Table 1.
Patient | Age/sex [years] [female/male] | Diagnosis | Quality of life [score] | Symptoms duration to PET 1 [years] | Clinical features 1: stance/gait/sitting ataxia 2: ocular motor dysfunction 3: dysarthria 4: limb ataxia |
SARA PET 1 [points] | SARA PET 2 [points] | Delta SARA [points] | Responders |
---|---|---|---|---|---|---|---|---|---|
1 | 73 f | MSA-c | 0.25 | 4 | 1,2,3,4 | 20 | 14 | − 6 | Yes |
2 | 72 f | GAD-antibody encephalitis | 0.5 | 3 | 1,2,4 | 16 | 10.5 | − 5.5 | Yes |
3 | 28 m | Phenytoin associated CA | 0.5 | 1 | 1,2,3,4 | 16 | 17.5 | + 1.5 | No |
4 | 45 f | Ethyl toxic | 0.4 | 5 | 1,2,3,4 | 33 | 22.5 | − 10.5 | Yes |
5 | 52 f | Ethyl toxic | 0.7 | 3 | 1,2,3,4 | 17 | 14 | − 3 | Yes |
6 | 75 m | MSA-c | 0.3 | 4 | 1,2,3,4 | 12,5 | 9.5 | − 3 | Yes |
7 | 42 f | SCA 3 | 0.6 | 6 | 1,2 | 4,5 | 2,5 | − 2 | No |
8 | 61 f | SAOA | 0.3 | 4 | 1,2,3,4 | 7 | 9 | + 2 | No |
9 | 69 f | SAOA, small ischaemia | 0.2 | 9 | 1, 2,3,4 | 15,5 | 10 | − 5.5 | Yes |
10 | 64 f | SAOA | 0.3 | 13 | 1,2,3,4 | 12 | – | – | – |
11 | 64 m | SAOA | 0.4 | 14 | 1,2,4 | 14,5 | 12 | − 2.5 | Yes |
12 | 73 m | SAOA | 0.8 | 12 | 1,2,4 | 8 | 8 | 8.0 | No |
13 | 74 f | SAOA with DBN | 0.7 | 3 | 1,2,4 | 10,5 | – | – | – |
14 | 59 m | SCA 2 | 0.5 | 12 | 1,2,4 | 16 | 14.5 | − 1.5 | No |
15 | 44 m | SCA 1 | 0.5 | 6 | 1,2,3,4 | 19 | 15.5 | − 3.5 | Yes |
16 | 67 f | SAOA mit DBN | 0.4 | 20 | 1,2,3,4 | 15,5 | 13.5 | − 2.0 | No |
17 | 57 f | SAOA | 0.5 | 7 | 1,2,3,4 | 13,5 | 6 | − 7.5 | Yes |
18 | 69 m | Puratophin1-gene-mutation | 0.6 | 9 | 1,2,3,4 | 12 | 8.5 | − 3.5 | Yes |
19 | 74 m | SAOA | – | 6 | 1,2 | 4,5 | 1.5 | − 3.0 | Yes |
20 | 74 m | SAOA | – | 5 | 1,2,4 | 13 | 12 | − 1.0 | No |
21 | 49 f | Multiple sclerosis | 0.6 | 2 | 1,2,3,4 | 10 | 7 | − 3.0 | Yes |
22 | 77 f | SAOA with DBN | – | 5 | 1,2 | 3 | 2 | − 1.0 | No |
n = 22 | 61.9 ± 13.1 y | 0.2–0.8 | 7.0 ± 5.5 y | Mean 13.3 | Mean 10.5 | Mean 4.5 | Responders = 12 | ||
f = 13 | Mean 0.48 | f = 13 | Non-responders = 8 | ||||||
m = 9 | Drop out = 2 |
DBN downbeat nystagmus, GAD glutamate acid decarboxylase, MSAc multiple system atrophy of cerebellar dysfunction subtype, SCA spinocerebellar ataxia, SAOA sporadic adult-onset ataxia of unknown aetiology