运动障碍疾病MovementDisorders

1、运动障碍疾病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 symptomsn Muscular hypotonus associated with hyperkinesia(F1)n Muscular hypertonus characterized with hypokinesia(F2) Parkinsons disease-conceptionAlso called paralysis agitans. n a common degeneration disease in nervous systemn becomes increasingly common with advancing a

3、gen characterized 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 fa

4、ctor: alpha-synuclein gene -susceptibility Parkinsons disease-Pathologyv loss of pigmentation and cells in the substantia nigrav Lewy body in the basal gangalia, sympathetic ganglia and brainstem.Parkinsons disease-Biochemistryn L- tyrosine (blood) TH L-Dopa DDC DA MAO and COMT HVAn Nigrostriatal pa

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

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

7、signParkinson diseases-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. F

8、estinating gait(late sign).Parkinson diseases-Clinical findingsOther features: Myersons sign, oily face, intractable constipation, postural hypotension, cognitive disturbance, hallucination, depressionParkinsons disease-Differential diagnosisnParkinsonism: postencephalitic parkinsonism, drug or toxi

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

10、ogic diseases: Diffus 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 rigid

11、ity. Artane(12mg, tid).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 inhibitorS

12、urgery: pallidotomy 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

13、 young adult life. Clinical findings involuntary chorea dystonia weakness automatic action disturbance emotional changesSydenham Chorea- Etiology vRelated with the infection of Hemolytic Streptococcus A.v Anti-neuron antibodies can react with the neurons in caudate or subthalamic nucleus.v Maybe rel

14、ate with endocrine changes.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( facia

15、l grimacing and tongue 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 feve

16、r findings. Self-limitationSydenham 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 diagnosisnOnset age, triad , rheumatic fev

17、er findingsn1. Habit spasm: stereotyped action on the same muscle2. Congenital chorea: earlier onset n3. 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 h

18、istory5. Torsion spasm: continuous, hypermyotonia when torsion happensSydenham Chorea- Treatment & Prognosisnrest, keep quiet, avoid stimulationn penicillin or other antibiotics(1014 days) and deltacortone(until the symptoms disapper)n apozepam(5mg, bid/tid), haloperidol(0.51mg, bid/tid). side e

19、ffects of extrapyramidal system Self-limited 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 c

20、ircle on cornea Wiloson Disease- Conception & EtiologyWiloson Disease- Clinical findingsqchildhood/young adult lifeq 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 findingsqMay progress rapaidly(younger); often gradually progress with periods of remission