Dysautonomia, uncertain aetiology

Gabrielle Morris

29F with cerebral palsy for revision of baclofen pump

Background:

  • Cerebral palsy
    • Non-verbal
    • Mobilises with electric wheelchair, requires a carer to operate
    • Severe spasticity and contractures involving all four limbs
    • Significant scoliosis and restrictive lung disease
    • Gastrostomy tube for feeding
    • Central sleep disordered breathing?
      • Abnormal breathing pattern – crescendo/decrescendo pattern
      • Assumed to have significant restrictive lung disease. No formal spirometry
      • Recent overnight oximetry showed SaO2 maintained >90%

Issues:

  • >1 year of episodic flushing, diaphoresis, tachycardia, tachypnoea 
    • Autonomic dysfunction?
    • Variable severity and duration of symptoms
    • Baseline tachycardia 110bpm (previous HR 100 so may be chronic) 
  • Differentials include: 
    • Central dysautonomia – potential syrinx development  given cerebral pathophysiology
    • Hyperthyroidism 
    • Phaeochromotcytoma
    • Baclofen withdrawal due to malfunctioning pump
    • Pain and Anxiety component?
    • Atropine – PO secretions, episodes don’t appear to correlate with dose
    • Subclinical seizure activity

Discussion:

Perioperative Optimisation

  • Exclude hyperthyroidism and pheochromocytoma preoperatively
  • Co-ordinating investigations while under GA 
    • MRI – can be performed safely with baclofen pump?
    • Discussed with rehabilitation physician  – all baclofen pumps are MRI compatible, however, the magnetic field and associated increased temperature can cause the pump to malfunction. See attached article
  • Consider change atropine to glycopyrrolate

Plan:

  • Pathology testing for TFT’s, plasma, and urine catecholamines
  • Consider MRI under GA 
  • Discuss with treating teams – can we perform any further investigations while under GA/inpatient