A clinical and neurophysiologic study of upper motor neuron-dominant amyotrophic lateral sclerosis / 中华神经科杂志

ABSTRACT

Objective To investigate the clinical and electrophysiological characteristics of upper motor neuron-dominant amyotrophic lateral sclerosis (UMN-D ALS ).Methods The clinical and electrophysiological characteristics were analyzed retrospectively in 76 patients with UMN-D ALS and 19 patients with primary lateral sclerosis (PLS).Electrophysiological study included the examination of median nerve,ulnar nerve,tibial nerve,peroneal nerve and sural nerve except for the electromyogram of bulbar region,cervical region,thoracic region and lumbosacral region.Results The diagnosis in 8 PLS patients were changed to UMN-D ALS after detailed review.In UMN-D ALS patients,there were more females in age group of older than 40 (malefemale =1∶1.37) and 32 patients (38.1％) had onset with bulbar dysfunction.UMN-D ALS patients showed electromyograph evidence of denervation at 30 months (median)after onset,and clinical lower motor neuron (LMN) signs 6 months later.Seventy-seven patients (91.6％)developed LMN sign by 4 years from symptom onset.ALS functional rating score-revised changed from 40 ±3 to 32 ±4 in 4 years in U MN-D ALS patients (t =1.83,P ＜ 0.05 ).The amplitude of motor unit action potential (MUAP) on the first interosseus dorsalis was higher (( 1003.7 ± 25.2) μV vs (353.5 ±21.5) μV,t=2.34,P ＜0.05) and the duration of MUAP was longer ((19.8 ±2.3) ms vs (9.6 ±1.3) ms,t =1.85,P ＜0.05 ) in UMN-D ALS patients than in PLS patients.Conclusions UMN-D ALS occurs more in female cases,with more bulbar onset and with faster progression than PLS does.It also presents focal denervation in electromyograph.