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Cortical excitability changes distinguish the motor neuron disease phenotypes from hereditary spastic paraplegia.

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Title: Cortical excitability changes distinguish the motor neuron disease phenotypes from hereditary spastic paraplegia.
Authors: Geevasinga, N., Menon, P., Sue, C. M., Kumar, K. R., Ng, K., Yiannikas, C., Kiernan, M. C., Vucic, S.
Source: European Journal of Neurology. May2015, Vol. 22 Issue 5, p826-826. 8p.
Subjects: Paraplegia, Motor neuron diseases, Amyotrophic lateral sclerosis, Genetic mutation, Phenotypes
Abstract: Background and purpose Cortical hyperexcitability has been identified as an important pathogenic mechanism in motor neuron disease ( MND). The issue as to whether cortical hyperexcitability is a common process across the MND phenotypes, including amyotrophic lateral sclerosis ( ALS) and primary lateral sclerosis ( PLS), remains unresolved. Separately, the clinical distinction between PLS and 'mimic disorders' such as hereditary spastic paraparesis ( HSP) may be difficult, potentially delaying diagnosis. Consequently, the aim of the present study was to determine the nature and spectrum of cortical excitability changes across the MND phenotypes, and to determine whether the presence of cortical dysfunction distinguishes PLS from HSP. Methods Cortical excitability studies were undertaken on a cohort of 14 PLS, 82 ALS and 13 HSP patients with mutations in the spastin gene. Results Cortical hyperexcitability, as heralded by reduction of short interval intracortical inhibition ( PLS 0.26%, −3.8% to 1.4%; ALS −0.15%, −3.6% to 7.0%; P < 0.01) and cortical silent period duration ( CSPPLS 172.2 ± 5.4 ms; CSPALS 178.1 ± 5.1 ms; P < 0.001), along with an increase in intracortical facilitation was evident in ALS and PLS phenotypes, although appeared more frequently in ALS. Inexcitability of the motor cortex was more frequent in PLS ( PLS 71%, ALS 24%, P < 0.0001). Cortical excitability was preserved in HSP. Conclusions Cortical dysfunction appears to be an intrinsic process across the MND phenotypes, with cortical inexcitability predominating in PLS and cortical hyperexcitability predominating in ALS. Importantly, cortical excitability was preserved in HSP, thereby suggesting that the presence of cortical dysfunction could help differentiate PLS from HSP in a clinical setting. [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 excitability changes distinguish the motor neuron disease phenotypes from hereditary spastic paraplegia.
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  Data: &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;%22Menon%2C+P%2E%22&quot;&gt;Menon, P.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Sue%2C+C%2E+M%2E%22&quot;&gt;Sue, C. M.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Kumar%2C+K%2E+R%2E%22&quot;&gt;Kumar, K. R.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Ng%2C+K%2E%22&quot;&gt;Ng, K.&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;. May2015, Vol. 22 Issue 5, p826-826. 8p.
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  Data: &lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Paraplegia%22&quot;&gt;Paraplegia&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Motor+neuron+diseases%22&quot;&gt;Motor neuron diseases&lt;/searchLink&gt;&lt;br /&gt;&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;%22Genetic+mutation%22&quot;&gt;Genetic mutation&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Phenotypes%22&quot;&gt;Phenotypes&lt;/searchLink&gt;
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  Data: Background and purpose Cortical hyperexcitability has been identified as an important pathogenic mechanism in motor neuron disease ( MND). The issue as to whether cortical hyperexcitability is a common process across the MND phenotypes, including amyotrophic lateral sclerosis ( ALS) and primary lateral sclerosis ( PLS), remains unresolved. Separately, the clinical distinction between PLS and &#39;mimic disorders&#39; such as hereditary spastic paraparesis ( HSP) may be difficult, potentially delaying diagnosis. Consequently, the aim of the present study was to determine the nature and spectrum of cortical excitability changes across the MND phenotypes, and to determine whether the presence of cortical dysfunction distinguishes PLS from HSP. Methods Cortical excitability studies were undertaken on a cohort of 14 PLS, 82 ALS and 13 HSP patients with mutations in the spastin gene. Results Cortical hyperexcitability, as heralded by reduction of short interval intracortical inhibition ( PLS 0.26%, −3.8% to 1.4%; ALS −0.15%, −3.6% to 7.0%; P &lt; 0.01) and cortical silent period duration ( CSPPLS 172.2 &#177; 5.4 ms; CSPALS 178.1 &#177; 5.1 ms; P &lt; 0.001), along with an increase in intracortical facilitation was evident in ALS and PLS phenotypes, although appeared more frequently in ALS. Inexcitability of the motor cortex was more frequent in PLS ( PLS 71%, ALS 24%, P &lt; 0.0001). Cortical excitability was preserved in HSP. Conclusions Cortical dysfunction appears to be an intrinsic process across the MND phenotypes, with cortical inexcitability predominating in PLS and cortical hyperexcitability predominating in ALS. Importantly, cortical excitability was preserved in HSP, thereby suggesting that the presence of cortical dysfunction could help differentiate PLS from HSP in a clinical setting. [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.12669
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        Text: English
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      – SubjectFull: Paraplegia
        Type: general
      – SubjectFull: Motor neuron diseases
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      – SubjectFull: Amyotrophic lateral sclerosis
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      – SubjectFull: Genetic mutation
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      – SubjectFull: Phenotypes
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              Text: May2015
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