| Title |
Primary lateral sclerosis and the amyotrophic lateral sclerosis–frontotemporal dementia spectrum
|
|---|---|
| Published in |
Journal of Neurology, June 2018
|
| DOI | 10.1007/s00415-018-8917-5 |
| Pubmed ID | |
| Authors |
Smriti Agarwal, Elizabeth Highton-Williamson, Jashelle Caga, José M. Matamala, Thanuja Dharmadasa, James Howells, Margaret C. Zoing, Kazumoto Shibuya, Nimeshan Geevasinga, Steve Vucic, John R. Hodges, Rebekah M. Ahmed, Matthew C. Kiernan |
| Abstract |
To investigate whether primary lateral sclerosis (PLS) represents part of the amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD) spectrum of diseases. Comprehensive assessment was taken on 21 patients with PLS and results were compared to patients diagnosed with pure motor ALS (n = 27) and ALS-FTD (n = 12). Clinical features, Addenbrooke's Cognitive Examination (ACE) scores, Motor Neuron Disease Behaviour (Mind-B) scores, motor disability on the ALS functional rating scale (ALSFRS) and survival times were documented. Motor cortex excitability was evaluated using transcranial magnetic stimulation (TMS). Global cognition was impaired in PLS (mean total ACE score 82.5 ± 13.6), similar to ALS-FTD (mean total ACE score 76.3 ± 7.7, p > 0.05) while behavioural impairments were not prominent. TMS revealed that resting motor threshold (RMT) was significantly higher in PLS (75.5 ± 6.2) compared ALS-FTD (50.1 ± 7.2, p < 0.001) and ALS (62.3 ± 12.6, p = 0.046). Average short-interval intracortical inhibition (SICI) was similar in all three patient groups. The mean survival time was longest in PLS (217.4 ± 22.4 months) and shortest in ALS-FTD (38.5 ± 4.5 months, p = 0.002). Bulbar onset disease (β = - 0.45, p = 0.007) and RMT (β = 0.54, p = 0.001) were independent predictors of global cognition while motor scores (β = 0.47, p = 0.036) and SICI (β = 0.58, p = 0.006) were significantly associated with ALSFRS. The cognitive profile in PLS resembles ALS-FTD, without prominent behavioural disturbances. A higher RMT in PLS than ALS and ALS-FTD is consistent with differential cortical motor neuronal abnormalities and more severe involvement of corticospinal axons while SICI, indicative of inhibitory interneuronal dysfunction was comparable with ALS and ALS-FTD. Overall, while these findings support the notion that PLS lies on the ALS-FTD spectrum, the mechanisms underlying slow disease progression are likely to be distinct in PLS. |
X Demographics
The data shown below were collected from the profile of 1 X user who shared this research output. Click here to find out more about how the information was compiled.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 1 | 100% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 1 | 100% |
Mendeley readers
The data shown below were compiled from readership statistics for 56 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 56 | 100% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Ph. D. Student | 11 | 20% |
| Researcher | 6 | 11% |
| Other | 5 | 9% |
| Student > Bachelor | 5 | 9% |
| Student > Master | 5 | 9% |
| Other | 6 | 11% |
| Unknown | 18 | 32% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 12 | 21% |
| Psychology | 9 | 16% |
| Neuroscience | 7 | 13% |
| Nursing and Health Professions | 4 | 7% |
| Agricultural and Biological Sciences | 2 | 4% |
| Other | 4 | 7% |
| Unknown | 18 | 32% |
Attention Score in Context
This research output has an Altmetric Attention Score of 10. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 31 October 2018.
All research outputs
#3,247,404
of 23,090,520 outputs
Outputs from Journal of Neurology
#760
of 4,533 outputs
Outputs of similar age
#67,781
of 329,882 outputs
Outputs of similar age from Journal of Neurology
#11
of 71 outputs
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So far Altmetric has tracked 4,533 research outputs from this source. They typically receive more attention than average, with a mean Attention Score of 8.1. This one has done well, scoring higher than 81% of its peers.
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We're also able to compare this research output to 71 others from the same source and published within six weeks on either side of this one. This one has done well, scoring higher than 80% of its contemporaries.