Oliviamsutton at 17:49, 7 December 2021

2021-12-07T17:49:16Z

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	~~Provide~~ a ~~brief summary~~ of ~~this comorbidity here~~.		Congenital myasthenic syndromes (CMS) are a genotypically and phenotypically heterogeneous group of rare neuromuscular disorders that cause impaired neuromuscular transmission. Inheritance can be autosomal dominant or recessive.

	== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. --> ==		== Anesthetic implications<!-- Briefly summarize the anesthetic implications of this comorbidity. --> ==

	=== Preoperative optimization<!-- Describe how this comorbidity may influence preoperative evaluation and optimization of patients. --> ===		=== Preoperative optimization<!-- Describe how this comorbidity may influence preoperative evaluation and optimization of patients. --> ===
			Preop evaluation may involve planning with a neurologist and geneticist to determine the patient's subtype and what medications may be effective.

	=== Intraoperative management<!-- Describe how this comorbidity may influence intraoperative management. --> ===		=== Intraoperative management<!-- Describe how this comorbidity may influence intraoperative management. --> ===
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	== Pathophysiology<!-- Describe the pathophysiology of this comorbidity. Add subsections as needed. --> ==		== Pathophysiology<!-- Describe the pathophysiology of this comorbidity. Add subsections as needed. --> ==
			Caused by mutations for proteins involved in neuromuscular transmission (organization, maintenance, function or modification of motor endplate). These mutations result in either gain or loss of function causing a change in the magnitude of the response to acetylcholine. Subtypes are classified based on where the defect occurs: presynaptic, synaptic, and postsynaptic. The most common type is post-synaptic, secondary to a mutation in synaptic vesicles, acetylcholinesterase, or nicotinic acetylcholine receptors.

			These differ from [[myasthenia gravis]] and [[Lambert-Eaton syndrome\|Lambert-Eaton,]] which are mediated by autoantibodies.

	== Signs and symptoms<!-- Describe the signs and symptoms of this comorbidity. --> ==		== Signs and symptoms<!-- Describe the signs and symptoms of this comorbidity. --> ==
			Signs: ophthalmoplegia, ptosis, dysphonia, swallowing disturbance, facial paresis, muscle fatigability, recurrent apnea

			Acute respiratory failure may occur triggered by infections episodes

	== Diagnosis<!-- Describe how this comorbidity is diagnosed. --> ==		== Diagnosis<!-- Describe how this comorbidity is diagnosed. --> ==
			Tensilon test (EMG with repetitive nerve stimulation), muscle biopsy, genetic analysis may be used to confirm diagnosis.

	== Treatment<!-- Summarize the treatment of this comorbidity. Add subsections as needed. --> ==		== Treatment<!-- Summarize the treatment of this comorbidity. Add subsections as needed. --> ==

	=== Medication<!-- Describe medications used to manage this comorbidity. --> ===		=== Medication<!-- Describe medications used to manage this comorbidity. --> ===
			Administration of medication without knowing subtype of CMS is unadvised.

			Acetylcholinesterase inhibitors are most frequently used, if the subtype is appropriate. 4-diaminopyridine is an alternative, which increases amount of acetylcholine released in the synaptic cleft.

			Other medications that may be used include salbutamol, albuterol, ephedrine, fluoxetine, and other experimental choices.

			=== Nonmedical therapy<!-- Describe surgical procedures used to treat this comorbidity. --> ===
			Physiotherapy, speech and occupational therapy, invasive treatment such as feeding tubes and mechanical ventilation may be required.

	~~=== Surgery<!-- Describe surgical procedures used~~ to ~~treat this comorbidity~~. ~~--> ===~~		Unlike in Myasthenia Gravis, plasmapheresis is not effective as the pathology is not due to antibodies.

	=== Prognosis<!-- Describe the prognosis of this comorbidity --> ===		=== Prognosis<!-- Describe the prognosis of this comorbidity --> ===
			Pregnancy has been reported to exacerbate clinical symptoms.

	== Epidemiology<!-- Describe the epidemiology of this comorbidity --> ==		== Epidemiology<!-- Describe the epidemiology of this comorbidity --> ==
			Estimated 1:500,000 prevalence

	== References ==		== References ==

	[[Category:Comorbidities]]		[[Category:Comorbidities]]

Oliviamsutton: Created page with "{{Infobox comorbidity | other_names = | anesthetic_relevance = | specialty = | signs_symptoms = | diagnosis = | treatment = | image = | caption = }} Provide a brief s..."

2021-12-07T16:59:40Z

Created page with "{{Infobox comorbidity | other_names = | anesthetic_relevance = | specialty = | signs_symptoms = | diagnosis = | treatment = | image = | caption = }} Provide a brief s..."

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{{Infobox comorbidity
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| anesthetic_relevance =
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Provide a brief summary of this comorbidity here.

== Anesthetic implications ==

=== Preoperative optimization ===

=== Intraoperative management ===

=== Postoperative management ===

== Related surgical procedures ==

== Pathophysiology ==

== Signs and symptoms ==

== Diagnosis ==

== Treatment ==

=== Medication ===

=== Surgery ===

=== Prognosis ===

== Epidemiology ==

== References ==

[[Category:Comorbidities]]

Congenital myasthenic syndromes - Revision history

Oliviamsutton at 17:49, 7 December 2021

Oliviamsutton: Created page with "{{Infobox comorbidity | other_names = | anesthetic_relevance = | specialty = | signs_symptoms = | diagnosis = | treatment = | image = | caption = }} Provide a brief s..."