DIAGNOSTIC DELAY OF ENCEPHALITIS IN A CHILD WITH QUADRAPLEGIC CEREBRAL PALSY: COGNITIVE BIAS IN ACTION.

Background: In children with complex backgrounds, clinicians consistently under-recognise deterioration from baseline, resulting in delays to diagnosis and management. Assessing children with disabilities and understanding when they deviate from their norm requires a thoughtful, inquisitive, and consistent approach.

In this cas,e we discuss a child who, due to a background of cerebral palsy and the resulting cognitive biases, had a delayed diagnosis of encephalitis.

Case Presentation Summary: A 17-year-old male presents to the emergency department (ED) with 5 days of high temperatures, reduced responsiveness, and ‘not himself’. There is significant parental concern that he is having new vacant episodes, is unable to hold a conversation, and is refusing fluids and medications. He has a background of quadraplegic cerebral palsy, periventricular leukomalacia, and is ex-premature (34+0 weeks). He is triaged, followed by early senior assessment by a consultant. CT head is negative.

Over the proceeding 24 hours he was assessed 8 times, by 2 consultants, 2 resident doctors and 1 registrar. Parents repeatedly escalate their concerns, but are told it is constipation, he is treated with enemas and advised to go home once bowels are open. After 24 hours without improvement, parents re-escalate their concerns once again, resulting in assessment by the most junior doctor in the department. This examination reveals the child flat in bed, unable to speak, GCS 11, large pupils (8mm), L pupil dilating to light, R pupil responding appropriately, new bilateral nystagmus, periodic absent gaze, unable to fix and follow, tender abdomen, constipation palpable, and a new erythematous macular rash.

On opening a discussion with the parents, they insist he is very far from baseline, explaining that he usually eats independently, holds conversation, self-propels in a wheelchair, and has no history of absence seizures. These concerning features of new neurology prompt a direct escalation of concerns to a senior, finally resulting in admission to the hospital under paediatric care. He is treated for viral encephalitis and remains an inpatient for 1 month. Happily, he recovers to baseline and is discharged home.

Learning Points Discussion: All 6 doctors who reviewed this child presumed his neurological state was in keeping with quadraplegic cerebral palsy. Confirmation and attribution bias puts all clinicians, regardless of seniority, at risk of dismissing symptoms in children with disabilities as ‘part of their baseline’, relying on pre-conceptions about a child and the condition which bear no reference to the actuality of the situation. Remaining cognizant of this helps to improve our clinical judgment.

Challenging seniors and escalating concerns as a junior doctor is difficult. Having an awareness of conformity bias helps us overcome this, allowing us to provide the care we wish to deliver.

In children who cannot advocate for themselves, anchoring bias is a risky pitfall. Listening to a parent/carer’s knowledge cannot be underestimated- appreciating their insight and making a conscious effort to avoid this bias may be the most important step you take in sharpening your clinical acumen.