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Role of Child Neurologists In Evaluating and Treating Children With Autonomic Dysregulation Including Pots and Neuropathic Pain

Administrator: Nancy Kuntz

Small fiber peripheral neuropathy and neuropathic pain in Children
Nancy Kuntz

Studies of children and adolescents with debilitating, unexplained widespread pain syndromes referred to tertiary centers for evaluation have demonstrated a high proportion with abnormalities noted on simple non-invasive autonomic testing (heart rate response to deep breathing, Valsalva maneuver, head up tilt and quantitative sudomotor axon reflex testing) as well as more invasive evaluation of small fiber dysfunction (such as sural nerve biopsies and intraepidermal nerve fiber density on skin punch biopsies). (Oaklander A and Klein MM, Pediatr 2013).  Both genetic and acquired toxic/metabolic causes of small fiber dysfunction can lead to autonomic dysfunction and neuropathic pain.  Quality of life studies in long term survivors of childhood cancer have been demonstrated to have pain and autonomic dysfunction likely secondary to the impact of chemotherapeutic agents on their peripheral nerves.  This talk will cover the relationship between pathophysiology of autonomic dysfunction and pain with small fiber peripheral neuropathies both genetic and acquired.  Clinical presentation, testing and treatment will be addressed.

 

Orthostatic intolerance in children--recognition, testing, treatment and comorbidities
Kelsey Klaas 

Postural dizziness, nausea, tachycardia, chest pain, shortness of breath, abdominal pain and anxiety can all be manifestations of Postural Orthostatic Tachycardia Syndrome (POTS).  Studies have shown that the heart rate increment to assuming upright posture in typically developing children and adolescents is higher than previously estimated and has led to requiring a greater than 40 bpm increment to be considered elevated (Singer W et al, J Pediatr 2012). Significant overlap exists between adolescents with orthostatic intolerance and those with gastrointestinal dysmotility/slowing causing nausea, early satiety and abdominal pain.  Mast cell deregulation and hyperextensible joints also appear to be comorbid conditions with autonomic dysregulation.  This talk will focus on recognizing the presentation, evaluating the symptoms and recognizing comorbid conditions.  Increased incidence of orthostatic intolerance/POTS has been recognized post COVID-19 pandemic.  Brain "fog" associated with POTS may reflect failure of cerebral autoregulation in adolescent POTS. 

 

Recognition and evaluation of autonomic dysfunction in children and adolescents
William Whitehouse

At different ages, children and adolescents are frequently unable or unwilling to provide a cogent description of their clinical symptoms.  In the youngest children, the barrier is frequently limitations in expressive language. In school aged children, their need to not be different or embarrassed creates a barrier to their self-reporting.  Adolescents can sometimes be self-absorbed reporting all of the innate sensations occurring in their bodies making it difficult to know whether the reported symptoms are normal physiology or alarming. It is in this context that recognition of autonomic symptoms and laboratory testing of autonomic function becomes important. From Breath-holding spells in infants and toddlers to orthostatic intolerance in adolescents, autonomic dysfunction presents with a broad phenotype and differential diagnosis.  This presentation will review clinical signs and symptoms from bedside examination that assess autonomic status.  Non-invasive methods of autonomic testing will be reviewed.