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An updated review of PANDAS/PANS disease also known as idiopathic autoimmune encephalitis: What the allergist should know

Hannah Hardin B.S., Wenhai Shao Ph.D., and Jonathan A. Bernstein M.D. published “An updated review of PANDAS/PANS disease also known as idiopathic autoimmune encephalitis: What the allergist should know”, in Annals of Allergy, Asthma & Immunology. 2023 Aug; ISSN 1081-1206.

Background
Pediatric Acute-onset Neuropsychiatric Syndrome (PANS), further subcategorized as Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus (PANDAS), is a form of Idiopathic Autoimmune Encephalitis (IAE). Post-streptococcal autoimmunity seen in IAE manifests as various neuropsychiatric symptoms such as obsessive rituals, tics, anxiety, depression, and many others. IAE has clinically heterogeneous phenotypes that make accurate diagnosing difficult, though diagnostic testing such as the Cunningham Panel increases the likelihood of finding effective treatments. Current treatments recommended include psychiatric medication, behavioral intervention, antibiotics, anti-inflammatory therapy, and immunomodulating therapy.

Objective
To provide an updated review on the diagnosis, management, and treatment of Pediatric Autoimmune Neuropsychiatric Disorder Associated with Streptococcus (PANDAS) and Pediatric Autoimmune Neuropsychiatric Syndrome (PANS), also referred to as idiopathic autoimmune encephalitis (IAE).

Results
Information from 47 sources were used to outline current knowledge of IAE pathophysiology, clinical manifestations, and epidemiology, as well as diagnostic recommendations and current treatment guidelines. Gaps in knowledge as well as current controversy was also outlined to provide a thorough background of this condition and future needs for IAE research.

RELATED READING

The PANDAS/PANS disorders. Is it time for more allergist-immunologists to get involved?
Author: Bellanti, Joseph A.
Source: Allergy and Asthma Proceedings, Volume 44, Number 5, 1 September 2023, pp. 296-305(10)

PANDAS and its later iterative form, PANS, continue to challenge clinicians, patients, and their families. Although the precise reason why these disorders develop remains unknown, both are considered to have an autoimmune basis related to the production of antibodies directed at antigens of the putative causative infectious disease agents that are cross-reactive with antigenic epitopes on selected brain nuclei, which lead to the neuroinflammatory sequelae responsible for the neuropsychiatric symptoms of these conditions, a phenomenon referred to as molecular mimicry.