癲癇是一種最常見的神經系統疾病,特點為多數發作無誘因且難以預測。發作可導致合并癥,包括外傷及癲癇猝死(Sudden unexpected death in epilepsy,SUDEP),并致生活質量下降。過去20年廣泛研究了發作的預警和報警,開發很多方法及設備,如頭皮腦電圖、顱內腦電圖、肌電圖、皮膚電變化、心率和心率變異性(Heart rate variability,HRV)。其中HRV是最有前景的方法。發作發放通過網絡導致交感神經和副交感神經間不平衡并且改變了自主神經發放合并心率異常。過去20年用計算機方法開發了HRV的譜分析。HRV的變化早于腦電圖發作和臨床發作的開始。HRV可能是癲癇發作的預警和報警的指標。現在雖有很多關于癲癇的HRV算法,但是缺少標準的對于癲癇患者的方案,并且沒有固定的監測模式,使之難以轉化為臨床實用,解決這個問題是十分重要的。總結出一個HRV評估的最低方案可用于所有癲癇患者的研究十分必要,可使HRV成為預警癲癇發作的有用工具。
2002年Burger等首先報道癲癇患者的大腦標本中有少突膠質細胞增生。2013年Coras等認為是一個新的臨床病理學實體稱之增殖性少突膠質細胞伴癲癇(Proliferative oligodendroglial hyperlasia in epilepsy,POGHE)。2017年Schurr等詳細研究其病理學后確認為輕度皮質發育畸形伴少突膠質細胞增生及癲癇(Mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy,MOGHE)。迄今國外文獻報道92例,國內尚無報道及介紹,本文對92例進行分析并復習文獻。均為兒童藥物難治性部分發作,發作年齡≤15歲者96.4%,≤10歲者83.1%。臨床表現多種多樣。電臨床多定位于額葉(81.5%),少數在顳頂或顳枕區。磁共振成像異常類似于局灶性皮質發育不良(Focal cortical dysplasia,FCD),尤其是FCDⅡa。均為藥物難治性癲癇,并做外科切除性治療。組織病理學均有不同于FCD的特點,即灰白質交界處有簇狀或層狀少突膠質細胞增生及異位神經元。但皮質分層無異常。
Currently, about one-third of patients with anti-epilepsy drug or resective surgery continue to have sezure, the mechanism remin unknown. Up to date, the main target for presurgical evaluation is to determene the EZ and SOZ. Since the early nineties of the last century network theory was introduct into neurology, provide new insights into understanding the onset, propagation and termination. Focal seizure can impact the function of whole brain, but the abnormal pattern is differet to generalized seizure. Brain network is a conception of mathematics. According to the epilepsy, network node and hub are related to the treatment. Graphy theory and connectivity are main algorithms. Understanding the mechanism of epilepsy deeply, since study the theory of epilepsy network, can improve the planning of surgery, resection epileptogenesis zone, seizure onset zone and abnormal node of hub simultaneously, increase the effect of resectiv surgery and predict the surgery outcome. Eventually, develop new drugs for correct the abnormal network and increase the effect. Nowadays, there are many algorithms for the brain network. Cooperative study by the clinicans and biophysicists instituted standard and extensively applied algorithms is the precondition of widely used clinically.
Drug-resistant epilepsy (DRE) not only have disruption of the patients by seizure, but also influence the quality of life, cognitive function and social association, as well as development delay even retrogression for children. Epilepsy surgery is the only curative treatment currently available for focal lesional pharmacoresistant epilepsy, five years complete seizure freedom rates was around 60% after surgery. The criterion of surgical intervention at present is achievement for the diagnosis of DRE, thereafter consideration of early epilepsy surgery, but these maybe a long-term duration. Recent advance in examine methods and surgical techniques have improved the surgical treatment of epilepsy, to such patient with focal lesional structure abnormality, before the DRE emergence, under the discussion of the multidisciplinary team. Children under 3 years old, the brain have greater neural plasticity, early surgical treatment is expected at allow the healthy brain to recover and develop the language function and quality of life. Numerous cause may pose abstracts to the delay of surgical intervention: (1) diagnosis delay; (2) patient himself and their familiar recognize that there have same risk of surgical treatment; (3) the primary doctor firmly believe that epilepsy surgery is the ultimate methods; (4) special problems of the patient, such including: age, comorbidity, and the location of symptom, EEG as well as imaging non-conformation.
Intracranial electrographic recording, especially stereoencephalography (SEEG), remains the gold standard for preoperative localization in epilepsy patients. However, this method is invasive and has low spatial resolution. In 1982, magnetoencephalography (MEG) began to be used in epilepsy clinics. MEG is not affected by the skull and scalp, can provide signals with high temporal and spatial resolution, and can be used to determine the epiletogensis zone (EZ) and the seizure onset zone (SOZ). Magnetic source imaging (MSI) is a method that superimposes the MEG data on a magnetic resonance image (MRI) and has become a major tool for presurgical localization. The applicability of MEG data has been largely improved by the development of many post-MRI processing methods in the last 20 years. In terms of the sensitivity of localization, MEG is superior to VEEG, MRI, PET and SPECT, despite inferiority to SEEG. MEG can also assist in the intracranial placement of electrodes and improve preoperative planning. Limitations of MEG include high cost, insensitivity to radiation source, and difficulty in locating deep EZ in the medial regions of the brain. These limitations could be overcome by new generations of equipment and improvement of algorithmics.