Table 1.
Clinical features of the 45 patients with late-onset MADD
| Clinical features | Numbers of patients |
|---|---|
| Gender | |
| Male | 27/45 (60.0) |
| Median age (years), range | 28 (6, 44) |
| Median onset ages (years), range | 21 (4, 44) |
| Muscle weakness | |
| Proximal limbs | 45/45 (100) |
| Distal limbs | 0/45 (0) |
| Neck | 45/45 (100) |
| Mastication | 21/45 (46.7) |
| Exercise intolerance | 45/45 (100) |
| Myalgia | 21/45 (46.7) |
| Muscle atrophy | 10/45 (22.2) |
| Gastrointestinal symptoms | 26/45 (57.8) |
| Electromyography | |
| Myopathic changes | 28/42 (66.7) |
| Neurogenic changes | 2/42 (4.8) |
| No obvious abnormality | 12/42 (28.6) |
| Fatty liver | 6/31 (19.4) |
| CK (U/L), mean (range) | 888 (168, 2526) |
| LDH (U/L), mean (range) | 962 (253, 3507) |
| AST (U/L), mean (range) | 139 (37, 447) |
Values are presented as n/N (%). MADD: Multiple acyl-coenzyme A dehydrogenase deficiency; CK: Creatinine kinase; AST: Aspartate aminotransferase; LDH: Lactate dehydrogenase. Upper limit of normal: CK 140 U/L, LDH 245 U/L, and AST 40 U/L.