Table 1.
Age (years) | 44.2±20 |
Men | 19 (43%) |
Age at diagnosis (years) | 36.0±20 |
Duration of ERT therapy (years) | 5.4±3 |
Mean Total SFNSL score | 11.6±9 (95% CI 9.0–14.2) |
% FVC (upright) | 64.0±20 |
Shoulder abduction (MRC scale) | 4.4 |
Hip flexion (MRC scale) | 3.3 |
ERT – enzyme replacement therapy; SFNSL – small fiber neuropathy screening list; %FVC – forced vital capacity; MRC – Medical Research Council.