Validation of the Paediatric Autoimmune Encephalitis Severity Score (PASS) in children with autoimmune encephalitis

Project LeadDr. Priyanka Madaan, Pediatric Neurology Unit, Department of Pediatrics, Post graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India

Awarded 2021

Lay Summary

Autoimmune encephalitis is a common cause of encephalitis in children. It is a condition in which the child’s own immune system acts against the brain cells, leading to dysfunction of the brain. Affected children can lose awareness of self and surroundings, and be unable to sleep or communicate. They can also have seizures or other violent involuntary movements. The condition can be very disabling and lead to a state where the child is bed-bound and dependent. Fortunately, there are drugs that when used appropriately can result in complete recovery in a significant number of children.

The measurement of severity of autoimmune encephalitis using a standard score is important to better understand the severity of the disease, to aid in decision-making regarding treatments, and to compare the experiences of various canters across the world. Although the Clinical Assessment Scale in Autoimmune Encephalitis (CASE) has been validated for rating the severity of autoimmune encephalitis in adults, it does not consider the differences between childhood and adult presentations, or the developmental context of age-appropriate skills in childhood. Hence, the development of an objective autoimmune encephalitis severity assessment tool for children is required. The Paediatric Autoimmune Encephalitis Severity Score (PASS) is a score recently developed by a consensus of experts across the world. In this study we aim to validate this scoring tool to document the severity of autoimmune encephalitis in children. This score is anticipated to guide documentation of clinical severity during the active phase of the disease. It will serve as a numerical measure of severity for systematically evaluating treatments and comparing severity and outcomes across centers. Better and uniform evaluation of disease severity will ultimately lead to improvement in the care and outcomes in children with autoimmune encephalitis.