Ictal piloerection is associated with high-grade glioma and autoimmune encephalitis—Results from a systematic review

Abstract Purpose To comprehensively analyze ictal piloerection (IP) in a large number of subjects. Methods We performed a systematic review on case report studies of patients diagnosed with IP (1929–2017) with additional cases included from the Department of Neurology of University of Pecs, the National Institute of Clinical Neurosciences, and Odense University Hospital. Each included case was characterized regarding patient history, IP seizure characteristics, diagnostic work-up, and therapy. Comparative analyses were also carried out based on sex and pathology. Results Altogether, 109 cases were included. We observed a strong male predominance (p  Conclusion IP was particularly associated with autoimmune encephalitis and high-grade glioma, suggesting IP’s particular clinical importance in directing diagnostic work-up.