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Normally weak, weaker during pregnancy: An interesting case of Congenital Myasthenic Syndrome
Abstract Number: SA-71
Abstract Type: Case Report/Case Series
Introduction: Congenital Myasthenic Syndromes(CMS)a heterogenous group of genetic diseases of AR inheritance, characterized by a dysfunction of neuromuscular transmission. Prevalence is estimated at 1 in 500,000.1
Case report: A 24 years old primiparous lady presented with a history of CMS. She was diagnosed at the age of 11 years and was wheel chair bound since. She was started on fluoxetine at the age of 15, which caused depression and was changed to Quinidine which improved her symptoms. During her pregnancy her weakness got worse and she started using the wheel chair more often due to fatiguability. She had bilateral ptosis, complex ophthalmoplegia, proximal muscle weakness. She was seen in the high risk anaesthetic clinic at 36 weeks and a multidisciplinary plan was made by anaesthetists, neurologists and obstetricians.
The anaesthetic plan was to continue Quinindine peripartum, provide early epidural in labour, regional for Caesarean section and in an unlikely event of Caesarean section needing GA to avoid or restrict the use of muscle relaxants.
Discussion: CMS is classified as presynaptic (defects in Acetylcholine synthesis or release), synaptic (anomaly of the cholinesterase collagen tail),postsynaptic defects (decreased expression of acetylcholine receptors, fast and slow channel CMS).2
Diagnosis1- decreased response of the compound muscle action potential (CMAP, absence of anti-acetylcholine receptor and anti-MuSK antibodies in the serum, lack of improvement with immunosuppressive therapy and absence of pathology in a skeletal muscle biopsy.
Treatment is based on the type of CMS. Most benefit from Acetylcholinestrase inhibitors like pyridostigmine, 3,4-diaminopyridine increases the release of ACh. Ephedrine & Albuterol for refractory cases. Quinidine & fluoxetine for slow channel CMS. Our patient was diagnosed with slow channel type of CMS.
Our patient's symptoms worsened during pregnancy and she was wheelchair bound. Prevention of secondary complications and monitoring side effects of drugs used is important.
Neuraxial block is preferred to avoid general anaesthesia (NM blocking drugs and anticholinesterses). Down regulation of acetylcholine receptors makes them resistant to depolarising muscle relaxants and sensitive to non-depolarising relaxants.
Labour and delivery can cause fatiguablity. To avoid stress epidural was sited early in labour. Total labour time was kept short by augmentation and ventouse delivery.
Summary: A proper balance should be achieved between managing pregnancy, safety of the mother and the foetus from the drugs used in its management. Multi-disciplinary clinics are essential in the managing complex conditions like CMS.
1.Congenital myasthenic syndromes. Daniel Hantai et al. Current Opinion in Neurology 2004, 17:539–551
2.Obstetric and Anesthetic Management of Severe Congenital Myasthenia Syn-drome.Terblanche et al Anesthesia & Analgesia: October 2008; volume 107;issue 4: 1313-1315.