Clinical features reported previously in SCA 8
Cerebellar signs | Pyramidal signs | Sensory signs | Cognitive impairment | Neurophysiological abnormality | |||||
---|---|---|---|---|---|---|---|---|---|
Nystagmus | Dysarthria | Limb ataxia | Gait ataxia | Hyperreflexia | Extensor plantar | ||||
*Current series, | |||||||||
–, not reported. | |||||||||
Day4 | 8/11 | 11/11 | 11/11 | 10/11 | 8/11 | 2/11 | 5/11 | 0/11 | 7/7 |
Ikeda7 | 4/6 | 6/6 | 6/6 | 6/6 | 1/6 | 0/6 | 2/6 | 0/6 | – |
Silveira5 | – | 7/7 | 6/7 | – | – | – | 3/7 | – | |
Juvonen16 | 5/15 | 14/15 | 12/15 | 13/15 | 9/15 | 5/15 | 4/15 | 6/15 | – |
Jardim32 | 1/1 | 1/1 | 1/1 | 1/1 | 0/1 | 0/1 | 1/1 | 0/1 | – |
Sempere33 | 0/1 | 1/1 | 1/1 | 1/1 | 1/1 | 0/1 | 0/1 | 0/1 | |
Brusco27 | 2/4 | ¾ | ¾ | 4/4 | 2/4 | 2/4 | ¼ | 0/4 | 0/1 |
Topisirovic29 | – | 1/1 | 1/1 | 1/1 | 1/1 | – | – | 0/1 | 0/1 |
Tazon28 | 2/3 | 3/3 | 3/3 | 3/3 | – | 0/2 | 0/2 | 0/1 | 0/3 |
Izumi30 | – | – | 15/15 | 9/15 | 1/15 | 4/15 | – | ||
Zeman* | 1/7 | 5/7 | 6/7 | 7/7 | 4/7 | 1/7 | 3/7 | 5/7 | – |
Total | 23/48 | 52/56 | 44/49 | 67/71 | 35/61 | 11/62 | 16/45 | 18/68 | 7/13 |
Total (%) | 48% | 93% | 90% | 94% | 57% | 18% | 36% | 26% | 54% |