Skip to main content

Table 2 Group 1, 2 and 3 symptoms

From: The Brown-Vialetto-Van Laere and Fazio Londe syndrome revisited: natural history, genetics, treatment and future perspectives

 

Group 1 (N=21)

%

Group 2 (N=21)

%

Group 3 (N=28)

%

Total (N=73)

%

Bulbar palsy (CN IX-XII)

18

86%

21

100%

25

89%

67

92%

Hearing loss

14

67%

19

90%

24

86%

59

81%

Facial weakness

10

48%

20

95%

23

82%

56

77%

Respiratory compromise

18

86%

13

62%

13

46%

47

64%

Muscle weakness

14

67%

10

48%

14

50%

40

55%

Upper motor neuron signs

3

14%

10

48%

11

39%

26

36%

Palsy of CN III, IV or VI

9

43%

5

24%

7

25%

23

32%

Stridor

10

48%

3

14%

4

14%

18

25%

Palsy of the trigeminal nerve (V)

1

5%

6

29%

6

21%

14

19%

Lower motor neuron signs

4

19%

5

24%

4

14%

13

18%

Diaphragm weakness/palsy

4

19%

3

14%

4

14%

11

15%

Palsy of the optic nerve (II)

2

10%

3

14%

5

18%

10

14%

Ataxia

2

10%

3

14%

3

11%

9

12%

Tremor

2

10%

1

5%

3

11%

6

8%

Behavioral changes

1

5%

0

0%

2

7%

3

4%

Mental retardation

0

0%

0

0%

3

11%

3

4%

Epilepsy

0

0%

0

0%

2

7%

3

4%

Autonomic dysfunction

0

0%

0

0%

2

7%

2

3%