Table 2.
Clinical characteristics of patients with SCA1, SCA2 and unknown genetic etiology
|
Genetic Diagnosis |
N |
Mean age symptom onset (years) |
Disability level* |
SARA** |
INAS*** |
Clinically relevant depression ***** |
|---|---|---|---|---|---|---|
| (0–40) | (0–16) | |||||
| SCA1 |
21 |
34.8 ± 10 |
4 ± 2 |
18.8 ± 9.7 |
3.6 ± 2.4 |
68.4% |
| SCA2 |
1 |
34.0 |
6 |
26 |
6 |
100% |
| Unknown genetic etiology | 12 | 32.7 ± 9.8 | 3 ± 2.7 | 13.7 ± 7.1 | 3.8 ± 2.1 | 75% |
N = patient number included in the study.
*Disability level was quantified using the SPATAX score. (0 = No functional handicap, 1 = No functional handicap but signs at examination, 2 = mild unable to run, walking unlimited, 3 = moderate unable to run, limited walking without aid, 4 = severe walking with one stick, 5 = walking with two sticks, 6 = unable to walk requiring wheelchair, 7 = confined to bed).
**SARA = Scale of Assessment and Rating of Ataxia.
***INAS = Inventory of Non ataxia Symptoms.
****Clinically relevant depression as percentage of total subtype patient population - assessed using the Patient Health Questionnaire 9 (PHQ9).