Publication details

Anestezie a nervosvalová onemocnění

Title in English Anaesthesia and neuromuscular disorders
Authors

VOHÁŇKA Stanislav ŠTOURAČ Petr KLINCOVÁ Martina

Type Article in Periodical
Magazine / Source Česká a slovenská neurologie a neurochirurgie
MU Faculty or unit

Faculty of Medicine

Citation
Doi http://dx.doi.org/10.14735/amcsnn2018501
Keywords anaesthesia; myasthenia gravis; muscular dystrophy; myotonic dystrophy; neuromuscular blockade; malignant hyperthermia
Description Anaesthesia is a complex situation during the treatment of patients with neuromuscular diseases. On the one hand, concerns about breaking the fragile balance can lead to postponing of necessary interventions, but on the other hand, it can lead to serious risks with improper approaches. Myasthenia is a disease where there is no risk of malignant hyperthermia, and there is an altered sensitivity to peripheral myorelaxants. We prefer to avoid benzodiazepines as a premedication, and after the procedure we always place the patient on a monitored bed with the possibility of artificial ventilation. We do not give suxamethonium to muscular dystrophy and myotonic dystrophy patients and do not use volatile gases due to risk of rhabdomyolysis. There is also no risk of malignant hyperthermia in this group. Malignant hyperthermia is a pharmacogenetic disorder manifested by abnormal hypermetabolic response when exposed to halogenated inhalation anesthetics (halothane, isoflurane, desflurane, sevoflurane) or peripheral muscle relaxants of the depolarizing type (suxamethonium). It can rarely occur even after physical, excessive or heat stress. It is associated with electromechanical coupling and most often occurs with mutations in the ryanodine receptor, but rarely with some other mutations in the genes that are related to calcium metabolism.