The deficiency of muscle Carnitine secondary to chronic dialysis frequently induces lipid storage in striated muscles associated with progressive myocardial involvement. Serial EMG recordings disclose the presence of several motor unit potential families in tibialis anterior muscle related to damage of peripheral nervous fibres and muscular districts due to the storage of lipid vacuoles. The administration of d,1-Carnitine to twenty chronically dialyzed uraemic patients significantly improved distal latency of the M response of the external peroneal nerve at the EDB muscle and the MUP properties suggesting a Carnitine-dependent amelioration of fatty acid oxidative processes both in muscle and Schwann cells. The main side effects following d,1-Carnitine oral administration (3 g/daily) is a myasthenia-like symptom complex that is promptly reversed on drug interruption.