Pediatric Neuroscience Pathways Fall 2012 - Cleveland Clinic

the scope of pediatric autonomic disorders is currently not well
recognized as most publications on various aspects of autonomic
function tend to concentrate mainly on adult disorders. in recent
years, however, investigators have begun to appreciate the value of
childhood genetic autonomic disorders as models to advance the
understanding of pathophysiologic mechanisms involved in autonomic
dysfunction. 1 it is therefore incumbent on pediatric caregivers
to be more aware of the existence of this expanding spectrum of
pediatric autonomic disorders, as early recognition is essential for
appropriate investigation and management of this group of pediatric
patients who may otherwise suffer debilitating symptoms and signs.
symptoms of autonomic dysfunction in children
Because the autonomic nervous system innervates multiple organ
systems, the clinical manifestations of autonomic disorders are
extremely varied and include abnormalities in the cardiovascular,
respiratory, gastrointestinal, ophthalmologic, neurologic, sudomotor
and urologic systems.
Pediatric Neurology
Postural Orthostatic Tachycardia Syndrome in Children – An Emerging Entity
Manikum Moodley, MD
At Cleveland Clinic, children and adolescents with postural orthostatic tachycardia syndrome (POTS) may
receive state-of-the-art care from specialists dedicated to diagnosis and treatment of syncope and autonomic
disorders. this multidisciplinary collaboration between pediatric neurology and cardiology offers the most
modern approach to this complex group of disorders.
table 1. symptoms of pediatric Pots
orthostatic symptoms
• Lightheadedness or dizziness
• Presyncope or near faint
• Blurred vision
• Generalized bodily weakness
• Shortness of breath
• Headache
• Nausea
• Excessive fatigue
sympathetic overactivity
• Palpitation
• Migraine
• Chest pain
• Diarrhea/constipation
• Vomiting
• Tremulousness
• Anxiety or panic attacks
• Bladder symptoms
• Hyperhydrosis
Postural orthostatic tachycardia syndrome in children
The list of pediatric autonomic disorders is ever-expanding, with
many presenting in early childhood as congenital/genetic disorders
and others, like POTS, presenting in the adolescent/teen age
group. POTS is a well-known entity in adult patients but less
well-known in children. Fortunately, it has been increasingly
recognized in children and adolescents as of late.
POTS in adults is defined as the development of orthostatic
symptoms associated with sustained increment of heart rate ≥ 30
beats per minute (bpm) and/or an absolute heart rate ≥ 120 bpm,
within 10 minutes of active standing or head-up tilt test. 2 in children
and adolescents, a heart rate increment of 35 or 40 bpm is
considered excessive. 3-6
symptoms of Pediatric Pots
the symptoms of orthostatic intolerance and sympathetic
overactivation in POTS are listed in Table 1. In addition, a
spectrum of somatic symptoms may occur, including fatigue,
nausea, abdominal pain, migraine, sleep disturbance, widespread
myofascial pain and cognitive dysfunction, as well as psychiatric
symptoms of depression and anxiety. the most common
adolescent presentation involves teenagers within one to three
years of their growth spurt.
diagnostic evaluation
the most important part of the evaluation of patients with suspected
Pots is a detailed history and clinical examination followed
by several diagnostic studies, as listed in Table 2.
treatment
Both nonpharmacological and pharmacological interventions are
useful in the management of Pots (table 3).
conclusion
POTS is common among adolescents, and its presence should be
recognized by family physicians, pediatricians and child neurologists
so that appropriate and timely interventions can be instituted.
Its pathophysiological basis is complex, with multiple interacting
models explaining its myriad manifestations. common mechanisms
are denervation (neuropathic POTS), hyperadrenergic states
and deconditioning. Many questions still surround pediatric POTS;
24 Pediatric NeuroscieNce Pathways | 2012 – 2013