Diagnositc Criteria Associated with a Cohort of Children Identified with Symptoms of Postinfectious Autoimmune Encephalopathy (PAE)

Abstract

Postinfectious Autoimmune Encephalopathy (PAE) is well defined in the literature; however, a provider may be unaware of its existence or have a difficult time distinguishing the disorder from other neuropsychiatric conditions, such as OCD and anxiety (Leon, et. al, 2018). Availability of a valid and reliable screening tool would contribute to improved accuracy in the secondary prevention of PAE. (Helm & Blackwood, 2015). The primary purpose of this project is to determine the frequency that patients clinically identified as having PAE present with diagnostic criteria described in the literature to begin the first step developing such a tool.

PAE is a condition in which an infection triggers an autoimmune reaction that targets the nervous system, leading to changes in neurologic function, mood, and behavior. PAE includes disorders such as PANDAS, PANS, CANS, PiTANDS, and Sydenham Chorea (Pruss, 2017), and has been recognized as a diagnostic entity since 1995 (Swedo et al., 1998). A strategic search was done at a university library utilizing web-based search engines. Articles retained suggest that the criteria for diagnosis of PAE are well defined and utilized as a standard for research regarding this disorder. However, a patient referral from primary care sources can be unpredictable (Helm & Blackwood, 2015).

A non-experimental chart review was done applying investigator-designed extraction questions to a database consisting of patient information specifically seen for evaluation of PAE. The list of extraction questions is computer-based and consists of “yes” or “no” questions targeting primary, secondary and tertiary symptoms identified through a literature review. Descriptive statistics, Pearson’s chi-square testing, and cluster analysis were performed to determine symptoms associated with PAE.

One hundred and three patient records entered into a REDCap CPAE Registry were reviewed using the extraction questions. Compulsive behavior was the most frequent presentation. Urinary symptoms, dysgraphia, and emotional lability displayed differences based on PAE Diagnosis (CPAE 1= meets diagnosed, CPAE 2= meets diagnosis by major or minor criteria, and CPAE 3= does not meet diagnosis). The project concluded that further investigation is necessary to determine the symptoms associated with PAE.