Table 1.
Description of the sample: demographic and clinical variables and Aco2 activity. Our sample consisted of 26 ALS patients (15 men and 11 women) and 22 healthy controls who were matched for age and sex. Four of the ALS cases displayed the characteristics of bulbar onset ALS, while twenty-two displayed signs of the spinal onset variety. The ALS patients were at different stages of disease development and exhibited different degrees of severity. ALS: Amyothophic lateral sclerosis, UMN: Upper motor neuron, LMN: Lower motor neuron, VCM: Vital capacity maneuver, BMI: Body mass index, Aco 2: Aconitase 2.
| ALS | Control | |
|---|---|---|
| Sex (male/female) | 15/11 | 11/11 |
| Age (years) | 58.24 ± 14.85 | 57.60 ± 11.13 |
| Riluzole | 26 | 0 |
| Survival > 36 months | 14 | - |
| Survival > 48 months | 6 | - |
| Site of onset (bulbar/spinal) | 4/22 | - |
| UMN/LMN at onset | 6/20 | - |
| VCM < 50% | 10 | - |
| BMI < 20 | 2 | - |
| ALSFRS-r High Medium Mild |
6 11 9 |
- |
| El Escorial criteria Definite Probable Laboratory-supported probable |
13 10 3 |
|
| Aco2 activity | 257.65 ± 111.1 mU/mg | 375.41 ± 166 mU/mg |