Tremolizzo Lucio, Susani Emanuela, Aliprandi Angelo, Salmaggi Andrea, Ferrarese Carlo, Appollonio Ildebrando
Neurology Unit, San Gerardo Hospital/Monza and DCMT, University of Milano-Bicocca , Italy.
Amyotroph Lateral Scler Frontotemporal Degener. 2014 Dec;15(7-8):546-50. doi: 10.3109/21678421.2014.913636. Epub 2014 May 27.
Ultrasound detection of muscle fasciculations was recently proposed for assessing lower motor neuron (LMN) dysfunction in ALS patients. Given the continuum between ALS and frontotemporal degeneration (FTD), the aim of the present study was to evaluate muscle ultrasound (MUS) in FTD both for feasibility and prevalence of fasciculations. Twenty-two FTD patients were examined (five muscles bilaterally: biceps brachii, first dorsalis interosseous, T10 paraspinalis, vastus lateralis, tibialis anterior) with a 7-MHz linear array transducer and a fasciculation score (FS) computed. Twenty-two matched cognitively-intact control subjects and six ALS patients were also included. Results showed that MUS was feasible, reliable and well tolerated in all subjects. Two FTD/MND patients displayed very high FS values, similar to those in ALS patients. The remaining 20 FTD patients displayed a mean FS value significantly higher than the control group with six patients (30%) having FS values out of the range of controls. Disease progression rate correlated with the FS. In conclusion, MUS can be easily applied to FTD patients and represents a non-invasive technique for defining LMN involvement in these patients. LMN dysfunction is a frequent condition in FTD and might identify a subset of patients with a different clinical course.
最近有人提出用超声检测肌肉束颤来评估肌萎缩侧索硬化症(ALS)患者的下运动神经元(LMN)功能障碍。鉴于ALS与额颞叶变性(FTD)之间存在连续性,本研究的目的是评估FTD患者的肌肉超声(MUS)检查,以确定其可行性以及束颤的发生率。对22例FTD患者进行了检查(双侧检查五块肌肉:肱二头肌、第一骨间背侧肌、胸10椎旁肌、股外侧肌、胫骨前肌),使用7兆赫线阵换能器并计算束颤评分(FS)。还纳入了22例匹配的认知功能正常的对照受试者和6例ALS患者。结果显示,MUS在所有受试者中都是可行、可靠且耐受性良好的。2例FTD/运动神经元病(MND)患者的FS值非常高,与ALS患者相似。其余20例FTD患者的平均FS值显著高于对照组,6例患者(30%)的FS值超出对照组范围。疾病进展率与FS相关。总之,MUS可以很容易地应用于FTD患者,是一种用于确定这些患者LMN受累情况的非侵入性技术。LMN功能障碍在FTD中很常见,可能会识别出临床病程不同的患者亚组。