Case | Gender | Diagnosis | age at CTS surgery (months) | age at ERT (months) | Age at ENG (months) | Distal Motor Latency MNL (msec) | Distal Sensory Latency SNL (msec) | Distal Motor Amplitude CMAP (mVlt) | Distal Sensory Amplitude SEPA (μVlt) | ||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
right | left | right | left | right | left | right | left | ||||||
N°1 | M | Hunter | 84 | 125 | 55 | 3.8 (+2.5 SD) | 4.2 (+3 SD) | 2.6 (+2 SD) | 3.6 (+4 SD) | 3.7 (77%) | 3.0 (62%) | 9.4 (47%) | 8.7 (43%) |
70 | 4.7 (+3.5 SD) | 5.8 (+6 SD) | 3.2 (+3 SD) | absent | 0.6 (12%) | 1.1 (23%) | 9.5 (47%) | absent | |||||
79 | 4.5 (+3 SD) | 6.6 (+7 SD) | 2.7 (+2 SD) | absent | 8.8 (183%) | 6.2 (125%) | 5.0 (22%) | absent | |||||
N°2 | M | Hunter | 141 | 145 | 146 | 5.0 (+3 SD) | 3.5 (0 SD) | 3.0 (+2 SD) | 2.5 (0 SD) | NA | NA | NA | 4.1 (0%) |
179 | 2.3 (-1 SD) | 3.5 (+0.8 SD) | absent | absent | 3.1 (76%) | 3.2 (77% ) | absent | absent | |||||
N°3 | M | Hunter | 136 | 141 | 142 | 6.7 (+6 SD) | 6.0 (+5 SD) | 3.7 (+4 SD) | 3.2 (+4 SD) | 4.5 (110%) | 4.5 (110%) | 4.7 (31%) | 8.3 (56%) |
167 | 4.1 (+1.5 SD) | 3.7 (+1 SD) | 2.8 (+1.5 SD) | 2.5 (+0.5 SD) | 3.8 (93% ) | 3.9 (95%) | 11.0 (74%) | 23.3 (156%) | |||||
N°4 | M | Hunter | 78 | 35 | 31 | normal | normal | normal | normal | normal | normal | normal | normal |
57 | 3.0 (+1 SD) | 4.8 (+5 SD) | 3.2 (+3 SD) | 3.4 (+3.5 SD) | 3.8 (80%) | 5.3 (110%) | 13.3 (66%) | 14.0 (70%) | |||||
N°5 | M | Hunter | 78 | 109 | 69 | 13.2 (+18 SD) | 10.0 (+13 SD) | absent | absent | 0.6 (13%) | 0.6 (15%) | absent | absent |
76 | 11.0 (+14 SD) | 10.7 (+14 SD) | absent | absent | 0.7 (14%) | 0.2 (4%) | absent | absent | |||||
102 | 5.3 (+3 SD) | 3.7 (+1 SD) | 4.2 (+7 SD) | 3.8 (+6 SD) | 2.5 (73%) | 5.6 (164%) | 4.9 (38%) | 5.0 (38%) | |||||
N°6 | M | Hunter | 120 | 129 | 134 | 3.0 (0 SD) | 4.0 (+1.5 SD) | 3.4 (+3 SD) | 3.12 (+2.5 SD) | 4.9 (120%) | 4.8 (117%) | 1.9 (12%) | 19.6 (130%) |
N°7 | M | Hunter | 68 | 115 | 4.5 (+2 SD) | 4.3 (+2 SD) | 3.3 (+4 SD) | 3.3 (+4 SD) | 8.3 (265%) | 2.5 (74%) | 8.5 (65%) | 5.0 (38%) | |
N°8 | M | Hunter | 72 | 3.7 (+3 SD) | 3.5 (+2.5 SD) | absent | absent | 0.6 (15%) | 4.4 (110%) | absent | absent | ||
N°9 | M | Hunter | 44 | 5.4 (+5 SD) | 4.7 (+4 SD) | 3.8 (+5 SD) | 3.0 (+1 SD) | 1.9 (48%) | 0.5 (13%) | 10.6 (53%) | 8.4 (42%) | ||
N°10 | M | Hunter | 53 | 43 | 4.2 (+3 SD) | 4.9 (+7 SD) | 2.4 (+1 SD) | 3.8 (+5 SD) | 4.3 (107%) | 2.5 (62%) | 25.0 (120%) | 17.1 (85%) | |
N°11 | M | Hurler | 56 | 5.1 (+4.5 SD) | 4.1 (+3 SD) | 4.1 (+5 SD) | 2.5 (+1.5 SD) | 1.0 (20%) | 2.9 (60%) | 3.7 (18%) | 12.8 (65%) | ||
N°12 | F | Hurler | 12 | 18 | 2.4 (0 SD) | 2.2 (0 SD) | 128.0 (-2 SD) | 1.1 (-2 SD) | 7.2 (185%) | 4.8 (125%) | 29.6 (116%) | 42.6 (167%) | |
N°13 | M | Hurler-Sheie | 74 | 93 | 51 | 2.5 (0 SD) | ND | absent | ND | 5.2 (108%) | ND | absent | ND |
64 | 2.0 (+0.5 SD) | 2.2 (0 SD) | absent | absent | 2.3 (49%) | 4.6 (95% ) | absent | absent | |||||
176 | 4.5 (+ 2.5 SD) | 3.6 (+1 SD) | absent | absent | 2.1 (51%) | 5.9 (144%) | absent | absent |