Extrapyramidal diseases

1. Extrapyramidal diseases

2. Ⅰ.Formation of basal ganglia *Corpus striatum:/caudate nucleus \ neostriatum \lentiform/putamen nucleus / nucleus\globus pallidus- paleostriatum *Red nucleus *Substantia nigra *Subthalamic nucleus

3. Ⅱ Function Regulation of voluntary motor activity

4. Ⅲ.clinical features 1.Poverty of movement-hypertonia syndrome position: substantia nigra ;globus pallidus ex : Parkinson’ s disease 2.Increasing of movement-hypotonia syndrome position: caudate nucleus ;putamen nucleus ex : Chorea minor

5. Parkinson’s Disease

6. Ⅰ.Definition It is also called paralysis agitans. It is a slowly progressive degeneration disease of the extrapyramidal system. It is characterized by tremor、rigidity、bradykinesia 、abnormal gait and attitude.

7. Ⅱ.Etiology and Pathogenesis 1.age:>40years old \ 2.Environmental factors :MPTP degeneration of DA neurons 3.Genetic factor / (>50%)

8. Ⅲ.Pathology and biochemical pathology 1.Pathology (1)Macroscopic changes: no special changes (2)Microscopic changes:widespread cellular degeneration, in substantia nigra, Lewy’s body

9. 2.Biochemical pathology HVA  tyrosine ↓ dopadecar ↓ decomposition hydroxylase - boxylase ↑MAO,COMT Levo-tyrosine →→→→ Levodopa →→→→ dopamine ↓ ↓↘ Caudate nucleus ←←←←←←←←←↓ ↘ ↙Substantia nigra- Ach ↑ putamen↙ corpus striatum path ↓ nucleus ↓ clinical features

10. Ⅳ.Clinical Features 1.tremor:usually first occur(60-70%) “pill-rolling” static tremor : the tremor is present when the patient is at rest. When the patient is nervous ,the tremor increase . When the patient move voluntarily or sleep , the tremor is suppressed or disappears entirely.

11. 2.rigidity Cog-wheel rigidity lead-pipe rigidity

12. 3.bradykinesia (1).Voluntary movements are reduced (2).Masked face (3).micrographia

13. 4.Abnormal gait and attitude (1).generalized flexion (2).festinating gait

14. 5.Other symptoms (1).autonomic disturbances : hypersteatosis:oily face; hyperhidrosis; constipation sphincter disturbance is rare (2).mental symptoms: dementia ;depression (3).dysarthria (4).salivation

15. Ⅴ.Diagnosis 1.It occurs in old people ,the onset is insidious and progress gradually. 2.Clinical features: tremor 、rigidity 、 bradykinesia、abnormal attitude and gait .

16. Ⅵ.Differential Diagnosis 1.Secondary parkinson’s disease (Parkinsonism) (1).infection: encephalitis lethargica (2).poisoning: CO,Mn (3).drugs (4).cerebral arteriosclerosis (5).trauma

17. 2.Others (1).Depression (2).Essential tremor (3).multiple system atrophy: OPCA (4).thyrotoxicosis,alcoholism.

18. Ⅶ.Treatment 1.Drug treatment (1).anticholinergic drugs Artane 2mg tid po;kemadrin 2.5mg tid po Side-effects: retention of urine; enlarged pupil; hypohidrosis; confusion Contraindications:hypertrophy of prostrate glaucoma

19. (2).amantadine: 50mg tid po Side-effects :insomnia ; confusion; hallucination Contraindications: epileptic ;hepatic and renal dysfunction

20. (3)Levodopa L-dopa + dopadecarboxylase inhibitor(DCI) Ex : Madopar ; Sinemet

21. Side-effects 1.Peripheral side-effects: nauea ;vomiting Postural hypotension; cardiac arrhythmia

22. 2.Central side-effects (1).motor fluctuation a.End of dose deterioration b.on-off phenomenon (2).dyskinesia a.peak-dose dyskinesia b.Biphasic dyskinesia c.Dystonia (3).mental symptoms:confusion;hallucination

23. (4)Dopamine receptor stimulant drug: bromocriptine(5)monoamine oxidase B inhibitor(6)COMT inhibitor 2.Surgical treatment 3.Cell transplantation and gene therapy 4.Rehabilitation treatment

24. Chorea minor

25. Ⅰ.Definition It is also called Sydenham chorea. It is a common nervous system manifestation of rheumatic fever, it often occurs in children . It is characterized by involuntarily choretic movements、 hypotonia、weakness、 mental symptoms.

26. Ⅱ Etiology Infection of A hemolytic streptococus

27. Ⅲ.Clinical features 1.onset:5-15 years old ; F>M; subacute or insidious 2.Choretic movements 3.Muscular tension and power are reduced 4.Mental symptoms 5.Symptoms of rheumatic fever: cardiac disease; fever; WBC↑;rheumatic arthritis; subcutaneous nodule ;blood sedimentation↑;ASO↑

28. Ⅳ.Diagnosis 1.The age of onset 2.Clinical features 3.Symptoms of rheumatic fever

29. Ⅴ.Treatment 1.Etiological treatment Penicillin 10-14 days 2.Symptomatic treatment Valii; luminal; chlorpromazine

30. Hepatolenticular Degeneration (HLD)

31. Ⅰ.Definition It is also called Wilson’s disease.It is an autosomal recessive inheritant disease caused by copper metabolic disorder. The affected areas mainly are liver and basal ganglia. It is characterized by progressive extrapyramidal symptoms、cirrhosis of the liver 、mental symptoms and K-F ring.

32. Ⅱ.Etiology and Pathogenesis  Copper  + 2 globulins  ceruloplasmin enzyme   bile,urine,sweet    liver cirrhosis of the liver abnormal  basal ganglia  extrapyramidal deposition symptoms  kidney  renal dysfunction  cornea  K-F ring

33. Ⅲ.Pathology Degeneration: liver basal ganglia kidney cornea

34. Ⅳ.Clinical Features 1.nervous symptoms (1)extrapyramidal symptoms: choretic action;tremor;rigidity;bradykinesia (2)mental symptoms:intelligent deficiency 2.symptoms of cirrhosis of the liver 3.K-F ring: 95% 4.others: renal dysfunction

35. Ⅴ.Investigation 1.ceruloplasmin of serum:↓0.26-0.36g/L 2.copper oxidase activity:↓

36. Ⅵ.Diagnosis 1.the symptoms of liver and extrapyramidal system. 2.the levels of CP and copper oxidase activity are reduced. 3.K-F ring of cornea 4.positive family history

37. Ⅶ.Treatment 1.low-copper diet 2.drug treatment first -choice: D-penicillamine 3.symptomatic therapy 4.surgical treatment