Electrophysiological and Structural Changes in Chinese Patients with LHON
Table 1
Summary of genetic, functional, and structural findings in patients with LHON.
Patient number
Gender
Eye
Age
Gene mutation
Snellen BCVA
PVEP
PERG
mfERG
OCT
Peak time delayed (ms)
P50 amplitude reduced
N95/P50 amplitude reduced
Peak time shortening
Ring 1-2 amplitude decreased
GCL + IPL thickness thinning in macula
RNFL thickness thinning in macula
RNFL around optic disc
Acute
1
M
L
16
11778
6/120
35
−
+
−
+
+
+
−
Chronic
1
M
R
16
11778
6/300
−
+
+
+
+
+
+
Acute
2
M
R
14
11778
6/150
20
−
+
−
+
+
−
Acute
2
M
L
14
11778
6/40
16
−
−
−
+
+
−
Acute
3
M
R
13
3635, 3316
6/150
12
−
+
−
+
+
−
−
Acute
3
M
L
13
3635, 3316
6/200
12
−
+
−
−
+
+
−
Acute
4
M
R
38
3497
CF
Acute
4
M
L
38
3497
6/600
Chronic
5
M
R
19
11778
6/200
−
+
+
+
+
+
+
Chronic
5
M
L
19
11778
6/200
−
+
+
+
+
+
+
Chronic
6
F
R
18
3460
6/40
18
−
+
+
+
+
+
Chronic
6
F
L
18
3460
6/60
19
−
+
+
+
+
+
Chronic
7
M
R
15
11778
CF
27
−
+
+
+
+
+
+
Chronic
7
M
L
15
11778
6/600
25
−
+
+
+
+
+
+
Chronic
8
F
L
43
3460
6/20
+
Chronic
8
F
R
43
3460
6/120
+
Chronic
9
M
L
38
11778
6/300
−
+
+
+
+
+
+
Chronic
9
M
R
38
11778
CF
+
+
+
+
+
+
+
Chronic
10
M
L
8
3394
6/600
+
+
+
+
Chronic
10
M
R
8
3394
6/60
+
+
+
+
Chronic
11
M
L
29
3497
6/9
+
+
+
+
Chronic
11
M
R
29
3497
6/60
+
+
+
Chronic
12
M
L
18
11778
6/60
−
+
+
+
+
+
+
Chronic
12
M
R
18
11778
CF
−
+
+
+
+
+
+
Chronic
13
M
L
41
11778
6/600
Chronic
13
M
R
41
11778
6/60
Notes: “−” signifies normal findings; “+” signifies abnormal findings; absence of data indicates that tests were not performed; “CF” indicates “counting fingers visual acuity”; PVEP peak time delay indicates the time above the upper limit of normal in the age-matched control group.
