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Table 2 Nerve conduction study findings of the four cerebrotendinous xanthomatosis patients

From: Neuromuscular abnormality and autonomic dysfunction in patients with cerebrotendinous xanthomatosis

Case

 

Median

Ulnar

Superficial radial

Peroneal

Tibial

Sural

H-reflex (N < 27.6)

  

Motor

Sensory

Motor

Sensory

     

I-1

R DL (ms)

4.4 (N < 3.6)

2.8

2.9 (N < 2.8)

2.2

1.9

4.0 (N < 4.2)

2.7 (N < 4.9)

Absent

31.0

 

L DL (ms)

4.4

3.0

2.5

2.9

1.7

4.0

3.7

Absent

30.4

 

R CV (m/s)

50 (N > 57.8)

50 (N > 51.7)

50 (N > 61.4)

56 (N > 56.3)

63 (N > 50)

39 (N > 48.6)

45 (N > 50.5)

Absent (N > 47.4)

 
 

L CV (m/s)

49

47

54

49

71

42

40

Absent

 
 

R Amp

7.6 (N > 11.9)

47 (N > 33)

11 (N > 11.7)

32 (N > 26.1)

23 (N > 15)

4.1 (N > 6.0)

5.5 (N > 13.1)

Absent (N > 12.5)

 
 

L Amp

7.1

21

8.1

38

33

4.5

9.6

Absent

 
 

R F waves

26.8-0.7 (N < 25.4)

 

27.0-0.8 (N < 25.0)

  

49.8-0.4 (N < 44.7)

50.2-0.8 (N < 45.0)

  
 

L F waves

27.9-0.8

 

26.4-1.0

  

47.4-0.4

50.4-0.8

  

I-2

R DL (ms)

4.4

2.7

3.2

2.6

2.1

4.2

5.0

Absent

32.4

 

L DL (ms)

4.0

2.8

3.0

2.2

1.8

3.9

3.3

Absent

32.6

 

R CV (m/s)

49

53

54

46

57

39

41

Absent

 
 

L CV (m/s)

51

50

51

54

67

41

40

Absent

 
 

R Amp

8.6

23

7.3

26

34

2.9

10.5

Absent

 
 

L Amp

9.2

31

8.8

13

60

3.8

6.7

Absent

 
 

R F waves

30.0-0.5

 

28.8-1.0

  

50.5-1.0

51.8-1.0

  
 

L F waves

28.3-1.0

 

28.4-1.0

  

50.1-0.6

53.9-1.0

  

II-1

R DL (ms)

4.3

2.8

3.0

2.4

2.1

Absent

4.5

Absent

38.4

 

L DL (ms)

4.1

3.0

2.8

2.5

2.0

4.9

5.1

Absent

37.5

 

R CV (m/s)

44

50

42

50

56

Absent

32

Absent

 
 

L CV (m/s)

46

47

43

48

60

30

34

Absent

 
 

R Amp

10.3

42

11.5

20

25

Absent

4.0

Absent

 
 

L Amp

11.7

47

9.9

29

32

2.4

2.6

Absent

 
 

R F waves

32.6-1.0

 

32.1-1.0

  

Absent

70.6-0.6

  
 

L F waves

30.4-0.9

 

30.4-1.0

  

79.2-0.2

75.7-0.9

  

II-2

R DL (ms)

4.0

2.5

2.8

2.0

1.7

4.4

3.4

Absent

31.9

 

L DL (ms)

4.2

2.7

3.0

2.3

1.7

3.8

3.8

Absent

31.6

 

R CV (m/s)

52

56

58

60

71

39

41

Absent

 
 

L CV (m/s)

52

52

50

53

71

43

39

Absent

 
 

R Amp

8.5

29

6.6

38

16

3.2

4.7

Absent

 
 

L Amp

7.4

50

6.7

40

29

4.7

2.0

Absent

 
 

R F waves

29.8-1.0

 

30.4-0.8

  

54.2-0.3

53.3-1.0

  
 

L F waves

29.6-1.0

 

31.6-0.9

  

50.9-0.8

56.5-0.9

  
  1. N = normal; DL = distal latency; CV = conduction velocity; Amp = amplitude (mV in motor nerves and μV in sensory nerves); F waves = minimal latency-persistent rate; H-reflex = soleus H-reflex in minimal latency; AT = anterior tibialis; FDI = 1st dorsal interosseous muscle; R = right; L = left