The ALSFRSr predicts survival time in an ALS clinic population.
OBJECTIVE
To determine whether the Amyotrophic Lateral Sclerosis Functional Rating Scale-revised (ALSFRSr), a predictor of survival time in ALS clinical trials, predicts survival time in an ALS clinic population.


METHODS
The authors prospectively evaluated 267 consecutive patients with ALS at first visit to an ALS clinic using the ALSFRSr and pulmonary function testing. The association of ALSFRSr score at baseline with death or tracheostomy in ALS was examined using Cox proportional hazards models, adjusting for age at baseline, sex, and symptom duration.


RESULTS
Of 267 patients with ALS, 103 (39%) reached the endpoint, defined as either death (79 patients) or tracheostomy (24 patients), during a mean follow-up of 1.0 +/- 0.7 years. Among the 103 patients who reached the endpoint during follow-up, 77 (75%) had a baseline ALSFRSr score of less than 38 (the median baseline score of all patients), compared to 53 of 164 (32%) who remained alive without tracheostomy. Patients with a total ALSFRSr score below the median had a 4.4-fold increased risk of death or tracheostomy compared to those who scored above the median (HR: 4.38, 95% CI: 2.79 to 6.86, p < 0.001). Both the total ALSFRSr score at baseline (HR: 0.94, 95% CI: 0.91 to 0.98, p < 0.001) and forced vital capacity at baseline (HR: 0.99, 95% CI: 0.98 to 1.00, p = 0.02) were associated with death or tracheostomy when included in the same Cox model.


CONCLUSIONS
In an ALS clinic population, the total Amyotrophic Lateral Sclerosis Functional Rating Scale-revised score at baseline is a strong predictor of death or tracheostomy independently of forced vital capacity and after adjustment for age at baseline, sex, and symptom duration.
Kaufmann
P
P
Department of Neurology, College of Physicians and Surgeons, The Neurologic Institute, Columbia University, 710 W 168th Street, New York, NY 10032, USA. pk88@columbia.edu
Levy
G
G
Thompson
J L P
JL
Delbene
M L
ML
Battista
V
V
Gordon
P H
PH
Rowland
L P
LP
Levin
B
B
Mitsumoto
H
H
D017668:Age of Onset; D000690:Amyotrophic Lateral Sclerosis / Q000401:mortality* / Q000503:physiopathology* / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D016016:Proportional Hazards Models; D011446:Prospective Studies; D012129:Respiratory Function Tests / Q000379:methods; D018434:Sickness Impact Profile; D015996:Survival Rate; D014139:Tracheostomy / Q000379:methods