神经病学课件 运动障碍疾病

Parkinson’s disease Huntington’s disease Dystonia Restless Leg Syndrome Psychiatric diseases (OCD)
Parkinson’s disease Huntington’s disease Wilson disease Multiple system atrophy: SND,OPCA,SDS Dystonia Restless leg syndrome
Mentation, behavior, and mood Activities of daily living Motor Complications of therapy Hoehn-Yahr Stage Schwab and England Activities of Daily Living Scale
Dystonia sustained muscle contractions often causing twisting movements or abnormal postures
General consideration Pathogenesis Prevention Clinical findings Different diagnosis treatment
1.5 men/women ratio Main risk factor : age Young onset: before 50 y.o. Rare familiar forms: genetic clues
World Health Organization estimates for incidence and prevalence follow:
and words that emerge from a background of normal motor activity.
Spasticity: continue clonic contraction, upper motor neuron( hereditary spastic paraplegia,HSP)
Tremor: Video. PD.avi a rhythmic oscillatory movement best characterized by its relationship to voluntary motor
activity
Intension: cerebellar rest : PD(4-6 Hz)..PDbrother 012.mpg action: Essential and cerebellar postural: metabolism(8-12 Hz)
▪ Exclusion of several causes of secondary
Parkinsonism
The Unified Parkinson's Disease Rating Scale (UPDRS)
The patient is evaluated by interview and clinical assessment in the following categories:
Myoclonus: a single sudden jerk or a short series occurring in slow : CJD(CreutzfeldtJakob disease(Prion disease), AIDS dementia complex
Athetosis : in contrast to chorei form movements these are slower writing,resembling the actions like a worm or snake
Neuropathology of PD
▪ Eosinophilic, round intracytoplasmic inclusions called
lewy bodies and Lewy neurites.
▪ First described in 1912 by a German
neuropathologist - Friedrich Lewy.
agents,Calcium-channel blockers ,Valproic acid ,Fluoxetine
Rare causes
Hypoparathyroidism with basal ganglia calcification , Hypothyroidism and hyperthyr源自文库idism
Miscellaneous: Repeat trauma (notably from boxing)
Structural lesions: Tumors ,Infarctions ,Hydrocephalus
Dauer W, Przedborski S.. Parkinson's disease: mechanisms and models. Neuron. 2003 39:889-909.
James Parkinson, in “An Essay on the Shaking Palsy.”
▪ The famous French neurologist, Charcot, further
described the syndrome in the late 1800s.
Parkinson Disease Motor system disorder in the brain. Results from the loss of Dopamine. First described by James Parkinson in 1817.
Chorea: denotes rapid irregular muscle jerks that occur involuntarily and unpredictably in different parts of the body(Video:)HDchorea.MPG
Tic: Sudden,repetitive,stereotyped,purposeless brief actions,gestures,sounds
Diagnosis of PD
▪ Anamnesis and clinical examination
▪ No disease-specific biological marker available
▪ Positron Emission Tomography (PET) or Single-
photon Emission Computed Tomography (SPECT) with dopaminergic radioligands
It comprise a spectrum of abnormalities that range from the hypokinetic disorders (of which Parkinson's disease is the best-known example) at one extreme to the hyperkinetic disorders (exemplified by Huntington's disease and hemiballismus) at the other.
▪ The most common movement disorder
affecting 1-2 % of the general population over the age of 65 years.
▪ The second most common
neurodegenerative disorder after Alzheimer´s disease (AD).
▪ Inclusions particularly numerous in the substantia
nigra pars compacta.
Neuropathology of PD: Lewy bodies
▪ Not limited to substantia nigra only; ▪ also found in the motor nucleus of the vagus nerve, ▪ the hypothalamus, the nucleus basalis of Meynert ▪ the cerebral cortex, the olfactory bulb and the autonomic
Jame parkinson’s founding Biochemistry founding MPTP Mutaion of Gene
History of Parkinson´s disease (PD)
▪ First described in 1817 by an English physician,
There’s about 170,000 PD patient in China.
May be less prevalent in China and other Asian countries, and in African-Americans.
Prevalence rates in men are slightly higher than in women; reason unknown, though a role for estrogen has been debated.
wemen: 4.9/100,000 men: 5.8/100,000 0-19 years: 0.0/100,000 20-64 years: 3.9/100,000 65 years and over: 49.3/100,000
wemen: 6/100,000 men: 8/100,000
0-19 years: 0/100,000 20-64 years: 6/100,000 65 years and over: 70/100,000
nervous system.
▪ Confined largely to neurons; glial cells only rarely affected.
causes:
Common causes
Primary (75%): Idiopathic Parkinson's disease
Secondary (25%): Vascular disease Infectious and postinfectious Postencephalitic Neurosyphilis AIDS Toxins:Manganese ,Cyanide ,Methanol , Carbon monoxide ,MPTP,Pesticides Medications:Neuroleptics , Dopamine-depleting
浙江大学医学院附属第二医院
Second Affiliated Hospital, Zhejiang University College of Medicine
welcome
Movement disorder disease is induced by basal ganglia damage with different kind causes, such as : toxin, degeneration, infalmanntary, infarction , genetics, ect.
Symptoms (Primary) Tremor of one or more than one limb. Rigidity . Bradykinesia. Postural instability.
Dopamine Pathways
Nolte, Fig 8-33
Epidemiology of PD