Sex Difference | M/F = 23/22 |
---|---|
Average age at onset | 37.0 ± 17.9 (n = 36), Range: 12.5-62 |
Clinical features | Â |
   Bulbar symptom | 35/45 (78%) |
   Pyramidal tract signs | 33/45 (73%) |
   Ataxia | 31/44 (71%) |
   Palatal myoclonus | 15/38 (39%) |
MRI findings | Â |
   Marked medullary atrophy | 37/42 (88%) |
   Deep white matter abnormalities | 21/43 (49%) |
Brainstem signal change (including nodular lesions) | 16/36 (44%) |