Extrapyramidal diseExtrapyramidal diseasesases

ⅠⅠ.Formation of basal ganglia.Formation of basal ganglia

**Corpus striatum:/caudate nucleus \ neostriatumCorpus striatum:/caudate nucleus \ neostriatum \lentiform/putamen nucleus / \lentiform/putamen nucleus / nucleus\globus pallidus- paleostriatumnucleus\globus pallidus- paleostriatum

**Red nucleusRed nucleus

**Substantia nigraSubstantia nigra

**Subthalamic nucleusSubthalamic nucleus

Ⅱ Ⅱ FunctionFunction

Regulation of voluntary motor Regulation of voluntary motor activityactivity

ⅢⅢ.clinical features.clinical features

1.Poverty of movement-hypertonia syndrome1.Poverty of movement-hypertonia syndrome position: substantia nigra ;globus pallidusposition: substantia nigra ;globus pallidus ex : Parkinsonex : Parkinson’ ’ s s diseasedisease2.Increasing of movement-hypotonia syndrome2.Increasing of movement-hypotonia syndrome position: caudate nucleus ;putamen nucleusposition: caudate nucleus ;putamen nucleus ex : Chorea minorex : Chorea minor

Parkinson’s DiseaseParkinson’s Disease

ⅠⅠ.Definition.Definition

It is also called paralysis agitans. It is a sloIt is also called paralysis agitans. It is a slowly progressive degeneration disease of thwly progressive degeneration disease of the extrapyramidal system. It is characterized e extrapyramidal system. It is characterized by tremorby tremor 、、 rigidityrigidity 、、 bradykinesia bradykinesia 、、 ababnormal gait and attitude.normal gait and attitude.

ⅡⅡ.Etiology and Pathogenesis.Etiology and Pathogenesis

1.age:>40years old 1.age:>40years old

\\2.Environmental factors :MPTP degeneration 2.Environmental factors :MPTP degeneration of DA neuronsof DA neurons

3.Genetic factor 3.Genetic factor / / (>50%)(>50%)

ⅢⅢ.Pathology and biochemical .Pathology and biochemical pathologypathology

1.Pathology1.Pathology(1)Macroscopic changes: no special changes(1)Macroscopic changes: no special changes(2)Microscopic changes:widespread cellular(2)Microscopic changes:widespread cellular degeneration, in substantia nigra, degeneration, in substantia nigra, Lewy’s bodyLewy’s body

2.Biochemical pathology2.Biochemical pathology HVAHVA

tyrosine tyrosine ↓ ↓ dopadecar dopadecar ↓↓ decomposition decomposition hydroxylase - boxylase ↑MAO,COMThydroxylase - boxylase ↑MAO,COMTLevo-tyrosine →→→→ Levodopa →→→→ dopamine ↓Levo-tyrosine →→→→ Levodopa →→→→ dopamine ↓ ↓↘ ↓↘ Caudate Caudate nucleusnucleus ←←←←←←←←←↓ ↘ ←←←←←←←←←↓ ↘ ↙ ↙Substantia nigra- Substantia nigra- Ach Ach ↑↑ putamenputamen ↙ ↙ corpus striatum pathcorpus striatum path ↓ ↓ nucleus ↓nucleus ↓ clinical featuresclinical features

ⅣⅣ.Clinical Features.Clinical Features1.1.tremor:tremor:usually first occur(60-70%)usually first occur(60-70%) “ “pill-rolling”pill-rolling” static tremorstatic tremor : the tremor is present : 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.rigidity2.rigidity

Cog-wheel rigidityCog-wheel rigidity

lead-pipe rigiditylead-pipe rigidity

3.bradykinesia3.bradykinesia

(1).Voluntary movements are reduced(1).Voluntary movements are reduced

(2).Masked face(2).Masked face

(3).micrographia(3).micrographia

4.Abnormal gait and attitude4.Abnormal gait and attitude

(1).generalized flexion(1).generalized flexion

(2).festinating gait (2).festinating gait

5.Other symptoms5.Other symptoms

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

ⅤⅤ.Diagnosis.Diagnosis

1.It occurs in old people ,the onset is insidiou1.It occurs in old people ,the onset is insidious and progress gradually.s and progress gradually.

2.Clinical features: tremor 2.Clinical features: tremor 、、 rigidity rigidity 、 、 bradbradykinesiaykinesia 、、 abnormal attitude and gait . abnormal attitude and gait .

ⅥⅥ.Differential Diagnosis.Differential Diagnosis

1.Secondary parkinson’s disease1.Secondary parkinson’s disease (Parkinsonism)(Parkinsonism)(1).infection: encephalitis lethargica(1).infection: encephalitis lethargica(2).poisoning: CO,Mn(2).poisoning: CO,Mn(3).drugs(3).drugs(4).cerebral arteriosclerosis (4).cerebral arteriosclerosis (5).trauma(5).trauma

2.Others2.Others

(1).Depression(1).Depression

(2).Essential tremor(2).Essential tremor

(3).multiple system atrophy: OPCA(3).multiple system atrophy: OPCA

(4).thyrotoxicosis,alcoholism.(4).thyrotoxicosis,alcoholism.

ⅦⅦ.Treatment.Treatment1.Drug treatment1.Drug treatment(1).anticholinergic drugs(1).anticholinergic drugsArtane 2mg tid po;kemadrin 2.5mg tid poArtane 2mg tid po;kemadrin 2.5mg tid poSide-effects: retention of urine; Side-effects: retention of urine; enlarged pupil; enlarged pupil; hypohidrosis; confusionhypohidrosis; confusionContraindications:hypertrophy of prostrateContraindications:hypertrophy of prostrate glaucomaglaucoma

(2).amantadine: 50mg tid po (2).amantadine: 50mg tid po

Side-effects :insomnia ; confusion; Side-effects :insomnia ; confusion; hallucinationhallucination

Contraindications: epileptic ;hepaticContraindications: epileptic ;hepatic and renal dysfunction and renal dysfunction

(3)Levodopa(3)Levodopa

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

Side-effectsSide-effects

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

2.Central side-effects2.Central side-effects

(1).motor fluctuation (1).motor fluctuation a.End of dose deteriorationa.End of dose deteriorationb.on-off phenomenonb.on-off phenomenon(2).dyskinesia(2).dyskinesiaa.peak-dose dyskinesiaa.peak-dose dyskinesiab.Biphasic dyskinesiab.Biphasic dyskinesiac.Dystoniac.Dystonia(3).mental symptoms:confusion;hallucination(3).mental symptoms:confusion;hallucination

(4)Dopamine receptor stimulant drug:(4)Dopamine receptor stimulant drug: bromocriptine bromocriptine(5)monoamine oxidase B inhibitor(5)monoamine oxidase B inhibitor(6)COMT inhibitor(6)COMT inhibitor2.Surgical treatment2.Surgical treatment

3.Cell transplantation and gene 3.Cell transplantation and gene therapytherapy

4.Rehabilitation treatment4.Rehabilitation treatment

CChorea minorhorea minor

ⅠⅠ.Definition.Definition

It is also called Sydenham chorea. It is a comIt is also called Sydenham chorea. It is a common nervous system manifestation of rheummon nervous system manifestation of rheumatic fever, it often occurs in children . It is atic fever, it often occurs in children . It is characterized by involuntarily choretic movcharacterized by involuntarily choretic movementsements 、 、 hypotoniahypotonia 、、 weaknessweakness 、 、 mentamental symptoms.l symptoms.

Ⅱ Ⅱ EtiologyEtiology

Infection of A hemolytic streptoInfection of A hemolytic streptococuscocus

ⅢⅢ.Clinical features.Clinical features1.onset:5-15 years old ; F>M; subacute 1.onset:5-15 years old ; F>M; subacute or insidiousor insidious2.Choretic movements2.Choretic movements3.Muscular tension and power are reduced3.Muscular tension and power are reduced4.Mental symptoms4.Mental symptoms5.Symptoms of rheumatic fever: cardiac5.Symptoms of rheumatic fever: cardiac disease; fever; WBC↑;rheumaticdisease; fever; WBC↑;rheumatic arthritis; subcutaneous nodule ;blood arthritis; subcutaneous nodule ;blood sedimentation↑;ASO↑sedimentation↑;ASO↑

ⅣⅣ.Diagnosis.Diagnosis

1.The age of onset1.The age of onset

2.Clinical features2.Clinical features

3.Symptoms of rheumatic fever3.Symptoms of rheumatic fever

ⅤⅤ.Treatment.Treatment

1.Etiological treatment1.Etiological treatment Penicillin 10-14 days Penicillin 10-14 days 2.Symptomatic treatment2.Symptomatic treatment

Valii; luminal; chlorpromazineValii; luminal; chlorpromazine

Hepatolenticular DegenerationHepatolenticular Degeneration

(HLD) (HLD)

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

ⅡⅡ.Etiology and Pathogenesis.Etiology and Pathogenesis

Copper Copper + + 2 globulins 2 globulins ceruloplasmin ceruloplasmin enzymeenzyme bile,urine,sweet bile,urine,sweet liver liver cirrhosis of the livercirrhosis of the liverabnormalabnormal basal ganglia basal ganglia extrapyramidal extrapyramidal deposition symptomsdeposition symptoms kidney kidney renal dysfunction renal dysfunction cornea cornea K-F ring K-F ring

ⅢⅢ.Pathology.Pathology DegenerationDegeneration:: liver liver

basal gangliabasal ganglia

kidneykidney

corneacornea

ⅣⅣ.Clinical Features.Clinical Features 1.nervous symptoms1.nervous symptoms (1)extrapyramidal symptoms: choretic (1)extrapyramidal symptoms: choretic action;tremor;rigidity;bradykinesiaaction;tremor;rigidity;bradykinesia (2)mental symptoms:intelligent deficiency(2)mental symptoms:intelligent deficiency 2.symptoms of cirrhosis of the liver2.symptoms of cirrhosis of the liver 3.K-F ring: 95%3.K-F ring: 95% 4.others: renal dysfunction4.others: renal dysfunction

ⅤⅤ.Investigation.Investigation

1.ceruloplasmin of serum:↓0.26-0.36g/L1.ceruloplasmin of serum:↓0.26-0.36g/L

2.copper oxidase activity:↓2.copper oxidase activity:↓

ⅥⅥ.Diagnosis.Diagnosis

1.the symptoms of liver and extrapyr1.the symptoms of liver and extrapyramidal system.amidal system.2.the levels of CP and copper oxidas2.the levels of CP and copper oxidase activity are reduced.e activity are reduced.3.K-F ring of cornea3.K-F ring of cornea4.positive family history4.positive family history

ⅦⅦ.Treatment.Treatment

1.low-copper diet1.low-copper diet2.drug treatment2.drug treatmentfirst -choice: D-penicillaminefirst -choice: D-penicillamine3.symptomatic therapy3.symptomatic therapy4.surgical treatment4.surgical treatment