Sugammadex is changing the paradigm in neuromuscular blockade in patients with myotonic dystrophy

2019 ◽  
Vol 29 (10) ◽  
pp. 337-340 ◽  
Author(s):  
Joana Teixeira ◽  
Bernardo Matias ◽  
Irene Ferreira ◽  
Tiago Taleço ◽  
João S Duarte

Myotonic dystrophy type 1 is a rare neuromuscular disease that represents a challenge to anaesthetic management. Most of the literature does not recommend the usage of neuromuscular blocking agents, if general anaesthesia is needed in these patients. Depolarising neuromuscular blocking agents like suxamethonium are contraindicated, and there might be an increased sensitivity to non-depolarising agents like rocuronium with greater risk of postoperative residual neuromuscular blockade and consequent respiratory failure. Reversing neuromuscular blockade is also problematic as neostigmine can induce myotonic crisis, impairing normal ventilation. We discuss the use of sugammadex for neuromuscular blockade reversal, from a clinical case of a patient with myotonic dystrophy type 1 for laparoscopic cholecystectomy. The patient had a general anaesthesia with neuromuscular blockade with rocuronium. After the surgical procedure, neuromuscular blockade was safely reversed with sugammadex, guided by neuromuscular monitoring without any perioperative complications.

2018 ◽  
Vol 97 (1) ◽  
pp. 78-81
Author(s):  
E.A. Mamaeva ◽  
◽  
L.A. Fedorova ◽  
S.E. Voronovich ◽  
V.D. Nazarov ◽  
...  

2021 ◽  
pp. 1-10
Author(s):  
Elisabet Hammarén ◽  
Lena Kollén

Background: Individuals with myotonic dystrophy type 1 (DM1) are known to stumble and fall, but knowledge is scarce regarding dynamic stability in this disorder. Objective: To describe disease progress regarding muscle force, dynamic stability and patient reported unintentional falls during a ten-year period, in individuals with DM1. Methods: Quantification of isometric muscle force in four leg muscle groups and assessment of Timed 10-meter-walk in maximum speed (T10max), Timed Up&Go (TUG) and Step test (STEP) were performed at three occasions in a DM1 cohort, together with self-reported falls. Results: Thirty-four people (m/f:11/23, age:50.2 + /–9.4) participated. The muscle force loss after ten years was large in the distal ankle muscles. A steeper force decrease was seen in most muscles between year five and ten compared to the former five-year period. Males reported more falls than females, 91%vs 35%had fallen last year. A positive correlation, ρ= 0.633, p <  0.001, was shown between walking time (T10max) and number of falls. Frequent fallers were only seen among those with slower walk (T10max >  10seconds), and fewer steps in the STEP test (STEP≤5 steps). Conclusions: A diminishing leg muscle strength and worse dynamic stability were seen in the group, with a steeper decrease in the latter five years. Weak ankle dorsiflexors, a slower walk and difficulties to lift the forefoot were related to frequent falls.


2014 ◽  
Vol 1 (1) ◽  
pp. 95-98 ◽  
Author(s):  
Benjamin Gallais ◽  
Cynthia Gagnon ◽  
Jean Mathieu ◽  
Louis Richer ◽  
Stéphane Jean ◽  
...  

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