ABSTRACT
Objective To evaluate the value of neurophysiological index ( NI) in evaluating the rate of progression of amyotrophic lateral sclerosis ( ALS ) . Methods Forty-eight patients with ALS were enrolled in Zhongda Hospital of Southeast University from January 2010 to August 2016, of whom 36 patients met the ALS definite diagnostic criteria , 12 patients met the ALS probable diagnostic criteria ( following study confirmed those patients met the ALS definite diagnostic criteria ) , including 8 bulbar-onset and 40 upper extremity onset forms of the disease .Fifty-four age-and sex-matched healthy subjects served as controls.After evaluated by the ALS Functional Rating Scale-revised ( ALSFRS-R) , all subjects underwent electrophysiological examination in the Electromyography Lab of the hospital .The rate of disease progression (ΔFS) =48-total ALSFRS-R score at initial visit/symptom duration (months).The relevance between the complex muscle action potential (CMAP), F frequency, distal motor latency (DML), NI and the ΔFS was investigated respectively.Results In ALS group, the ALSFRS-R score was 14.56 ±10.10, the duration from onset to diagnosis was (14.56 ±10.10) months, and theΔFS was 1.54 ±1.18 per month.The median nerve NI in ALS group was 0.60 ±0.76, in control group was 2.56 ±0.78, with statistically significant difference between two groups (t=-12.5, P<0.01).The ulnar nerve NI in ALS group was 0.70 ±0.55, in control group was 0.96 ±0.10, also with statistically significant difference between two groups (t=-0.31, P=0.003).The median nerve NI and ulnar nerve NI both were negatively correlated withΔFS (r=-0.63, P=0.000; r=-0.506, P=0.007).The ΔFS was exponentially based on median nerve NI (R2 =0.668, P<0.01).Conclusion NI is an objective electrophysiological index , which could be used to evaluate the rate of disease progression in ALS , and to evaluate the prognosis of the disease .
ABSTRACT
BACKGROUND: Neurophysiological Index (NI) is a sensitive measure of changes during the course of amyotrophic lateral sclerosis (ALS). However, NI applied at the ulnar nerve has limitation in that the initial manifestation of ALS is different among individual patients. This study was aimed to evaluate the efficiency of NI applied to the posterior tibial nerve system in a prospective study of progression in ALS patients. METHODS: The subjects of the study were 22 patients with definite or probable ALS based on revised EL Escorial criteria. NI applied to the ulnar nerve/abductor digiti minimi muscle and the posterior tibial nerve/abductor hallucis muslce, revised ALS functional rating scale, Norris scale, and grip power were obtained at four and eight months of the follow-up. RESULTS: Overall, NI at both ulnar and posterior tibial nerve systems showed a significant interval change at 4 and 8 months. Although NI in both nerve systems showed significant changes at an interval of 4 months in patients with upper limb onset, NI obtained from the ulnar nerve did not show a difference from the baseline in the lower limb onset patients. Moreover, there was a significant change of NI in the early stage ALS patients, compared to the late stage patients. CONCLUSIONS: In the lower limb onset patients, NI obtained from the posterior tibial nerve is more useful in evaluating the disease progression pattern. Moreover, NI is more effective in the evaluation of the patients in the early stage.
Subject(s)
Humans , Amyotrophic Lateral Sclerosis , Disease Progression , Hand Strength , Lower Extremity , Muscles , Prospective Studies , Tibial Nerve , Ulnar Nerve , Upper ExtremityABSTRACT
BACKGROUND: Neurophysiological Index (NI) is derived from compound muscle action potentials, distal motor latency and F-wave frequency in the ulnar nerve/abductor digiti minimi. Recent studies suggested that NI could be used as a sensitive measure of change during the course of ALS. However, the NI has several limitations which include being derived only from the ulnar nerve territory and is less valuable when atrophic change of the abductor digiti minimi is severe. Thus, this study aimed to evaluate the correlation of NI with the functional status of ALS patients and the availability of NI obtained from median and posterior tibial nerve territory. METHODS: The subjects of the study were 25 patients with ALS. The NI, Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) and grip power were obtained during the same period and NI values were compared with ALSFRS-R and grip power. RESULTS: A significant correlation was observed between ALSFRS-R and various NI values. Moreover, NI values derived from the ulnar nerve/abductor digiti minimi and the median nerve/abductor pollicis brevis on the dominant hand were statistically correlated with fine motor function scores in ALSFRS-R, and NI values from posterior tibial nerve/abductor hallucis were significantly correlated with lower limb function scores. CONCLUSIONS: Our results support the previous findings that NI is a useful neurophysiological measurement in ALS patients. Moreover, we might suggest NI values from the median nerve/abductor pollicis brevis and the posterior tibial nerve/abductor hallucis also could be used in measuring the functional status of ALS patients.