Rodrigo Rocamora1, Juan L Becerra2, Pilar Fossas3, María Gomez4, Rosa M Vivanco-Hidalgo5, José A Mauri6, Albert Molins7. 1. Epilepsy Monitoring Unit, Department of Neurology, Hospital del Mar, Barcelona, Spain. Electronic address: rrocamora@parcdesalutmar.cat. 2. Epilepsy Monitoring Unit, Department of Neurology Hospital Germans Trias i Pujol, Barcelona, Spain. 3. Department of Neurology, Hospital de Mataró, Barcelona, Spain. 4. Department of Neurology, Hospital San Pedro, Logroño, Spain. 5. Epilepsy Monitoring Unit, Department of Neurology, Hospital del Mar, Barcelona, Spain. 6. Department of Neurology, Hospital Clínico Universitario, Zaragoza, Spain. 7. Department of Neurology, Hospital Dr. Josep Trueta, Girona, Spain.
Abstract
PURPOSE: Ictal piloerection is an infrequent seizure semiology that is commonly overlooked as an ictal epileptic manifestation. Piloerection is considered to be principally caused by temporal lobe activity although frontal and hypothalamic seizure origins have been reported. The described etiology has shown a wide variety of structural causes such as mesial temporal sclerosis, tumors, posttraumatic, cavernomas and cryptogenic epilepsies. METHODS: We retrospectively reviewed the incidence of ictal piloerection in the clinical records of patients who underwent video-EEG monitoring (VEEGM) between 2007 and 2013 in a multicenter cooperative study. All patients presented refractory epilepsies and were evaluated with a protocol that included brain MRI, neuropsychology and VEEGM. RESULTS: A total of 766 patients were evaluated in four tertiary centers in Spain. Five patients showed piloerection as principal seizure semiology (prevalence 0.65%). The mean age at seizure onset was 39.6 years and the average epilepsy duration was 5.2 years (range 2-14) before diagnosis. Four patients were additionally examined with FDG-PET and/or SPECT-SISCOM. All presented temporal lobe epilepsy (TLE), three right-sided and two left-sided. A typical unilateral hippocampal sclerosis was described in 3 cases. The etiology detected in all cases was limbic encephalitis. Three had LGI1, one anti-Hu, and another Ma2 antibodies. CONCLUSION: Our series describes a so far not well-recognized autoimmune association of pilomotor seizures to limbic encephalitis. This etiology should be ruled out through a comprehensive diagnostic work-up even in cases of long-lasting TLE with typical hippocampal atrophy on MRI.
PURPOSE: Ictal piloerection is an infrequent seizure semiology that is commonly overlooked as an ictal epileptic manifestation. Piloerection is considered to be principally caused by temporal lobe activity although frontal and hypothalamic seizure origins have been reported. The described etiology has shown a wide variety of structural causes such as mesial temporal sclerosis, tumors, posttraumatic, cavernomas and cryptogenic epilepsies. METHODS: We retrospectively reviewed the incidence of ictal piloerection in the clinical records of patients who underwent video-EEG monitoring (VEEGM) between 2007 and 2013 in a multicenter cooperative study. All patients presented refractory epilepsies and were evaluated with a protocol that included brain MRI, neuropsychology and VEEGM. RESULTS: A total of 766 patients were evaluated in four tertiary centers in Spain. Five patients showed piloerection as principal seizure semiology (prevalence 0.65%). The mean age at seizure onset was 39.6 years and the average epilepsy duration was 5.2 years (range 2-14) before diagnosis. Four patients were additionally examined with FDG-PET and/or SPECT-SISCOM. All presented temporal lobe epilepsy (TLE), three right-sided and two left-sided. A typical unilateral hippocampal sclerosis was described in 3 cases. The etiology detected in all cases was limbic encephalitis. Three had LGI1, one anti-Hu, and another Ma2 antibodies. CONCLUSION: Our series describes a so far not well-recognized autoimmune association of pilomotor seizures to limbic encephalitis. This etiology should be ruled out through a comprehensive diagnostic work-up even in cases of long-lasting TLE with typical hippocampal atrophy on MRI.
Authors: Christoph Baumgartner; Johannes Koren; Martha Britto-Arias; Simone Schmidt; Susanne Pirker Journal: Clin Auton Res Date: 2019-02-25 Impact factor: 4.435
Authors: Sergio L Schmidt; Juliana J Schmidt; Julio C Tolentino; Carlos G Ferreira; Sergio A de Almeida; Regina P Alvarenga; Eunice N Simoes; Guilherme J Schmidt; Nathalie H S Canedo; Leila Chimelli Journal: J Med Case Rep Date: 2016-07-20