MethodszzStructural changes were examined using a region-of-interest approach, applyingvoxel-based morphometry for gray-matter changes and diffusion tensor imaging for white-matter changes. Functional changes in the motor system were elucidated using threshold-trackingtranscranial magnetic stimulation (TMS) measurements of upper motor-neuron excitability.ResultszzThe structural analyses showed that in ALS there were more white-matter changes inthe corticospinal and motor-cortex regions and more gray-matter changes in the cerebellum incomparison to controls. bvFTD showed substantial gray- and white-matter changes across virtually all motor-system regions compared to controls, although the brainstem was affected lessthan the other regions. Direct comparisons across patient groups showed that the gray- andwhite-matter motor-system changes inclusive of the motor cortex were greater in bvFTD thanin ALS. By contrast, the functional integrity of the motor system was more adversely affected inALS than in bvFTD, with both patient groups showing increased excitability of upper motorneurons compared to controls.ConclusionszzCross-correlation of structural and functional data further revealed a neuraldissociation of different motor-system regions and tracts covarying with the TMS excitabilityacross both patient groups. The structural and functional motor-system integrities appear to bedissociated between ALS and bvFTD, which represents useful information for the diagnosis ofmotor-system changes in these two disorders
JS, Bae
01cf80e1-fbd5-4cc8-a5e3-13aaeaa2a994
Ferguson, M
7863cff2-b6de-449d-8431-45177b864a95
Tan, R
d95992a7-805d-4ec7-8b3b-571c8c64706e
Mioshi, E
5310242a-e90b-476d-a02d-51f13f973c8e
Simon, N
dc33ac06-7da3-46b3-b005-f4670c865104
Burrell, J
23629bf3-31aa-4d14-b988-d2612999c00c
Vucic, S
916aa133-994f-4c38-8941-0e7756645a5a
JR, Hodges
936bf0c6-b9ab-46eb-a3ed-2a6b719019aa
MC, Kiernan
00e2d9c6-7249-4e39-bdfd-d39ea40412bf
Hornberger, M
a48c1c63-422a-4c11-9a51-c7be0aa3026d
February 2016
JS, Bae
01cf80e1-fbd5-4cc8-a5e3-13aaeaa2a994
Ferguson, M
7863cff2-b6de-449d-8431-45177b864a95
Tan, R
d95992a7-805d-4ec7-8b3b-571c8c64706e
Mioshi, E
5310242a-e90b-476d-a02d-51f13f973c8e
Simon, N
dc33ac06-7da3-46b3-b005-f4670c865104
Burrell, J
23629bf3-31aa-4d14-b988-d2612999c00c
Vucic, S
916aa133-994f-4c38-8941-0e7756645a5a
JR, Hodges
936bf0c6-b9ab-46eb-a3ed-2a6b719019aa
MC, Kiernan
00e2d9c6-7249-4e39-bdfd-d39ea40412bf
Hornberger, M
a48c1c63-422a-4c11-9a51-c7be0aa3026d
JS, Bae, Ferguson, M, Tan, R, Mioshi, E, Simon, N, Burrell, J, Vucic, S, JR, Hodges, MC, Kiernan and Hornberger, M
(2016)
Dissociation of structural and functional integrities of the motor system in amyotrophic lateral sclerosis and behavioral-variant frontotemporal dementia.
Journal of Clinical Neurology.
(doi:10.3988/jcn.2016.12.2.209).
Abstract
Background and PurposezzThis study investigated the structural and functional changes inthe motor system in amyotrophic lateral sclerosis (ALS; n=25) and behavioral-variant frontotemporal dementia (bvFTD; n=17) relative to healthy controls (n=37).MethodszzStructural changes were examined using a region-of-interest approach, applyingvoxel-based morphometry for gray-matter changes and diffusion tensor imaging for white-matter changes. Functional changes in the motor system were elucidated using threshold-trackingtranscranial magnetic stimulation (TMS) measurements of upper motor-neuron excitability.ResultszzThe structural analyses showed that in ALS there were more white-matter changes inthe corticospinal and motor-cortex regions and more gray-matter changes in the cerebellum incomparison to controls. bvFTD showed substantial gray- and white-matter changes across virtually all motor-system regions compared to controls, although the brainstem was affected lessthan the other regions. Direct comparisons across patient groups showed that the gray- andwhite-matter motor-system changes inclusive of the motor cortex were greater in bvFTD thanin ALS. By contrast, the functional integrity of the motor system was more adversely affected inALS than in bvFTD, with both patient groups showing increased excitability of upper motorneurons compared to controls.ConclusionszzCross-correlation of structural and functional data further revealed a neuraldissociation of different motor-system regions and tracts covarying with the TMS excitabilityacross both patient groups. The structural and functional motor-system integrities appear to bedissociated between ALS and bvFTD, which represents useful information for the diagnosis ofmotor-system changes in these two disorders
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0145JCN_jcn-12-209
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Accepted/In Press date: 1 November 2015
Published date: February 2016
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Local EPrints ID: 505194
URI: http://eprints.soton.ac.uk/id/eprint/505194
PURE UUID: 86813608-7833-48be-9e13-9cad6987ad2d
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Date deposited: 01 Oct 2025 16:44
Last modified: 02 Oct 2025 02:19
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Author:
Bae JS
Author:
M Ferguson
Author:
R Tan
Author:
E Mioshi
Author:
N Simon
Author:
J Burrell
Author:
S Vucic
Author:
Hodges JR
Author:
Kiernan MC
Author:
M Hornberger
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