1. The force produced by isometric contractions of the quadriceps muscle have been studied during maximal voluntary contractions and when a substantial part of the muscle was electrically stimulated via surface electrodes. 2. In normal children and adults, the force of a maximal voluntary contraction of the quadriceps was proportional to body weight. 3. The function of the quadriceps has been described in terms of the force/frequency curve, speed of relaxation and the rate of loss of force during 18 s stimulation at 30 Hz and 100 Hz. 4. The functional characteristics of adductor pollicis when stimulated via the ulnar nerve were essentially similar to those of the quadriceps. 5. Studies of the function of these two muscles are complementary since quadriceps femoris is amenable to needle biopsy investigations of its structure and chemistry whereas adductor pollicis is more suitable for electrophysiological studies.

1. The fatigue of force that occurs during the first 60 s of a maximum voluntary contraction of the human quadriceps has been examined by comparing the voluntary force with that obtained by brief tetanic stimulation at 50 Hz in nine healthy subjects. In three subjects the voluntary force declined in parallel with the tetanic force whereas in the remainder it fell more rapidly, suggesting that central fatigue was present. 2. For those subjects who showed little or no central fatigue, surface electromyograph (EMG) activity remained approximately constant while the force declined by about 60%. In the others, EMG activity and force declined in parallel but when an extra effort was made the subjects could briefly increase their force and this was accompanied by a proportionately greater increase in EMG activity (generally up to the original value). 3. It is concluded that in sustained maximum voluntary contractions of the quadriceps (a) central fatigue may account for an appreciable proportion of the force loss, (b) surface EMG recordings provide no evidence that neuromuscular junction failure is the limiting factor determining the loss of force in this muscle.

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A severely mentally retarded boy with two normal siblings was persistently found to excrete elevated amounts of 3-hydroxybutyrate and acetoacetate. Enzyme analysis in cultured fibroblasts revealed a probable deficiency in cytosolic acetoacetyl-CoA thiolase which was about half the control activity with normal mitochondrial thiolase activities. Treatment with reduced dietary fat was initiated and a rapid reduction of the ketosis to biochemical normality was demonstrated. Shortly after initiating dietary treatment he presented with severe gastrointestinal problems and the histological features of colitis cystica superficialis. This appeared to respond to intravenous hydrocortisone therapy with an apparent complete recovery.