Managing pregnancy and anaesthetics in patients with skeletal muscle channelopathies

•Two thirds of patients with skeletal muscle channelopathies had worsening of symptoms during pregnancy.•Some patients have worsening of symptoms following anaesthetic but none had malignant hyperthermia.•Patients do not have any difference in antenatal and delivery complications compared to the nor...

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Bibliographic Details
Published in:Neuromuscular disorders : NMD 2020-07, Vol.30 (7), p.539-545
Main Authors: Raja Rayan, Dipa L, Hanna, Michael G
Format: Article
Language:English
Subjects:
Non-dystrophic myotonia
  Periodic paralysis
  Myotonia congenita
  Paramyotonia congenita
  Andersen Tawil syndrome
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Summary:•Two thirds of patients with skeletal muscle channelopathies had worsening of symptoms during pregnancy.•Some patients have worsening of symptoms following anaesthetic but none had malignant hyperthermia.•Patients do not have any difference in antenatal and delivery complications compared to the normal populations.•Neonates with sodium channel mutations require careful monitoring for complications in the post-partum period. The skeletal muscle channelopathies are a group of rare diseases and include non-dystrophic myotonia and periodic paralysis. Given their rarity, little has been published on the management of anaesthesia and pregnancy in this cohort despite being important aspects of care. We have conducted a large study of over 70 patients who underwent anaesthesia and 87 pregnancies to investigate the problems encountered following anaesthesia or during pregnancy. This was performed via patient surveys sent out to genetically confirmed channelopathy patients seen at the National Hospital for Neurology and Neurosurgery. Most significantly in our cohort, patients frequently experienced a worsening or precipitation of symptoms during pregnancy (75%) or following anaesthetic (31%). None of our patients developed malignant hyperthermia, although there are confirmed reports of this in patients with periodic paralysis and mutations in RYR1. There was a significantly higher number of miscarriages compared to the normal population. There was no significant difference in antenatal or delivery complications compared to the general population. However, three neonates did have complications, all of whom were found to carry mutations in SCN4A. This study highlights the importance of counselling patients and clinicians for the possibility of worsening symptoms during pregnancy or anaesthesia and the careful management of neonates following delivery.
ISSN:0960-8966
1873-2364
DOI:10.1016/j.nmd.2020.05.007