Sunday, December 20, 2015


Jeffrey B. Anderson, Martha Willis, Heidi Lancaster RN, , Karen Leonard RN, Cameron Thomas. The Evaluation and Management of Pediatric Syncope.  Pediatric Neurology, in press.

Syncope is a common problem in children and adolescents. It is typically caused by benign neurally mediated hypotension, but other, more concerning, etiologies of syncope must be considered. In most cases, the underlying cause of syncope in the pediatric patient can be determined by obtaining a thorough history and physical examination. Attention to the cardiac, neurological, and psychological history and examination can rule out more rare causes of loss of consciousness. Most individuals with neurally mediated hypotension can be treated with lifestyle measures including aggressive hydration, dietary salt, and an exercise program. In instances where lifestyle modification fails, medications may offer symptomatic improvement.

From the article:

Regardless of the underlying etiology, multiple studies confirm that syncope decreases one's quality of life to a degree similar to other chronic illnesses. In one study of pediatric patients with syncope, PedsQL Total Scores were lower than in patients with diabetes and similar to those in patients with asthma, end-stage renal disease, and obesity, as seen in the Figure .  Attention to and management of pediatric syncope can decrease patient and family anxiety as well as improve quality of life.

There are likely to be multiple reasons for the diminished quality of life in patients experiencing syncope. Their dizziness, light-headedness, and nausea or other presyncopal symptoms can inhibit not only their school attendance but also their ability to participate in extracurricular activities. The inability to predict when syncopal events may occur escalates anxiety and results in further isolation. Grief over the loss of the ability or perceived ability to participate in regular activities can occur and eventually lead to depression...

A standardized approach to the child or adolescent presenting with syncope is important. The American Heart Association, the American College of Cardiology, and the European Society of Cardiology have created guidelines for the evaluation of syncope. These guidelines review the current evidence on the diagnosis and management of patients presenting with syncope in an attempt to improve the quality of clinical practices and utilization of heath care resources. These guidelines recommend that the essential components of evaluation of children who present with syncope include a comprehensive medical and family history, a physical examination, and electrocardiography. Further initial diagnostic testing, including blood work and cardiac or neurological imaging tests, is not recommended...

Typically a cardiac examination and electrocardiography with normal findings and a history consistent with NMS are sufficient to rule out a serious cardiac cause...

Rarely, in cases where the mechanism of syncope is believed to be NMS but symptoms are atypical, a tilt-table test may be ordered. In the tilt-table test the child or adolescent is placed on a table and tilted to 70 degrees while being monitored by electrocardiography and an automated blood pressure monitor. This test creates an artificial orthostatic stress that often provokes a drop in heart rate and/or blood pressure, which can be suggestive of a diagnosis of NMS. Tilt-table testing results should be interpreted with caution, however, because false-positive results are common...

Once a diagnosis of NMS is made, a standardized approach to the management of this problem is employed. The first-line treatments for pediatric patients with NMS include reassurance, adequate fluid and salt intake, and regular exercise.  We recommend a goal of 100 ounces of fluid per day for our adolescent patients. Care must be taken to balance other medical problems, and if a patient has underlying cardiac disease or hypertension then increased dietary salt should be avoided. Reassurance is paramount because patients and families are often concerned that a serious or life-threatening medical problem may have caused the syncopal event.
In patients with recurrent NMS despite adequate hydration and salt intake medications may be of some benefit. Although a number of medications have been used in the management of NMS, β-blockers, fludrocortisone, and midodrine are the most common prescribed for this problem. The Prevention of Syncope Trial (POST) studied the efficacy of metoprolol compared with placebo in the management of adults with NMS and found no efficacy for β-blockers in preventing recurrent syncope. β-Blockers and fludrocortisone have both been shown to have mixed results in pediatric patients with NMS.  Midodrine has been shown to be effective in adults with severely symptomatic NMS. In patients who have failed lifestyle changes our approach has been to start with fludrocortisone in patients with refractory NMS, because of the ease of use of this medication, and then to add midodrine in cases where the fludrocortisone is ineffective. When using fludrocortisone it is important to monitor for hypokalemia, which can be seen in patients using this medication.                     


  1. Blad H, Lamberts RJ, Gert van Dijk J, Thijs RD. Tilt-induced vasovagal syncope and psychogenic pseudosyncope: Overlapping clinical entities. Neurology. 2015 Dec 8;85(23):2006-10.



    To describe the combination of tilt-induced vasovagal syncope (VVS) and psychogenic pseudosyncope (PPS) and aid its clinical recognition.


    We identified people with tilt-induced VVS/PPS from 2 tertiary syncope referral centers. For each case, 3 controls with tilt-induced VVS were selected at random from the same center. Clinical characteristics were compared between both groups adjusting for multiple comparisons.


    Of 1,164 tilt-table tests, 23 (2%) resulted in VVS/PPS; these 23 cases were compared with 69 VVS controls. VVS and PPS coincided more often than chance would predict: 2% vs 0.6%, p < 0.001. Typical VVS prodromes and triggers were reported in all people with VVS/PPS and in controls with VVS. Attack frequency was significantly higher in the VVS/PPS (2 per month, range 0.1-60) than in the VVS group (0.25 per month, range 0.02-4; p < 0.001). Delayed recovery of consciousness was more frequently reported in the VVS/PPS group (likelihood ratio [+LR] 8.14, 95% confidence interval [CI] 3.94-16.84), as well as episodes without prodromes (+LR 5.57, 95% CI 2.53-12.26), atypical triggers (+LR 5.00, 95% CI 2.04-12.24), eye closure (+LR 3.75, 95% CI 1.68-8.35), and apparent loss of consciousness >1 minute (+LR 2.86, 95% CI 1.98-4.13).


    VVS/PPS presents with a complex phenotype. High attack frequency, delayed recovery of consciousness, apparent loss of consciousness >1 minute, ictal eye closure, atypical triggers, and the absence of prodromes may serve as indicators that PPS coincides with VVS.

  2. Raj V, Rowe AA, Fleisch SB, Paranjape SY, Arain AM, Nicolson SE. Psychogenic pseudosyncope: diagnosis and management. Auton Neurosci. 2014 Sep;184:66-72.


    Psychogenic pseudosyncope (PPS) is the appearance of transient loss of consciousness (TLOC) in the absence of true loss of consciousness. Psychiatrically, most cases are classified as conversion disorder, which is hypothesized to represent the physical manifestation of internal stressors. The incidence of PPS is likely under-recognized and the disorder is under investigated in the unexplained syncope population, yet it can be diagnosed accurately with a focused history and confirmed with investigations including head-up tilt testing (HUTT), electroencephalogram (EEG; sometimes combined with video) or, in some centers, transcranial Doppler (TCD). Patients are more likely to be young females with an increased number of episodes over the past 6months. They frequently experience symptoms prior to their episodes including light-headedness, shortness of breath and tingling. Conversion disorder is associated with symptomatic chronicity, increased psychiatric and physical impairment, and diminished quality of life. Understanding the epidemiology, biological underpinnings and approach to diagnosis of PPS is important to improve the recognition of this disorder so that patients may be managed appropriately. The general treatment approach involves limiting unnecessary interventions, providing the patient with needed structure, and encouraging functionality. While there are no treatment data available for patients with PPS, studies in related conversion disorder populations support the utility of psychotherapy. Psychotropic medications should be considered in patients with comorbid psychiatric disorders.

  3. Benbadis SR, Sutton R. Psychogenic symptoms are not only for the epileptologist: All physicians be aware! Neurology. 2015 Dec 8;85(23):2000-1.

    Among all psychogenic symptoms, psychogenic nonepileptic events (PNEE) are by far the best studied. We will use the term “events” in order to avoid the confusing term “seizures” here. Within neurology, movement disorders are also relatively well-documented and studied. In cardiology, common psychogenic symptoms are noncardiac chest pain and psychogenic pseudosyncope (PPS). The pathophysiology, or rather psychopathology, is similar for psychogenic symptoms regardless of the presenting symptoms. For patients with spells or fits, whether they are labeled PPS or PNEE depends mostly on what they resemble more (shaking = seizure, limp = syncope) and to which specialist they present...

    In this issue of Neurology, Blad et al. report a sizeable group of patients with both vasovagal syncope (VVS) and PPS. The 2 coexisted more than they should by chance. Not surprisingly, the red flags that suggested a psychogenic origin were similar to those that are well established for PNEE.

    This study is an important illustration of embellishment, or exaggeration of organic symptoms. Thus, the symptoms here can be viewed as partially psychogenic. The tilt-table procedure, like any medical procedure, acts like a provocative technique both in suggestible patients, those with PPS,and in those constitutionally vulnerable to vasodepression. Embellishment is similar to subconsciously learning from VVS symptoms and signs to manifest PPS...
    The findings of Blad et al. have important practical implications. Internists and cardiologists who evaluate syncope should consider the diagnosis of PPS more often and earlier, just like most neurologists have learned to consider the diagnosis of PNEE in patients with refractory seizures. ..

    Another important lesson is that tilt-table tests would offer enhanced diagnostic capability by being performed routinely with EEG monitoring. Ictal recording easily distinguishes organic syncope (of any cause) and PPS, due to a predictable and sensitive series of changes. For routine EEG monitoring during tilt to be achieved, close cooperation between neurologist and cardiologist is necessary and is usually lacking. A potential limitation of the benefit of EEG monitoring is that EEG changes may not be present (false-negative) in incomplete or presyncope (e.g., dizziness, lightheadedness), but this is no different from ictal EEG being negative in mild or limited simple partial seizures...(continued)

  4. (continued)If a standing provocation test were performed on all patients with syncope unexplained by history, physical, and 12-lead ECG, a notable and larger proportion with PPS might be found and coincidence with VVS may be less. In parallel, most patients with PNEE do not have coexisting epilepsy.

    While psychogenic symptoms or signs exist in all specialties, PNEE are the most provable of all psychogenic symptoms, so they have been well-studied. Since the advent of EEG-video monitoring, a consistent finding at epilepsy centers is that about 30% of patients with refractory seizures actually have PNEE.

    All specialties have their equivalent psychogenic symptoms: shortness of breath and cough in pulmonary medicine, constipation and abdominal pain in gastroenterology, blindness in ophthalmology, dysphonia or globus in otolaryngology. Interestingly, pain is the least provable psychogenic symptom, so much so that the diagnosis of psychogenic pain is no longer accepted. Chronic pain conditions, such as fibromyalgia, continue to be controversial as to whether they are organic or psychogenic. Common features of psychogenic symptoms exist, whichever the specialty. They are first diagnosed as, and may coexist with, the mimicked organic illness. In general, most patients with psychogenic symptoms are not consciously faking (factitious, malingering) but rather fall under the unconscious category (formerly somatoform disorders, now somatic symptom disorders). It then follows that if 30% of refractory seizures are psychogenic, it is possible that 30% of refractory unexplained syncope is psychogenic, or partly embellished by a psychogenic component.

    The last and unfortunate feature of psychogenic symptoms across all specialties is that it is difficult to find good treatment. At least for PNEE, there is recent evidence that selective serotonin reuptake inhibitors and cognitive-behavioral therapy can be effective. This may not be the case for PPS, but successful treatment depends on rarely encountered mental health professionals, most of whom pay little attention and show little interest in this category of disorders.