1. 运动障碍疾病 Movement Disorders
Neurology Department
The Second Affiliated Hospital
Harbin Meical University

2. Extrapyramidal Disorders
General Conception: The dysfunction of basal ganglia
putamen globus pallidus lentiform nucleus caudate nucleus subthalamus corpus striatum substantia nigra basal ganglia

3. General Conception Two types of symptoms
Muscular hypotonus associated with hyperkinesia(F1)
Muscular hypertonus characterized with hypokinesia(F2)

4. Parkinson’s disease---conception
Also called paralysis agitans.
a common degeneration disease in nervous system
becomes increasingly common with advancing age
characterized by rest tremor, bradykinesia, rigidity, abnormal gait and posture

5. Parkinson’s 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 death
Genetic factor: alpha-synuclein gene -----susceptibility

6. Parkinson’s disease---Pathology
loss of pigmentation and cells in the substantia nigra
Lewy body in the basal gangalia, sympathetic ganglia and brainstem.

7. Parkinson’s disease---Biochemistry
L- tyrosine (blood) TH L-Dopa DDC DA MAO and COMT HVA
Nigrostriatal pathway is the most important.
dopamine depletion dopaminergic nigrostriatal system
Extrapyramidal Pathway

8. dopamine acetylcholine

9. Parkinson disease’s---Clinical findings
Over middle aged & insidious onset and gradual progression.
Tremor: often appear as the first sign.
Characters: 4~6Hz, conspicuous at rest, increase at times of emotional stress, improves during voluntary activity, stop during sleep, begin from hand , “N” progression.

10. Parkinson disease’s---Clinical findings
Rigidity(F1/F2): lead pipe / cogwheel phenomenon. Minimal rigidity: move the contralateral limb, head dropping test, road mark sign

11. Parkinson disease’s--Clinical findings
Bradykinesia: 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(late sign).

12. Parkinson disease’s--Clinical findings
Other features: Myerson’s sign, oily face, intractable constipation, postural hypotension, cognitive disturbance, hallucination, depression

13. Parkinson’s disease---Differential diagnosis
Parkinsonism: postencephalitic parkinsonism, drug or toxin-induced parkinsonism(CO, Mn), arteriosclerotic parkinsonism.
Depression: a trial of antidepressant drug treatment may be helpful.

14. Parkinson’s disease---Differential diagnosis
Essential(benign, familial) tremor: family history, early onset, nod or head shake.
Parkinsonism associated with other neurologic diseases: Diffus Lewy body disease(hallucination), Wilson disease, Huntington chorea, multiple system atrophy(MSA), PSP, cortical basal ganglionic degeneration(CBGD).

15. Parkinson’s disease---Treatment
Begin from low dose and individualized. Anticholinergic drugs: helpful in alleviating tremor and rigidity. Artane(1~2mg, tid).
Amantadine: for mild parkinsonism. 50mg, bid~tid.
Levodopa: L-Dopa/DCI(4:1)---compound; side effects: nausea, hypotension(peripheral) dyskinesia and motor fluction(central)
wearing off or on-off phenomenon

16. Parkinson’s disease---Treatment
DA-R agonist: bromocriptine
MAOB/COMT inhibitor
Surgery: pallidotomy and deep brain stimulation(high frequency thalamic stimulation)
Physical therapy and aids for daily living
Prognosis dead from complications

17. Sydenham Chorea--- Conception
Also called rheumatic chorea. A usual manifestation of rheumatic fever in nervous system. Presents in childhood or young adult life. Clinical findings involuntary chorea dystonia weakness automatic action disturbance emotional changes

18. Sydenham 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 changes.

19. Sydenham Chorea--- Clinical findings
5~15ys.
G/B=3:1
Subacute or insidious onset
Early symptom: irritability, inattention, unsteady gait, easily dropping objects, et al.

20. Sydenham Chorea--- Clinical findings
Chorea: rapid, irregular, involuntarily, unpredictably
obvious on face( facial grimacing and tongue movements)
prominent when nervous and disappeared during sleep.

21. Sydenham Chorea--- Clinical findings
Muscle 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-limitation

22. Sydenham Chorea--- Testing
1.Serum test: rapid erythrocyte sedimentation, increased wbc EEG: unspecific. 3. Image test: 29~85% patients present low density focus in caudate nucleus on CT.

23. Sydenham Chorea--- Diagnosis&Differential diagnosis
Onset age, triad , rheumatic fever findings
1. Habit spasm: stereotyped action on the same muscle 2. Congenital chorea: earlier onset
3. Tourette syndrome: tics on face accompanied by strange voice and dirty words.

24. Sydenham Chorea--- Diagnosis&Differential diagnosis
4.Huntington Chorea: onset after middle age, dementia, familial history
5. Torsion spasm: continuous, hypermyotonia when torsion happens

25. Sydenham Chorea--- Treatment & Prognosis
rest, keep quiet, avoid stimulation
penicillin or other antibiotics(10~14 days) and deltacortone(until the symptoms disapper)
apozepam(5mg, bid/tid), haloperidol(0.5~1mg, bid/tid).
side effects of extrapyramidal system
Self-limited 3~6 months after the onset

26. Wiloson Disease--- Conception & Etiology
Also 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

27. Wiloson Disease--- Clinical findings
childhood/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 psychology

28. Wiloson Disease--- Clinical findings
May progress rapaidly(younger); often gradually progress with periods of remission and exacerbation
Nonneurologic findings:
ocular and hepatic abnormalities

29. Wiloson Disease--- Differential diagnosis
multiple sclerosis
juvenile-onset Huntington disease

30. Wiloson Disease--- Investigative studies
Serum copper and ceruloplasmin(CP) are low, and 24h urinary copper excretion is generally increased.
Abnormal liver function blood test and aminoaciduria
Image test show cerebrocortical atrophy and abnormalities in the basal ganglia.

31. Wiloson Disease--- Treatment
D-Penicillamine(1~1.5g/d): allergic test
Restriction of dietary copper
zinc sulfate(200mg/d) decrease absorbance
Treatment must be continued for life. Early treatment can give a better recovery.