Introduction of Quantitative Scale for Fasciculations in ALS Patients
Sara Palacios1, julieta casen1, cecilia quarraccino1, Gabriel Rodriguez1
1Hospital Ramos Mejia
Objective:
Develop a scale regarding the location and frequency of fasciculations in ALS patients.
Background:
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with different diagnostic challenges. Fasciculations are one of the signs of lower motor neuron impairment. It is currently uncertain whether their extent and duration are associated with relevant clinical or demographic characteristics of ALS patients. Nowadays there is no clinical scale to grade them.
Design/Methods:

Eleven corporal segments are evaluated: face-tongue, neck-thorax, back, proximal upper right and left limb, distal upper right and left limb, proximal lower right and left limb, distal lower right and left limb.  We proposed a score according to the following parameters: 0: absence, 1: isolated contraction, 2: multiple non-continuous contractions, 3: continuous contractions; with a possible total score ranging from 0 to 33 points. We performed a physical exam and filmed 30-seconds videos in 11 consecutive ALS patients (probable or definite diagnosis according to El Escorial criteria) that had fasciculations at the moment of their regular medical consultation during July 2022. The videos were scored by the developers of the scale (ALB and GER) and later sent to five neurology residents and five neurologists to independently rate them. 

Results:

The final score obtained by the creators of the scale was 15. Interobserver variability was analyzed: 30% scored 17 points, 30% 16 points, 10% 19 points, 10% 15 points, 10% 12 points and 10% 11 points. In only two videos all of the observers agreed on the points and marked “absence of fasciculations”. The highest rate of variability was seen between options “2: multiple non-continuous contractions” and “3: continuous contractions”.

Conclusions:

Even though we observed a marked interobserver variability, this study proposes an easily appliable measurement method for fasciculations. A larger study would confirm if this scale has an acceptable variability rate.

10.1212/WNL.0000000000203277