Two Distinct Clinical Phenotypes of Bulbar Motor Impairment in Amyotrophic Lateral Sclerosis.
Two Distinct Clinical Phenotypes of Bulbar Motor Impairment in Amyotrophic Lateral Sclerosis.
复制标题
DOI：
10.3389/fneur.2021.664713
复制
发表时间：
2021
影响因子：
3.4
通讯作者：
Green JR
中科院分区：
文献类型：
--
作者：
Stipancic KL;Yunusova Y;Campbell TF;Wang J;Berry JD;Green JR
Objective: Understanding clinical variants of motor neuron diseases such as amyotrophic lateral sclerosis (ALS) is critical for discovering disease mechanisms and across-patient differences in therapeutic response. The current work describes two clinical subgroups of patients with ALS that, despite similar levels of bulbar motor involvement, have disparate clinical and functional speech presentations. Methods: Participants included 47 healthy control speakers and 126 speakers with ALS. Participants with ALS were stratified into three clinical subgroups (i.e., bulbar asymptomatic, bulbar symptomatic high speech function, and bulbar symptomatic low speech function) based on clinical metrics of bulbar motor impairment. Acoustic and lip kinematic analytics were derived from each participant's recordings of reading samples and a rapid syllable repetition task. Group differences were reported on clinical scales of ALS and bulbar motor severity and on multiple speech measures. Results: The high and low speech-function subgroups were found to be similar on many of the dependent measures explored. However, these two groups were differentiated on the basis of an acoustic measure used as a proxy for tongue movement. Conclusion: This study supports the hypothesis that high and low speech-function subgroups do not differ solely in overall severity, but rather, constitute two distinct bulbar motor phenotypes. The findings suggest that the low speech-function group exhibited more global involvement of the bulbar muscles than the high speech-function group that had relatively intact lingual function. This work has implications for clinical measures used to grade bulbar motor involvement, suggesting that a single bulbar measure is inadequate for capturing differences among phenotypes.
登录
查看更多内容
DOI：
10.3109/21678421.2013.778548
发表时间：
2013-05
影响因子：
2.8
作者：
Ravits J;Appel S;Baloh RH;Barohn R;Brooks BR;Elman L;Floeter MK;Henderson C;Lomen-Hoerth C;Macklis JD;McCluskey L;Mitsumoto H;Przedborski S;Rothstein J;Trojanowski JQ;van den Berg LH;Ringel S
通讯作者：
Ringel S
影响因子：
2.8
作者：
Rong P;Yunusova Y;Wang J;Green JR
通讯作者：
Green JR
影响因子：
4.4
作者：
Cedarbaum, JM;Stambler, N;Nakanishi, A
通讯作者：
Nakanishi, A
DOI：
10.1080/21678421.2019.1697888
发表时间：
2019-12-04
影响因子：
2.8
作者：
Barnett, Carolina;Green, Jordan R.;Yunusova, Yana
通讯作者：
Yunusova, Yana
影响因子：
1.2
作者：
KENT, RD;KENT, JF;ROSENBEK, JC
通讯作者：
ROSENBEK, JC