Cortical inexcitability defines an adverse clinical profile in amyotrophic lateral sclerosis.
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| Title: | Cortical inexcitability defines an adverse clinical profile in amyotrophic lateral sclerosis. |
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| Authors: | Dharmadasa, T. (AUTHOR), Howells, J. (AUTHOR), Matamala, J. M. (AUTHOR), Simon, N. G. (AUTHOR), Burke, D. (AUTHOR), Vucic, S. (AUTHOR), Kiernan, M. C. (AUTHOR) |
| Source: | European Journal of Neurology. Jan2021, Vol. 28 Issue 1, p90-97. 8p. |
| Subjects: | Amyotrophic lateral sclerosis, Evoked potentials (Electrophysiology), Transcranial magnetic stimulation, Survival analysis (Biometry), Motor cortex |
| Abstract: | Background and purpose: In amyotrophic lateral sclerosis, studies using threshold‐tracking transcranial magnetic stimulation (TMS) have identified corticomotoneuronal dysfunction as a key pathogenic mechanism. Some patients, however, display no motor response at maximal TMS intensities, termed here an 'inexcitable' motor cortex. The extent to which this cortical difference impacts clinical outcomes remains unclear. The aim of this study was to determine the clinical profile of patients with inexcitability to TMS. Methods: Motor cortex excitability was evaluated using TMS. Patients in whom a motor evoked potential could not be recorded in one or more limbs at maximal TMS intensities were classified as four‐limb or partially inexcitable. Demographic information, clinical variables and survival data were analysed. Results: From 133 patients, 40 were identified with inexcitability. Patients with four‐limb inexcitability were younger (P = 0.03) and had lower‐limb disease onset (64%), greater functional disability (P < 0.001) and faster disease progression (P = 0.02), particularly if inexcitability developed within 1 year of symptoms (P < 0.01). Patients with partial inexcitability had higher resting motor thresholds compared to the excitable cohort (P < 0.01), but averaged short‐interval intracortical inhibition was similar (P = 0.5). Mean survival was reduced if inexcitability involved all limbs within 12 months of symptom onset (P = 0.04). Conclusion: Amyotrophic lateral sclerosis patients with inexcitability of all four limbs to TMS have a distinct clinical profile of younger age and lower‐limb onset. Importantly, these patients display a more malignant disease trajectory, with faster progression, greater functional disability and reduced survival when occurring in early disease. This measure may provide an important prognostic marker in amyotrophic lateral sclerosis. [ABSTRACT FROM AUTHOR] |
| Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.) | |
| Database: | Psychology and Behavioral Sciences Collection |
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| Header | DbId: pbh DbLabel: Psychology and Behavioral Sciences Collection An: 147773133 AccessLevel: 6 PubType: Academic Journal PubTypeId: academicJournal PreciseRelevancyScore: 0 |
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| Items | – Name: Title Label: Title Group: Ti Data: Cortical inexcitability defines an adverse clinical profile in amyotrophic lateral sclerosis. – Name: Author Label: Authors Group: Au Data: <searchLink fieldCode="AR" term="%22Dharmadasa%2C+T%2E%22">Dharmadasa, T.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Howells%2C+J%2E%22">Howells, J.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Matamala%2C+J%2E+M%2E%22">Matamala, J. M.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Simon%2C+N%2E+G%2E%22">Simon, N. G.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Burke%2C+D%2E%22">Burke, D.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Vucic%2C+S%2E%22">Vucic, S.</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Kiernan%2C+M%2E+C%2E%22">Kiernan, M. C.</searchLink> (AUTHOR) – Name: TitleSource Label: Source Group: Src Data: <searchLink fieldCode="JN" term="%22European+Journal+of+Neurology%22">European Journal of Neurology</searchLink>. Jan2021, Vol. 28 Issue 1, p90-97. 8p. – Name: Subject Label: Subjects Group: Su Data: <searchLink fieldCode="DE" term="%22Amyotrophic+lateral+sclerosis%22">Amyotrophic lateral sclerosis</searchLink><br /><searchLink fieldCode="DE" term="%22Evoked+potentials+%28Electrophysiology%29%22">Evoked potentials (Electrophysiology)</searchLink><br /><searchLink fieldCode="DE" term="%22Transcranial+magnetic+stimulation%22">Transcranial magnetic stimulation</searchLink><br /><searchLink fieldCode="DE" term="%22Survival+analysis+%28Biometry%29%22">Survival analysis (Biometry)</searchLink><br /><searchLink fieldCode="DE" term="%22Motor+cortex%22">Motor cortex</searchLink> – Name: Abstract Label: Abstract Group: Ab Data: Background and purpose: In amyotrophic lateral sclerosis, studies using threshold‐tracking transcranial magnetic stimulation (TMS) have identified corticomotoneuronal dysfunction as a key pathogenic mechanism. Some patients, however, display no motor response at maximal TMS intensities, termed here an 'inexcitable' motor cortex. The extent to which this cortical difference impacts clinical outcomes remains unclear. The aim of this study was to determine the clinical profile of patients with inexcitability to TMS. Methods: Motor cortex excitability was evaluated using TMS. Patients in whom a motor evoked potential could not be recorded in one or more limbs at maximal TMS intensities were classified as four‐limb or partially inexcitable. Demographic information, clinical variables and survival data were analysed. Results: From 133 patients, 40 were identified with inexcitability. Patients with four‐limb inexcitability were younger (P = 0.03) and had lower‐limb disease onset (64%), greater functional disability (P < 0.001) and faster disease progression (P = 0.02), particularly if inexcitability developed within 1 year of symptoms (P < 0.01). Patients with partial inexcitability had higher resting motor thresholds compared to the excitable cohort (P < 0.01), but averaged short‐interval intracortical inhibition was similar (P = 0.5). Mean survival was reduced if inexcitability involved all limbs within 12 months of symptom onset (P = 0.04). Conclusion: Amyotrophic lateral sclerosis patients with inexcitability of all four limbs to TMS have a distinct clinical profile of younger age and lower‐limb onset. Importantly, these patients display a more malignant disease trajectory, with faster progression, greater functional disability and reduced survival when occurring in early disease. This measure may provide an important prognostic marker in amyotrophic lateral sclerosis. [ABSTRACT FROM AUTHOR] – Name: AbstractSuppliedCopyright Label: Group: Ab Data: <i>Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.) |
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| RecordInfo | BibRecord: BibEntity: Identifiers: – Type: doi Value: 10.1111/ene.14515 Languages: – Code: eng Text: English PhysicalDescription: Pagination: PageCount: 8 StartPage: 90 Subjects: – SubjectFull: Amyotrophic lateral sclerosis Type: general – SubjectFull: Evoked potentials (Electrophysiology) Type: general – SubjectFull: Transcranial magnetic stimulation Type: general – SubjectFull: Survival analysis (Biometry) Type: general – SubjectFull: Motor cortex Type: general Titles: – TitleFull: Cortical inexcitability defines an adverse clinical profile in amyotrophic lateral sclerosis. Type: main BibRelationships: HasContributorRelationships: – PersonEntity: Name: NameFull: Dharmadasa, T. – PersonEntity: Name: NameFull: Howells, J. – PersonEntity: Name: NameFull: Matamala, J. M. – PersonEntity: Name: NameFull: Simon, N. G. – PersonEntity: Name: NameFull: Burke, D. – PersonEntity: Name: NameFull: Vucic, S. – PersonEntity: Name: NameFull: Kiernan, M. C. IsPartOfRelationships: – BibEntity: Dates: – D: 01 M: 01 Text: Jan2021 Type: published Y: 2021 Identifiers: – Type: issn-print Value: 13515101 Numbering: – Type: volume Value: 28 – Type: issue Value: 1 Titles: – TitleFull: European Journal of Neurology Type: main |
| ResultId | 1 |
