Table 2.
Frequency of symptoms and characteristics stratified by gender and age of onset
| Male 48.9%(272/556) | Female 51.1% (284/556) | Congenital 8.8% (16/182) | Infantile 5.5% (10/182) | Juvenile 27.5% (50/182)) | Adult 39% (71/182) | Late adult 19.2% (35/182) | Total n = 556 | |
|---|---|---|---|---|---|---|---|---|
| Myotonia, % (No.) | 82.9 (218/263) | 73.1 (204/279) | 68.8 (11/16) | 80 (8/10) | 89.4 (42/47) | 78.3 (54/69) | 54.3 (19/35) | 77.9 (422/542) |
| Severe | 26.1 (57/218) | 17.6 (49/204) | 9.1 (1/11) | 12.5 (1/8) | 23.8 (10/42) | 25.9 (14/54) | 36.8 (7/19) | 25.1 (106/422) |
| Fatigue, % (No.) | 79.6 (215/270) | 78.6 (217/276) | 75 (12/16) | 90 (9/10) | 83.7 (41/49) | 72.1 (49/68) | 74.3 (26/35) | 79.1 (432/546) |
| Severe | 23 (62/270) | 32.2 (89/276) | 8.3 (1/12) | 11.1 (1/9) | 39 (16/41) | 46.9 (23/49) | 23.1 (6/26) | 35 (151/432) |
| Dysphagia, % (No.) | 51 (132/259) | 45.5 (122/268) | 33.3 (5/15) | 33.3 (3/9) | 44.9 (22/49) | 34.4 (22/64) | 62.5 (20/32) | 48.2 (254/527) |
| Ambulatory assisted or non-ambulant, % (No.) | 35.2 (94/267) | 34.9 (98/281) | 31.3 (5/16) | 30 (3/10) | 26.5 (13/49) | 37.1 (26/70) | 31.4 (11/35) | 35 (192/548) |
| Wheelchair use, % (No.) | 26.8 (72/269) | 25.5 (71/278) | 43.8 (7/16) | 20 (2/10) | 20 (10/50) | 26.1 (18/69) | 22.9 (8/35) | 26.1 (143/547) |
| Heart condition, % (No.) | 50.9 (58/114) | 45.5 (50/110) | 30.8 (4/13) | 50 (5/10) | 46.9 (23/49) | 51.4 (36/70) | 53.1 (17/32) | 48.2 (108/224) |
| Non-invasive ventilation, % (No.) | 16.2 (19/117) | 13.6 (16/118) | 12.5 (2/16) | 0 (0/10) | 10 (5/50) | 20 (14/70) | 14.7 (5/34) | 14.9 (35/235) |
| Cataract surgery, % (No.) | 22.5 (25/111) | 29.5 (33/112) | 0 (0/16) | 0 (0/10) | 14.9 (7/49) | 36.8 (25/68) | 48.6 (17/35) | 26 (58/223) |
Age of onset has been determined based upon the age provided by the treating clinician and was classified into: (1) congenital form, onset from birth to 1 month old; (2) infantile form, onset from 1 month to 10 years; (3) juvenile form, onset at 11–20 years; (4) adult form, onset at 21–40 years, and (5) late adult form, onset after the age of 40 years
Fractions give the absolute number of patients divided by the number of patients with available clinical information for each item