课件：神经病学（英文）1.symptomatology of neurological disea.ppt

Neurology,Department of Neurology, The 2nd affiliated hospital, Harbin Medical University,Chapter 1. Introduction,神经病学 ( Neurology),The Objects of Neurology: CNS、PNS and muscular disorders The contents of study: Etiology and Pathogenesis Pathology Clinical features Diagnosis and Differential diagnosis, Treatment and Prevention Prognosis,Nervous system,Central nervous system: brain spinal cord Peripheral nervous system: cranial nerves spinal nerves,Nervous system,Neurology is a part of neuroscience, including: Neuroanatomy, Neurophysiology, Neurobiochemistry, Neuropathology, Neurogenetics, Neuroimmunology, Neuroepidemiology, Neuroiconography神经影像学, Neurophamacology, Neuropsychology, Experimental Neurology, Neurobiology, Molecular Biology,Catalogue of the neurological diseases,Vascular diseases Infectious diseases Tumors Traumatic diseases,Catalogue of the neurological diseases,Autoimmune diseases(some of them are demyelinative diseases脱髓鞘疾病) Hereditary and metabolic disorders Congenital dysplasia先天性发育障碍 Intoxication Nutritional disturbances,Symptoms of Nervous System could divided to four classes:,Deficit symptoms deficits or loss on the normal functions (hemiparalysis, aphasia) Irritative symptoms excessive excitements that nervous structures appeared when they were stimulated (seizures, radical pain),Symptoms of Nervous System could divided to four classes:,Liberated symptoms When the higher centers were impaired, the function of the lower center that normally controlled by the former was liberated(pyramidal signs锥体束征).,Symptoms of Nervous System could divided to four classes:,Shock Symptoms CNS急性局部严重病变，引起与之功能相关的远隔部位神经功能短暂缺失 Brain shock: cerebral hemorrhage Spinal shock: in the acute stage of total cord transverse, there is a flaccid paralysis with loss of tendon and other reflexes, accompanied by sensory loss below the level of the lesion and by urinary and fecal retention.,Supplemented exam in neurological diseases,1. Lumbar puncture and CSF analysis: Appearance, Pressure, Dynamics Routine exam Biochemical examinations CSF-IgG index, OB Cytologic exam Specific antibodies(MBP, AChR),Supplemented exam,2. Imaging studies: plain X-rays of the skull and the spine, myelography CT, MRI (magnetic resonance imaging), MRA DSA (digital subtraction angiography),Supplemented exam,3. Electrophysiologic studies: EEG(electroencephalography) EMG (electromyography) NCV(nerve conduction velocity) VEP(visual evoked potentials) BAEP(brianstem auditory evoked potentials) SEP(somatosensory evoked potentials) 4. Transcranial doppler(TCD),Supplemented exam,Radioisotope examinations(放射性同位素) SPECT(single photon computed tomography) PET(positron emission tomography) Immunologic and virologic detections(免疫学及病毒学检测)：such as MBP、AChR and cysticercus antibodies, (HSV)PCR Biopsy: muscles, nerves and brain,Chapter 2. Symptomatology of the Neurological Diseases,Section 1. Disorders of Consciousness,Disturbances of the Level of Consciousness,Consciousness is awareness of the internal or external world. 意识(awareness) 指大脑的觉醒(arousal)程度，是机体对自身和周围环境的感知和理解功能，并通过语言、躯体运动和行为表达出来；是CNS对内、外环境刺激应答反应的能力。 该能力减退或消失就意味着不同程度的意识障碍(disorders of consciousness)。,意识(consciousness)-Concept,Consciousness describes that sets of neural processes that allow an individual to perceive, comprehend, and act upon the internal and external environments. It is usually envisioned in two parts: arousal and awareness.,Arousal describes the degree to which the individual appears to be able to interact with these environments; the contrast between waking and sleeping is a common example of two different states of arousal.,Arousal requires the interplay of both the reticular formation and the cerebral hemispheres. The reticular components necessary for arousal reside in the midbrain and diencephalon; the pontine reticular formation is not necessary for arousal.,Awareness reflects the depth and content of the aroused state. Awareness is dependent on arousal, since one who cannot be aroused appears to lack awareness. Awareness does not imply any specificity for the modality of stimulation. This stimulation may be external (e.g., auditory) or internal (e.g., thirst).,Attention depends on awareness and implies the ability to respond to particular types of stimuli (modality-specific).,Stupor refers to a condition in which the patient is less alert than usual, but can be stimulated into responding.,Obtundation (意识模糊) describes a patient who appears to be asleep much of the time when not being stimulated. This eyes-closed state is not electroencephalographic sleep, however.,Stuporous/obtunded patients will respond to noxious stimuli by attempting to deflect or avoid the stimulus.,Patient with Coma lies with eyes closed and does not make an attempt to avoid noxious stimuli. Such a person may display various forms of reflex posturing, but does not actively try to avoid the stimulus.,Vegetative state, in which the eyes open and close, the patient may appear to track objects about the room, and may chew and swallow food placed in the mouth.,However, the vegetative patient does not respond to auditory stimuli, and does not appear to sense pain, hunger, or other stimuli. This is a state in which there is arousal but no awareness.,Delirium is defined as a disturbance of consciousness that is accompanied by a change in cognition that cannot be better accounted for by a preexisting or evolving dementia.,The disturbance develops over a short period of time, usually hours or days, and tends to fluctuate during the course of the day.,There is evidence from the history, physical examination, or laboratory tests that the delirium is a direct physiological consequence of a general medical condition, substance intoxication or withdrawal, use of a medication, or toxin exposure, or a combination of these factors.,Disorders of Consciousness,Anatomical basis of alerting system(维持意识清醒的重要结构): 脑干上行性网状激活系统 (ascending reticular activating system) 广泛的大脑皮质神经元的完整性 (Cerebral cortex and the afferent pathways) (中枢整合机构) The maintenance of consciousness requires a fine balance of activity between the cerebral cortex and the reticular system.,Disorders of consciousness -Clinical classification,意识障碍：指意识水平下降 嗜睡(somnolent): 患者处于睡眠状态，唤醒后定向力基本完整，但注意力不集中，记忆稍差，如不继续对答，又进入睡眠。 The early stage of consciousness disorder, it is often a feature of raised intracranial pressure.,Disorders of consciousness -Clinical classification,昏睡状态(stupor)：处于较深睡眠状态，较重的疼痛或言语刺激方可唤醒，作简单模糊的回答，旋即熟睡。 The patient can be roused only briefly by pain stimulation or loud speech.,Disorders of consciousness- Clinical classification 昏迷(coma): the patient is unresponsive and unarousable) 意识丧失，对言语刺激 无应答反应， 可分为浅、中、深昏迷。,Disorders of consciousness -Clinical classification,Disorders of consciousness affecting the contents of consciousness 意识模糊(confusion)或朦胧状态(twilight state) 意识轻度障碍，表现意识范围缩小，常有定向力障碍，突出表现是错觉，幻觉较少见，情感反应与错觉相关，可见于癔症。,Disorders of consciousness -Clinical classification,Disorders of consciousness affecting the content of consciousness 谵妄状态(delirium state) 定向力(orientation)、自知力障碍，注意力涣散(attention)，不能与外界正常接触。常有hallucinations、delusions，以错视为主，形象生动逼真，可有恐惧、外逃或伤人行为。 Acute: fever, intoxication such as Atropine Chronic: chronic alcoholism,Disorders of consciousness -Clinical classification,特殊类型意识障碍-醒状昏迷(coma vigil) 1. 去皮层综合征(decorticate) 无意识睁眼闭眼，光、角膜反射(corneal reflex)存在，对外界刺激无反应，去皮层强直状态(decorticate rigidity)，病理征(+) 上行网状激活系统未受损，保持觉醒-睡眠周期，无意识咀嚼和吞咽 缺氧性脑病、大脑皮质广泛损害CVD及外伤等,Disorders of consciousness -Clinical classification,2. 无动性缄默症(akinetic mutism)：对外界刺激无意识反应，四肢不能动，不语。无目的睁眼或眼球运动，睡眠-醒觉周期可保留。伴自主神经功能紊乱，体温高、心跳或呼吸节律不规则、多汗、尿便潴留或失禁，无锥体束征。 脑干上部或丘脑网状激活系统及前额叶-边缘系统损害。,Disorders of consciousness -Clinical classification,Differential diagnosis (1) 意志缺乏症 清醒状态，但不讲话，无自主活动。对刺激无反应、无欲望，严重淡漠状态。双侧额叶病变。,闭锁综合征(locked-in syndrome),脑桥基底部病变，皮质核束&皮质脊髓束双侧受损(Lacunar infarct, Multiple sclerosis) 表现几乎全部运动功能丧失 Quadriplegic cranial nerves palsy that come from pons or below the pons,闭锁综合征(locked-in syndrome),They are conscious by opening their eyes or moving their eyes vertically on command, but they are speechless, motionless and they cant swallow.,Section 2. Aphasia,Aphasia-Concept,失语症(aphasia)：脑损害所致的语言交流能力障碍，后天获得性各种语言符号(口语、文字、手语等)表达及认识能力受损或丧失。 Points for diagnosis： Alert, normal mental state, no severe intelligent disturbances No visual and auditory deficits, no palsy or ataxia on the muscles of vocal organs (mouth, pharynx and larynx),Aphasia-classification,目前国内常用的失语症分类 外侧裂周围失语综合征 共同点：病灶都在外侧裂周区, 共同特点是均有复述障碍(repetition disorder)。 -Broca 失语(Broca aphasia, BA) -Wernicke失语(Wernicke aphasia, WA) -传导性失语 (conduction aphasia, CA）,Aphasia-classification,经皮层性失语(transcortical aphasia)-分水岭区失语综合征 病灶位于分水岭区, 共同特点是复述相对保留。 经皮层运动性失语(transcortical motor aphasia, TCMA) 经皮层感觉性失语(transcortical sensory aphasia, TCSA) 经皮层混合性失语(mixed transcortical aphasia, MTA),Aphasia-classification,完全性失语 (global aphasia, GA) 命名性失语 (anomic aphasia, AA) 皮层下失语综合征 (subcortical aphasia syndrome) 丘脑性失语 (thalamic aphasia, TA) 底节性失语 (basal ganglion aphasia, BaA),Broca Aphasia -Clinical features,obvious expression disturbance typically nonfluent, paucity of speech, difficult to talk, difficult to give words, telegraphic, loss of grammar, disorders of repetition, naming, reading and writing.,Broca Aphasia -Lesions,Broca aphasia 累及优势半球Broca区(额下回后部) 相应皮层下白质 脑室周围白质及顶叶 岛叶损害,Wernicke Aphasia -Clinical features,no comprehension fluent，dash along，speech no difficult clear pronunciation，normal tune a lot semantic paraphasia(语义错语，如帽子袜子) neologism (新语)，答非所问 与理解一致的复述、听写障碍(dictation disorder),Wernicke Aphasia -Lesions,Wernicke aphasia 位于优势半球Wernicke区 (颞上回后部),Conduction Aphasia -Clinical features,repetition lost n lost(不成比例地) preserved spontaneous speech normal understanding 不能讲出自发讲话时较易说出的词或句子，或以错语复述-语音错语(铅笔“先北”)，找词困难、犹豫、停顿,Conduction Aphasia -Lesions,优势半球缘上回皮质或深部白质内弓状纤维,Transcortical Aphasia -Clinical features,复述较其它语言功能好，甚至是不成比例地好,Transcortical Aphasia -Lesions,因病变部位不同，临床表现亦不同，临 床特点及病变部位如表2-3,Anomic Aphasia -Clinical features,以命名不能为主要特征 呈选择性命名障碍，找词困难，赘语 在所给的供选择名称中能选出正确的名词,Anomic Aphasia -Lesions,在优势半球颞中回后部或颞枕交界区,Global Aphasia -Clinical features,所有语言功能 口语、听理解、复述、命名、阅读、书写均严重障碍 表现为哑，刻板性语言（吗、吧、哒等）,Global Aphasia -Lesions,优势半球大范围病变，如大脑中动脉区大病灶,Subcortical Aphasia -Clinical features,皮层下病变产生失语较皮质病变少见，症状不典型，但仔细观察仍可发现其特点 Classification: -thalamic aphasia：表现为音量小、语调低、表情淡漠、不主动讲话，且有找词困难，可伴错语。 -basal ganglion aphasia：表现自发性言语受限、音量小、语调低,Apraxia-Concept,Apraxia: inability to perform previously learned task 企图作有目的或细巧动作时，不能准确执行所了解的随意性动作。如不能按要求做伸舌、吞咽、洗脸、刷牙、划火柴和开锁等简单动作 但病人在不经意时却能自发地做这些动作 脑部疾患时，患者无瘫痪、共济失调、肌张力障碍和感觉障碍，无意识及智能障碍 病变部位多在左侧缘上回,Agnosia -Concept,Agnosia：不能通过某种感觉辨认以往熟悉的物体，却能通过其它感觉通道识别 如看到手表不知为何物，触摸表外形或听表走动声音，可知是手表 脑损害时，无视觉、听觉、触觉、智能及意识障碍。是少见的神经心理学障碍,Agnosia-classification,Tactile Agnosia: cannot recognize a familiar objects placed in his hands if his eyes are closed. Visual Agnosia: there is impairment of recognition of familiar objects,symbols or persons Auditory Agnosia: cannot appreciate the significance of well known sounds,Disturbances of Vision and Eye Movements,Section 3.,Disturbances of Vision -Anatomy and Physiology,Visual pathways：retinaoptic nerveoptic chiasm(a)optic tractlateral geniculate nucleioptic radiationscalcarine cortex(posterior poles of the occipital lobes) 视神经、视束及视放射纤维均按严格的排列顺序与视网膜的每一点有精确的对应关系。 视交叉处视神经纤维的重组则成为偏盲或象限盲的基础如图2-2。,Disturbances of Vision -Anatomy and Physiology,Decreased visual acuity in one eye: Acute loss of vision: obstruction of the ophthalmic artery or the central retinal artery) Transient monocular blindness: TIA of internal carotid artery, ocular migraine Progressive loss of vision: several hours, days (ON、MS) 不规则视野缺损，之后视力障碍或失明 compressive lesions on the optic nerves: tumors, aneurysm, Foster-Kennedy syndrome,Disturbances of Vision -Anatomy and Physiology,Decreased visual acuity in both eyes: Transient recurrent amaurosis: TIA of visual centers on bilateral occipital lobes, obstruction can led to cortical blindness (positive pupillary reaction to light ) Progressive blindness： intoxication optic neuropathy caused by innutrition primary optic atrophy papilledema (tumor, hemorrhages, inflammation, increased intracranial pressure),Disturbances of Vision -Anatomy and Physiology,Visual field defects Anatomical bases:图2-2 双颞侧偏盲(Bitemporal hemianopia) 垂体瘤、颅咽管瘤等使视交叉中部受损.,Disturbances of Vision -Anatomy and Physiology,对侧同向性偏盲(homonymous hemianopia ) Clinical features： 双眼病变对侧视野的同向偏盲 Lesions：lateral geniculate bodies, whole damage of optic radiation and calcarine cortex 图2-2,Disturbances of Vision -Anatomy and Physiology,对侧视野同向象限盲(homonymous quadrantanopia) 双眼同向上象限盲(homonymous superior quadrantanopia):见于颞叶后部病变使视辐射下部受损所致 双眼对侧视野同向下象限盲(homonymous inferior quadrantanopia):见于顶叶病变(肿瘤或血管病)使视辐射上部受损引起,Eye Movement Disorders- Clinical features,解剖生理基础:图2-3 眼肌麻痹(ocular palsy) Peripheral ocular palsy 动眼神经麻痹(oculomotor nerve palsy): ptosis, outward deviation, diplopia, 瞳孔散大、光反射消失，loss of reaction to accommodation,Eye Movement Disorders- Clinical features,解剖生理基础:图2-3 眼肌麻痹(ocular palsy) Peripheral ocular palsy trochlear nerve palsy：The superior oblique muscle palsy, diplopia is most pronounced when the patient looks downward abducens nerve palsy：inward deviation, failure of attempted abduction, diplopia,Eye Movement Disorders- Clinical features,Nuclear ophthalmoplegia 合并邻近神经结构损害：展神经核受损常累及面神经和锥体束等. 产生分离性眼肌麻痹：动眼神经核性损害更可选择性损害个别眼肌,也可累及双侧眼肌。 The lesions are usually vascular diseases of brain stem, inflammatory diseases, or tumors.,Eye Movement Disorders- Clinical features,核间性眼肌麻痹(internuclear ophthalmoplegia) 前核间性眼肌麻痹 Lesions: lesions lie in the medial longitudinal fasciculus, an unilateral ascending pathway in the brain stem 图2-4 Clinical features: On lateral gaze, excursion of the abducting eye is full(with nystagmus or not), but adduction of the contralateral eye is impaired. Convergence is preserved.,Eye Movement Disorders- Clinical features,后核间性眼肌麻痹 一侧内侧纵束下行纤维受损 On lateral gaze, the adduction of the contralateral eye is full, but the abduction of the ipsilateral eye is impaired.,Eye Movement Disorders- Clinical features,一个半综合征(one and a half syndrome) 一侧脑桥被盖部病变引起该侧副外展神经核或PPRF受损， A symptom that combines internuclear ophthalmoplegia with an inability to gaze towards the side of the lesion. 图2-4,Eye Movement Disorders- Clinical features,中枢性-核上性眼肌麻痹(Supranuclear opthalmoplesia) Lesions：皮层眼球水平同向运动中枢(lateral gaze center) Clinical features：palsy of conjugate horizontal movement 双眼水平同向运动障碍即凝视麻痹(gaze palsy)，即双眼向病灶侧凝视 刺激性病灶引起双眼向病灶对侧的同向偏斜 图2-4,Eye Movement Disorders- Clinical features,帕里诺(Parinaud syndrome) Clinical features: up gaze paralysis Lesions：上丘眼球垂直同向运动皮质下中枢损害,Eye Movement Disorders- Clinical features,Pupillary abnormalities anatomy and physiology Pupils size: In a brightly illuminated examining room, normal pupils are 24mm in diameter in adults. 瞳孔调节: 支配瞳孔括约肌的动眼神经副交感纤维 支配瞳孔散大肌的来自superior cervical ganglion交感纤维共同调节,Eye Movement Disorders- Clinical features,Pupillary light reflex: Pathway: retinaoptic nervesoptic chiasmaoptic tractpretectal area(中脑顶盖前区) Edinger-Westphal nucleusoculomotor nervesciliary ganglion(睫状神经节)postgangliar fiberspupillary constrictor muscles(瞳孔括约肌) 光反射传入纤维，外侧膝状体之前视觉径路病变、中脑病变、传出纤维即动眼神经损害均可使光反射减弱或消失,Eye Movement Disorders- Clinical features,Reaction to accommodation(调节反射, 集合反射) When the eyes converge to focus on a nearer object, the pupils normally constrict 缩瞳反应和会聚动作不一定同时受损，调节反射路径尚不确切 阿罗(Argyll-Robertson)瞳孔 negative pupillary light reflex, positive accommodation reflex 顶盖前区光反射径路受损所致 neurosyphilis is the usual cause.,Eye Movement Disorders- Clinical features,艾迪瞳孔 又称强直性瞳孔(tonic pupil) Clinical features: Unilateral larger pupil, reacts sluggishly and only to persistent bright light 光照停止后瞳孔缓慢散大。调节反射同样缓慢出现，缓慢恢复,Eye Movement Disorders- Clinical features,霍纳征(Horner sign) clinical features: unilateral small pupil(myosis), ptosis(眼裂变小: 睑板肌麻痹)、 enophthalmus(眼球内陷: 眼眶肌麻痹)， lack of sweating in the ipsilateral face. Lesions：见于颈上交感神经径路损害及脑干网状结构的交感纤维损害(图2-5) 。,Section 4. Vertigo and Auditory Disorders,Vertigo-concept,眩晕(vertigo) is the illusion of movement of the body or the environment. 患者主观感觉自身或外界物体呈旋转感或升降、直线运动、倾斜、头重脚轻等感觉。是对自身平衡觉和空间位象觉的自我体会错误 头晕(dizziness) 常缺乏自身或外界物体的旋转感，sensations of light-headedness, faintness or giddiness,Vertigo-Clinical features and classification,1. Systemic vertigo Etiology：caused by lesions on vestibule system, main cause of vertigo, accompanied by equilibrium disorder, 眼球震颤 and dysaudia。 (1) Peripheral vertigo (真性眩晕) 病变见于前庭感受器及前庭神经颅外段(未出内听道)，如迷路炎、中耳炎、前庭神经元炎、内耳眩晕症(Meniere病)等。,Vertigo-Clinical features and classification,(2) Central vertigo (假性眩晕) 病变在前庭神经颅内段、前庭核（vestibular nuclei）、核上纤维、内侧纵束及皮质和小脑的前庭代表区 图2-6 Usually occur in transient ischaemia of vertibro-basal arteries; tumors in cerebellum, brain stem, and the fourth ventricle; increased intracranial pressure; auditory neuroma; epilepsy et al. 系统性眩晕的鉴别,Vertigo-Clinical features and classification,2. Non-systemic vertigo etiology：caused by other somatic diseases, for instance eye diseases, anemia, hematonosis, heart failure, infection, intoxication and neurasthenia(神经功能失调), and so on. features：是头晕眼花或轻度站立不稳，无眩晕感，seldom accompanying nausea、vomiting，no nystagmus.,Vertigo-Clinical features and classification,耳聋 (deafness) Conductive deafness (传音性耳聋) 外耳道和中耳病变，如外耳道异物或耵聍、骨膜穿孔和中耳炎等。 Perceptive deafness (感音性耳聋) 内耳、听神经、蜗神经核核上听觉通路病变所致 Mixed hearing loss 传导性及神经性耳聋同时存在,Auditory Disorders -Clinical features,耳鸣(tinnitus) Concept: 无外界声音刺激，患者却主观听到持续性声响。 Lesions: 是由听感受器及其传导径路病理性刺激所致的主观性耳鸣。,Auditory Disorders -Clinical features,听觉过敏 (acoustic hyperesthesia, hyperacusis) Concept 声音呈病理性增强，即患者感觉到的声音较真正听到的强。 Lesions 常见于面神经麻痹时，因镫骨肌瘫痪使微弱的声波振动即导致内淋巴强烈震荡而引起。,Section 5. Syncope and Seizure,Syncope -Concept,晕厥(syncope) Pathogenesis: The loss of consciousne