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1、Movement Disorders,CHEN, Guojun Department of Neurology Chongqing Medical University,Introduction to movement disorders,“movement disorders ” is often used with “extrapyramidal or basal ganglia (BG)” disease Lesions of basal ganglia or pyramidal system are related to some, but not all, of the moveme
2、nt disorders,Pathways of extrapyramidal system 1. Cortex-cortex 2. Substatia nigra-striatum 3. Striatum-globus pallidus,Major neurotransmitters,Dopamine, acetylcholine GABA glutamate some neuropeptides such as ENK and Substance P They work synchronously to maintain normal excitation and inhibitions.
3、,Normal function of extrapyramidal system,Regulates muscle tone Maintains posture Co-ordinates voluntary movement,Basal Ganglia,Damage to the basal ganglia: Produces either too much activation (hyperkinetic) responses= twitches, movements bursts, jarring, etc. OR Produces too little force (hypokinet
4、ic)=rigidity Parkinsons disease,Pink=inhibition Blue=excitation,Common symptoms of movement disorders,Akinesia-rigid Parkinsonism-brdykinesia Dyskinesia Chorea Dystonia Tremor tics,Chorea,Chorea is a rapid, purposeless, irregular, jerky movement that seems to flow randomly from one part of the body
5、to another.,Dystonia and Athetosis,Dystonia is a syndrome of sustained muscle contractions causing abnormal postures or twisting and repetitive movements. Athetotic movements are complex, wormlike, irregular, non propositional and predominate over postural anomalies and on the distal parts of limbs
6、and face.,tics,Twitches of the face, limbs or the whole body. Can be reproduced voluntarily Patient has partial control Do not interfere with voluntary activity May persist in sleep,Common forms of movement disorders,Parkinsons Disease Wilsons Disease (Hepatolenticular degeneration) Essential tremor
7、 Sydenhams Chorea (Rheumatic chorea) Tourettes Syndrome,Parkinsons Disease（PD）,Is also called paralysis agitans Is characterized by a neuronal accumulation of -synuclein and neuronal loss in SN Presents with bradykinesia, tremor, rigidity, shuffling gait, and flexed posture,History of PD First descr
8、ibed in 1817 by an English physician, James Parkinson, in “An Essay on the Shaking Palsy.” The famous French neurologist, Charcot, further described the syndrome in the late 1800s.,Epidemiology of PD,1% of those 55 years Risk factors: ageing, positive family history, male gender, head injury, exposu
9、re to pesticides, consumption of well water, and rural living. Factors linked to reduced incidence: coffee drinking, smoking, nonsteroidal anti-inflammatory drugs, and estrogen.,Dopamine pathways in human brain,Dopamine synthesis,DA metabolism,DA degradation,Lewy bodies,Etiopathogenic mechanisms,Mec
10、hanisms-summary,Cell death may be caused by -synuclein aggregation proteosomal and lysosomal system dysfunction, reduced mitochondrial activity. Excitotoxicity and inflammation are likely to play a relevant role in progressive neuronal degeneration.,Clinical features,Motor features Bradykinesia, tre
11、mor, rigidity, shuffling gait, and flexed posture Non-motor features Depression and anxiety, cognition, sleep disturbances, sensory, and autonomic dysfunctions,Parkinsons Disease（PD）,Motor features,Bradykinesia. Slow in movement. micrographia (hypophonia)-soft speech “Masked face” Tremor at rest. 4-
12、6Hz. “pill rolling”. The lips, tongue, and jaw may be involved but spares the head. Rigidity .“cogwheeling ” or “lead-pipe”. Gait disturbance: shuffling short steps. Festinating (hurried) gait. At later stage, freezing of gait (start hesitation).,Non-motor features,Depression and anxiety affects 50%
13、 of patients, intrinsic cognitive impairment sleep disturbances sensory abnormalities and pain, loss of smell (anosmia) disturbances of autonomic function,Diagnosis,5055 years, develops slowly At least two of the following: tremor at rest, rigidity, bradykinesia or gait disturbance. Is responsible t
14、o dopamine Exclude other parkinsonisms secondary parkinsonisms Parkinson-plus syndromes,Differential diagnosis,Secondary Parkinsonism Parkinsons-plus syndromes Parkinsonism with abnormal metabolisms of TAU (Taupathies) and -synuclein (-synucleinopathies) Wilsons Disease (Hepatolenticular degeneratio
15、n) Essential tremor (ET),Secondary Parkinsonism,Vascular parkinsonism Seen in lacunar infarction Poor response to L-dopa Drug-induced parkinsonism (DIP).,Drug-induced parkinsonism (DIP),Is due to neuroleptics, some atypical antipsychosis, lithium carbonate, or antiemetic agents (especially metochlop
16、romide). Less common: valproic acid, fluoxetine (antidepressant). Antihypertensive agents such as reserpine and alpha-methyldopa. Exposure to toxins such as CO, disulfide, cyanide and methanol can also lead to parkinsonism. DIP may respond to anticholinergic agents, amantadine, and L-dopa.,Differential diagnosis,Secondary Parkinsonism Parkinsons-plus syndromes Parkinsonism with abnormal metabolisms of TAU (Taupathies) and -synuclein (-synucleinopathies) Wilsons Disease
