Disease onset is typically in early childhood and progression consists in delayed acquisition of motor milestones, in particular, walking due to unsteadiness in toddlers, followed by lower-limb spasticity and peripheral neuropathy. Onset has also been observed in childhood or adolescence. Other clinical symptoms like ocular disturbances, dysarthria, and upper-limb ataxia develop at a slower rate. Finally, mild intellectual disability, hearing loss, and incontinence have also been documented in some individuals. Individuals affected by ARSACS are often wheel-chair bound by the age of 40.
SACS