Signs

Frequency (n)

Percentage (%)

Motor signs

Myoclonus

14

9

Tremor

39

24.8

Asterixis

7

4.5

Fasciculations

3

1.9

Muscle tone abnormalities

Spastic hypertonia

2

1.3

Plastic hypertonia

4

2.5

Hypotonia

2

1.3

Reflex jerk of the lower limbs

Hyporeflexia/areflexia

76

48.4

Hyperreflexia

5

3.2

Sensitivity abnormality

Monofilament perception

Reduced

46

44.58

Absent

16

11.94

Perception test on diapason

Reduced

50

43.3

Absent

17

12.69

Cranial nerve involvement

Nerve I

5

3.2

Nerve VII

6

3.8

Nerve VIII

14

8.9

Orthostatic disturbances

Orthostatic hypotension

25

15.9

Orthostatic vertigo

21

13.4

Other syndromes

Pyramidal syndrome

10

6.4

Cerebellar syndrome

4

2.5

Spinal cord compression

1

0.6