Severity and progression of disorder vary. Often associated with diabetes and heart disease. Disease progression varies. Weakness and fatigue of hip muscles with aching back and thigh muscles common. Lung tumor is often present.
Progression varies with success of drug therapy and treatment of any malignancy.
For infants, disease is generalised and severe with heart, liver and tongue enlargement common. Adult form involves weakness of mid-body and respiratory muscles. Varied weakness of shoulder, hip, face and neck muscles. Often a secondary metabolic condition, progression varies and carnitine supplementation can be effective. Inability to sustain moderate prolonged exercise.