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Motor function and behaviour across the ALS-FTD spectrum.

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Title: Motor function and behaviour across the ALS-FTD spectrum.
Authors: De Silva, D., Hsieh, S., Caga, J., Leslie, F. V. C., Kiernan, M. C., Hodges, J. R., Mioshi, E., Burrell, J. R.
Source: Acta Neurologica Scandinavica. May2016, Vol. 133 Issue 5, p367-372. 6p.
Subjects: Amyotrophic lateral sclerosis, Frontotemporal dementia, Motor ability, Behavior disorders, Disease progression, Follow-up studies (Medicine)
Abstract: Background Behavioural/functional disturbances, characteristic of frontotemporal dementia ( FTD), are also a feature of amyotrophic lateral sclerosis ( ALS) and patients with combined ALS and FTD ( FTD- ALS). Aim of the study To investigate the progression of behavioural disturbances in ALS and FTD using the FTD functional rating scale ( FTDFRS). Methods Patients with ALS, FTD- ALS and FTD were recruited from specialist clinics. Baseline assessments included the FTDFRS and the ALS functional rating scale-revised ( ALSFRS-R). Baseline assessments were included, as were longitudinal assessments in a proportion of patients. Results In total, 21 ALS, 12 FTD- ALS and 14 behavioural variant FTD (bv FTD) patients were included in the study. Moderate or severe behavioural disturbance was common in patients with ALS at baseline (47.6%), although less frequent than in bv FTD patients; patients with FTD- ALS displayed intermediate impairment. The ALSFRS-R showed the opposite pattern and did not correlate with the FTDFRS. During the follow-up period, significant ( P < 0.05) behavioural deterioration was demonstrated in patients with bv FTD and FTD- ALS, with a trend for decline in patients with ALS ( P = 0.06). Conclusion Motor disturbance is the primary marker of disease severity in ALS, but behavioural and functional impairment are common, and may decline independently of motor function. As such, the FTDFRS may provide valuable information in the assessment and monitoring of ALS. [ABSTRACT FROM AUTHOR]
Copyright of Acta Neurologica Scandinavica 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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  Data: Motor function and behaviour across the ALS-FTD spectrum.
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  Data: &lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22De+Silva%2C+D%2E%22&quot;&gt;De Silva, D.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Hsieh%2C+S%2E%22&quot;&gt;Hsieh, S.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Caga%2C+J%2E%22&quot;&gt;Caga, J.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Leslie%2C+F%2E+V%2E+C%2E%22&quot;&gt;Leslie, F. V. 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;%22Hodges%2C+J%2E+R%2E%22&quot;&gt;Hodges, J. R.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Mioshi%2C+E%2E%22&quot;&gt;Mioshi, E.&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Burrell%2C+J%2E+R%2E%22&quot;&gt;Burrell, J. R.&lt;/searchLink&gt;
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  Data: &lt;searchLink fieldCode=&quot;JN&quot; term=&quot;%22Acta+Neurologica+Scandinavica%22&quot;&gt;Acta Neurologica Scandinavica&lt;/searchLink&gt;. May2016, Vol. 133 Issue 5, p367-372. 6p.
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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;%22Frontotemporal+dementia%22&quot;&gt;Frontotemporal dementia&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Motor+ability%22&quot;&gt;Motor ability&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Behavior+disorders%22&quot;&gt;Behavior disorders&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Disease+progression%22&quot;&gt;Disease progression&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Follow-up+studies+%28Medicine%29%22&quot;&gt;Follow-up studies (Medicine)&lt;/searchLink&gt;
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Background Behavioural/functional disturbances, characteristic of frontotemporal dementia ( FTD), are also a feature of amyotrophic lateral sclerosis ( ALS) and patients with combined ALS and FTD ( FTD- ALS). Aim of the study To investigate the progression of behavioural disturbances in ALS and FTD using the FTD functional rating scale ( FTDFRS). Methods Patients with ALS, FTD- ALS and FTD were recruited from specialist clinics. Baseline assessments included the FTDFRS and the ALS functional rating scale-revised ( ALSFRS-R). Baseline assessments were included, as were longitudinal assessments in a proportion of patients. Results In total, 21 ALS, 12 FTD- ALS and 14 behavioural variant FTD (bv FTD) patients were included in the study. Moderate or severe behavioural disturbance was common in patients with ALS at baseline (47.6%), although less frequent than in bv FTD patients; patients with FTD- ALS displayed intermediate impairment. The ALSFRS-R showed the opposite pattern and did not correlate with the FTDFRS. During the follow-up period, significant ( P &lt; 0.05) behavioural deterioration was demonstrated in patients with bv FTD and FTD- ALS, with a trend for decline in patients with ALS ( P = 0.06). Conclusion Motor disturbance is the primary marker of disease severity in ALS, but behavioural and functional impairment are common, and may decline independently of motor function. As such, the FTDFRS may provide valuable information in the assessment and monitoring of ALS. [ABSTRACT FROM AUTHOR]
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  Data: &lt;i&gt;Copyright of Acta Neurologica Scandinavica 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/ane.12471
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        Text: English
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      – SubjectFull: Amyotrophic lateral sclerosis
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      – SubjectFull: Behavior disorders
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              Text: May2016
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