书山论剑(神经病)个人整理大题答案.doc

书山论剑——神经病大题答案（个人整理版）
04级八年制
名解：
1、 Lhermitte’s sign：典型的低头曲颈触电样征P239：多发性硬化患者被动屈颈时会诱导刺痛感或触电样感觉，从颈部放射至背部甚至到下肢的放射性触电感。头复位时，症状消失；屈颈动作愈迅速有力，触电感亦愈强烈，如屈颈动作缓慢，触电感则较轻微。是多发性硬化的特征性表现之一。It is an electrical sensation that runs down the back and into the limbs which is elicited诱出 by bending the head forward. It can also be evoked引起 when the neck is flexed。( From Wikipedia) It is a sensation of "electricity" that is felt by the patient on passive or active flexion of the neck. It indicates a lesion of the posterior columns in the cervical portion of the spinal cord. （来自课件）
2、 Bell sign：P118常见于特发性面神经麻痹又称Bell麻痹，由于茎乳孔（面神经管）内面神经非特异性炎症导致周围性面神经瘫痪，表现为患者闭目时眼球向外上方转动而露出巩膜，称Bell征。While closing the eye, the eyeball screws upward to expose the white sclera. this is a reflex upward deviation of eyes in response to forced closure of the eyelidsis.（来自课件）
3、 皮质盲cortical blindness：皮质盲是大脑枕叶皮质受到毒素影响或血管痉挛缺血而引起的一种中枢性视功能障碍，以血管痉挛性损害最为常见。临床表现为双眼视觉完全丧失，瞳孔光反射正常，眼底正常，可有偏瘫等。本病常见于2～6岁小儿。Cortical blindness is the total or partial loss of vision in a normal-appearing eye caused by damage to the visual area in the brain's occipital cortex. This damage is most often caused by loss of blood flow to the occipital cortex from either unilateral or bilateral posterior cerebral artery blockage (ischemic stroke). ( From Wikipedia)
4、 面具脸mask-like face：是帕金森的典型症，面部肌肉强直，面部表情肌肉运动减少，眨眼及双眼转动减少，表情呆滞，像戴了面具一样，俗称“面具脸。It is a typical symptom of Parkinson’s disease, the manifestations are rigor muscle of face, decreased motor of facial expression muscle, wink and rotation of two eyes reduced, expression sluggishness, just like wearing a mask. (Edit by myself)
问答
1、 ALS肌萎缩侧索硬化amyotrophic lateral sclerosis的临床表现。P238
答：（1）多在40岁以后发病，男性多于女性；（2）大多数患者以单侧上肢的下运动神经元损害症状起病，表现为手指运动不灵和力弱，伴同侧伸腕困难；（3）部分患者以整个或上肢近端无力起病，随后大、小鱼际肌和蚓状肌等手部小肌肉萎缩，渐向前臂、上臂及肩胛带肌发展，伸肌无力较屈肌显著。（4）逐渐出现下肢痉挛性瘫痪、剪刀步态、肌张力增高、腱反射亢进和Babinski征阳性等。（5）少数病例从下肢起病，渐延及双上肢。上肢起病者约为下肢起病者的2倍，远端肌无力较近端更常见。肌束震颤是最常见的症状之一，可在多个肢体及科舌部发生。（6）延髓麻痹通常较晚出现，可于手部肌肉萎缩不久后出现，表现为构音障碍、讲话含糊不清、吞咽和咀嚼困难、舌肌萎缩伴纤颤。（7）部分患者可出现假性球麻痹性情感障碍，如强哭强笑等。（8）肢体主观感觉异常如麻木、疼痛等，但无客观感觉障碍。（9）病程持续进展，最终因呼吸肌麻痹或并发呼吸道感染死亡。
The disorder causes muscle weakness and atrophy throughout the body caused by degeneration of the upper and lower motor neurons. Unable to function, the muscles weaken and atrophy. Affected individuals may ultimately lose the ability to initiate and control all voluntary movement, although bladder and bowel sphincters and the muscles responsible for eye movement are usually, but not always, spared.
Cognitive function is generally spared for most patients although some (~5%) also have frontotemporal dementia. A higher proportion of patients (~30-50%) also have more subtle cognitive changes which may go unnoticed but are revealed by detailed neuropsychological testing. Sensory nerves and the autonomic nervous system, which controls functions like sweating, are generally unaffected but may be involved for some patients. （From Wikipedia）
2、 GBS的诊断标准。P130
答：诊断要点是：（1）病前1-3周有感染史，急性病或亚急性起病并在4周内进展的对称性四肢弛缓性瘫痪和脑神经损害，轻微感觉异常；（2）脑脊液蛋白-细胞分离现象；（3）肌电图检查，早期可见F波或H波反射延迟或消失；（4）神经传导速度减慢，远端潜伏期延长，动作电位波幅正常或下降。
课件答案（英文）：（1）Acute or subacute development of symmetric motor or sensorimotor neuropathy after a viral illness, delivery, or surgery；（2）Compatible EMG alterations；（3）CSF: Elevated protein content with normal cell count.
3、 癫痫持续状态（status epilepticus, SE）的定义。P301
答：传统定义认为SE指“癫痫全身性发作在两次发作间意识不清楚，单次发作持续30分钟或在短时间内频繁发作”。2001年，国际抗癫痫联盟提出了新的定义：“超过大多数这种发作类型患者的发作持续时间后，发作仍然没有停止的临床征象，或反复的癫痫发作，在发作间期中枢神经系统的功能没有恢复到正常基线”。在没有办法确定“大多数患者发作持续时间”的情况下，倾向性的看法是“连续发作超过5分钟就是癫痫持续状态”。
课件（英文整理）：Repetitive epileptic seizures, in which the patient does not recover normal alert state between attacks.
Traditionally it is defined as one continuous unremitting seizure lasting longer than 30 minutes, or recurrent seizures without regaining consciousness between seizures for greater than 30 minutes. (From Wikipedia)
论述
1、 蛛网膜下腔出血（subarachnoid hemorrhage，SAH）临床表现。P175
Answers: (1). Generally, common occurrence age is 40-70 years (lower than in cerebral hemorrhage) Sudden onset while the patient is up and active； (2). Headache, nausea, vomiting；(3). Symptoms of meningeal irritation(Stiff僵硬 neck, Brudzinski sign, Kernig sign)；
(4). Disturbance of consciousness, seizure；(5). In most case, there is no local neurologic deficit except for third nerve palsy (occular movement disturbance)；(6). A delayed hemiplegia偏瘫 may occur 4-10 days after onset (attributed to vascular spasm); (7). Examination of fundi基底 reveals preretinal视网膜前的or subhyaloid透明膜下的hemorrhage.[来自课件的答案]
2、 C5脊髓在不同地方破坏出现的感觉症状。

05八年制
名解
1、 Wernicke aphasia听觉性失语P62：优势侧半球颞上回后部的感觉性语言中枢病变引起，患者听力正常，但不能理解他人和自己的言语。It is a type of aphasia traditionally associated with neurological damage to Wernicke’s area in the brain, (Brodmann area 22, in the posterior part of the superior temporal gyrus of the dominant hemisphere). The patients have normal hearing but can’t understand language of themselves and other people. ( From Wikipedia)
2、 Dysarthria构音障碍P62：是和发音相关的中枢神经、周围神经或肌肉疾病导致的一类言语障碍的总称。It is a motor speech disorder resulting from neurological injury (including central nerve and peripheral nerve injury) of the motor component of the motor-speech system or muscle disease. ( From Wikipedia and translation)
3、 Trigger spots触发点P117；三叉神经痛患者面部某个区域可能特别敏感，易触发疼痛，如上下唇、鼻翼外侧、舌侧缘等，这些区域称之为“触发点”。For the patients of trigeminal neuralgia, some areas on the face may be specially sensitive that is likely to trigger pain. Such as lower and upper lips, lateral ala nasi, margo lateralis linguae and so on. These areas are called “Trigger spots”.
4、 Jackson epilepsy杰克逊[皮质]性癫痫: 癫痫局灶性运动性发作表现之一，表现为异常运动从局部开始，沿皮层功能区移动，如从手指—腕—前臂—肘—肩—口角—面部逐渐发展。称为贾克森癫痫。It’s one kind of partial epileptic seizures, manifesting that abnormal movements began in region and moved along cortical function areas. For example, gradually develop from fingers, wrist, forehand, elbow, shoulder, angle of mouth to face.
简答
1、 GBS吉林-巴雷综合征（Guillain-Barre syndrome） differential diagnosis. P130
Answers:（1）Acute poliomyelitis:急性脊髓灰质炎
1）acute, generalized disease caused by destruction of motor neurons in the spinal cord；
2）It is distinguished by asymmetry of flaccid松弛的 paralysis, signs of meningeal irritation, fever, and CSF pleocytosis脑脊液[淋巴]细胞异常增多；
3）no dysfunction of sense( anaesthesia麻木; hyperaesthesia感觉过敏)。
（2）Acute myelitis:急性脊髓炎
1）a transverse myelitis interrupting both motor and sensory tracts at one level, usually thoracic.
2）It usually begins with localized back or radicular pain神经根痛followed by abrupt onset of bilateral paresthesia感觉异常 in the legs, an ascending sensory level；
3）Urinary bladder and bowel involvement occurs early and is prominent突出的；
4）Both cell count and Protein content in CSF are mildly elevated or normal。
（3）Myasthenia Gravis重症肌无力：
1）characterized by fluctuating波动 weakness（肌）无力；
2）affected muscles involve: ocular, facial, oropharyngeal muscles. Limb and neck weakness is also common；
3）Myasthenia weakness is the clinic response to cholinergic drugs；4）CSF are normal。
（4）Periodic paralysis (hypokalemic)周期性麻痹：
1）characterized by episodic bouts侵袭，发作 of limb weakness；
2）Familial history of transient短暂的 attack of weakness；
3）Low potassium in the serum, and low potassium changes in the ECG；4）CSF are normal；5）Weakness can be terminated解除 by administration of potassium salts。
（5）白喉diphtheria和肉毒中毒botulism：Laryngeal test and serological test should be done to differentiate these two diseases.
2、 Weber syndrome大脑脚综合征 – damage structures and symptoms. P46
Answers: (1). Structures: oculomotor nerve and pyramidal tract (including substantia nigra, corticospinal fibers, corticobulbar皮质延髓的fibers). (2). Symptoms: characterized by the presence of an oculomotor nerve palsy and contralateral hemiplegia (including central facial paralysis and lingual muscle paralysis).
3、 Brown-Sequard syndrome脊髓半切综合征 – damage structures and symptoms. P141
Answers: (1). Structures: the upper motor neuron pathway of the corticospinal tract, one or both dorsal columns, the spinothalamic tract. (2). Symptoms: motor (muscle) paralysis on the same (ipsilateral同侧的) side as the lesion and deficits in pain and temperature sensation on the opposite (contralateral) side. This is called ipsilateral hemiplegia and contralateral pain and temperature sensation deficits. The loss of sensation on the opposite side of the lesion is because the nerve fibers of the spinothalamic tract (which carry information about pain and temperature) crossover交叉 once they meet the spinal cord from the peripheries（神经）末梢区域。.
4、 PD’s typical symptoms. P259
Answers: (1). Resting tremor: “pill-rolling” 搓丸样动作etc; (2). Rigidity: increased tone, micrographia小写症 etc; (3). Bradykinesia运动迟缓：masked face etc; (4). Postural instability: festinant gait慌张步态 etc; (5). Other symptoms: non-motor symptoms, autonomic nerve symptoms and sensory symptoms.
论述
1、 Status epilepsy – definition and treatment. P301
Answers:
(1). Definition: Traditionally it is defined as one continuous unremitting seizure lasting longer than 30 minutes, or recurrent seizures without regaining consciousness between seizures for greater than 30 minutes. (From Wikipedia)
(2). Treatment:
1) An airway should be inserted and oxygen given.
2) The patient should be protected from injury during the convulsive episode and placed on a flat, soft surface with side rails up and padded to avoid injury.
3) Convulsion should be controlled as quickly as possible
4) It is important to ensure adequate respiratory exchange and oxygenation. If existence of respiratory depression, the patient should be intubated.
5) The complication with status epilepticus should be treated .
6) Treating the underlying cause.
7) The patient with unconsciousness should be injected luminal (100mg q8-12 hours) into muscle; and with consciousness taken AEDs. （来自课件）
2、 脑血栓cerebral thrombosis、脑出血cerebral hemorrhage、蛛网膜下腔出血subarachnoid hemorrhage的鉴别。P162
Answers: (1). Cerebral thrombosis(embolism) diagnosis: 1)Characteristically sudden onset and rapid progression; 2). Focal neurologic deficits; 3). With or without headache, mind confusion, convulsion at the onset; 4). Primary diseases related to embolus; 5). CT/MRI results. (2). Cerebral hemorrhage&(3). Subarachnoid hemorrhage.看课本吧。
06八年制
名解
1、 Todd paralysis：常见于癫痫局灶性运动性发作，表现为身体某一局部不自主的抽动，大多见于一侧眼睑、口角、手或足趾，也可涉及一侧面部或肢体，严重者发作后可留下短暂性肢体瘫痪，称为Todd麻痹。It is focal weakness in a part of the body after a seizure. This weakness typically affects appendages附属物 and is localized to either the left or right side of the body. It usually subsides completely within 48 hours. Todd's paresis may also affect speech, eye position (gaze凝视), or vision. (From Wikipedia)
2、 Bell sign：见前述。
3、 Horner’s syndrome P43：患侧眼裂变小，眼睑低垂，瞳孔缩小，眼球内陷，同侧额面部少汗或无汗，常见于颈部交感神经受压受损。It is the combination of drooping下垂 of the eyelid (ptosis下垂) and constriction of the pupil (miosis瞳孔缩小), sometimes accompanied by decreased sweating of the face on the same side; redness发红充血 of the conjunctiva结膜 of the eye is often also present. It indicates a problem with the sympathetic nervous system. (From Wikipedia)
4、 Foster-Kennedy syndrome P35：表现为病变侧因肿瘤压迫而出现视神经萎缩，对侧颅内压增高导致视神经乳头水肿。见于额叶底面肿瘤。It is defined as one-sided optic nerve atrophy with the compression of a mass and papilledema in the other eye resulting from the increased cranial pressure. (From Wikipedia)
5、 Trigger spots：见前述。
简答
1、 Manifestation of decortical syndrome去皮质综合征. P59
答：大脑皮质广泛损害导致皮质功能丧失，而皮质下结构的功能仍然存在。表现：（1）双眼凝视gaze或无目的purposeless活动，无任何自发言语，呼之不应，貌似清醒consciousness，实无意识。（2）存在觉醒-睡眠awareness-sleep周期，但时间是紊乱disturbance的。（3）缺乏随意运动voluntary motion，原始反射primary reflexion活动保留reserve。（4）情感反应缺乏，偶有无意识哭叫或自发性spontaneous强笑forced laughing。（5）四肢腱反射tendon reflex亢进hyperfunction，病理反射阳性。（6）大小便urination and defecation失禁incontinence，腺体分泌亢进。（7）觉醒时交感神经sympathetic nerves功能亢进，睡眠时副交感神经parasympathetic nerves功能占优势be in the ascendance。（8）表现特殊的身体姿势posture，双前臂屈曲flexion和内收adduction，腕及手指屈曲，双下肢伸直straight，足跖voix pedis屈。（来自课本）
2、 Briefly describe the informations of upper motor neuron lesion and lower motor neuron lesion of facial nerve, and the difference between them. P24
答：（1）上运动神经元瘫痪：亦称痉挛性瘫痪spastic paralysis或中枢性瘫痪central paralysis。肌张力增高increased tension，腱反射亢进，出现病理反射，无肌肉萎缩，病程长者可出现失用性apraxia肌肉萎缩。急性严重病变时，由于断联休克作用，瘫痪开始是弛缓的atonic，无病理反射，休克期过后逐渐转为痉挛性瘫痪。（2）下运动神经元瘫痪：亦称弛缓性瘫痪或周围性瘫痪peripheral paralysis。特点是肌张力降低，腱反射减弱或消失，肌肉萎缩，无病理反射。如脊髓前角刺激性irritating病变可伴有肌束震颤fasciculation，肌电图electromyogram(EMG)显示神经传导conduction异常和失神经电位denervation potential。（3）鉴别见课件的表2-5.
3、 Differential diagnosis of GBS.
答：见前述。
4、 Tell the difference between extradural and intradural locations of extramedullary lesion in compressive myelopathy. P141
答：见P141页的表7-2.
问答
1、 Diagnosis of Parkinson disease. P259
Answers：(1). Not easy in early stage; (2). Four dominating symptoms; (3). Excluding Parkinsonism and Parkinsonian plus syndromes; (4). SPECT or PET: Dopamine Transporter(DAT) ↓ 。（来自课件）
答：（1）中老年发病，缓慢进行性病程；（2）四项主征（静止性震颤、肌强直、运动迟缓、姿势步态异常）中必备运动迟缓一项，其余三项至少具备其中之一。（3）左旋多巴治疗有效；（4）患者无眼外肌麻痹、小脑体征、体位性低血压、锥体系损害和肌萎缩等。【来自课本，其余的2006诊断标准，看书吧。】
2、 Treatment of subarachnoid hemorrhage. P175
Answers: Stop bleeding, prevent vascular spasm, remove cause and prevent re-occurrence (in principle). Treatments contains:
(1). Absolute bed rest for 4-6 weeks, relieve and stop pain, avoid making too strenuous费劲的efforts.
(2). Therapy for intracranial hypertension.
(3). Stop bleeding: EACA 6-氨基己酸, PAMBA对氨甲基苯甲酸for 7-10 days.
(4). Prevention and treatment for vascular spasm: Nimodipine尼莫地平.
(5).Surgical operation: 1). Removal of the aneurysm动脉瘤 or AVM动静脉型血管畸形; 2)Intravascular approach is a good way.
(6). Prevention of recurrence: treatment for the cause, avoidance of inducement诱因.（来自课件）
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