Charcot-Marie-Tooth Disease Neuropathy Score of Shy et al (CMTNS)

sensory symptoms in the lower extremities

none

0

limited to toes

1

above toes up to ankles

2

above ankles up to knees

3

above knees

4

motor symptoms in the legs

none

0

trips, slaps feet, catches toes

1

ankle-foot orthosis on at least 1 leg or ankle support

2

cane, walker, ankle surgery

3

wheelchair most of the time

4

motor symptoms in the arms

none

0

difficulty with buttons and/or zippers

1

unable to do buttons or zippers but can write

2

cannot write or use keyboard

3

affecting proximal arms

4

pin sensibility

normal

0

reduced in fingers or toes

1

reduced up to wrists or ankles

2

reduced at elbows or knees

3

reduced above elbows or knees

4

vibration sensibility

normal

0

reduced in fingers or toes

1

reduced up to wrists or ankles

2

reduced at elbows or knees

3

reduced above elbows or knees

4

lower extremity strength

normal

0

4+, 4 or 4- on foot dorsiflexion

1

<= 3 on foot dorsiflexion

2

<= 3 dorsiflexion and plantar flexion

3

proximal weakness

4

upper extremity strength

normal

0

4+, 4 or 4- on intrinsics or finger extensors

1

<= 3 intrinsics or finger extensors

2

< 5 wrist extensors

3

weak above elbows

4

ulnar CMAP

> 6 mV

0

4.0 to 5.9 mV

1

2.0 to 3.9 mV

2

0.1 to 1.9 mV

3

absent

4

ulnar SNAP

> 9 µV

0

6.0 to 8.9 µV

1

3.0 to 5.9 µV

2

0.1 to 2.9 µV

3

absent

4

0

none

1 to 10

mild

11 to 20

moderate

21 to 36

severe