Acute quadriplegic myopathy with myosin-deficient muscle fibres after liver transplantation - Defining the clinical picture and delimiting the risk factors

Background. In the last few years, rare cases of acute quadriplegic myopath y (AQM*) with myosin deficient muscle fibres occurring after solid organ tr ansplantation has been reported. The aim of the present study was to review all cases of AQM with myosin deficient-fibres seen at our institution amon g a large series of patients after orthotopic liver transplants (OLT), with special attention to clinical aspects and associated risk factors. Additio nally, an extensive review of all ultrastructurally demonstrated cases of A QM in transplant recipients is also included. Patients and Methods. Among patients involved in 281 consecutive Liver tran splant procedures performed in a 4-year period, 3 men and 1 woman developed an arreflexic, flaccid quadriplegia in the immediate postoperative period of OLT, After ruling out other causes of weakness, a muscle biopsy was perf ormed and a loss of thick (myosin) filaments was confirmed by ultrastructur al analysis in all cases. Accurate clinical, epidemiological, and evolutive data were recorded. Results, Corticosteroids had been used at usual dosage given to liver trans plant recipients; all four patients had several intra- and postoperative co mplications leading to receiving significantly higher amounts of hemoderiva tes, to develop renal failure in all cases, and to require a significantly higher number of reoperations within a few days after transplantation than our contemporaneous global series of liver transplant recipients. AQM patie nts required a significantly longer intensive care unit and hospital stay. Muscular recovery was the rule, but currently a mild myopathic gait remains in three patients. These and other reported cases of AQM do not histologic ally and clinically differ from AQM seen in other critically ill patients w ho have not had transplants. Conclusions, Patients with a complicated intra- and postoperative course of OLT who develop newly acquired acute muscle weakness should be suspected a s having acute AQM with myosin-deficient muscle fibres, In this setting, di fferential diagnosis with other causes of weakness should be carried out, b ecause the prognosis of this myopathy is good with early muscle rehabilitat ion therapy.