Dalma Tényi1, Beáta Bóné1, Réka Horváth1, Sámuel Komoly1, Zsolt Illés2, Christoph P Beier3, Anna Kelemen4, Norbert Kovács5, Gergely Darnai1, József Janszky6. 1. Department of Neurology, University of Pécs, H-7623, Rét u. 2., Pécs, Hungary. 2. Department of Neurology, University of Pécs, H-7623, Rét u. 2., Pécs, Hungary; Department of Neurology, Odense University Hospital, Odense, and Department for Clinical Research, University of Southern Denmark, Odense C, DK-5000, Winsløwparken 19, Denmark. 3. Department of Neurology, Odense University Hospital, Odense, and Department for Clinical Research, University of Southern Denmark, Odense C, DK-5000, Winsløwparken 19, Denmark. 4. National Institute of Clinical Neurosciences, H-1145, Amerikai út 57, Budapest, Hungary. 5. Department of Neurology, University of Pécs, H-7623, Rét u. 2., Pécs, Hungary; PTE-MTA Clinical Neuroscience MR Research Group, H-7623, Rét u. 2, Hungary. 6. Department of Neurology, University of Pécs, H-7623, Rét u. 2., Pécs, Hungary; PTE-MTA Clinical Neuroscience MR Research Group, H-7623, Rét u. 2, Hungary. Electronic address: janszky.jozsef@pte.hu.
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 Pécs, 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 < 0.001). The mean age at onset of epilepsy was 39.5 ± 20.7 years (median: 38, IQR:24-57). The seizure onset zone was temporal (p < 0.001), and was lateralized to the ipsilateral hemisphere in unilateral localization (p = 0.001). The seizure was accompanied by cold shiver in 53%, and by other autonomic symptoms in 47% of cases. In 53% of patients, IP never progressed into complex partial or generalized tonic-clonic seizure; 16% of the patients reported occasional, and 31% regular generalization. Seizure frequency was higher among females (median:25/day, IQR:3-60) than among males (median:3/day, IQR:1-11) (p = 0.017). The two most common underlying pathologies were limbic encephalitis (23%) and astrocytoma (23%, among them 64% WHO III-IV astrocytoma). CONCLUSION: IP was particularly associated with autoimmune encephalitis and high-grade glioma, suggesting IP's particular clinical importance in directing diagnostic work-up.
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 Pécs, 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 < 0.001). The mean age at onset of epilepsy was 39.5 ± 20.7 years (median: 38, IQR:24-57). The seizure onset zone was temporal (p < 0.001), and was lateralized to the ipsilateral hemisphere in unilateral localization (p = 0.001). The seizure was accompanied by cold shiver in 53%, and by other autonomic symptoms in 47% of cases. In 53% of patients, IP never progressed into complex partial or generalized tonic-clonic seizure; 16% of the patients reported occasional, and 31% regular generalization. Seizure frequency was higher among females (median:25/day, IQR:3-60) than among males (median:3/day, IQR:1-11) (p = 0.017). The two most common underlying pathologies were limbic encephalitis (23%) and astrocytoma (23%, among them 64% WHO III-IV astrocytoma). CONCLUSION: IP was particularly associated with autoimmune encephalitis and high-grade glioma, suggesting IP's particular clinical importance in directing diagnostic work-up.