Characteristic | II 1 | II3 | II5 | II7 | III4 |
---|---|---|---|---|---|
Age at examination | 59 | 53 | 49 | 48 | 26 |
Age at symptom onset | 29 | 30 | 28 | 26 | 24 |
Symptoms at onset | gait instability | gait instability | gait instability | gait instability | gait instability |
SARA | 20 | 10 | 12 | 22 | 5 |
Dysarthria | mild | no | mild | moderate | no |
Ocular signs | no | no | saccadic pursuit, smooth pursuit defective | saccadic pursuit, smooth pursuit defective | no |
Reflexes of | Slight | ||||
Upper limb | normal | normal | hyperreflexia | normal | normal |
Lower limb | normal | normal | Slight | normal | normal |
hyperreflexia | |||||
Cerebellar signs in ULs | no | no | moderate | moderate | mild |
Gait ataxia | mild | mild | moderate | moderate | mild |
Muscle tone | normal | normal | normal | normal | normal |
Involuntary movement | no | no | no | no | no |
Babinski sign | negative | negative | positive | positive | negative |
Cerebral MRI | cerebellar atrophy | NA | cerebellar atrophy | cerebellar atrophy | NA |
Other complications | weakness of LLs | no | memory decline | vertigo | no |
MoCA | 23 | 24 | 18 | 25 | 28 |