[Progression rate of MUNE at diagnosis: a prognostic factor of survival in patients with amyotrophic lateral sclerosis].
OBJECTIVE
To identify the correlation between the progression rate of motor unit number estimate (MUNE) at diagnosis and survival.


METHODS
We included 129 patents with amyotrophic lateral sclerosis (ALS) enrolled in our hospital from January 2002 to December 2005. We recorded clinical features, ALS functional rating scale (ALSFRS), forced vital capacity (FVC) and electrophysiological data at diagnosis. The patients were monitored every 3 months from visit to death or tracheotomy.


RESULTS
Mean age at onset was (52.19+/-11.00) years. The median survival time from symptom onset was 45.71 months (95% CI=35 to 51). In univariate analysis of Kaplan-Meier method, outcome was significantly related to progression rate of MUNE (P<0.05). In the Cox multivariate model, progression rate of MUNE was a significant prognostic factor of survival (P<0.01). Using 2.9/month as cutoff point, the value beyond this point at diagnosis tended to have a shorter survival (P<0.05). A 1-unit decrease in the progression rate of MUNE was associated with a 34.2% decrease risk of death.


CONCLUSIONS
Progression rate of MUNE at diagnosis can provide the information of disease progression at one particular point, which may help to stratify patients and affect survival. It is the strongest prognostic factor of survival in patients with ALS compared with age, onset site and time from onset to diagnosis.
Liu
Xiao Xuan
XX
Department of Neurology, Peking University Third Hospital, Beijing, China.
Fan
Dong Shen
DS
Zhang
Jun
J
Zheng
Ju Yang
JY
Zhang
Shuo
S
D000200:Action Potentials; D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology / Q000503:physiopathology*; D004576:Electromyography / Q000379:methods; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology*; D018485:Muscle Fibers, Skeletal / Q000502:physiology*; D009410:Nerve Degeneration / Q000209:etiology / Q000503:physiopathology; D011379:Prognosis; D016019:Survival Analysis