Extrapyramidal diseases,.Formation of basal ganglia,*Corpus striatum:/caudate nucleus neostriatumlentiform/putamen nucleus / nucleusglobus pallidus- paleostriatum*Red nucleus*Substantia nigra*Subthalamic nucleus, Function,Regulation of voluntary motor activity,.clinical features,1.Poverty of movement-hypertonia syndromeposition: substantia nigra ;globus pallidusex : Parkinson s disease 2.Increasing of movement-hypotonia syndromeposition: caudate nucleus ;putamen nucleusex : Chorea minor,Parkinsons Disease,.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.,.Etiology and Pathogenesis,1.age:40years old 2.Environmental factors :MPTP degeneration of DA neurons 3.Genetic factor / (50%),.Pathology and biochemical pathology,1.Pathology (1)Macroscopic changes: no special changes (2)Microscopic changes:widespread cellulardegeneration, in substantia nigra, Lewys body,2.Biochemical pathology,HVAtyrosine dopadecar decompositionhydroxylase - boxylase MAO,COMT Levo-tyrosine Levodopa dopamine Caudate nucleus Substantia nigra- Ach putamen corpus striatum path nucleus clinical features,.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.,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;hyperhidrosis; constipationsphincter disturbance is rare (2).mental symptoms: dementia ;depression (3).dysarthria (4).salivation,.Diagnosis,1.It occurs in old people ,the onset is insidious and progress gradually.2.Clinical features: tremor 、rigidity 、 bradykinesia、abnormal attitude and gait .,.Differential Diagnosis,1.Secondary parkinsons disease(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.,.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 prostrateglaucoma,(2).amantadine: 50mg tid po,Side-effects :insomnia ; confusion; hallucinationContraindications: epileptic ;hepaticand renal dysfunction,(3)Levodopa,L-dopa + dopadecarboxylase inhibitor(DCI)Ex : Madopar ; Sinemet,Side-effects,1.Peripheral side-effects:nauea ;vomitingPostural hypotension;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,Chorea minor,.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., Etiology,Infection of A hemolytic streptococus,.Clinical features,1.onset:5-15 years old ; FM; subacute or insidious 2.Choretic movements 3.Muscular tension and power are reduced 4.Mental symptoms 5.Symptoms of rheumatic fever: cardiacdisease; fever; WBC;rheumaticarthritis; subcutaneous nodule ;blood sedimentation;ASO,.Diagnosis,1.The age of onset 2.Clinical features 3.Symptoms of rheumatic fever,.Treatment,1.Etiological treatmentPenicillin 10-14 days 2.Symptomatic treatmentValii; luminal; chlorpromazine,Hepatolenticular Degeneration (HLD),.Definition,It is also called Wilsons 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.,.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,.Pathology,Degeneration: liverbasal gangliakidneycornea,