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EPILEPSY 【Objective and demand】 1. to master the definition of epilepsy and epileptic seizure. 2. to master the international classifications of epileptic seizure. 3. to master the clinical features ,diagnosis and treatment of epilepsy. 4. to master the definition and therapy of status epilepticus.

1.The definition of epilepsy and epileptic seizure

(1)Epilepsy
 Epilepsy is a group of disorders resulting from abnormal , sudden, excessive electrical discharges arising from cerebral neurons. These discharges are self-terminating but have a tendency to recur.

(2). Epileptic seizure It is a transient disturbance of cerebral function caused by an abnormal neuronal discharge . (3) Relation between epilepsy and seizure Epilepsy is a total term of a group of diseases and syndrome. Epileptic seizure is a kind of symptom. Seizure often means a process of 1 time. If there are one or a few kinds of seizure that attack to one patient repeatedly, we call this epilepsy. One patient of epilepsy can have several kinds of seizures. 2.Etiology According to cause, divided to (1) idiopathic Epilepsy (2) symptomatic epilepsy (3)Cryptogenic epilepsy隐源性癫痫

2.Etiology (1) idiopathic Epilepsy Except possible hereditary tendency，there is no other clear cause. The first onset often takes place at a specific age. Distinctive clinical features and EEG

(2) symptomatic epilepsy In some patients, some causes can be found. These causes are some abnormalities of CNS. Such as : (A) Cranial trauma and surgery (B) Infection of CNS (C) Cerebrovascular diseases (D) Intracranial tumors (E) Degenerative diseases (F) abnormal chromosome (G) Prenatal产前的 and perinatal围产期的 factors. (H) Toxication

(3)Cryptogenic epilepsy隐源性癫痫 Clinical features support the diagonsis of symptomatic epilepsy, but the certain causes can not be found. there is no distinctive clinical features and EEG. 3. influencing factors (1)age: 60-80%<20y (2)hereditary factors (3)sleep (4)the change of internal environment Endocrine :hypoglycemia electrolyte disturbance :hyponatremia Embolism Some factors :fatigue, short of sleep, hungry, constipation, drinking , flash… 3.Classifications (1)International Classification of Epileptic Seizure(proposed by International League Against Epilepsy,1981 ) Partial seizures (seizures beginning focally) (A) simple partial seizures (consciousness not impaired) With motor symptoms With somatosensory or special sensory symptoms With autonomic symptoms With psychic symptoms

Common characteristics 1.Episodic: symptoms occur suddenly, and recover radidly after a while ,intermittent period is normal 2.Transient: seizures last for a short time, usually several seconds or minutes ,no more than half an hour expept status epilepticus

Common characteristics 3.Repeatability: seizures come for the second or more times through different time intervals after the first attack 4.Stereotype: clinical manifestations of each episode are almost the same

(B) complex partial seizures (with impairment of consciousness)

 a) Beginning as a simple partial seizure and progressing to a complex partial seizure
 b) Impairment of consciousness from the beginning
         only conscious disturbance 
         conscious disturbance and automatism自动症

(C) partial seizure becoming secondarily generalized seizure

Generalized seizures ( seizures beginning with both sides symmetrically )

(A) tonic – clonic seizure ( grand mal ) 
(B) tonic seizure 
(C) clonic seizure 
(D) myoclonic seizure 
(E) atonic 失张力的 seizure
(F) absence seizure ( typical / atypical )

Some other seizures that can not be classified 4. Clinical features

(1)EPILEPTIC SEIZURE Partial seizures (seizures beginning focally)

 (A) simple partial seizures 

(consciousness not impaired) .

     There are 4 subtypes.
motor seizure
      it means that the tic of the regional body. This is more 

often seen at unilateral corner of the mouth, eyelid, finger and toe. Sometimes the region is one side of face or distal site of one limb.

     The lesion locates the cortical motor area ( anterior central gyrus ) on  the opposite hemisphere.

Jackson's epilepsy :

      It is a special type of simple partial motor seizure. The seizure begins with one part of  body, then stretches  along  the distribution of cortical motor area . For example , the tic begins from  a thumb of one side , then involves wrist , elbow , shoulder and so on , step by step.

Todd paralysis : a transient remnant paralysis after seizure, commonly disappear in 0.5---36 h .

Sensory seizure (With somatosensory or special sensory symptoms)

The somatosensory symptoms   usually are numbness or acanthesthesia针刺感. These are more often seen at unilateral corner of the mouth, tongue, finger and toe.    
 The lesion locates the cortical sensory area( posterior central gyrus )  of  the opposite hemisphere. 

Special sensory seizures include : visual seizure : simple visual hallucination. for example :flare (lesion location : occipital lobe)

Auditory seizure: simple auditory hallucination. For example : bombus (lateral temporal lobe or insular gyrus )

Olfactory seizure: simple olfactory hallucination .for example: strange odor. (uncinate gyrus )

Gustatory seizure: simple gustatory hallucination

Vertiginous seizure : spinning sensation ,floating sensation, sinking sensation.

Autonomic seizure: Nausea , vomit, pale, Polydipsia (烦渇）……

It is seldom the only finding of seizure.

The lesion locates amygdala（杏仁核）, insular gyrus, and cingulate gyrus扣带回.

psychic seizure Memory change ( deja vu似曾相识, jamais vu似不相识, reviewing bygone quickly 快速回顾往事 ) obsessional thoughts 强迫思维 Abnormal affection (reasonless fear, anger, depression ,euphoria欣快 ) illusion and hallucination

The lesion locate limbic system 边缘系统.

(B) complex partial seizures ( with impairment of consciousness)

 a) Beginning as a simple partial seizure and progressing to a complex partial seizure
   the simple partial seizure is looked as aura.
   The aura先兆  can be any kind of simple partial seizure, especially psychic and special sensory symptoms. 

b) Impairment of conscious from the beginning I) only conscious disturbance

        confusional state is common, and loss of consciousness is not seen frequently.

II) conscious disturbance and automatism

         Automatism：the motor symptoms of complex partial seizure are characterized by unconscious activities that are harmonicous协调的  and adaptable and can not be remembered when the patients regain consciousness.
     

Automatism can be classified into several types:

    Eating automatism 
    Imitativeness automatism
    Gesture automatism
    Verbal automatism
    Walking automatism
    Half automatic action
    Etc

(C) partial seizure becoming secondarily generalized seizure

simple partial seizure becoming secondarily generalized seizure complex partial seizure becoming secondarily generalized seizure

Generalized seizures ( seizures beginning with both sides symmetrically)

            The clinical features and electroencephalogram in the beginning of this kind of  seizure can give us a piece of  information that the lesion locates  bilateral cerebral hemisphere. 
            

General specialities: Consciousness:

         Most of the subtypes are with unconsciousness.  
         While, myoclonic seizure can have not  unconsciousness.

Motor symptoms:

         often are bilateral, but not always are generalized in the bodies, maybe only involve two upper limbs or two lower limbs.

Clinical features and EEG:

        There are significant  characteristic.

(A) generalized tonic-clonic seizure (GTCS) !!!

      GTCS is a kind of attack in  which consciousness is lost , usually without aura or other warning. 

a) Tonic phase The initial manifestations are unconsciousness and tonic contractions of limb muscles for 10-30 seconds , producing extension of the extremities and arching of the body in apparent opisthotonos 角弓反张.

Tonic contraction of the respiratory  muscles may produce an expiration-induced vocalization发音 (cry or moan) and cyanosis紫绀
contraction of masticatory 咀嚼的 muscles may cause tongue trauma. 

The patient falls to the ground and may be injured.

b) Clonic phase

  The tonic phase is followed by a clonic (alternating muscle contraction and relaxation) phase of symmetric limb jerking that persists for an additional 30-60 seconds – or longer. With time , the jerking becomes less frequent , until finally all movements  cease and the muscles are flaccid. 

Breath come back after the tonic phase, and cyanosis clears. The mouth may froth泡沫 with saliva 唾液. Sphincteric muscle括约肌 relaxation or detrusor muscle 逼尿肌contraction may produce urinary incontinence尿失禁.

In the first two phases, pupil dilatation 扩大 ,raised blood pressure and heart rate, positive pathologic sign.

c) Recovery As the patient regains consciousness, they may be sleepy, have problems with vision or speech, and may have a bad headache, fatigue, or body aches. commonly takes 10-30 minutes

GTCS EEG Each of these phases of GTCS has its characteristic EEG accompaniment. Tonic phase: repetitive spikes or spike-wave discharges lasting a few seconds,followed by an approximately 10-s period 10-Hz spikes. Clonic phase:slow waves and polyspike-and-wave pattern Recovery phase: EEG traceing is nearly flat for a variable time

Tonic phase: repetitive spikes or spike-wave discharges

Clonic phase:slow waves and polyspike-and-wave pattern

Recovery phase: EEG traceing is nearly flat for a variable time

(B) absence seizure a) typical absence seizure

   These seizures are characterized by unconsciousness and staring episodes. Typically, the posture of the patient is maintained during the seizure. The mouth or face may move ，and  the eyes may blink. The seizure usually lasts 5-10 seconds. When the seizure is over, the person can not recall what just occurred and may go on with his / her activities, acting as though nothing happened. 

These seizures may occur several times a day. They are uncommon before the age of 5 and often stop before puberty青春期. The EEG shows a bilateral synchronous同步的 symmetrical 3 to 3.5 Hz spike and slow wave pattern. Commonly ,there is no manifestation of brain damage. VPA(valproic acid) is

  effective

bilateral synchronous symmetrical 3 to 3.5 Hz spike and slow wave pattern

b) atypical absence seizure The beginning and cessation of consciousness disorder in atypical absense seizure are slower than those in typical absence seizure. The change of muscular tension is obvious. EEG: 2.0-2.5 Hz irregular spike-slow wave or sharp-slow wave. It is often seen in the diffuse brain damage. The prognosis is not good.

atypical absence :irregular spike-slow wave or sharp-slow wave

(C) tonic seizures

    They are characterized by continuing muscle contraction that can lead to fixation固定 of the limbs and to deviation 偏斜 of the head and eyes to one side; the accompanying arrest停止 of ventilatory movements leads to cyanosis. Consciousness is lost, and there is no clonic phase to these seizure. 

tonic seizure: polyspike wave

(D) clonic seizures They are characterized by repetitive clonic jerking accompanied by loss of consciousness. There is no initial tonic period. Often infant EEG have no characteristic:slow wave or irregular spike-slow wave

 (E) myoclonic  seizures 

They are sudden , brief , involuntary movements or jerks of the trunk and extremities. They occur without warning and if severe enough may cause the patient to fall to the ground.

Most people experience similar myoclonic jerks  normally while falling asleep. 

EEG have no characteristic:polyspike slow wave or slow wave or irregular spike-slow wave

(2)EPILEPSY AND EPILEPSY SYNDROME Epilepsy is a total name of a group of diseases and syndromes. Up to now, there are about 30 types that are classified clearly; also, new types are found continuously. Some types have their special clinical features, etiological factors, prognosis individually, even the causative genes. Others can only be classified according to clinical features, and their etiological factors and prognosis maybe are very different. 5.Diagnosis A seizure problem should be evaluated to determine : whether the attacks are seizures or another paroxysmal disorder ( TIA / Migraine/ trigeminal neuralgia) , what types of seizures they are: clinical features , EEG. whether a cause for epilepsy can be established or not : PE (general and NS), Auxiliary examination.

(1) The detailed and accurate history is the main proof for diagnosis. Because it is often impossible that the physician or nurse has witnessed a patient’s attacks, the episodes must be described in detail as much as possible in a carefully taken history.

(2)Investigations to aid diagnosis (The observation to seizure and auxiliary examination).

  the observation to seizure (including physical examination)
        observing seizure is a direct and effective method . 

Auxiliary examination

      (A) EEG
            The EEG is extremely useful if recorded during an attack , but interical发作间期 EEGs are very often normal, sometimes abnormal.            
            There is the characteristic wave pattern, such as sharp wave, spike wave, sharp slow wave, spike slow wave, and so on. 
          However, anormal or nonspecifically abnormal EEG  never excludes the diagnosis of seizures.

      (B) Brain imaging
            CT and MRI may reveal structural lesions that have caused the seizures

(3)Differential diagnosis It is very important to distinguish epilepsy from other paroxysmal diseases or loss of consciousness. The most common differentials are as follow:

Syncope

  The loss of consciousness is due to a reduced supply of blood to the cerebral hemisphere or brainstem (ascending reticular activating system and cerebral cortex ).

Classification：

Reflexsyncope（vasovagal syncope，postural hypotension syncope， micturition syncope，cough syncope，shy-Drarger syndrome）
cardiac origin of syncope
cerebullar origin of syncope , Other(crying syncope,hypoglycemia syncope)

  there is usually prodromal 前驱的dizziness, pallor, nausea, and Sweating(10seconds-1minute).The patient recovers consciousness very rapidly(seconds to a few minutes).Postictal confusional state with disorientation does not occur.

TIA  : aged people ; the history of arteriosclerosis, hypertension , paralysis or numbness  
Pseudoseizures :  some patients may have attacks that resemble seizures after psychic stimulation. it is  more often  seen among young women who receive a mental stimulation before onset.

It can usually be distinguished both clinically and by EEG findings. there in no loss of consciousness,no postictal confusion, EEG show no discharges wave.

     psychiatric referral may be helpful. 

6. Treatment (1) Preventive measures:

   To prevent the etiological factors, such as : birth trauma , craniocerebral trauma ,and many kinds of intracranial  infectious diseases.
    To adjust some activities in  daily life ,avoid inducing factors: alcohol , fatigue,  resting and movements, preventing accidents. 

(2)etiological treatment Searching and treating etiological factors actively ; at the same time , having the anti-epileptic treatment according to the type of seizures.

(3) symptomatic treatment Deciding to use the AEDs or not

Selecting antiepileptic drugs: A. The introduction of antiepileptic drugs: (A) traditional antiepileptic drugs

Carbamazepine (CBZ)  卡马西平
Valproate  (VPA)  丙戊酸钠
Phenytoin (PHT)  苯妥英
phenobarbital (PB)  苯巴比妥
primidone  (PMD)  扑痫酮
ethosuxamide (ESX)  乙琥胺
clonazepam (CNZ)  氯硝安定

(B) new AEDs : (a) topiramate (TPM) 托吡酯（妥泰） (b) lamotrigine (LTG) 拉莫三嗪 (c) gabapentin (GBP) 加巴喷丁 (d) felbamate (FBM) 非氨酯 vigabatrin (VGB) 氨己烯酸 Levetiracetam(LEV) 左乙拉西坦

B. It depends on the type of seizure mainly. (A) partial seizure:

        The first-line antiepileptic drugs: CBZ, VPA, PHT, PB, PMD

(B) GTCS

    a) primary GTCS:
           The first-line drugs: CBZ, PB, VPA, PHT, PMD.
    b) primary GTCS with absence seizure:
        The first-line drugs : 
            firstly, VPA; secondly, PHT, PB.
    c) Secondary or unclear reason GTCS:
        The first-line drugs: CBZ, VPA, PHT.

(C) absence seizure: VPA , ESX. (D) Tonic seizure: CBZ, PB, PHT.

C. Also, we should consider the toxicity of drugs.

D. Additionally, the age, physical condition, and economic condition should be considered.

Single drugs therapy:

            Only one  drug at one time for an adequate length of time should be used to begin therapy.
            when a kind of drugs has gotten the effective blood drug concentration but has not clear effect to control seizure, or has brought too much side-effects to patients, this drug should be changed. 
            The course that the drugs replace should last 1 week (overlap period) at least, unless very serious side effect has taken place.
            Additionally, drug combination should only be seen in some special conditions.
            For example, intractable epilepsy (seizures still frequently attack---more than 4 times one month--- after formal treatment of 2 years with  every kinds of main AEDs). 
            Also, if the patient is restless and agitated, he/she can be used diazepam.

Medication methods

    The drugs should be built up slowly from the minimal effective dose according to the drug-kinetics 药物动力学characteristics. 
           Measurement of blood drug concentration is very important to advoid toxicity.
    Because most AEDs can cause some gastrointestinal tract  reaction, everyday  dose should be divided, and be given to patients for several times  in order to reduce the reaction.
          For example , because PHT has  strong alkalinity碱性 , it should be given after meal.
          PB can be taken once a day.

drug monitoring (therapeutic effect and side/toxic effect)

                Measurement of blood drug concentration is very important to advoid toxicity.
             We should pay a lot of attention to the side effect during the medication course.
             blood routine, urine routine, hepatic function and renal function should be checked before medication in order to contrast.
             something other are also very necessary, as follow:  having physical examination regularly, checking blood routine once a month, and checking blood biochemistry every 3 months.

Side effect

         every kinds  of AEDs have a few sorts of side effects. 

Long-term regular therapy

          idiopathic epilepsy : achieve remission for 1-2 years , then be considered withdrawal  
          not idiopathic epilepsy: achieve remission for 3-5 years 
          some patients: whole life 

Withdrawal of AEDs

          in view of the many adverse reactions associated with AEDs, a patient who has achieved remission for over 1-2 years should be considered for drug withdrawal. However, there is the risk of recurrence of seizures, especially in some forms of epilepsy, and this has important consequences for driving, employment, and self-esteem. 
          Thus, the final decision to attempt withdrawal must be made by patient, and if undertaken, must be carried out very slowly, with gradually decreasing doses.
          If the disease get worse (seizures relapse or EEG deterioration) , the dose should be recovered to the former therapeutic level .	
         40% can get the success of drug withdrawal at last. 

Refractory seizures Definition In some patients, disabling seizures persist despite trials of all major anticonvulsants , alone and in combination-and at the highest doses the patient can tolerate for 2 years, episodes more than 4 times per month. Refractory seizures Evaluation for possible surgical therapy should be considered. Patients with complex partial seizures arising from s single temporal lobe are the most frequent surgical candidates. Hemispherectomy and corpus callosum section are also sometimes used to treat intractable epilepsy.

7. Status epilepticus (1) definition It is defined arbitrarily as seizures that continue for more than 30 minutes without ceasing spontaneously, or which recur so frequently that full consciousness is not restored between between successive episodes. Status epilepticus is a life-threatening state

SE is a medical emergency because it can lead to permanent brain damage- from hyperpyrexia, circulatory collapse, or excitotoxic neuronal damage-if untreated. 

(2) treatment Control the epilepsy Terminating status epilepticus: (a) diazepam:

this is the principal medicine.
to adults, 10-20mg i. v. (intravenous injection) , no more than 20mg in single time (to children, 0.3-0.5 mg/kg). the  Speed is 3-5 mg/min i. v.
after 15 minutes, the patient can be given such medicine in same dose again, or 100-200 mg diazepam with 5% glucose saline (GS)  intravenous drip (i. v. drip)  in 12 hours slowly.

PAY ATTENTION TO: The total doses of diazepam given to a patient in 12 hours are no more than 200mg 。Diazepam can cause respiratory depression occasionally, at that time, this medicine should be stopped at once.

(b)10% Chloral Hydrate 水合氯醛: To adults, 25-30ml (to children, 0.5-0.8 ml/kg), retention enema保留灌肠 .

(c) clonazepam (CNZ) 氯硝安定

(d) Amobarbital 异戊巴比妥

(e) lidocaine 利多卡因

(B) the treatment after terminating status (transition and sustaining )

   long-acting AEDs should be used after terminating status .

   PB is often used in early course. To adults, 0.2g i.m. (intramuscular injection ), 3-4 times per day ( to children, the doses should be decreased suitably) .  Lasting 3-4 days.
   At the same time, effective antiepileptic drugs taken orally or by nasal feeding should be chosen to give patients according to the type of seizure. After that, the long term therapy is beginning. 

other symptomatic treatment and Supportive treatment : Keeping the respiratory tract unobstructed Reducing the temperature if there is a fever Treating pulmonary infection Relieving cerebral edema: mannitol Treating the electrolyte disturbances and acid-base imbalance

1)concerning neuroanatomy b)the oculomoter nerve runs in close proximity to the posterior communcation artery c)the superior colliculus is found in the mid brain 2)the following cranial nerve carry parasympathetic fiver a)oculomotor c)facial e)vagus 3)subdural haematomas can cause a)dementia b)pupilary change c)bradycardia d)changing level of counciousness 4)myasthenia gravis a)is cause by antibodies to the acetylecholine recepter in the majority of cases c)may show duarnal variation in symptoms e)may present with opthalmoplegia 5)in a young woman with aspastic paraparesis,the following suggest a diognosis of multiple sclerosis a)delayed visual evoked potentials d)oligoclonal bands in the CSF e)periventricular white matter lesions on magnetic resonance imaging(MRI) of the brain 6)the causes of a mixed upper and lower motor neuron picture include c)syringomyelia d)motor neuron disease e)Toboparesis 7)Unilateral facial weakness is a recognized feature of a)herpes zoster infection c)Acoustic disease d)cholesteatoma 8)bilateral lower motor neuron facial weakness may occur in a)sarcoidosis b)Guillain-Barre syndrome c)lyme disease d)lymphoma 9)the following are true about headaches c)Amaurosis fugax may be caused by temporal arteritis e)cluster headache are more common in men than in women 10)in idiopathic parkinson’s disease d)Anticholinergic drugs are most effective in relieving tremor 11) The following drugs can produce parkinsonism: a)chlorpromazinre d)metaclopramide e)haloperidol 12) the following features suggest that increased tone is due to rigidity : a)tone is increased equally in flexors and extensors c)associated pill-rolling tremor e)tone increases with synkinesis 13)concerning movement disorders: a)Huntington’s chorea presents with progressive dementia and chorea in middle age b)myoclonus is a feature of subacute sclerosing panencephallitis e)alcohol reduces benign essential tremor 14)causes of a small pupil include : a)horner’s syndrome c)tabes dorsalis e)pilocarpine eye-drops 15)concerning papilloedema : a)there is lodd of venous pulsation on funduscopy b)there may be enlargement of the blind spot c)intracranial pressure may be normal d) hypocalcaenia is a recognized cause e)it is a recognized feature in guillain-barre syndrome 16)concerning optic neutritis : b)white-matter abnormalities on MR imaging increase the likehood of developing multiple sclerosis in the future c)after recovery , some impariment of red-green color vision may remain e)it causesa delay in visual evoked potentials 17)Ptosis may be a feature of : a)myotonic dystrophy b)horner’s syndrome d)oculomotor nerve (third nerve ) palsy e)myasthenia gravis 18)the following may causes a third nerve palsy: a)aneurysm of the posterior communicating artery b) diabetes d)herniation of the uncus of the temporal lobe 19)concerning the brown –sequard syndrome : a) there is ipsilateral corticospinal loss below the lesion b)there is ipsilateral loss of joint –position sense below the lesion c)there is ipsilateral loss of two –point discrimination below the level of the lesion 20)the following typically occur within the first 24 hours of complete cervical cord transection: b)fall in blood pressure c) loss of bladder control e)gastric dilatation 21)in motor neruon disease : b)there may be atrophy of the betz cells in the motor cortex c)electromyography shows chronic partial denervation d)there should be no signs of sensory loss 22)concerning the brachial plexus a)in brachial neuritis ,severe pain around the shoulder precedes rapid wasting c)Erb’s palsy is caused by a lesion to C5/C6-derived regions of the brachial plexus d)a brachial plexus lesion and an ipsilateral horner’s syndrome may indicate a pancoast tumor e)vaccination may precipitate brachial neuritis 23)causes of a mononeuropathy include : a)diabetes c)polyarteritis nodosa e) lead poisoning 24)causes of a polyneuropathy include : a)diabetes b)Guillain –Barre syndrome c)may be caused by neurosyphilis d)may affect the shoulders in syringomyella 25)charcot joints: a)may affect the feet in diabetes c)may be caused by neurosyphilis d)may affect the shoulders in syringomyelia 26)a lesion to the comon peroneal nerve at the fibular head causes : a)weakness of eversion of the foot b)decreased sensation over the dorsum of the foot d)if long term wasting of tibialis anterior 27)hyposmia may arise secondary to : a)a head injury d)antibiotic therapy e) a front meningioma 28)brainstem death may be confirmed by : a) extensor response of the limbsto painful stimuli b)absent corneal reflex e)absent doll’s eye reflexes 29)the following are causes of acute transient visual impariment : b)amaurosis fugax c)papilloedema d)migrainous aura 30)a homonymous hemianopia may arise from a lesion of : a) the optic tract b)the occipital cortex e)the optic radiation 31)the following may be features of frontal lobe dysfunction a)depression b)social disinhibition c)apraxia of gait e)a grasp reflex 32)dysarthia may resutl from a lesion of: a)the cerebellum c)the hypoglossal nerve d)the balsal ganglia 33)the following may give rise to a pseudobulbar palsy e)multiple sclerosis 34)the following are clinical features of cerebellar dysfunction b)hypotonia d)titubation e)impairedrapid altering movements 35)facial sensory loss may occur with a lesion of : a)the cerebellopontine angle c)the gausserian ganglion e)the cavernous sinus 36)the following clinical features may help differentiate between a syncopalattack and a seizure: a)upright posture at the onset c)a bitten tongue d)urinary incontinence 37)sensorineural deafness may occur secondary to : a)loud noise b)gentamicin therapy c)meniere’a disease d)an acoustic neuroma 38)the following are fartures of a subarachnoid haemorrhage a)fever b)thundrclap c)photophobia d)positive kernig’s sign e)neck stiffness 39)choreic movements are: c)brief ,jeukyand irregular 40)a physiological tremor is : b)worsened bu anxiety d)improved by beta blockers 41)features of an upper motor neuron lesion are( all false ) Brisk abdominal and cremasteric reflexes Wasted muscle Eakness of individual muscle Hypotonia Fatigue muscle strength 42)a lesionof the medulla on one side may give rise to : b)a contealateral hemiparesis d)contealateral easkness of the tongue 43) a small pupil may be seen in : b)elderly patients c)horner’s syndrome d)terminally ill patients taking morphine for analgesia e)a pontine lesion 44)the following may be seen in a patient with a lesionof the third nerve or nucleus A ) a fixed dilatedpupil b)ptosis c)diplopia in all positions of gaze d)a history of diabetes mellitus e)a contrallateral hemiplegia 45)nystagmus may be seen in: a) a patient with an internuclear ophthalmoplegia b)a lesion of the pons c)a patient who is blind d)a patient with cerebellar dysfunction e)a lesion of the foramen magnum 46)in a patient with a sensory ataxia: a)vibrationmay be imparied c)romberg’s test may be positive d)a history of alcohol abuse may be implicated in the aetiology 47)clinical features of a unilateral lesion of the cerebellopontine angle may be (all false ) Conductuve deafness on the same side An ipsilateral hemiparesis Ipsilateral weakness of the lower face A pseudobulbar dysarthria Vertigo as a prominent early symptom 48)a patient with herpes zoster infectonofthe geniculate ganglion may present with c) hyperacusis d)altered perception of taste e)pain from the auditory meatus 49)the fibers of the dorsal column pathway c)are affected in the deficiency of vitamin B12 d)when damaged may result in a positive Romberg’s test e)are spared following occlusionof the anterior spinal artery 50)a dissociated sensory loss may be seen in a)syringomyelia

. Three kinds of crisis of myasthenia gravis (MG) are myasthenic crisis, cholinergic crisis, and brittle crisis. 2. According to the evolution of symptoms and signs, cerebral thrombosis can be divided into three completed stroke, progressive stroke, reversible ischemic neurologic deficit (RIND). 3. The extent of MND (motor neuron disease) include: anterior horn cell, motor nucleus of cranial nerves, pyramidal neuron of cortex, cortico-bulbar tract, cortico-spinal tract 4. There are two kinds of bulbar palsy: bulbar palsy and pseudo-bulbar palsy. The most important difference between them is Lesion position: nucleus ambigus; glossopharyngeal nerve and vagus nerve and gag reflex (-) and Lesion position of pseudo bulbar palsy: bilateral cortico-bulbar tract and gag reflex (+) 5. Multiple Sclerosis is a kind of autoimmune diseases characterized by demyelination of CNS. The characteristics of MS are multiple in space and multiple in time. 6. Main cause of cerebral thrombosis atherosclerosis, cerebral hemorrhage, hypertension, subarachnoid hemorrhage, saccular (berry) aneurysms, cerebral embolism, rheumatic heart disease 7. The main pathology of HSV-1 encephalitis is inflammation and necrosis in the temporal lobes and frontal cortex. Drug for the treatment of HSV encephalitis- Acyclovir 8. Cardinal feature of Parkinson disease are: tremor; rigidity; akinesia & bradykinesia; postural instability 9. Peripheral neuropathy pathological process: Wallerian degeneration, axonal degeneration, neural degeneration, segmental demyelination 10. CSF flows in between: arachnoidmater and piamater. 11. In Status epilepticus treatment, total doses of Diazepam given to patient in 12 hr no more than 200 mg. 12. Viral encephalitis main symptoms: Meningeal signs, Kernig sign, Brudzinski sign 13. According to the clinical features，especially neurological imaging: cerebral thrombosis can be divided into- Large area cerebral infarction, Cerebral watershed infarction, hemorrhagic infarct, mutiple infarct. 14. Subtypes of motor neuron disease: amyotrophic lateral sclerosis, progressive bulbar palsy, progressive spinal muscular atrophy, primary lateral sclerosis 15. In general, lesion of broca aphasia locates in the posterior of inferior frontal gyrus of dominant hemisphere. Lesion of Wernicke aphasia is the posterior part of superior temporal gyrus of dominant hemisphere. 16. Three main features of MG are: Fluctuating weakness and easy fatigability of skeletal muscle; Distribution of the weakness; Response to anticholinesterase drugs. 17. Posterior intracranial circulation system and anterior intracranial circulation system supply the blood circulation of brain. 18. If the lesion is in the internal capsule, clinical findings include -- hemiplegia, hemianesthesia and homonymous hemianopia of the opposite side of the body. 19. The main cause of Subarachnoid Hemorrhage is saccular (berry) aneurysms (70%). Others are degenerative and hypertensive aneurysms (10 %); arteriovenous malformations (AVMs) (10%); not defined (10%). 20. International Classification of Epileptic Seizure includes-- Partial seizures, generalized seizures and unclassified seizures. 21. Diazepam is the principal medicine to treat epileptic status. 22. The main signs of intracranial hypertension are headache, vomiting, pupil edema. 23. GBS diagnostic features: weakness; sensory disorder; tendon reflex loss; dysfunction of autonomic nerve; monophase course 24. Vertebrobasilar and Internal carotid artery supply brain. 25. Main cause of cerebral hemorrhage: hypertension. Others: - atherosclerosis, saccular aneurysm, hematopathy , A-V malformation, Moyamoya disease 26. Motor neuron disease commonest subtype: amyotrophic lateral sclerosis(ALS) 27. The diagnostic criteria for Guillain-Barre syndrome are often follows 1-4 weeks after infection, acute or subacute onset, the lower motor neuron paralysis of symmetric four limbs, sensory disorders of ending-brush nerves & the disabilities of cranial nerves, Albumino-cytological(protein -cell) dissociation in CSF, the delay of F wave or H reflection in the earlier period. 28. Main cause of cerebral thrombosis: atherosclerosis 29. MS (ant) motor symptom due to corticospinal tract

Aphasia:-Disorders of communication with others in language caused by lesions in cerebral.Disorders of repetition, naming, reading and writing. Apraxia: inability to perform previously learned task.Can’t do some simple motions such as being asked to extend his tongue, swallow, wash his face, brushing, unlock ect. Ascending myelitis:- Developing from thoracic cord to cervical cord or medulla oblongata rapidly in several hours to 2 days. Patient occurs with the weakness of all limbs ,respiratory and bulbar palsy and so on . Acute myelitis:- is an acute inflammation of one or more segments of the spinal cord, which is associated with infections and vaccinations disorders. Acute transverse impairments caused by demyelination or necrosis of the myelin on white matter Arousal:The degree to which the individual appears to be able to interact with the environment. Broca aphasia -patient knows what he or she wants to say but has difficult finding the correct words, often product the wrong word. The ability to repeat words is better than spontaneous speech. Brown-sequard syndrome-Also called Spinal cord hemisection syndrome; deep sensory disorder and spastic paralysis on the same side below the level of lesion; pain and temperature loss of the opposite side below the level of lesion Bitemporal hemianopia loss of the temporal side of visual field in both eyes.Caused by the optic chiasm lesion such as pituitary tumor、craniopharyngioma. Bell’s palsy:- acute LMN facial palsy, usually unilateral weakness of lower half of face & spare eye closure, forehead movement. Brachial plexus:- The large network of nerve fibers which distributes the innervation of the upper extremity. The brachial plexus extends from the neck into the axilla. In humans, the nerves of the plexus usually originate from the lower cervical and the first thoracic spinal cord segments (C5-C8 and T1), but variations are not uncommon. Brudzinski sign: keep the patient with supine position without pillow, flex the head, if the patient flexes his legs involuntary at the same time, it means the test is positive. Coma: The most severe degree of depressed consciousness. Unresponsive and unarousable. eyes closed and does not make an attempt to avoid noxious stimuli, display forms of reflex posturing. Consciousness is awareness of the internal or external world.It is a kind of abilities for a person to understand himself (herself) or environment around him. Cerebral embolism refers to the cases in which various emboli enter into the cerebral artery along the blood flow from other parts of the body, then occlude the cerebral vessels , and produce local neurological deficits. Cerebral thrombosis:- is the most common type of the cerebral infarction. Most cerebral thrombosis can be attributed to atherosclerosis and chronic hypertension, which occlude cerebral artery and result in clinical symptoms. accounts for 80% of CVDs. Crisis:- Rapidly progressive weakness of the medulla oblongata and respiratory muscles leading to dysphagia, dyspnea and respiratory failure is termed as crisis, a life-threatening event. Cervical enlargement:- C5-T2 ->supplying upper limbs --- brachial plexus CSF protein –cell dissociation:- a characteristic abnormality of GBS; increased concentration of protein but a normal cell count in the 2rd and 3th week . Test of CSF is normal in the first week. Consciousness is the state of being aware of self and the environment. The level of consciousness is the first state evaluated in the neurological examination. Epilepsy:¬¬¬¬¬-¬¬¬¬¬¬ Epilepsy is a chronic disorder, or a group of chronic disorders caused by abnormal, sudden , excessive, electrical discharges of cerebral neurons which lead to transient central nervous system disfunction . Epileptic seizure:- It can be defined as an intermittent, stereotyped disturbance of motor function, or sensation, consciousness, emotion, behavior, autonomic nerve function, arising from abnormal neuronal discharges. abnormal cerebral neuronal discharges are patho-physiological base of epileptic seizure. Guillain-Barré syndrome: is an acute inflammatory demyelinating polyneuropathy characterized by progressive muscle weakness and areflexia. Guillain-Barré syndrome is a neurological disorder in which the body's immune system attacks parts of the peripheral nervous system. Gordon's sign an abnormal variation of Babinski's reflex, elicited by compressing the calf muscles, characterized by dorsiflexion of the great toe and fanning of the other toes. It is evidence of disease of the pyramidal tract. Gonda's sign is a clinical sign in which flexing and then suddenly releasing the 4th toe elicits an extensor plantar reflex. It is found in patients with pyramidal tract lesions, and is one of a number of Babinski-like responses. Hoffmman sign: The test involves tapping the nail or flicking the terminal phalanx of the third or fourth finger. A positive response is seen with flexion of the terminal phalanx of the thumb. Hypokalemic periodic paralysis (Hopp):- is an autosomal dominant familial disorder characterized by recurrent episodes of skeletal muscle weakness associated with the decrease of serum potassium levels. homonymous hemianopia -loss of the opposite side of visual field in both eyes.The lesion is in lateral geniculate bodies, whole damage of optic radiation and calcarine cortex. Horner's syndrome:- lesion : lateral horn of C8~T1; constricted pupil-Pupil smaller; Ptosis; endophthalmos -eyeball embole; facial anhidrosis -Absent or deficient sweat of the affected face Hunt syndrome:--lesions: above geniculate ganglion. (1)Hyperacusis (hypersensitivity to sound in the affected ear); (2)Lacrimation; (3)Dysgeusia (loss of taste on the front two-thirds of the tongue); (4)Lower motor neuron facial palsy; (5)Pain of Near mastoid process at the effected face; (6)Loss of pinna and auditory canal’s sensation; (7)Herpes of periost Jackson's epilepsy:- It is a special type of simple partial motor seizure. The seizure begins with one part of body, then stretches along the distribution of cortical motor area . For example , the tic begins from a thumb of one side , then involves wrist , elbow , shoulder and so on ,step by step. Kernig sign: keep the patient with supine position without pillow, flex the hip and knee joint at 90°, elevate a leg, if the patient raises his head or has painful expression, it indicates the test is positive. Lacunar infarction refers to small infarcts (0.2-20mm) in the deep white matter of cerebral hemisphere and brainstem. It is caused by ischemia and necrosis of the cerebral tissue because of hypertensive arteriolosclerosis (the diameter of small artery:100-200μm ). It is the most common cerebrovascular lesion in elderly hypertensive patients. In the past time, belong to cerebral thrombosis, but now we can find some differences between them. Lumbar enlargement:- L1- S2 -> innervation of the lower limbs--- lumbosacral plexus Miller-Fisher syndrome:- a proximal variant of GBS involving ocular muscles. ophthalmoplega, ataxia, areflexia Migraine is a kind of paroxysmal headache, and characterized by unilateral or bilateral throbbing headache repeatedly. Myasthenia Gravis is a autoimmune disease in the NMJ that depends on cellular immunity , and is mediated by AChR-Ab , complements take part in the formation of this disease characterized by weakness of cranial and skeletal muscles. A marked reduction in the number of AChR on the postsynaptic membrane of the NMJ -----the basic defect in MG. Motor neuron disease (MND) is a kind of progressive degeneration disease that selectively encroach on the whole or one part of motor system. Motor neuron disease (MND) is a rare condition caused by the breakdown of the nerve cells in the brain that control the muscles. currently no cure Multiple Sclerosis is a kind of autoimmune diseases characterized by demyelination of CNS.Its characteristic is multiple demyelinated plaques in white matter of CNS.multiple in space (a few focuses) and in time (occur repeatedly). The most common lesion positions are as follow: White matter surrounding ventricle, optic nerve, conductive tract in spinal cord, brain stem, and cerebellum. Myopathies:- Muscular diseases are intrinsically primary diseases of skeletal muscle (striated muscle). Myopathy may be inherited or acquired. The common symptoms are weakness, fatigue, cramps, stiffness, myalgia (pain), spasm, and twitching Weakness : usually most marked proximally rather than distally Neuromuscular junction diseases:- a sets of diseases caused by circulating factors such as neurotoxins or auto antibodies which bind with high affinity to specific proteins at the NMJ and disturb the neuromuscular transmission. Neck stiffness (nuchal rigidity): keep the patient with supine position without pillow, flex his head, if resistance exists, it means the test is positive. Oppenheim's sign is dorsiflexion of the big toe elicited by irritation downward of the medial side of the tibia Parkinson’s disease(paralysis agitans):-a common degeneration disease in nervous system. becomes increasingly common with advancing age. characterized by rest tremor, bradykinesia,　　rigidity, abnormal gait and posture. Periodic paralysis are known as skeletal muscle channelopathies and be familial (autosomal dominant inheritance). Periodic paralysis is characterized by intermittently flaccid weakness or paralysis that may be associated with abnormalities of the plasma potassium level. Primary Intracerebral Hemorrhage is also called cerebral hemorrhage which is caused by spontaneous(non-traumatic) rupture of blood vessels, usually arteries. Rossolimo's sign is a clinical sign in which percussion of the tips of the toes causes an exaggerated flexion of the toes. Schaefer's sign is a clinical sign in which squeezing the Achilles tendon elicits an extensor plantar reflex. Somnolent: The early stage of consciousness disorder.Often a feature of raised intracranial pressure. Stupor refers to a condition in which the patient is less alert than usual, but can be stimulated into responding Status epilepticus (SE) is a life-threatening state that be defined as continuous epileptic seizures for more than half an hour or recurrent epileptic seizures with failure to regain consciousness between seizures. Syncope It is a syndrome of transient and recurrent loss of consciousness, and the patient often fall to ground because of decreased muscular tension of all body. Seizure is a transient disturbance of cerebral function caused by an abnormal neuronal discharge Subarachnoid hemorrhage (SAH) refers to a spontaneous(rather than traumatic) arterial bleeding into the subarachnoid space. It is the fourth frequent cerebral vascular disorder following atherothrombosis, embolism, and cerebral hemorrhage. Stroke – describes an acute event, in which a neurological deficit appears, and is related to the impairment of the blood supply to one part of the brain. Stroke includes: transient ischemic attack(TIA), cerebral thrombosis, cerebral embolism, lacunar infarction, primary cerebral hemorrhage and subarachnoid hemorrhage, etc. Tic douloureux:- In the serious cases, during onset there are the reflective spasm of facial muscle with blushing, the increasing of skin temperature and tearing. The mouth corner of patient is drawn to the affected side. It attacks in the day and night The patient has a sleepless night because of pain .(insomnia ) Trigger points:- Trigger points can be anywhere on the face or in the mouth or nose, such as the cheek, nose , mouth , tongue . They are generally not in the same place as the pain. The pain can be triggered by touching facial trigger points or by moving, such as talking , eating ,washing the face, shaving, chewing, brushing the teeth, putting on makeup , even a slight breeze on the face. Transient Ischemic Attack:- refers to an episode of an acute focal neurological deficit which lasts no more than 24 hours and is followed by complete functional recovery. But it may re-occur. Transverse myelopathy lesions:- Cause-Lesions affecting all or most tracts of the spinal cord at ≥1 segmental levels. S/S-Deficits in all functions mediated by the spinal cord (because all tracts are affected to some degree) Trigeminal neuralgia:- is a facial-pain syndrome of unknown cause that develops in middle to late life, and is a transient, repeated attack of agonia in the sensory distributions of the 5th cranial nerve. Viral encephalitis is an acute inflammatory process involving to meninges, and variable degree, brain parenchyma, which is caused by many various kinds of viruses. Willis’ circle is a potential effective intracranial anastomotic pathway that compensates for sudden focal reductions of blood flow at the base of the brain. Congenital asymmetry of the VAs and narrow or absence of its anterior or posterior communicating segments are commonly seen, and all above may reduce the circle’s anatomic effectiveness. Wallenberg syndrome Major cerebral infarction syndromes caused by Posterior inferior cerebellar artery occlusion, Characterized by 1.severe vertigo, vomiting and nystagmus (vestibular nucleus), 2.crossing sensory disorder 3.homolateral Horner sign, 4. Bulbar palsy, 5. homolateral cerebellar ataxia inferior cerebellar peduncle and cerebellum.