Extrapyramidal diseases

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

Ⅱ Function Regulation of voluntary motor activity

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

Parkinson’s Disease

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.

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

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

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

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

2.rigidity Cog-wheel rigidity lead-pipe rigidity

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

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

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

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.

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

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

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

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

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

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

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

(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

C horea minor C horea minor

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.

Ⅱ Etiology Infection of A hemolytic streptococus

Ⅲ.Clinical features 1.onset:5-15 years old ; F>M; subacute or insidious 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 disease; fever; WBC↑;rheumatic arthritis; subcutaneous nodule ;blood arthritis; subcutaneous nodule ;blood sedimentation↑;ASO↑ sedimentation↑;ASO↑

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

Ⅴ.Treatment 1.Etiological treatment 1.Etiological treatment Penicillin days Penicillin days 2.Symptomatic treatment 2.Symptomatic treatment Valii; luminal; chlorpromazine Valii; luminal; chlorpromazine

Hepatolenticular Degeneration (HLD)

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.

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

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

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

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

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

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