title: Medial temporal lobe epilepsy associated with hippocampal sclerosis is a distinctive syndrome.
abstract: Epileptic syndromes are distinctive disorders with specific features, which when taken together, permit a specific diagnosis. There is actually a debate on that medial temporal lobe epilepsy with hippocampal sclerosis is an epileptic syndrome. To address this issue, we searched for discriminative semiological features between temporal lobe epilepsy patients with hippocampal sclerosis (TLE-HS patients or group 1), TLE patients with medial structural lesion other than hippocampal sclerosis or in MRI-negative cases with medial onset on further investigations (group 2) and lateral TLE patients (LTLE or group 3). We retrospectively collected data from medical and EEG-video records of 523 TLE patients, referred for surgery to the Pitié-Salpêtrière Epileptology Unit between 1991 and 2014. We identified 389 patients belonging to group 1, 61 patients belonging to group 2, and 73 patients belonging to group 3 and performed a comparative analysis of their clinical data and surgical outcomes. TLE-HS patients (group 1): (1) began epilepsy earlier (11 ± 9 vs. 20 ± 10 vs. 15 ± 9 years); (2) exhibited more frequently early febrile convulsions (FC) (59 vs 7 vs 5%); (3) presented more: ictal gestural automatisms (90 vs 54 vs 67%), dystonic posturing (47 vs 20 vs 23%), and secondary generalized tonic-clonic seizures (GTCS) (70 vs 44% vs 48%) as compared to both groups 2 and 3 patients (all p < 0.001). With respect to auras, abdominal visceral auras were more reported by TLE-HS than by LTLE patients (49 vs 16%). Three cardinal criteria correctly classified 94% of patients into TLE-HS group: history of FC, dystonic posturing, and secondary GTCS. Postoperative outcome was significantly better in TLE-HS group than in the two other groups (p = 0.03 and 0.003). Our study demonstrates that cardinal criteria are reliably helpful to distinguish patients with TLE-HS from those with other TLE and may allow considering TLE-HS as a distinctive syndrome.
authors.affiliation: Rehabilitation Unit, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France.
authors.forename: Young Joo
authors.initials: YJ
authors.lastname: No
authors.affiliation: Rehabilitation Unit, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France.
authors.forename: Chiara
authors.initials: C
authors.lastname: Zavanone
authors.affiliation: Department of Neuropathology, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France.|Brain and Spine Institute (ICM; INSERM UMRS1127, CNRS UMR7225, UPMC), 75013, Paris, France.|Sorbonne University, UPMC Univ. Paris 06, 75005, Paris, France.
authors.forename: Franck
authors.initials: F
authors.lastname: Bielle
authors.affiliation: Department of Clinical Neurophysiology, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France.
authors.forename: Vi-Huong
authors.initials: VH
authors.lastname: Nguyen-Michel
authors.affiliation: Stroke Unit, Hôpital de la Pitié-Salpêtrière, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France.|Brain and Spine Institute (ICM; INSERM UMRS1127, CNRS UMR7225, UPMC), 75013, Paris, France.|Sorbonne University, UPMC Univ. Paris 06, 75005, Paris, France.
authors.forename: Yves
authors.initials: Y
authors.lastname: Samson
authors.affiliation: Brain and Spine Institute (ICM; INSERM UMRS1127, CNRS UMR7225, UPMC), 75013, Paris, France.|Epileptology Unit, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France.
authors.forename: Claude
authors.initials: C
authors.lastname: Adam
authors.affiliation: Department of Clinical Neurophysiology, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France.|Brain and Spine Institute (ICM; INSERM UMRS1127, CNRS UMR7225, UPMC), 75013, Paris, France.|Sorbonne University, UPMC Univ. Paris 06, 75005, Paris, France.|Epileptology Unit, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Navarro
authors.affiliation: Rehabilitation Unit, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France. sophie.dupont@aphp.fr.|Brain and Spine Institute (ICM; INSERM UMRS1127, CNRS UMR7225, UPMC), 75013, Paris, France. sophie.dupont@aphp.fr.|Sorbonne University, UPMC Univ. Paris 06, 75005, Paris, France. sophie.dupont@aphp.fr.|Epileptology Unit, AP-HP, GH Pitie-Salpêtrière-Charles Foix, 75013, Paris, France. sophie.dupont@aphp.fr.|Epileptology Unit and Rehabilitation Unit, Hôpital de la Salpêtrière, 47, boulevard de l'Hôpital, 75651, Paris Cedex 13, France. sophie.dupont@aphp.fr.
authors.forename: Sophie
authors.identifier: http://orcid.org/0000-0001-6516-4688
authors.initials: S
authors.lastname: Dupont
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D004569:Electroencephalography; D004833:Epilepsy, Temporal Lobe / Q000150:complications* / Q000000981:diagnostic imaging*; D005260:Female; D006624:Hippocampus / Q000000981:diagnostic imaging* / Q000473:pathology*; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging; D008297:Male; D012598:Sclerosis / Q000150:complications / Q000473:pathology; D014741:Video Recording; D055815:Young Adult
keywords: Hippocampal sclerosis; Lateral lobe epilepsy; Medial temporal lobe epilepsy; Syndrome