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. Author manuscript; available in PMC: 2021 Apr 2.
Published in final edited form as: Mol Genet Metab. 2017 Aug 20;122(1-2):18–32. doi: 10.1016/j.ymgme.2017.08.006

Table 10.

Autonomic dysfunction [106,107,110].

System Examples of dysfunction Potential evaluation and management
Genital-urinary system
  • Urinary retention

  • Urinary incontinence

  • Nocturia

  • Evaluation by urology

  • Management of urinary retention by bladder training, catheterization, and/or medications

  • Routine assessment for urinary tract infections

Gastrointestinal system
  • Feeding difficulties

  • Dysphagia

  • Esophageal dys-motility

  • Delayed gastric emptying

  • Recurrent abdominal pain

  • Intestinal dys-motility

  • Incontinence or constipation

  • Speech/occupational therapy evaluation of feeding

  • Assessment of nutritional status

  • Assessment for aspiration risk

  • Dietary management: optimize hydration and dietary fiber for constipation

  • Medical management (e.g. stool softeners and laxatives) for constipation

Cardiac and vascular systems
  • Arrhythmias

  • Tachycardia

  • Hypertension

  • Postural hypotension

  • Acrocyanosis

  • Evaluation by cardiology

  • Optimization of environmental factors for acrocyanosis

  • Optimization of hydration and salt intake for postural hypotension

Sudomotor function
  • Temperature regulation (Hyperthermia or Hypothermia)

  • Flushing

  • Sweating issues

  • Optimization of environmental factors

Ophthalmology
  • Alacrima

  • Pupillary changes

  • Ptosis

  • Evaluation by ophthalmology

  • Eye lubrication or eye patching at night to prevent cornea abrasions

Pulmonary
  • Apneas

  • Disordered breathing

  • Respiratory insufficiency

  • Evaluation by pulmonology

  • Consider sleep study for diagnosis

Neurology
  • Pain

  • Sleep cycle dys-regulation

  • Spasticity

  • Dystonia

  • Seizures

  • Irritability

  • Pain management medications

  • Sleep management interventions