Thom Maria
Departments of Neuropathology and Clinical and Experimental Epilepsy, Institute of Neurology, University College London, London, UK.
Neuropathol Appl Neurobiol. 2014 Aug;40(5):520-43. doi: 10.1111/nan.12150.
Hippocampal sclerosis (HS) is a common pathology encountered in mesial temporal lobe epilepsy (MTLE) as well as other epilepsy syndromes and in both surgical and post-mortem practice. The 2013 International League Against Epilepsy (ILAE) classification segregates HS into typical (type 1) and atypical (type 2 and 3) groups, based on the histological patterns of subfield neuronal loss and gliosis. In addition, granule cell reorganization and alterations of interneuronal populations, neuropeptide fibre networks and mossy fibre sprouting are distinctive features of HS associated with epilepsies; they can be useful diagnostic aids to discriminate from other causes of HS, as well as highlighting potential mechanisms of hippocampal epileptogenesis. The cause of HS remains elusive and may be multifactorial; the contribution of febrile seizures, genetic susceptibility, inflammatory and neurodevelopmental factors are discussed. Post-mortem based research in HS, as an addition to studies on surgical samples, has the added advantage of enabling the study of the wider network changes associated with HS, the long-term effects of epilepsy on the pathology and associated comorbidities. It is likely that HS is heterogeneous in aspects of its cause, epileptogenetic mechanisms, network alterations and response to medical and surgical treatments. Future neuropathological studies will contribute to better recognition and understanding of these clinical and patho-aetiological subtypes of HS.
海马硬化（HS）是内侧颞叶癫痫（MTLE）以及其他癫痫综合征在手术和尸检中常见的一种病理表现。2013年国际抗癫痫联盟（ILAE）分类根据海马亚区神经元丢失和胶质增生的组织学模式，将HS分为典型（1型）和非典型（2型和3型）组。此外，颗粒细胞重组以及中间神经元群体、神经肽纤维网络和苔藓纤维出芽的改变是与癫痫相关的HS的显著特征；它们有助于鉴别HS的其他病因，同时也凸显出海马癫痫发生的潜在机制。HS的病因尚不清楚，可能是多因素的；本文将讨论热性惊厥、遗传易感性、炎症和神经发育因素的作用。基于尸检的HS研究作为手术样本研究的补充，具有额外的优势，即能够研究与HS相关的更广泛的网络变化、癫痫对病理及相关合并症的长期影响。HS在病因、癫痫发生机制、网络改变以及对药物和手术治疗的反应等方面可能具有异质性。未来的神经病理学研究将有助于更好地认识和理解HS的这些临床和病理病因学亚型。
