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%)