Neurobehavioral Changes Associated with Rheumatic Fever and Rheumatic Heart Disease: A Narrative Review

David Mcmillan; Sarangan Ketheesan; Rukshan Ahamed Mohamed Rafeek; Riya Thapa; Mohammad Raguib Munif; Adam Hamlin; Phillip Tully; Robert Norton; Andrew White; Natkunam Ketheesan

doi:10.4103/hm.HM-D-23-00069

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Neurobehavioral Changes Associated with Rheumatic Fever and Rheumatic Heart Disease: A Narrative Review

Journal article

Open access

Peer reviewed

Neurobehavioral Changes Associated with Rheumatic Fever and Rheumatic Heart Disease: A Narrative Review

David Mcmillan, Sarangan Ketheesan, Rukshan Ahamed Mohamed Rafeek, Riya Thapa, Mohammad Raguib Munif, Adam Hamlin, Phillip Tully, Robert Norton, Andrew White and Natkunam Ketheesan

Heart and Mind, Vol.8(2), pp.89-99

2024

DOI: https://doi.org/10.4103/hm.HM-D-23-00069

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Abstract

acute rheumatic fever

group A streptococcus

pediatric automimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS)

poststreptococcal immune complications

rheumatic heart disease

Sydenham's chorea

UniSC Diversity Area - Disability and Inclusion

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) are autoimmune conditions triggered by Group A Streptococcus skin or throat infections. If ARF/RHD is undetected, misdiagnosed or antibiotic treatment is not provided early, patients may develop cardiac failure, leading to premature death. Although it is an easily preventable disease, ARF/RHD remains the most significant cause of heart disease-associated deaths in people under 25 years old, both in low- and middle-income countries and among First Nations in high-income countries. Up to 30% of the patients with ARF/RHD present with a neurobehavioral condition - Sydenham's chorea (SC). The clinical course of SC is mostly self-limiting and is characterized by the onset of involuntary choreiform movements and neuropsychiatric features such as obsessive-compulsive disorder, tics, depression and anxiety, psychosis, and attention-deficit hyperactivity disorder. While the precise mechanism as to why only a proportion of patients with ARF/RHD develop SC remains unknown, an impaired blood-brain barrier is considered to play a central role in its development. The most well-characterized neurobehavioral outcome is stroke which may occur in isolation or as part of systemic thromboembolism. Both infective endocarditis and mitral valve disease with or without aortic valve disease increase the embolic and ischemic stroke risk. ARF/RHD is known to significantly impact the quality of life with neuropsychiatric consequences. Another neurobehavioral syndrome which occurs in the absence of ARF/RHD is "pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections" (PANDAS). PANDAS has been categorized as a subset of pediatric acute-onset neuropsychiatric syndromes. However, establishing a diagnosis of PANDAS has been challenging. In this review, we discuss the current status of our understanding regarding the different manifestations of poststreptococcal neurobehavioral changes. Particular attention is given to ARF/RHD-associated SC, and we highlight the areas for further research to understand the association between poststreptococcal sequelae and neurobehavioral abnormalities.

Details

Title: Neurobehavioral Changes Associated with Rheumatic Fever and Rheumatic Heart Disease: A Narrative Review
Authors: David Mcmillan - University of the Sunshine Coast, Queensland, Centre for Bioinnovation
Sarangan Ketheesan - Royal Brisbane and Women's Hospital
Rukshan Ahamed Mohamed Rafeek - University of New England
Riya Thapa - University of New England
Mohammad Raguib Munif - University of New England
Adam Hamlin - University of New England
Phillip Tully - University of New England
Robert Norton - University of New England
Andrew White - Townsville Hospital
Natkunam Ketheesan (Corresponding Author) - University of the Sunshine Coast, Queensland, Centre for Bioinnovation
Publication details: Heart and Mind, Vol.8(2), pp.89-99
Publisher: Wolters Kluwer - Medknow Publications and Media Pvt. Ltd.
Date published: 2024
DOI: 10.4103/hm.HM-D-23-00069
ISSN: 2468-6484; 2468-6476
Copyright note: This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
Data Availability: All data generated or analyzed during this study are included in this published article
Grants: Preclinical evaluation of a streptococcal peptide based specific diagnostic for rheumatic fever and the identification of proteins responsible for the pathogenesis of rheumatic heart disease, 2010336, National Health and Medical Research Council (Australia, Canberra) - NHMRC
Organisation Unit: School of Science, Technology and Engineering; Centre for Bioinnovation
Language: English
Record Identifier: 991016298702621
Output Type: Journal article

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Collaboration types: Domestic collaboration
Web Of Science research areas: Cardiac & Cardiovascular Systems; Psychiatry

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