椎体外系疾病extrapyramidal disease神经病学双语课件

Extrapyramidal diseases .Formation of basal ganglia *Corpus striatum:/caudate nucleus neostriatum lentiform/putamen nucleus / nucleusglobus pallidus- paleostriatum *Red nucleus *Substantia nigra *Subthalamic nucleus Function Regulation of voluntary motor activity .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 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 cellular degeneration, in substantia nigra, Lewys body 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 .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; constipation sphincter 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 prostrate glaucoma (2).amantadine: 50mg tid po Side-effects :insomnia ; confusion; hallucination Contraindications: epileptic ;hepatic and renal dysfunction (3)Levodopa L-dopa + dopadecarboxylase inhibitor(DCI) Ex : Madopar ; Sinemet Side-effects 1.Peripheral side-effects: nauea ;vomiting Postural 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: cardiac disease; fever; WBC;rheumatic arthritis; subcutaneous nodule ;blood sedimentation;ASO .Diagnosis 1.The age of onset 2.Clinical features 3.Symptoms of rheumatic fever .Treatment 1.Etiological treatment Penicillin 10-14 days 2.Symptomatic treatment Valii; 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: liver basal ganglia kidney cornea .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 .Investigation 1.ceruloplasmin of serum:0.26-0.36g/L 2.copper oxidase activity: .Diagnosis 1.the sympto