1. New clinicopathological associations and histoprognostic markers in ILAE types of hippocampal sclerosis
- Author
-
Valérie Thuriès, Claude Adam, Pierre Levy, Vi-Huong Nguyen-Michel, Charles Duyckaerts, Stéphane Clemenceau, Véronique Samson, Michel Baulac, Virginie Lambrecq, Anne Bertrand, Ana Laura Calderón-Garcidueñas, Bertrand Mathon, Vincent Navarro, Sophie Dupont, Karima Mokhtari, Franck Bielle, Service de Neuropathologie [CHU Pitié Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Universidad Veracruzana, Université Pierre et Marie Curie - Paris 6 (UPMC), Institut Pierre Louis d'Epidémiologie et de Santé Publique (iPLESP), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute (ICM), Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-CHU Pitié-Salpêtrière [AP-HP], Unité fonctionnelle d'épilepsie [CHU Pitié-Salpêtrière], Service de Neurologie [CHU Pitié-Salpêtrière], IFR70-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-IFR70-CHU Pitié-Salpêtrière [AP-HP], Service de neurologie 1 [CHU Pitié-Salpétrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], and Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)
- Subjects
0301 basic medicine ,Adult ,Male ,Proto-Oncogene Proteins B-raf ,Pathology ,medicine.medical_specialty ,Antigens, CD34 ,Status epilepticus ,Hippocampal formation ,Hippocampus ,Pathology and Forensic Medicine ,Cohort Studies ,03 medical and health sciences ,Epilepsy ,0302 clinical medicine ,Immunophenotyping ,Intermediate Filament Proteins ,medicine ,Humans ,Gliosis ,Research Articles ,Hippocampal sclerosis ,Sclerosis ,biology ,business.industry ,General Neuroscience ,Electroencephalography ,medicine.disease ,Granule cell ,Prognosis ,3. Good health ,030104 developmental biology ,medicine.anatomical_structure ,Epilepsy, Temporal Lobe ,biology.protein ,Female ,Neurology (clinical) ,NeuN ,medicine.symptom ,business ,Corrigendum ,030217 neurology & neurosurgery ,Biomarkers ,[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology - Abstract
International audience; Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is a heterogeneous syndrome. Surgery results in seizure freedom for most pharmacoresistant patients, but the epileptic and cognitive prognosis remains variable. The 2013 International League Against Epilepsy (ILAE) histopathological classification of hippocampal sclerosis (HS) has fostered research to understand MTLE-HS heterogeneity. We investigated the associations between histopathological features (ILAE types, hypertrophic CA4 neurons, granule cell layer alterations, CD34 immunopositive cells) and clinical features (presurgical history, postsurgical outcome) in a monocentric series of 247 MTLE-HS patients treated by surgery. NeuN, GFAP and CD34 immunostainings and a double independent pathological examination were performed. 186 samples were type 1, 47 type 2, 7 type 3 and 7 samples were gliosis only but no neuronal loss (noHS). In the type 1, hypertrophic CA4 neurons were associated with a worse postsurgical outcome and granule cell layer duplication was associated with generalized seizures and episodes of status epilepticus. In the type 2, granule cell layer duplication was associated with generalized seizures. CD34+ stellate cells were more frequent in the type 2, type 3 and in noHS. These cells had a Nestin and SOX2 positive, immature neural immunophenotype. Patients with nodules of CD34+ cells had more frequent dysmnesic auras. CD34+ stellate cells in scarce pattern were associated with higher ratio of normal MRI and of stereo-electroencephalographic studies. CD34+ cells were associated with a trend for a better postsurgical outcome. Among CD34+ cases, we proposed a new entity of BRAF V600E positive HS and we described three hippocampal multinodular and vacuolating neuronal tumours. To conclude, our data identified new clinicopathological associations with ILAE types. They showed the prognostic value of CA4 hypertrophic neurons. They highlighted CD34+ stellate cells and BRAF V600E as biomarkers to further decipher MTLE-HS heterogeneity. This article is protected by copyright. All rights reserved.
- Published
- 2018
- Full Text
- View/download PDF