Cortical hyperexcitability and disease spread in amyotrophic lateral sclerosis.

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Title: Cortical hyperexcitability and disease spread in amyotrophic lateral sclerosis.
Authors: Menon, P., Geevasinga, N., Bos, M., Yiannikas, C., Kiernan, M. C., Vucic, S.
Source: European Journal of Neurology. Jun2017, Vol. 24 Issue 6, p816-824. 9p. 1 Chart, 4 Graphs.
Subjects: Amyotrophic lateral sclerosis, Pathological physiology, Transcranial magnetic stimulation, Motor cortex, Diagnosis, Patients
Abstract: Background and purpose Amyotrophic lateral sclerosis ( ALS) is characterized by focal disease onset with a predominantly contiguous pattern of disease spread. The pathophysiological mechanisms underlying disease progression in ALS have not been elucidated. Given that cortical hyperexcitability has been identified as an important pathogenic mechanism in ALS, the aim of the present study was to determine whether changes in cortical function could mediate disease spread in ALS. Methods Threshold-tracking transcranial magnetic stimulation was undertaken in 50 patients with sporadic ALS with recording of responses over both abductor pollicis brevis muscles, with results matched to clinical assessments and concurrent neurophysiological investigation of lower motor neuron function. Subsequently, patients were followed longitudinally to map patterns of clinical disease progression. Results Cortical dysfunction was evident over both motor cortices, with hyperexcitability more prominent over the dominant motor cortex, contralateral to the site of disease onset, with reduction of resting motor threshold ( F = 3.83, P < 0.05), short-interval intracortical inhibition ( F = 15.0, P < 0.0001) and cortical silent-period duration ( F = 8.01, P < 0.01), along with an increase in motor evoked potential amplitude ( F = 5.66, P < 0.01). In addition, patterns of cortical change were consistent with a contiguous pattern of disease progression. Conclusions Cortical hyperexcitability appears to be more prominent over the dominant motor cortex, contralateral to the side of symptom onset, and contributes to a contiguous pattern of spread in sporadic ALS. [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.)
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  Data: Cortical hyperexcitability and disease spread in amyotrophic lateral sclerosis.
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  Data: &lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Menon%2C+P%2E%22&quot;&gt;Menon, P.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Geevasinga%2C+N%2E%22&quot;&gt;Geevasinga, N.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Bos%2C+M%2E%22&quot;&gt;Bos, M.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Yiannikas%2C+C%2E%22&quot;&gt;Yiannikas, C.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Kiernan%2C+M%2E+C%2E%22&quot;&gt;Kiernan, M. C.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Vucic%2C+S%2E%22&quot;&gt;Vucic, S.&lt;/searchLink&gt;
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  Data: &lt;searchLink fieldCode=&quot;JN&quot; term=&quot;%22European+Journal+of+Neurology%22&quot;&gt;European Journal of Neurology&lt;/searchLink&gt;. Jun2017, Vol. 24 Issue 6, p816-824. 9p. 1 Chart, 4 Graphs.
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  Data: &lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Amyotrophic+lateral+sclerosis%22&quot;&gt;Amyotrophic lateral sclerosis&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Pathological+physiology%22&quot;&gt;Pathological physiology&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Transcranial+magnetic+stimulation%22&quot;&gt;Transcranial magnetic stimulation&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Motor+cortex%22&quot;&gt;Motor cortex&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Diagnosis%22&quot;&gt;Diagnosis&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Patients%22&quot;&gt;Patients&lt;/searchLink&gt;
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  Data: Background and purpose Amyotrophic lateral sclerosis ( ALS) is characterized by focal disease onset with a predominantly contiguous pattern of disease spread. The pathophysiological mechanisms underlying disease progression in ALS have not been elucidated. Given that cortical hyperexcitability has been identified as an important pathogenic mechanism in ALS, the aim of the present study was to determine whether changes in cortical function could mediate disease spread in ALS. Methods Threshold-tracking transcranial magnetic stimulation was undertaken in 50 patients with sporadic ALS with recording of responses over both abductor pollicis brevis muscles, with results matched to clinical assessments and concurrent neurophysiological investigation of lower motor neuron function. Subsequently, patients were followed longitudinally to map patterns of clinical disease progression. Results Cortical dysfunction was evident over both motor cortices, with hyperexcitability more prominent over the dominant motor cortex, contralateral to the site of disease onset, with reduction of resting motor threshold ( F = 3.83, P &lt; 0.05), short-interval intracortical inhibition ( F = 15.0, P &lt; 0.0001) and cortical silent-period duration ( F = 8.01, P &lt; 0.01), along with an increase in motor evoked potential amplitude ( F = 5.66, P &lt; 0.01). In addition, patterns of cortical change were consistent with a contiguous pattern of disease progression. Conclusions Cortical hyperexcitability appears to be more prominent over the dominant motor cortex, contralateral to the side of symptom onset, and contributes to a contiguous pattern of spread in sporadic ALS. [ABSTRACT FROM AUTHOR]
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  Data: &lt;i&gt;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&#39;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.&lt;/i&gt; (Copyright applies to all Abstracts.)
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        Value: 10.1111/ene.13295
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        Text: English
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        PageCount: 9
        StartPage: 816
    Subjects:
      – SubjectFull: Amyotrophic lateral sclerosis
        Type: general
      – SubjectFull: Pathological physiology
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      – SubjectFull: Transcranial magnetic stimulation
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      – TitleFull: Cortical hyperexcitability and disease spread in amyotrophic lateral sclerosis.
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              Text: Jun2017
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              Y: 2017
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