Anesthesiologic approach in neuromuscular diseases

Firstly it must be said that the neuromuscular diseases (in this case spinal muscular atrophy) usually are associated with cardiovascular and respiratory diseases of small or large entities. So before setting general anesthesia the doctor must perform a thorough anamnesis (the patient's medical history), and then take into account any of these diseases, which will be addressed in their own ways. If case history is negative for these or other illnesses, you can proceed to set a anesthesiologic program that takes into account the current state of the patient and the specific disease.
Much of the interventions can be performed by techniques that do not use muscle relaxants substances (drugs that can relax skeletal muscles), which in these cases are not recommended, if necessary it could be used so-called "competitive" drugs, in low-dose, and in the end, the relevant doses of neostigmine or fisiostigmine.
The use of this class of drugs can lead intraoperative occurrence of a syndrome known as "malignant hyperthermia", which seriously complicates the action and may also lead to heavy consequences! Fortunately it’s an occurrence very rare, but that should not be underestimated.
The use of drugs for induction and maintenance of anesthesia, whether intravenous or volatile, does not interfere on omeostasis (general physical balance) of the patient, although the doses must be carefully measured. The patient must be constantly monitored (controlled) in all his vital signs, and these must be adjusted to the depth and quantity of medicines to be administered. The awakening must be particularly followed to ensure that the active muscles have completely taken over their function.
In conclusion, patients of this type should be considered "sensitive patients” that bear less and worse than other anesthetics too deep or sudden changes in blood pressure.
While taking into account the initial premise, I feel in all conscience to reassure all those who should be subject to intervention under general anesthesia, provided that the anesthetist is experienced!
Finally, we must remember that some (small) interventions can also be performed under local anesthesia, with the same precautions for control and sensitive approach.

dott. Augusto Grube, Anesthetist, Trieste