**6. Ataxias scales in children**

Ataxia scales are used especially in clinical trials evaluating the potential effect of therapeutic agents. Different ataxia rating scales have been used in the paediatric age, the most commonly applied in children include the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for Ataxia Assessment and Rating (SARA) [81].

ICARS was initially developed to evaluate treatment efficacy in randomised clinical trials. ICARS is constituted by four clinical subscales domains including posture and gait, limb coordination, speech and oculomotor function [81]. ICARS ranges from 0 (optimal outcome) to 100 (most severely affected outcome). In children, some reports are suggesting an age-dependent ICARS score, with performance improving with age [82].

### **Figure 3.**

*MRI FLAIR sequence revealed diffuse white matter hyperintensity and interspersed areas of low signal intensities findings in classic vanishing white matter (VWM) disease.*

SARA includes eight items that range a total score from 0 (no ataxia) to 40 (most severe ataxia), assessing gait, stance, sitting, speech disturbance, finger chase, nose-to-finger test, fast alternating hand movements, and heel-shin slide. Studies also indicate an exponential decline of SARA scores [83] with age, and some authors do not recommend its use in children under 12 years of age [84].

In both mentioned scales, the presence of different types of movement disorders can influence scores, so its application in uniform phenotypes of movement disorders is suggested for an adequate interpretation [85].
