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Table 1 Electroneurography examination results

From: Early detection of median nerve compression by Electroneurography can improve outcome in children with Mucopolysaccharidoses

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

  1. CTS carpal tunnel syndrome, ND not done, NA not available, ERT Enzyme Replacement Therapy, ENG electroneurography evaluation, SD standard deviation, % percent of lower limit of normal (LLN)