1/2. myokymia and impaired muscular relaxation with continuous motor unit activity.We have studied two cases of the syndrome of myokymia and impaired muscular relaxation with continuous motor unit activity. Both patients complained of muscle twitching, weakness, stiffness, and hyperhydrosis during their illness. myokymia was present over the entire body in both. On repetitive testing of muscle strength each patient showed initial fatigue followed by increasing strength as he continued his efforts. Both patinets improved on phenytoin therapy at high blood levels. Nerve conduction velocities were decreased. Electromyograms showed continuous electrical activity at rest which persisted during sleep and spinal anaesthesia but was diminished by curare. Intravital staining with methylene blue in one case demonstrated sprouting and beading of motor nerve terminals with multiple innervation of muscle fibres. The neurophysiological and pathological findings in these two cases indicate an abnormality of peripheral nerve in this disorder.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/2. Twelve hour anaesthesia in a patient with epidermolysis bullosa.epidermolysis bullosa (EB), an inherited disorder presents clinically with recurrent cutaneous blister formation with possible involvement of mucous membranes and other organs. The sequelae of this disease pose multiple challenges to the anaesthetist and operating room team. Recent literature describes several anaesthetic techniques for the short surgical procedures this patient population may undergo. We describe the anaesthetic technique employed in a 28-yr-old women with recessive dystrophic epidermolysis bullosa who underwent 12 hr reconstructive surgery followed by a review of the literature that includes a recent description of the possible association of EB with at least two distinct neuromuscular diseases. A detailed description of airway and skin management is described in addition to preoperative concerns. We conclude that a prolonged operative procedure can be undertaken successfully in this population with minimal sequelae involving skin integrity and airway management.- - - - - - - - - - ranking = 4keywords = anaesthesia (Clic here for more details about this article) |