当前位置： SCI文献检索 > JOURNAL OF NEUROLOGY期刊下所有文献 > Primary lateral sclerosis and the amyotrophic lateral sclerosis-frontotemporal dementia spectrum.

Primary lateral sclerosis and the amyotrophic lateral sclerosis-frontotemporal dementia spectrum.

Abstract：

AIM:To investigate whether primary lateral sclerosis (PLS) represents part of the amyotrophic lateral sclerosis-frontotemporal dementia (ALS-FTD) spectrum of diseases. METHODS: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). RESULTS: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. CONCLUSION: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.

journal_name

J Neurol

journal_title

Journal of neurology

authors

Agarwal S,Highton-Williamson E,Caga J,Matamala JM,Dharmadasa T,Howells J,Zoing MC,Shibuya K,Geevasinga N,Vucic S,Hodges JR,Ahmed RM,Kiernan MC

doi

10.1007/s00415-018-8917-5

subject

Has Abstract

pub_date

2018-08-01 00:00:00

pages

1819-1828

issue

eissn

0340-5354

issn

1432-1459

pii

10.1007/s00415-018-8917-5

journal_volume

265

pub_type

杂志文章

在线工具

Primary lateral sclerosis and the amyotrophic lateral sclerosis-frontotemporal dementia spectrum.