Age (years) mean ± SD | 36 ± 10 |
Age at exam (years) mean ± SD | 21 ± 8 |
Age at onset (years) mean ± SD | 15 ± 6 |
Sex (male) | 49/72 (68%) |
Consanguinity | |
¾ FA | 16/33 (48.5%) |
¾ AVED | 7/11 (63.6%) |
Gait | |
¾ Walk without help | 40/72 (55.6%) |
¾ Confined to bed | 4/72 (5.6%) |
Abolished tendon reflexes | 63/72 (87.5%) |
LL weakness | 45/72 (62.5%) |
UL weakness | 13/72 (18.1%) |
Dysathria | 53/72 (73.6%) |
BB signs | 46/72 (63.9%) |
Amyotrophy | 10/72 (14%) |
Normal Glycemia | 36/37 (97%) |
Lipidogram abnormal | 7/37 (18%) |
CT scan | |
¾ Normal | 13/29 (44.8%) |
¾ Atrophy | 16/29 (55.1%) |