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Table 2 Results of nerve conduction studies in patients with Hereditary spastic paraplegia caused by a c.2155C > T, p.R719W mutation in DNM2

From: Adult-onset autosomal dominant spastic paraplegia linked to a GTPase-effector domain mutation of dynamin 2

Patients

III:3

III:5

IV:1

Normal values

Symptom duration (years) at time of study

17

32

7

 

Motor nerve conduction studies

    

n.Medianus

    

Distal motor latency (m/s)

3.6

ND

3.5

<4.2

Compound muscle action potential (mV)a

4.5

ND

8.7

>5.0

Motor nerve conduction velocity (m/s)

48.2

ND

57.1

>47.6

n.Peroneus

    

Distal motor latency (m/s)

4.49

NR

5.1

<6.5

Compound muscle action potential (mV)a

NR

0.04

0.21

>3.0

Motor nerve conduction velocity (m/s)

NR

NR

40.6

>40.5

n.Tibialis posterior

    

Distal motor latency (m/s)

NR

NR

6.2

<6.0

Compound muscle action potential (mV)a

NR

0.5

1.9

>5.0

Motor nerve conduction velocity (m/s)

NR

NR

39.7

>40.0

Sensory nerve conduction studies

    

n.Medianus

    

Distal sensory nerve conduction velocity (m/s)b

61.4

ND

64.5

>47.2

Sensory nerve action potential amplitude (μV)

10

ND

16.6

>5.0

n.Suralis

    

Distal sensory nerve conduction velocity (m/s)

NR

38.5

42.8

>33.2

Sensory nerve action potential amplitude (μV)

0.7

0.43

5.82

>6.0

  1. ND not done
  2. Bold: outside normal range
  3. aDistal motor response amplitude
  4. bOrthodromic stimulation
  5. NR no response