1、運動障礙疾病Movement Disorders Neurology Department The Second Affiliated Hospital Harbin Meical UniversityExtrapyramidal DisordersGeneral Conception: The dysfunction of basal ganglia putamen globus pallidus lentiform nucleus caudate nucleus subthalamus corpus striatum substantia nigra basal ganglia Gener

2、al Conception Two types of symptoms Muscular hypotonus associated with hyperkinesia(F1) Muscular hypertonus characterized with hypokinesia(F2) Parkinsons disease-conceptionAlso called paralysis agitans. a common degeneration disease in nervous system becomes increasingly common with advancing age ch

3、aracterized by rest tremor, bradykinesia, rigidity, abnormal gait and postureParkinsons disease-Etiology Aging: over middle aged persons Environmental toxin: MPTP(l-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine) in astrocyte oxidated MPP+ mitochondrion in substantia nigra neuronal deathGenetic factor:

4、alpha-synuclein gene -susceptibility Parkinsons disease-Pathology loss of pigmentation and cells in the substantia nigra Lewy body in the basal gangalia, sympathetic ganglia and brainstem.Parkinsons disease-Biochemistry L- tyrosine (blood) TH L-Dopa DDC DA MAO and COMT HVA Nigrostriatal pathway is t

5、he most important. dopamine depletion dopaminergic nigrostriatal system Extrapyramidal Pathway dopamine acetylcholine Parkinson diseases-Clinical findings Over middle aged & insidious onset and gradual progression.Tremor: often appear as the first sign. Characters: 46Hz, conspicuous at rest, increas

6、e at times of emotional stress, improves during voluntary activity, stop during sleep, begin from hand , “N” progression.Parkinson diseases-Clinical findingsRigidity(F1/F2): lead pipe / cogwheel phenomenon. Minimal rigidity: move the contralateral limb, head dropping test, road mark signParkinson di

7、seases-Clinical findingsBradykinesia: slowness and reduction of voluntary movement, difficult to initiate, masked face, hypophonia, micrographia.Abnormal gait and posture: flexed posture; difficult to get up, start walking, turn or stop. Shuffling gait and absence of the arm swing. Festinating gait(

8、late sign).Parkinson diseases-Clinical findingsOther features: Myersons sign, oily face, intractable constipation, postural hypotension, cognitive disturbance, hallucination, depressionParkinsons disease-Differential diagnosisParkinsonism: postencephalitic parkinsonism, drug or toxin-induced parkins

9、onism(CO, Mn), arteriosclerotic parkinsonism. Depression: a trial of antidepressant drug treatment may be helpful.Parkinsons disease-Differential diagnosisEssential(benign, familial) tremor: family history, early onset, nod or head shake. Parkinsonism associated with other neurologic diseases: Diffu

10、s Lewy body disease(hallucination), Wilson disease, Huntington chorea, multiple system atrophy(MSA), PSP, cortical basal ganglionic degeneration(CBGD).Parkinsons disease-TreatmentBegin from low dose and individualized.Anticholinergic drugs: helpful in alleviating tremor and rigidity. Artane(12mg, ti

11、d).Amantadine: for mild parkinsonism. 50mg, bidtid.Levodopa: L-Dopa/DCI(4:1)-compound; side effects: nausea, hypotension(peripheral) dyskinesia and motor fluction(central) wearing off or on-off phenomenonParkinsons disease-Treatment DA-R agonist: bromocriptineMAOB/COMT inhibitorSurgery: pallidotomy

12、and deep brain stimulation(high frequency thalamic stimulation)Physical therapy and aids for daily living Prognosis dead from complicationsSydenham Chorea- ConceptionAlso called rheumatic chorea. A usual manifestation of rheumatic fever in nervous system. Presents in childhood or young adult life. C

13、linical findings involuntary chorea dystonia weakness automatic action disturbance emotional changesSydenham Chorea- Etiology Related with the infection of Hemolytic Streptococcus A. Anti-neuron antibodies can react with the neurons in caudate or subthalamic nucleus. Maybe relate with endocrine chan

14、ges.Sydenham Chorea- Clinical findings515ys.G/B=3:1Subacute or insidious onsetEarly symptom: irritability, inattention, unsteady gait, easily dropping objects, et al.Sydenham Chorea- Clinical findingsChorea: rapid, irregular, involuntarily, unpredictably obvious on face( facial grimacing and tongue

15、movements) prominent when nervous and disappeared during sleep.Sydenham Chorea- Clinical findingsMuscle tone and power: reduced. Triad: limb weakness, chorea and ataxia. milkmaid grasp, wax-waning sign, dancing gait. Psychiatric symptom: agitation, hallucination Rheumatic fever findings. Self-limita

16、tionSydenham Chorea- Testing 1.Serum test: rapid erythrocyte sedimentation, increased wbc. 2. EEG: unspecific.3. Image test: 2985% patients present low density focus in caudate nucleus on CT.Sydenham Chorea- Diagnosis&Differential diagnosisOnset age, triad , rheumatic fever findings1. Habit spasm: s

17、tereotyped action on the same muscle2. Congenital chorea: earlier onset 3. Tourette syndrome: tics on face accompanied by strange voice and dirty words.Sydenham Chorea- Diagnosis&Differential diagnosis4.Huntington Chorea: onset after middle age, dementia, familial history5. Torsion spasm: continuous

18、, hypermyotonia when torsion happensSydenham Chorea- Treatment & Prognosisrest, keep quiet, avoid stimulation penicillin or other antibiotics(1014 days) and deltacortone(until the symptoms disapper) apozepam(5mg, bid/tid), haloperidol(0.51mg, bid/tid). side effects of extrapyramidal system Self-limi

19、ted 36 months after the onsetAlso called hepatolenticular degeneration(HLD) A kind of inherited disease extrapyramidal sign Autosomal recessive cirrhosis inheritance disease psychiatric symptom of copper metabolism kidney impairment disturbance-Etiology K-F circle on cornea Wiloson Disease- Concepti

20、on & EtiologyWiloson Disease- Clinical findingschildhood/young adult life reflect the disproportionate involvement of the caudate nucleus, putamen, cerebral cortex and cerebellum. resting/postural tremor, rigidity, facial grimacing, ataxia, choreiform movements of limbs, disorders of affect , behavior , personality or psychologyWiloson Disease- Clinical findingsMay progress rapaidly(younger); often gradually progress with periods of remissio