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Anaesthesia for Paramyotonia Congenita: A Narrative Review
Marianne Chee1, Suhitharan Thangavelautham2, Jing Hui Chen2
1Anaesthesiology and Critical Care, Singapore General Hospital, Singapore, SGP.
Paramyotonia congenita (PMC) is a rare, non-progressive neuromuscular disorder characterised by muscle stiffness and delayed relaxation following voluntary contraction or mechanical stimulation. Its rarity means many anaesthetists may encounter it only once, if ever, in their careers. This narrative review synthesises existing literature on anaesthetic management in these patients. We aim to provide anaesthetists with a clearer understanding of PMC, with particular focus on perioperative considerations, choice of anaesthetic agents, and reported outcomes. While anaesthetic techniques vary, several recurring management principles emerge, including avoidance of triggers for muscle stiffness, careful temperature control, and readiness to manage potential complications. Although there is no consensus on the optimal anaesthetic approach to PMC, current literature suggests that, with appropriate planning and intraoperative vigilance, safe anaesthesia is achievable.
Paramyotonia congenita (PMC) is a rare, non-progressive neuromuscular disorder characterised by muscle stiffness and delayed relaxation following voluntary contraction or mechanical stimulation. Its rarity means many anaesthetists may encounter it only once, if ever, in their careers. This narrative review synthesises existing literature on anaesthetic management in these patients. We aim to provide anaesthetists with a clearer understanding of PMC, with particular focus on perioperative considerations, choice of anaesthetic agents, and reported outcomes. While anaesthetic techniques vary, several recurring management principles emerge, including avoidance of triggers for muscle stiffness, careful temperature control, and readiness to manage potential complications. Although there is no consensus on the optimal anaesthetic approach to PMC, current literature suggests that, with appropriate planning and intraoperative vigilance, safe anaesthesia is achievable.
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