神经内科学：癫痫
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Closely related to heredity. Prevalence rate of close relative is high to 2-6%,
which is 70% in monovular twins.
2、Symptomatic epilepsy: by kinds of cerebral impairments and metabolic
结节性硬化
DNET
Sanjay M Sisodiya, 2004
低级别胶质瘤
血管畸形
影响癫痫发作的因素
Heredity: often involve many genes, which are only for预致性
(theprediction) or 敏感性(sensitivity )of seizure. The penetrance is limited by ages.
Environment: endocrine ,如经期性癫痫(menstrual epilepsy),妊娠性癫痫 (gravid epilepsy), sleep。 Flashing --myoclonus hyperventilation--absence seizure, over hydroposia--tonic-clonic seizure。
lymphoma
（9）Nutritional, metabolic diseases: Hypocalcemia, hypoglycemia, VitB6 deficiency.
（10） Degenerative, demyelinating diseases: AD, tuberous sclerosis, multiple sclerosis, Pick病。
Subcortical band hetero质-白质交接模糊）
FCD type IIb －额叶 Andrea Bernasconi, et al., 2011
thickening of the cortical gray matter and blurring of the gray–white matter transition (as a result of aggregates of large neurons, hypomyelination and Gliosis）
（2）Cerebral trauma 凹陷性骨折（ depressed fracture ）、硬脑膜撕裂
（ meninx fibrosa laceration ）、脑挫裂伤（ contusion and laceration of brain）等。
(3) Diseases in antenatal period and perinatal diseases。
Excitatory amino acids (aminoglutaminic acid, aminosuccinic acid) are excitatory transmitters,
GABA: Cl –
2、Change of membrane potential: to show off persistent depolarization → 阵发性去
Reflex epilepsy: photogenic epilepsy, chess epilepsy, startle epilepsy, 原发性阅读性癫痫。
Pathogenesis
Hyperexcitability It caused by the neurologic
disbalance between excitation and inhibition, and the abnormal stability of membrane potential.
Simple partial seizure
Motor symptoms : focal limbs（thumb or face) , Jacksonian
epilepsy (march), Todd’s paralysis, epilepsia partialis status。
Sensation symptoms : General sensation (numbness, pricking
Hydrocephalus, intracerebral hemorrhage and infaction which caused by头吸(head aspiration )or产钳 (obstetrical forceps), can make the局部脑软化(focal brain malacoma)。
• 儿童失神癫痫的基因定位于8q24。 • 良性家族性婴幼儿惊厥的基因被定位于19q。
4、轴突发芽：可能是神经元异常放电的形态学基础。
Classifications of seizure:
According to that discharge originate from unilateral or bilateral (EEG), consciousness is exist or not, seizures are classified three types
“Transmantle Sign”
Andrea Bernasconi, et al., 2011
Type II FCD可能(尚未手术证实)
报告： “左枕叶异常信号影，脱髓鞘病变考虑”。
Periventricular heterotopia
Subcortical band heterotopia
Ayala et al., 1973
3、Heredity:
• 常染色体显性遗传夜间发作性额叶癫痫（autosomal dominant nocturnal frontal lobe epilepsy)，位于 20q13.2。
• 青少年肌阵挛癫痫已发现3个基因位点，分别被定位于 6p21.3、15q14和8q24。
EPILEPSY 癫痫
名人与癫痫
拿破仑
牛頓
梵高
1. 癫痫的定义 2. 癫痫发作和癫痫和癫痫综合征的分类 3. 癫痫的诊断方法和鉴别诊断 4. 癫痫的药物治疗 5. 掌握癫痫持续状态
ILAE 癫痫新定义
一种脑部疾病 • 1: 存在癫痫反复发作的易感性 • 2: 这种疾病引起的神经生化、认知、心理和社会后
癫痫起源
只有大脑皮层，有些情况下的丘脑皮质相互作用系统 及脑干神经元的异常放电也会引起癫痫发作，而且这 种异常放电的特征为高度同步化。
Epidemiology
Incidence:0.5-1‰ Morbidity rate:5‰ Age dependent
<5岁占50％ <25岁占70-80％
subarachnoid hemorrhage, intracerebral hemorrhage, cerebral infarction, vascular malformation, et al.
（8）Intracranial tumors: Meningothelioma, astrocytoma, metastatic tumor,
nine subtypes（三大类九亚型）.
单纯部分发作（simple partial seizure）
Partial seizure 复杂部分性发作（complex partial seizure）
继发全面发作（secondary generalized
seizure）
Generalized
seizure
Classification by etiology
1.Idiopathic epilepsy (特发性癫痫）
Most common in children and adolescent, No obviously structural change and metabolic abnormality that induce seizure are found in patients’ brain.
pain)，Jackson感觉性癫痫。特殊感觉（嗅olfactory、味-gustatory、听-sounds、视vision）。
Autonomic symptoms : nausea, vomiting, 烦渴( polydipsia)、排尿欲
（urination desire)，sweating (出汗), cardiac arrhythmia。
Psychic symptoms : Amnestic disorder, sentiment abnormality(情感
异常)、 misconception(错觉), complicated hallucination, feelings of familiarity and unfamiliarity , et al.
极化飘移( paroxysmal depolarization shift).
The “Interictal Spike and Paroxysmal Depolarization Shift”
Intracellular and extracellular events of the paroxysmal depolarizing shift underlying the interictal epileptiform spike detected by surface EEG
（5）Infections Meninges or encephalon is infected by kinds of bacteria、
virus、parasites
（6）Toxicosis CO、汞（ hydrargyri, ）、乙醇（ alcohol）、灭鼠药
（ deratization durgs）等，以及尿毒症 (uremia)。 （7） Cerebrovascular diseases
(4) After febrile convulsion
Serious and lasting febrile convulsion can result in neuron hypoxia → impairments of neuron→ proliferation of gliocyte → seizure focus.
disorders.
（1）Congenital cerebral diseases 脑皮质发育障碍(Malformation cortical development), 先天性脑积水(congenital hydrocephalus ), 遗传代谢障碍: 卟啉病 (porphyrinopathy ), 苯丙酮尿症(phenylketonuria) 等。
果。 诊断三大要素： 脑部持续存在反复发作的易感性 至少一次癫痫发作的病史 发作伴发的神经生化、认知、心理及社会功能障
碍
癫痫发作 Epileptic seizure
是脑部神经元异常高度同步化活动所引起的， 由不同症状和体征组成的短暂性临床现象。
癫痫发作的三要素 脑部神经元高度同步化的异常活动、 发作的短暂性 特殊的临床现象。
Hypersynchrony Ability of firing neurons to
recruit neighboring cells and to transmit the electric signal to distant locations.
1、 Change of neurobiochemistry:
Complex partial seizure
The psychiatric symptoms or symptoms of special sense occur firstly, the secondary symptoms are impairments of consciousness, automatism and amnesia.
失神发作 （absence seizure） 肌阵挛发作（ myoclonic seizure） 阵挛性发作 （clonic seizure） 强直性发作（ tonic seizure）
强直-阵挛发作 （tonic-clonic seizure） 无张力性发作（ atonic seizure）
Unclassified seizure
which is 70% in monovular twins.
2、Symptomatic epilepsy: by kinds of cerebral impairments and metabolic
结节性硬化
DNET
Sanjay M Sisodiya, 2004
低级别胶质瘤
血管畸形
影响癫痫发作的因素
Heredity: often involve many genes, which are only for预致性
(theprediction) or 敏感性(sensitivity )of seizure. The penetrance is limited by ages.
Environment: endocrine ,如经期性癫痫(menstrual epilepsy),妊娠性癫痫 (gravid epilepsy), sleep。 Flashing --myoclonus hyperventilation--absence seizure, over hydroposia--tonic-clonic seizure。
lymphoma
（9）Nutritional, metabolic diseases: Hypocalcemia, hypoglycemia, VitB6 deficiency.
（10） Degenerative, demyelinating diseases: AD, tuberous sclerosis, multiple sclerosis, Pick病。
Subcortical band hetero质-白质交接模糊）
FCD type IIb －额叶 Andrea Bernasconi, et al., 2011
thickening of the cortical gray matter and blurring of the gray–white matter transition (as a result of aggregates of large neurons, hypomyelination and Gliosis）
（2）Cerebral trauma 凹陷性骨折（ depressed fracture ）、硬脑膜撕裂
（ meninx fibrosa laceration ）、脑挫裂伤（ contusion and laceration of brain）等。
(3) Diseases in antenatal period and perinatal diseases。
Excitatory amino acids (aminoglutaminic acid, aminosuccinic acid) are excitatory transmitters,
GABA: Cl –
2、Change of membrane potential: to show off persistent depolarization → 阵发性去
Reflex epilepsy: photogenic epilepsy, chess epilepsy, startle epilepsy, 原发性阅读性癫痫。
Pathogenesis
Hyperexcitability It caused by the neurologic
disbalance between excitation and inhibition, and the abnormal stability of membrane potential.
Simple partial seizure
Motor symptoms : focal limbs（thumb or face) , Jacksonian
epilepsy (march), Todd’s paralysis, epilepsia partialis status。
Sensation symptoms : General sensation (numbness, pricking
Hydrocephalus, intracerebral hemorrhage and infaction which caused by头吸(head aspiration )or产钳 (obstetrical forceps), can make the局部脑软化(focal brain malacoma)。
• 儿童失神癫痫的基因定位于8q24。 • 良性家族性婴幼儿惊厥的基因被定位于19q。
4、轴突发芽：可能是神经元异常放电的形态学基础。
Classifications of seizure:
According to that discharge originate from unilateral or bilateral (EEG), consciousness is exist or not, seizures are classified three types
“Transmantle Sign”
Andrea Bernasconi, et al., 2011
Type II FCD可能(尚未手术证实)
报告： “左枕叶异常信号影，脱髓鞘病变考虑”。
Periventricular heterotopia
Subcortical band heterotopia
Ayala et al., 1973
3、Heredity:
• 常染色体显性遗传夜间发作性额叶癫痫（autosomal dominant nocturnal frontal lobe epilepsy)，位于 20q13.2。
• 青少年肌阵挛癫痫已发现3个基因位点，分别被定位于 6p21.3、15q14和8q24。
EPILEPSY 癫痫
名人与癫痫
拿破仑
牛頓
梵高
1. 癫痫的定义 2. 癫痫发作和癫痫和癫痫综合征的分类 3. 癫痫的诊断方法和鉴别诊断 4. 癫痫的药物治疗 5. 掌握癫痫持续状态
ILAE 癫痫新定义
一种脑部疾病 • 1: 存在癫痫反复发作的易感性 • 2: 这种疾病引起的神经生化、认知、心理和社会后
癫痫起源
只有大脑皮层，有些情况下的丘脑皮质相互作用系统 及脑干神经元的异常放电也会引起癫痫发作，而且这 种异常放电的特征为高度同步化。
Epidemiology
Incidence:0.5-1‰ Morbidity rate:5‰ Age dependent
<5岁占50％ <25岁占70-80％
subarachnoid hemorrhage, intracerebral hemorrhage, cerebral infarction, vascular malformation, et al.
（8）Intracranial tumors: Meningothelioma, astrocytoma, metastatic tumor,
nine subtypes（三大类九亚型）.
单纯部分发作（simple partial seizure）
Partial seizure 复杂部分性发作（complex partial seizure）
继发全面发作（secondary generalized
seizure）
Generalized
seizure
Classification by etiology
1.Idiopathic epilepsy (特发性癫痫）
Most common in children and adolescent, No obviously structural change and metabolic abnormality that induce seizure are found in patients’ brain.
pain)，Jackson感觉性癫痫。特殊感觉（嗅olfactory、味-gustatory、听-sounds、视vision）。
Autonomic symptoms : nausea, vomiting, 烦渴( polydipsia)、排尿欲
（urination desire)，sweating (出汗), cardiac arrhythmia。
Psychic symptoms : Amnestic disorder, sentiment abnormality(情感
异常)、 misconception(错觉), complicated hallucination, feelings of familiarity and unfamiliarity , et al.
极化飘移( paroxysmal depolarization shift).
The “Interictal Spike and Paroxysmal Depolarization Shift”
Intracellular and extracellular events of the paroxysmal depolarizing shift underlying the interictal epileptiform spike detected by surface EEG
（5）Infections Meninges or encephalon is infected by kinds of bacteria、
virus、parasites
（6）Toxicosis CO、汞（ hydrargyri, ）、乙醇（ alcohol）、灭鼠药
（ deratization durgs）等，以及尿毒症 (uremia)。 （7） Cerebrovascular diseases
(4) After febrile convulsion
Serious and lasting febrile convulsion can result in neuron hypoxia → impairments of neuron→ proliferation of gliocyte → seizure focus.
disorders.
（1）Congenital cerebral diseases 脑皮质发育障碍(Malformation cortical development), 先天性脑积水(congenital hydrocephalus ), 遗传代谢障碍: 卟啉病 (porphyrinopathy ), 苯丙酮尿症(phenylketonuria) 等。
果。 诊断三大要素： 脑部持续存在反复发作的易感性 至少一次癫痫发作的病史 发作伴发的神经生化、认知、心理及社会功能障
碍
癫痫发作 Epileptic seizure
是脑部神经元异常高度同步化活动所引起的， 由不同症状和体征组成的短暂性临床现象。
癫痫发作的三要素 脑部神经元高度同步化的异常活动、 发作的短暂性 特殊的临床现象。
Hypersynchrony Ability of firing neurons to
recruit neighboring cells and to transmit the electric signal to distant locations.
1、 Change of neurobiochemistry:
Complex partial seizure
The psychiatric symptoms or symptoms of special sense occur firstly, the secondary symptoms are impairments of consciousness, automatism and amnesia.
失神发作 （absence seizure） 肌阵挛发作（ myoclonic seizure） 阵挛性发作 （clonic seizure） 强直性发作（ tonic seizure）
强直-阵挛发作 （tonic-clonic seizure） 无张力性发作（ atonic seizure）
Unclassified seizure