Myotonic Dystrophy

 

Issues:

Airway

·         Bulbar dysfunction

·         Delayed gastric motility

-> Secure airway

-> Awake extubation

Breathing

·         Restrictive lung disease (weakness)

·         Sleep apnoea (central and peripheral)

-> Beware T2RF

-> Beware opioids

-> Low threshold for HDU/ICU

Circulation

·         Conduction disease

·         Cardiomyopathy

Disability/neuro

·         Propensity for myotonic crisis

o  Phys: hypothermia/shivering, BP cuff, tourniquet

o  Pharm: sux, neo, not sure about volatiles

o  Surg: diathermy

o  Rx: IM lignocaine (NOT relaxant)

·         Sensitivity to CNS depressants

o  ↓MAC

o  ↓Propofol Cp50

Associated diseases

·         T2DM

·         Primary hypogonadism

·         Hypothyroidism

·         Adrenal insufficiency

·         Hypogammaglobulinaemia

·         Cancer

 

 

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