Narcolepsy and pediatric acute-onset neuropsychiatric syndrome: A case report that suggests a putative link between the two disorders

SUMMARY

“Narcolepsy and Pediatric Acute-Onset Neuropsychiatric Syndrome: A Case Report That Suggests a Putative Link Between the Two Disorders” explores a possible shared pathophysiological link between Narcolepsy Type 1 (NT1) and Pediatric Acute-Onset Neuropsychiatric Syndrome (PANS) through the case of a 13-year-old boy diagnosed with both conditions. It suggests that autoimmune neuroinflammation may affect both the orexinergic and dopaminergic systems, placing NT1 and PANS on a spectrum of related immune-mediated brain disorders.

Initial symptoms included sudden vocal tics (barking cough, cries), OCD-like symptoms, behavioral regression, school decline, anxiety, sadness, and sleep disturbances. Later symptoms included confusion, spontaneous falls, daytime sleepiness, muscle tone loss (cataplexy-like episodes), and ADHD-like attention difficulties.

Clinical findings indicated the following: high anti-streptolysin O (ASLO) titer, suggesting prior streptococcal infection; Normal MRI/EEG, but sleep study confirmed NT1 diagnosis; negative for HLA DQB1*06:02, rare in NT1 but documented; and severe tics and OCD symptoms confirmed through validated psychiatric scales.

Patient showed no clinical improvement with steroid immunotherapy but showed dramatic improvement in sleep quality when prescribed medications for narcolepsy, vocal tics and OCD symptoms, and anxiety and experienced full remission of cataplexy and improved daily functioning.

Authors propose NT1 and PANS may share autoimmune triggers (e.g., post-streptococcal) and reflect different manifestations of a shared immune-mediated pathogenesis: NT1: Driven primarily by orexin loss → sleep/wake and cataplexy symptoms, and PANS: Driven primarily by dopamine imbalance → tics, OCD, anxiety.

Both may involve overlapping dysfunction in hypothalamus (orexin) and basal ganglia (dopamine), possibly triggered by post-infectious immune response (e.g., to streptococcus). The concept suggests NT1, and PANS might not be distinct but rather part of a clinical and immunological continuum.

LINK TO PAPER: https://doi.org/10.1016/j.sleep.2024.06.025