**6.4. Cerebellar ataxia**

#### *6.4.1. Spinocerebellar ataxia 3 (SCA3)/Machado-Joseph disease*

We performed urodynamic studies in 11 patients with spinocerebellar ataxia 3 (SCA3) and performed EAS-EMG in 9. Six of the 9 patients showed neurogenic changes in MUPs.59

#### *6.4.2. Late cortical cerebellar atrophy (LCCA):*

We performed urodynamic studies in 7 patients with LCCA, which is a pure cerebellar ataxia without heredity, and EAS-EMG in 3 of them. However, none of the 3 patients exhibited neurogenic changes in the MUPs.

#### *6.4.3. Spinocerebellar ataxia 6 (SCA6)*

We performed urodynamic studies in 9 patients with spinocerebellar ataxia 6 (SCA6) and performed EAS-EMG in 8. Five of the 8 patients showed neurogenic changes in MUPs.

#### **6.5. Other diseases**

Sakuta et al. performed EAS-EMG in 30 patients with amyotrophic lateral sclerosis (ALS).62 None of them exhibited neurogenic changes in the MUPs, which contrasted with common neurogenic changes in the limb muscles in this disorder. These EMG findings correspond to the postmortem selective sparing of sacral Onuf's nucleus, which contrasts with severe loss of anterior horn cells innervating the limbs, tongue, and bulbar muscles2. Neurons in Onuf's nucleus demonstrate some morphological differences from the anterior horn cells innervating limb muscle 33. However, more recent studies have shown abnormalities in the Onuf's nucleus in most advanced cases with ALS27, particularly in patients under mechanical ventilation.
