Transcript 强直－阵挛发作（tonic-clonic seizure）

癫 痫（Epilepsies）
Department of Neurology , 2nd affiliated hospital ,
College of medicine, Zhengjiang university
癫 痫（Epilepsies）
definition:
Epilepsy is a group of chronic disorders
characterized by recurrent seizure, which is a
transient disturbance of cerebral function caused
by an abnormal neuronal discharge.
That are charcterized by recurrent 、
spontaneous、transient 、paroxysms.
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Because of different position and diffuse extent
of abnormal neuronal discharge, the neurologic
malfunction may reflect motor, sensation,
consciousness, behavior and autonomic nerve and
so on.
seizure
Epilepsy
epileptic syndromes
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classification by etiology:
1、Idiopathic epilepsy
for which most common in children and adolescent, No
obviously structural change and metabolic abnormality that
induce seizure are found in patients’ brain.
It may be closely related to heredity.
prevalence rate of close relative is high to 2-6%, which is
70% in monovular twins.
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2、Symptomatic epilepsy:
by kinds of cerebral impairments and metabolic disorders.
（1）： Congenital cerebral diseases:
脑皮质发育不全(agenesia corticalis )、先天性脑积水
(congenital hydrocephalus ), 遗传代谢障碍(genetic
metabolic disorders )，如卟啉病（ porphyrinopathy ）、
苯丙酮尿症（ phenylketonuria) 等。
（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。
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（3） Diseases in antenatal period and perinatal
diseases: are the most causes of infant epilepsy。
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), and those may become seizure focus several。
（4） After febrile convulsion：
Serious and lasting febrile convulsion can result in
neuron hypoxia → impairments of neuron→ proliferation
of gliocyte → seizure focus.
Medial temporal lobe is sensitive to hypoxia, which
lead to切迹硬化(incisure sclerosis)
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（5）Infections:
meninges or encephalon is infected by kinds of bacteria、 virus、
paresites, and then lead to encephalitis, meningitis,
cerebral abscess.
（6）Toxicosis:
CO、汞（ hydrargyri, ）、乙醇（ alcohol）、灭鼠药
（ deratization durgs）等，以及尿毒症（ uremia）、妊娠
中毒症(toxenia of pregnancy )。
（7） Cerebrovascular diseases:
subarachnoid hemorrhage, intracerebral hemorrhage,
cerebral infarction, vascular malformation, et al.
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（8） Intracranial tumors:
meningothelioma, astrocytoma, metastatic
tumor, lymphoma, et al.
（9) Nutritional, metabolic diseases:
hypocalcemia, hypoglycemia, VitB6 deficiency.
（10） Degeneral, demyelinating diseases:
AD, tuberous sclerosis, multiple sclerosis.
Pick病等。
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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.
enviroments:
endocrine ,如经期性癫痫(menstrual epilepsy),妊娠性癫痫
(gravid epilepsy), sleep。
causative factors ，如flashing --myoclonus,
overventilation--absence seizure, over hydroposia--tonic
clonic seizure。
reflex epilepsy: photogenic epilepsy, chess epilepsy等。
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pathogenesis:
It caused by the neurologic disbalance
between excitation and inhibition, and the
abnormal stability of membrane potential.
It may be increase of neuron excitation or
decrease of inhibition, and abnormality of
membrane.
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1、 Change of biochemistry:
excitatory amino acids (aminoglutaminic acid,
aminosuccinic acid) are excitatory transmitters,
which make calcium and sodium ions get in cell,
destroy the stability of membrane, prolong the
unpolarized time course.
GABA: Cl -
2、Change of membrane potential:
to show off persistent depolarization → 阵发性
去极化飘移( paroxysmal depolarization shift,PDS).
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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。
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。
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。
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classifications of seizure:
According to that discharge originate from
unilateral or bilateral (EEG), consciousness is
exist or not, seizures are classified three types
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）

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generalized
seizure
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
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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 pain)，
Jackson感觉性癫痫。特殊感觉（嗅-olfactory、味
-gustateory、听-sounds、视-vision）。
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Autonomic symptoms :
nausea(呕心） , vomiting(恶吐）, 烦渴
（ polydipsia）、排尿欲（urination desire），
sweating（出汗），cardiac arrhythmia, 等。
Psychic symptoms :
amnestic disorder, sentiment abnormality(情感异常)、
misconception(错觉), complicated hallucination,
feelings of familiarity and unfamiliarity , et al.
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complex partial seizure: or psychomotor seizure.
The psychiatric symptoms or symptoms of
special sense occur firstly, the secondary symptoms
are impairments of consciousness , automatism
and amnesia.
Automatism: the manifestations are partially or
completely poor contact with enviroments, and do
repetitive actions which are aimless or unconscious.
The eyes just agaze, accompany with吸吮(sucking),
摸索(groping), walking, speaking, running,
shouting and so on.
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secondary generalized seizure:
Begin with an aura.
Simple partial seizure
→ secondary generalized seizure;
Complex partial seizure
→ secondary generalized seizure;
Simple partial seizure
→ Complex partial seizure
→ secondary generalized seizure.
posture seizure,
adversive seizure
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absence seizure:
(petit mal)
Brief loss of consciousness for 5-10 seconds, no
reaction to call, the eyes agazing, begin and stop
abrutly. there are may be several to several hundred
seizure daily.
The EEG shows a characteristic 3/s spike-and-wave
pattern during the seizure.
The begin and stop of unconsciousness in atypical
absences is slower than in typical absences. The EEG
shows irregular spike-and-wave with abnormal
background.
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myoclonic seizure:
Showing abrupt, transient, quick muscle
contraction, which is generalized or focal.
clonic seizure:
Generally repetitive clonic seizure
tonic seizure:
The limbs straightly stretch, the head and eye deviate
to one side, accompany with greater pupils.
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tonic-clonic seizure: (grand mal)
loss of consciousness and convulsions
Tonic phase(强直期)：
Clonic phase(痉挛期）:
Postictal phase（惊厥后期）：
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Tonic phase:
• 上睑抬起(eyelids put up)，眼球上窜(eyes deviate) ，喉部痉挛
(laryngeal spasm, “epileptic cry”) ，The body is stiff ,the
elbows are flexed and the legs extend， limbs or hands
show a few small jerk.
This stage lasts for 10-20 seconds.
• Autonomic hyperactivity is prominent, such as心率加快
(cardiac arrhythmia) ; blood pressure elevation; bronchial
hypersecretion ; pupil dilation and the reflex to light
disappear ; breathing stops, the lipsand face cyanosis
(bluish).
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Clonic phase:
• The recurrent clonic muscular contractions are
produced , lasting for 1 or 2 minutes.
• Physiologic changes: cardiac arrhythmia ; blood
pressure elevation; bronchial hypersecretion ;
pupil dilation; the reflex to light disappear ; the
lips and face cyanosis (bluish). Bladder and bowel
funtion may be lost.
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Postictal phase:
• Breathing resumes firstly, foam(泡沫) around the
mouth, urinary and fecal incontinence.
• The pupil size, blood pressure, heart rate
gradually return to normal level .
• The patients gradually awake, headache, and
muscular pain are common.
• The patient does not recall the seizure itself.
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Status epilepticus，SE:
• More than 30 minutes of continuous seizure
activity ，or two or more sequential seizures
without full recovery of consciousness between
seizures.
• Usually with hyperthermia, dehydration ,
acidosis, WBC levations .
• SE is a common life-threatening neurological
disorder. It is essentially an acute, demanding
immediate treatment.
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• Convulsive SE：
– 强直阵挛发作状态 Tonic-clonic status epilepticus
– 强直性发作状态 Tonic status epilepticus
– 肌阵挛性癫痫状态 Myoclonic status epilepticus
– 单纯部分发作性状态 Simple partial status epilepticus
– 婴儿痉挛持续状态 Infantile spasm status epilepticus
• Non-convulsive SE：
– 失神状态 Absence status epilepticus
– 复杂部分性发作状态（颞叶癫痫持续状态）
Complex partial status epilepticus
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epileptic syndromes
• 良性儿童期中央回癫痫
（ Benign childhood epilepsy with centrotemporal spike），又称sylvian or rolandic
epilepsy.
• West综合征（ West syndrome）:又称婴儿
痉挛症（infantile spasms).
• Lennox-Gastaut综合征：
Lennox-Gastaut syndrome
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Benign childhood epilepsy with centro-temporal spike ：
An idiopathic age- and localization-related epileptic
syndrome .
Involving the face, pooling of saliva ,usually
secondary generalized.
The symptoms usually occur at night, usually have
an excellent prognosis, most seizures stopped by 15
years old.
EEG shows high-voltage central or temporal spikeand-wave discharges with normal background.
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West syndrome （infantile spasm）
The syndrome is usually symptom, may resulted by
prenatal ,perinatal ，metabolic disorders.
Characterized by begin with a sudden, rapid, tonic
contraction of trunk and limb, such as nodding , bowing, et
al. Usually occur in clusters, often several dozens,
separated by several seconds.
EEG shows consists of chaotic, high-voltage
polymorphic delta and theta rhythms (hypsarrhythmia-高峰
节律紊乱) with spike and sharp charges.
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Lennox-Gastaut syndrome：
The age at onset is preschool stage. The syndrome is
characterized by multiple types of seizures, the most
common seizure types are atypic absence, tonic,
myclonic and tonic-clonic seizure.
EEG show slow spike-and-wave discharges (1-2 Hz)
with abnormal background.
Usually have mental retardation or regression.
seizures often are resistant to therapy (refractory
epilepsy).
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Diagnosis and differentials：
The diagnosis of epileptic seizures is
made by analyzing the patient's detailed
clinical history.
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1、
Is it epilepsy?
hysteria,
syncope,
migraine,
TIA,
sleep disorders, et al.
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2、classifications
of seizure or
epileptic syndromes? ：
3. etiology：
根据history 、体征、辅助检查（EEG、脑
电图诱发试验、AEEG、VTMEEG、CT、MR、
MRS、fMR、SPECT、PET、DSA、 lumbar
puncture等）确定病因。
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prognosis：
prevention and treatment：
prevention：
treatable causes ：
对症治疗(pharmacologic therapy)：
身心卫生(mental health)：
外科治疗(Surgical Care)：
切除致痫灶或阻断癫痫发放传播途径
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对症治疗: pharmacologic therapy
发作间期的治疗(treatment during interictal
phase )：
发作期的治疗：
单次发作的治疗
持续状态的治疗
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treatment during interictal phase：
（1）何时用药:原则上一旦诊断明确,就开始规范治疗.
(2）合理用药:根据发作类型和癫痫综合症、不良反
应、年龄、性别、经济等个体状况选药。
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choose the appropriate drugs according to seizure type:
partial epilepsy-carbamazepine, phenytoin, henobarbital.
complex partial epilepsy—carbamazepine.
secondary generalized epilepsy—valproic acid
generalized epilepsy—valproic acid.
generalized tonic-clonic seizure also can choose
phenytoin, phenobarbital.
Absence epilepsy also can choose ethosuximide
and clonazepam
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Therapy Dose :
Begin with small dose, use the minimum dose to get
seizure free.
•Carbamazepine:
therapy dose range :0.3-1.0/day. Begin with 0.2/day,
increase the dose slowly if the seizure is not free.
effective dose concentration is 4-12mg/L.
•valproic acid:
0.6-1.6/day,begin with 0.6/day, the effective dose
concentration is 50-100mg/L.
•Phenytoin:
0.3/day,begin with 0.2/day, the effective dose
concentration is 10-20mg/L.
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Drug combination:
when the epilepsy is consider refractory epilepsy,
which can’t get seizure free by using mono-drug and
reach effective plasma concentration, usually consider to
combinate two or more anti-epilepsy drugs.
It is not appropriate to combinate anti-epilepsy drugs
with the same side effect and same mechanism.
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drug side effect:
• Carbamazepine: rash (Steveus-Johson
syndrome), acute ataxia, agranulocytosis, et al.
• Phenytoin: rash (Steveus-Johson syndrome),
acute or chronic ataxia, hyperplasia of gum
and hair, encephalopathy, et al.
( saturation dynamics)
• Valproic acid: gastrointestinal and hepatic
damage.
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Withdraw the drugs:
The conditions withdraw drugs :
2 to 5 years after seizure free.
Gradually decrease the drug dose. It usually
need slowly withdraw the drugs when the
therapy contains long duration ,large dose and
much kinds of drugs
6 to 12 months of the whole stage.
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卡马西平 Carbamazepine 0.1／片
得理多 Tegretol（进口）0.2／片
丙戊酸钠 Sodium valproate 0.2／片
德巴金控释片( Depakine )500mg／片
德巴金糖桨、 德巴金注射液(400mg/针)
鲁米那 Luminal（苯巴比妥 phenobarbital ）
30mg/片，或0.1/针
氯硝安定: Clonazepam
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• 妥泰 Topiramax（托吡酯 Topiramate ） 25mg/
片，200mg-400mg/天。
• 拉莫三嗪 Lamotrigine 50mg/片，200mg400mg/天。
• 加巴喷丁 Gabapentin:0.1/片，0.6~0.9/天。
• 奥卡西平 Oxcarbazepine：0.15/片，0.6~0.9/天。
• 开普兰（左拉西坦）：500mg bid
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treatment during seizure：
Lay the patient down, prevent the drop or
lingua biting, as well as the asphyxia(窒息)。
Treat the patient with high frequency and
long time convulsion by intramuscular
injection phenobarbital.
Patient with automatism should be take
care of ,in order to prevent injury.
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treatment of status epilepticus：
• Stop the seizure as quicklyer as better:
intravenous diazepam , phenytoin, clonazepam,
phenobarbital 。
• Keep the open of air way, give oxygen。
• Decrease the cerebral pressure.
• Infection precaution .
• Keep the balance of water, salt, acid-base and
allopathy.
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