Wahbi et al reported a score for predicting survival of a patient with myotonic dystrophy type 1. This can help to identify a patient who may require more aggressive managemen or a novel therapy. The authors are from multiple institutions in France.
Patient selection: myotonic dystrophy Type 1
Parameters:
(1) age in years
(2) diabetes
(3) need for walking support
(4) heart rate in beats per minute
(5) systolic blood pressure in mm Hg
(6) first-degree AV block
(7) bundle branch block
(8) vital capacity as percent of predicted
Parameter
Finding
Points
age in years
< 30 years
0
30 to 45 years
4
> 45 years
8
diabetes
no
0
yes
2
need for walking support
no
0
yes
3
heart rate
< 63
0
>= 63
1
systolic BP
<= 110
1
> 110
0
first-degree AV block
absent
0
present
1
bundle branch block
absent
0
present
1
vital capacity
< 60% of predicted
3
60 to 90%
1
> 90% of predicted
0
total score =
= SUM(points for all of the parameters)
Interpretation:
• minimum score: 0
• maximum score: 20
• The higher the score the worse the 10-year survival.
Score
10-Year Survival
0 to 2
98%
3
97%
4
95%
5
94%
6
91%
7
89%
8
85%
9
80%
10
73%
11
65%
12
56%
13
45%
14
34%
15 to 20
23%
Performance:
• The area under the ROC curve is 0.75.
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