Dissociation of Structural and Functional Integrities of the Motor System in Amyotrophic Lateral Sclerosis and Behavioral-Variant Frontotemporal Dementia
2
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Background and Purpose Tis study investigated the structural and functional changes in the motor system in amyotrophic lateral sclerosis (ALS; n=25) and behavioral-variant frontotemporal dementia (bvFTD; n=17) relative to healthy controls (n=37). Methods Structural changes were examined using a region-of-interest approach, applying voxel-based morphometry for gray-matter changes and difusion tensor imaging for white-matter changes. Functional changes in the motor system were elucidated using threshold-tracking transcranial magnetic stimulation (TMS) measurements of upper motor-neuron excitability. Results The structural analyses showed that in ALS there were more white-matter changes in the corticospinal and motor-cortex regions and more gray-matter changes in the cerebellum in comparison to controls. bvFTD showed substantial gray- and white-matter changes across virtually all motor-system regions compared to controls, although the brainstem was afected less than the other regions. Direct comparisons across patient groups showed that the gray- and white-matter motor-system changes inclusive of the motor cortex were greater in bvFTD than in ALS. By contrast, the functional integrity of the motor system was more adversely afected in ALS than in bvFTD, with both patient groups showing increased excitability of upper motor neurons compared to controls. Conclusions Cross-correlation of structural and functional data further revealed a neural dissociation of diferent motor-system regions and tracts covarying with the TMS excitability across both patient groups. Te structural and functional motor-system integrities appear to be dissociated between ALS and bvFTD, which represents useful information for the diagnosis of motor-system changes in these two disorders.
Abstract
Background and Purpose Tis study investigated the structural and functional changes in the motor system in amyotrophic lateral sclerosis (ALS; n=25) and behavioral-variant frontotemporal dementia (bvFTD; n=17) relative to healthy controls (n=37). Methods Structural changes were examined using a region-of-interest approach, applying voxel-based morphometry for gray-matter changes and difusion tensor imaging for white-matter changes. Functional changes in the motor system were elucidated using threshold-tracking transcranial magnetic stimulation (TMS) measurements of upper motor-neuron excitability. Results The structural analyses showed that in ALS there were more white-matter changes in the corticospinal and motor-cortex regions and more gray-matter changes in the cerebellum in comparison to controls. bvFTD showed substantial gray- and white-matter changes across virtually all motor-system regions compared to controls, although the brainstem was afected less than the other regions. Direct comparisons across patient groups showed that the gray- and white-matter motor-system changes inclusive of the motor cortex were greater in bvFTD than in ALS. By contrast, the functional integrity of the motor system was more adversely afected in ALS than in bvFTD, with both patient groups showing increased excitability of upper motor neurons compared to controls. Conclusions Cross-correlation of structural and functional data further revealed a neural dissociation of diferent motor-system regions and tracts covarying with the TMS excitability across both patient groups. Te structural and functional motor-system integrities appear to be dissociated between ALS and bvFTD, which represents useful information for the diagnosis of motor-system changes in these two disorders.
참고문헌 (32)
- Clark CM, "Frontotemporal lobar degeneration with motor neuron disease: a clinical and pathological spectrum", Arch Neurol, 63, pp.489~490 (2006)
- Kiernan MC, "Amyotrophic lateral sclerosis", Lancet, 377, pp.942~955 (2011)
- Mackenzie IR, "TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia", Lancet Neurol, 9, pp.995~1007 (2010)
- Lillo P, "Grey and white matter changes across the amyotrophic lateral sclerosis-frontotemporal dementia continuum", PLoS One, 7, pp.e43993~ (2012)
- Vucic S, "Novel threshold tracking techniques suggest that cortical hyperexcitability is an early feature of motor neuron disease", Brain, 129(Pt 9), pp.2436~2446 (2006)
- Vucic S, "Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosis", Brain, 131(Pt 6), pp.1540~1550 (2008)
- Burrell JR, "Motor neuron dysfunction in frontotemporal dementia", Brain, 134(Pt 9), pp.2582~2594 (2011)
- Rascovsky K, "Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia", Brain, 134(Pt 9), pp.2456~2477 (2011)
- Brooks BR, "El Escorial revisited:revised criteria for the diagnosis of amyotrophic lateral sclerosis", Amyotroph Lateral Scler Other Motor Neuron Disord, 1, pp.293~299 (2000)
- Cedarbaum JM, "The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III)", J Neurol Sci, 169, pp.13~21 (1999)
- Mioshi E, "The Addenbrooke’s Cognitive Examination Revised (ACE-R): a brief cognitive test battery for dementia screening", Int J Geriatr Psychiatry, 21, pp.1078~1085 (2006)
- Wedderburn C, "The utility of the Cambridge Behavioural Inventory in neurodegenerative disease", J Neurol Neurosurg Psychiatry, 79, pp.500~503 (2008)
- 배종석, "The Puzzling Case of Hyperexcitability in Amyotrophic Lateral Sclerosis", Journal of Clinical Neurology, 9(2), pp.65~74 (2013), 대한신경과학회[DOI]
- Vucic S, "Transcranial magnetic stimulation and amyotrophic lateral sclerosis: pathophysiological insights", J Neurol Neurosurg Psychiatry, 84, pp.1161~1170 (2013)
- Chow TW, "Magnetic resonance imaging in frontotemporal dementia shows subcortical atrophy", Dement Geriatr Cogn Disord, 26, pp.79~88 (2008)
- Garibotto V, "Subcortical and deep cortical atrophy in frontotemporal lobar degeneration", Neurobiol Aging, 32, pp.875~884 (2011)
- Looi JC, "Caudate nucleus volumes in frontotemporal lobar degeneration:differential atrophy in subtypes", AJNR Am J Neuroradiol, 29, pp.1537~1543 (2008)
- Cardenas VA, "Deformation-based morphometry reveals brain atrophy in frontotemporal dementia", Arch Neurol, 64, pp.873~877 (2007)
- Turner MR, "Biomarkers in amyotrophic lateral sclerosis", Lancet Neurol, 8, pp.94~109 (2009)
- Braak H, "Amyotrophic lateral sclerosis:dash-like accumulation of phosphorylated TDP-43 in somatodendritic and axonal compartments of somatomotor neurons of the lower brainstem and spinal cord", Acta Neuropathol, 120, pp.67~74 (2010)
- Chen Z, "Grey matter volume changes over the whole brain in amyotrophic lateral sclerosis: a voxel-wise meta-analysis of voxel based morphometry studies", Amyotroph Lateral Scler, 11, pp.549~554 (2010)
- Mezzapesa DM, "Whole-brain and regional brain atrophy in amyotrophic lateral sclerosis", AJNR Am J Neuroradiol, 28, pp.255~259 (2007)
- Mioshi E, "Cortical atrophy in ALS is critically associated with neuropsychiatric and cognitive changes", Neurology, 80, pp.1117~1123 (2013)
- Thivard L, "Diffusion tensor imaging and voxel based morphometry study in amyotrophic lateral sclerosis: relationships with motor disability", J Neurol Neurosurg Psychiatry, 78, pp.889~892 (2007)
- Mahoney CJ, "Frontotemporal dementia with the C9ORF72 hexanucleotide repeat expansion: clinical, neuroanatomical and neuropathological features", Brain, 135(Pt 3), pp.736~750 (2012)
- Sha SJ, "Frontotemporal dementia due to C9ORF72 mutations: clinical and imaging features", Neurology, 79, pp.1002~1011 (2012)
- Keil C, "Longitudinal diffusion tensor imaging in amyotrophic lateral sclerosis", BMC Neurosci, 13, pp.141~ (2012)
- Pearson JP, "Familial frontotemporal dementia with amyotrophic lateral sclerosis and a shared haplotype on chromosome 9p", J Neurol, 258, pp.647~655 (2011)
- Strick PL, "Cerebellum and nonmotor function", Annu Rev Neurosci, 32, pp.413~434 (2009)
- Snowden JS, "Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations", Brain, 135(Pt 3), pp.693~708 (2012)
- Whitwell JL, "Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin and sporadics", Brain, 135(Pt 3), pp.794~806 (2012)
- Josephs KA, "Clinically undetected motor neuron disease in pathologically proven frontotemporal lobar degeneration with motor neuron disease", Arch Neurol, 63, pp.506~512 (2006)