神经病学总论 16

Neurology,DepartmentofNeurology,The2ndaffiliatedhospital,kunmingMedicalcolleg,Introduction,神经病学(Neurology),TheObjectsofNeurology:CNS、PNSandmusculardisordersThecontentsofstudy:EtiologyandPathogenesisPathologyClinicalfeaturesDiagnosisandDifferentialdiagnosis,TreatmentandPreventionPrognosis,Nervoussystem,Centralnervoussystem:brainspinalcordPeripheralnervoussystem:cranialnervesspinalnerves,Nervoussystem,Neurologyisapartofneuroscience,including:Neuroanatomy,Neurophysiology,Neurobiochemistry,Neuropathology,Neurogenetics,Neuroimmunology,Neuroepidemiology,Neuroiconography,Neurophamacology,Neuropsychology,ExperimentalNeurology,Neurobiology,MolecularBiology,Catalogueoftheneurologicaldiseases,VasculardiseasesInfectiousdiseasesTumorsTraumaticdiseases,Catalogueoftheneurologicaldiseases,Autoimmunediseases(someofthemaredemyelinatingdiseases)HereditaryandmetabolicdisordersCongenitaldysplasiaIntoxicationNutritionaldisturbances,SymptomsofNervousSystemcoulddividedtofourclasses:,Deficitsymptomsdeficitsorlossonthenormalfunctions(hemiparalysis,aphasia)Irritativesymptomsexcessiveexcitementsthatnervousstructuresappearedwhentheywerestimulated(seizures,radicalpain),SymptomsofNervousSystemcoulddividedtofourclasses:,LiberatedsymptomsWhenthehighercenterswereimpaired,thefunctionofthelowercenterthatnormallycontrolledbytheformerwasliberated(pyramidalsigns).,SymptomsofNervousSystemcoulddividedtofourclasses:,ShockSymptomsCNS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Brainshock:cerebralhemorrhageSpinalshock:intheacutestageoftotalcordtransverse,thereisaflaccidparalysiswithlossoftendonandotherreflexes,accompaniedbysensorylossbelowthelevelofthelesionandbyurinaryandfecalretention.,Supplementedexaminneurologicaldiseases,1.LumbarpunctureandCSFanalysis:Appearance,Pressure,DynamicsRoutineexamBiochemicalexaminationsCSF-IgGindex,OBCytologicexamSpecificantibodies(MBP,AChR),Supplementedexam,2.Imagingstudies:plainX-raysoftheskullandthespine,myelographyCT,MRI(magneticresonanceimaging),MRADSA(digitalsubtractionangiography),Supplementedexam,3.Electrophysiologicstudies:EEG(electroencephalography)EMG(electromyography)NCV(nerveconductionvelocity)VEP(visualevokedpotentials)BAEP(brianstemauditoryevokedpotentials)SEP(somatosensoryevokedpotentials)4.Transcranialdoppler(TCD),Supplementedexam,Radioisotopeexaminations(放射性同位素)SPECT(singlephotoncomputedtomography)PET(positronemissiontomography)Immunologicandvirologicdetections(免疫学及病毒学检测)：suchasMBP、AChRandcysticercusantibodies,(HSV)PCRBiopsy:muscles,nervesandbrain,Chapter2.SymptomatologyoftheNeurologicalDiseases,Section1.DisordersofConsciousness,DisturbancesoftheLevelofConsciousness,Consciousnessisawarenessoftheinternalorexternalworld.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(disordersofconsciousness)。,意识(consciousness)-Concept,Consciousnessdescribesthatsetsofneuralprocessesthatallowanindividualtoperceive,comprehend,andactupontheinternalandexternalenvironments.Itisusuallyenvisionedintwoparts:arousalandawareness.,Arousaldescribesthedegreetowhichtheindividualappearstobeabletointeractwiththeseenvironments;thecontrastbetweenwakingandsleepingisacommonexampleoftwodifferentstatesofarousal.,Arousalrequirestheinterplayofboththereticularformationandthecerebralhemispheres.Thereticularcomponentsnecessaryforarousalresideinthemidbrainanddiencephalon;thepontinereticularformationisnotnecessaryforarousal.,Awarenessreflectsthedepthandcontentofthearousedstate.Awarenessisdependentonarousal,sinceonewhocannotbearousedappearstolackawareness.Awarenessdoesnotimplyanyspecificityforthemodalityofstimulation.Thisstimulationmaybeexternal(e.g.,auditory)orinternal(e.g.,thirst).,Attentiondependsonawarenessandimpliestheabilitytorespondtoparticulartypesofstimuli(modality-specific).,Stuporreferstoaconditioninwhichthepatientislessalertthanusual,butcanbestimulatedintoresponding.,Obtundation(意识模糊)describesapatientwhoappearstobeasleepmuchofthetimewhennotbeingstimulated.Thiseyes-closedstateisnotelectroencephalographicsleep,however.,Stuporous/obtundedpatientswillrespondtonoxiousstimulibyattemptingtodeflectoravoidthestimulus.,PatientwithComalieswitheyesclosedanddoesnotmakeanattempttoavoidnoxiousstimuli.Suchapersonmaydisplayvariousformsofreflexposturing,butdoesnotactivelytrytoavoidthestimulus.,Vegetativestate,inwhichtheeyesopenandclose,thepatientmayappeartotrackobjectsabouttheroom,andmaychewandswallowfoodplacedinthemouth.,However,thevegetativepatientdoesnotrespondtoauditorystimuli,anddoesnotappeartosensepain,hunger,orotherstimuli.Thisisastateinwhichthereisarousalbutnoawareness.,Deliriumisdefinedasadisturbanceofconsciousnessthatisaccompaniedbyachangeincognitionthatcannotbebetteraccountedforbyapreexistingorevolvingdementia.,Thedisturbancedevelopsoverashortperiodoftime,usuallyhoursordays,andtendstofluctuateduringthecourseoftheday.,Thereisevidencefromthehistory,physicalexamination,orlaboratoryteststhatthedeliriumisadirectphysiologicalconsequenceofageneralmedicalcondition,substanceintoxicationorwithdrawal,useofamedication,ortoxinexposure,oracombinationofthesefactors.,DisordersofConsciousness,Anatomicalbasisofalertingsystem脑干上行性网状激活系统(ascendingreticularactivatingsystem)广泛的大脑皮质神经元的完整性(Cerebralcortexandtheafferentpathways)(中枢整合机构)Themaintenanceofconsciousnessrequiresafinebalanceofactivitybetweenthecerebralcortexandthereticularsystem.,Disordersofconsciousness-Clinicalclassification,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。Theearlystageofconsciousnessdisorder,itisoftenafeatureofraisedintracranialpressure.,Disordersofconsciousness-Clinicalclassification,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。Thepatientcanberousedonlybrieflybypainstimulationorloudspeech.,Disordersofconsciousness-Clinicalclassification,昏迷(coma):thepatientisunresponsiveandunarousable)意识丧失，对言语刺激无应答反应，可分为浅、中、深昏迷。,Disordersofconsciousness-Clinicalclassification,Disordersofconsciousnessaffectingthecontentsofconsciousness意识模糊(confusion)或朦胧状态(twilightstate)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。,Disordersofconsciousness-Clinicalclassification,Disordersofconsciousnessaffectingthecontentofconsciousness谵妄状态(deliriumstate)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,intoxicationsuchasAtropineChronic:chronicalcoholism,Disordersofconsciousness-Clinicalclassification,特殊类型意识障碍-醒状昏迷(comavigil)1.去皮层综合征(decorticate)无意识睁眼闭眼，光、角膜反射(cornealreflex)存在，对外界刺激无反应，去皮层强直状态(decorticaterigidity)，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,Disordersofconsciousness-Clinicalclassification,2.无动性缄默症(akineticmutism)：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。,Disordersofconsciousness-Clinicalclassification,Differentialdiagnosis(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-insyndrome),脑桥基底部病变，皮质核束&皮质脊髓束双侧受损(Lacunarinfarct,Multiplesclerosis)表现几乎全部运动功能丧失Quadriplegiccranialnervespalsythatcomefromponsorbelowthepons,闭锁综合征(locked-insyndrome),Theyareconsciousbyopeningtheireyesormovingtheireyesverticallyoncommand,buttheyarespeechless,motionlessandtheycantswallow.,神经系统疾病：定位诊断和定性诊断(TopicalandE