What is teenage dysautonomia?

Teenage Dysautonomia: Understanding and Management

Teenage dysautonomia is a condition characterized by severe imbalance in the autonomic nervous system, which controls involuntary bodily functions including heart rate, blood pressure, temperature regulation, digestion, and other essential processes. 1

Definition and Types

• Dysautonomia encompasses a broad spectrum of disorders with abnormal function of the autonomic nervous system, which can manifest in various forms with fundamentally different underlying mechanisms 1, 2
• The autonomic nervous system consists of the parasympathetic and sympathetic nervous systems that integrate control of involuntary functions 1
• In teenagers, the most common presentation is Postural Orthostatic Tachycardia Syndrome (POTS), defined as an increase in heart rate of ≥40 beats/min in adolescents within 10 minutes of upright posture without orthostatic hypotension 1, 3

Clinical Manifestations in Teenagers

• The most common adolescent presentation involves teenagers within 1-3 years of their growth spurt who, after a period of inactivity from illness or injury, cannot return to normal activity levels because of symptoms induced by upright posture 4
• Orthostatic intolerance: Symptoms including dizziness, lightheadedness, nausea, dyspnea, and vision changes occurring specifically when assuming or maintaining upright position 1, 5
• Gastrointestinal symptoms: Nausea, abdominal pain, vomiting, diarrhea, bloating, and severe constipation 2
• Other common symptoms include headache, fatigue, palpitations, pre-syncope, and weakness 6, 3
• Symptoms can significantly impact quality of life and academic performance 3, 7

Diagnostic Approach

• Diagnosis requires documentation of excessive heart rate increase upon standing (≥40 bpm for adolescents) or heart rate ≥120 bpm within 10 minutes of standing 6
• The Schellong test (measuring heart rate and blood pressure when changing from supine to standing position) is useful for making the diagnosis 3
• Monitoring of heart rate and blood pressure with changes in position and respiration can demonstrate sympathetic withdrawal and excessive cholinergic aspects 8
• Differential diagnosis should rule out other causes of orthostatic intolerance including:
  • Neurogenic orthostatic hypotension 1
  • Reflex syncope 6
  • Chronic fatigue syndrome 6

Associated Conditions

• Dysautonomia in teenagers may be associated with Ehlers-Danlos syndrome, characterized by joint hypermobility and tissue fragility 7
• Following viral illnesses or periods of prolonged inactivity, teenagers may develop POTS symptoms 4
• Chronic fatigue syndrome represents a form of dysautonomia and has been renamed as a systemic disease of exercise intolerance 6

Management Approaches

• Nonpharmacologic treatment is the first-line approach and includes increasing aerobic exercise, lower-extremity strengthening, increasing fluid/salt intake, psychophysiologic training for management of pain/anxiety, and family education. 4
• Preventive measures include:
  • Postural care and good hydration 6
  • Higher salt intake 6, 4
  • Use of compression stockings and abdominal straps 6
  • Portioned meals 6
  • Supervised physical activity, mainly sitting, lying down or exercising in water 6
• Pharmacologic treatment should be considered on a case-by-case basis and may include:
  • Beta-blocking agents to blunt orthostatic increases in heart rate 4
  • Alpha-adrenergic agents to increase peripheral vascular resistance 4
  • Mineralocorticoid agents like fludrocortisone to increase blood volume 6, 4
  • Serotonin reuptake inhibitors for associated anxiety/depression 4

Prognosis and Long-term Considerations

• Many teenagers with POTS show improvement over time with appropriate management 4
• Early diagnosis and intervention are crucial to prevent development of chronic syndromes 8
• Regular monitoring is essential as symptoms may fluctuate and treatment may need adjustment 6
• The condition can be mistaken for malingering, depression, or anxiety disorders due to its complex presentation 7

Clinical Pitfalls to Avoid

• Delayed diagnosis is common due to the nonspecific nature of symptoms and lack of awareness among healthcare providers 8
• Inadequate monitoring of blood pressure when initiating treatment is a common pitfall 1
• Misattribution of symptoms to psychological causes rather than recognizing the underlying autonomic dysfunction 7
• Failure to consider comorbid conditions like Ehlers-Danlos syndrome that may require additional management 7