Therefore, in this study, we characterized and compared the clinical features (ALSFRS-R, progression rate, site of onset (spinal/bulbar), affection of upper and lower motor neurons (UMN or LMN), body mass index (BMI), sex, age of onset, diagnostic delay, and family history) of the most frequently occurring pathogenic variants of SOD1-ALS in Germany. The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.