Paroxysmal Sympathetic Hyperactivity after Acquired Brain Injury: Consensus on Conceptual Definition, Nomenclature, and Diagnostic Criteria

Abstract

A syndrome of paroxysmal, episodic sympathetic hyperactivity after acquired brain injury has been recognized for almost 60 years. This project sought to simplify the confused nomenclature for the condition (>31 eponyms) and simplify the nine overlapping sets of diagnostic criteria. A consensus-developed questionnaire based on a systematic review of the literature was circulated to a widely representative, international expert group utilizing a Delphi approach. Diagnostic criteria were dropped if group consensus failed to agree on their relative importance, with a goal of reaching a Cronbach α of 0.8 (suitable for research purposes). The resulting criteria were combined into an assessment measure for clinical and research settings. The consensus group recommend that the term "paroxysmal sympathetic hyperactivity" replace previous terms to describe the "syndrome, recognised in a subgroup of survivors of severe acquired brain injury, of simultaneous, paroxysmal transient increases in sympathetic [elevated heart rate, blood pressure, respiratory rate, temperature, sweating] and motor [posturing] activity." An 11 point probabilistic diagnostic scale was developed with reference to published criteria, yielding an acceptable Cronbach α of 0.8. These 11 items were proceduralized and combined with a symptom severity index to produce a diagnostic tool for use with adults (the paroxysmal sympathetic hyperactivity assessment measure [PSH-AM]). Development of a pediatric version of the scale and further research into the validity of the PSH-AM is recommended. The consensus position builds on previous literature to establish diagnostic definitions and criteria, an important move to standardize research and management of this condition.

Diagnosis and Management of Patients with Paroxysmal Sympathetic Hyperactivity following Acute Brain Injuries Using a Consensus-Based Diagnostic Tool: A Single Institutional Case Series

“…PSH can be clinical burden with therapeutic potential such as morbidity, weight loss, dehydration, infections, unnecessary tests and interventions, interfering with rehabilitation, and longer length of stay in ICU (Perkes et al 2010;Fernandez-Ortega et al 2012;Baguley et al 2014;Meyer 2014;Takahashi et al 2015). Although the prevalence of PSH is not uncommon in patients with acute brain damage, PSH remains an under-recognized condition with a diagnostic pitfall in the ICU settings in particular (Hughes and Rabinstein 2014).…”

“…Among the 394 survivors of them, 6 patients (1.5%) were diagnosed with PSH by using PSH-AM (Baguley et al 2014) (Fig. 1 and Table 2).…”

“…have been proposed, leading to diagnostic confusion in this disorder (Perkes et al 2010;Baguley et al 2014). Even conceptual definition has not been established and standardized clinical diagnostic criteria have never been developed.…”

“…In 2014, Baguley et al proposed that the term "paroxysmal sympathetic hyperactivity" (PSH) be used to identify the condition as defined ''a syndrome, recognized in a subgroup of survivors of severe acquired brain injury, of simultaneous, paroxysmal transient increases in sympathetic (elevated heart rate, blood pressure, respiratory rate, temperature, sweating) and motor (posturing) activity" (Baguley et al 2014). They also developed a diagnostic tool named "PSH-Assessment Measure (PSH-AM)" in order to establish standardized diagnostic criteria and to help to guide the clinical management of PSH (Baguley et al 2014) (Table 1).…”

“…They also developed a diagnostic tool named "PSH-Assessment Measure (PSH-AM)" in order to establish standardized diagnostic criteria and to help to guide the clinical management of PSH (Baguley et al 2014) (Table 1). PSH-AM is consisted of two components: 1) a diagnosis likelihood tool derived from 11 clinical characteristics of PSH, and 2) a clinical feature scale assigned to the severity of each sympathetic hyperactivity.…”