An isometric ischaemic intermittent m.biceps brachii exercise test is performed by 4 patients suffering from congenital myopathies which are characterized by a 100% type I fibre predominance, and by 26 healthy volunteers. Thirty contractions per minute are made at an 80% of maximal voluntary contraction level. It is found that type I muscle fibres have a fourfold lower force-generating capacity than type II muscle fibres. The EMG amplitude shows that more EMG voltage is needed per unit force in the patients with 100% type I fibres compared with controls. Under standardized fatiguing circumstances the power density frequency spectrum shows a weaker shift to lower frequencies in patients with 100% type I muscle fibres compared with controls. The muscle fibre conduction velocity (MFCV) of type I muscle fibres shows no decline during ischaemic exercise indicating an unimpaired muscle membrane excitability.