Table 1.
Summary of clinical data for the 73 patients with muscular dystrophy from 104 PSG studies
| Variables | All types | BMD | CMD | DM | DMD | LGMD |
| N (%) | N (%) | N (%) | N (%) | N (%) | N (%) | |
| Total patients | 73 (100) | 5 (100) | 7 (100) | 24 (100) | 27 (100) | 10 (100) |
| Male | 55 (75) | 5 (100) | 6 (86) | 10 (42) | 27 (100) | 7 (70) |
| Female | 18 (25) | 0 (0) | 1 (14) | 14 (58) | 0 (0) | 3 (30) |
| Vital status as of January 2023* | ||||||
| Alive | 58 (79) | 4 (80) | 5 (71) | 20 (83) | 21 (78) | 8 (80) |
| Deceased | 14 (19) | 1 (20) | 2 (29) | 3 (13) | 6 (22) | 2 (20) |
| Unknown | 1 (1) | 0 (0) | 0 (0) | 1 (4) | 0 (0) | 0 (0) |
| Tracheostomy* | ||||||
| Yes | 5 (7) | 0 (0) | 1 (14) | 3 (13) | 1 (4) | 0 (0) |
| No | 68 (93) | 5 (100) | 6 (86) | 21 (87) | 26 (96) | 10 (100) |
| Total studies† | 104 (100) | 7 (100) | 9 (100) | 37 (100) | 38 (100) | 13 (100) |
| Male | 76 (73) | 7 (100) | 8 (89) | 16 (43) | 38 (100) | 7 (54) |
| Female | 28 (27) | 0 (0) | 1 (11) | 21 (57) | 0 (0) | 6 (46) |
| Daytime hypoventilation (ABG) (pCO2 >45 mm Hg or bicarb >30 mEq/L)† |
||||||
| Present | 14 (13) | 0 (0) | 2 (22) | 7 (19) | 2 (5) | 3 (23) |
| Absent | 35 (34) | 3 (43) | 3 (33) | 8 (22) | 17 (45) | 4 (31) |
| Not recorded | 55 (53) | 4 (57) | 4 (44) | 22 (59) | 19 (40) | 6 (46) |
| Maximal inspiratory pressure† | ||||||
| Normal | 1 (1) | 0 (0) | 1 (11) | 0 (0) | 0 (0) | 0 (0) |
| Reduced (<75 cm H2O) | 27 (26) | 0 (0) | 4 (44) | 4 (11) | 14 (37) | 5 (38) |
| Not recorded | 76 (73) | 7 (100) | 4 (44) | 33 (89) | 24 (63) | 8 (62) |
| Maximal expiratory pressure† | ||||||
| Normal | 5 (5) | 0 (0) | 1 (11) | 1 (3) | 1 (3) | 2 (15) |
| Reduced (<60 cm H2O) | 23 (22) | 0 (0) | 4 (44) | 3 (8) | 13 (34) | 3 (23) |
| Not recorded | 76 (73) | 7 (100) | 4 (44) | 33 (89) | 24 (63) | 8 (62) |
| Echocardiogram† | ||||||
| Normal | 59 (57) | 4 (57) | 6 (67) | 14 (38) | 24 (63) | 11 (85) |
| Reduced (<50 NL LVEF) | 8 (8) | 1 (14) | 0 (0) | 0 (0) | 7 (18) | 0 (0) |
| Not recorded | 37 (36) | 2 (29) | 3 (33) | 23 (62) | 7 (18) | 2 (15) |
Clinical data were recorded within ±6 months of the corresponding PSG studies. Vital status was assessed up to January 2023. Clinical data for each patient nearest each PSG study are presented in online supplemental table 1.
*Percentages for all types use total number of patients (73) as the denominator; percentages for specific types use the number of patients in the total patients row for that specific type as the denominator. Percentages may not sum to 100 due to rounding.
†Percentages for all types use total number of PSG studies (104) as the denominator; percentages for specific types use the number of PSG studies in the total studies row for that specific type as the denominator. Percentages may not sum to 100 due to rounding.
ABG, arterial blood gas; BMD, Becker muscular dystrophy; CMD, congenital muscular dystrophy; DM, myotonic dystrophy; DMD, Duchenne muscular dystrophy; LGMD, limb-girdle muscular dystrophy; NL LVEF, normal left ventricular ejection fraction; pCO2, partial pressure of carbon dioxide; PSG, polysomnography.