Transcript PowerPoint 演示文稿

运动障碍疾病
Movement Disorders
Neurology Department
The Second Affiliated Hospital
Harbin Meical University
Extrapyramidal
Disorders
General Conception: The dysfunction of basal ganglia
putamen
globus pallidus
lentiform nucleus
subthalamus
caudate nucleus
corpus striatum
basal ganglia
substantia nigra
General Conception
Two types of symptoms

Muscular hypotonus associated with
hyperkinesia(F1)

Muscular hypertonus characterized with
hypokinesia(F2)
Parkinson’s disease---conception
Also called paralysis agitans.



a common degeneration disease in nervous
system
becomes increasingly common with
advancing age
characterized by rest tremor, bradykinesia,
rigidity, abnormal gait and posture


Parkinson’s disease---Etiology
Aging: over middle aged persons
Environmental toxin: MPTP(l-Methyl-4phenyl-1,2,3,6-tetrahydropyridine) in astrocyte
oxidated
MPP+
mitochondrion in
substantia nigra
neuronal death

Genetic factor: alpha-synuclein gene ----susceptibility
Parkinson’s disease---Pathology
 loss of pigmentation and cells in the
substantia nigra

Lewy body in the basal gangalia,
sympathetic ganglia and brainstem.
Parkinson’s disease---Biochemistry


L- tyrosine (blood) TH L-Dopa DDC
DA
MAO and COMT
HVA
Nigrostriatal pathway is the most important.
dopamine depletion
dopaminergic nigrostriatal system
Extrapyramidal
Pathway
dopamine
acetylcholine
Parkinson disease’s---Clinical findings
Over middle aged & insidious onset and
gradual progression.
 Tremor: often appear as the first sign.
Characters: 4~6Hz, conspicuous at rest, increase
at times of emotional stress, improves during
voluntary activity, stop during sleep, begin from
hand , “N” progression.
Parkinson disease’s---Clinical findings

Rigidity(F1/F2): lead pipe / cogwheel
phenomenon. Minimal rigidity: move the
contralateral limb, head dropping test,
road mark sign
Parkinson disease’s--Clinical findings

Bradykinesia: slowness and reduction of voluntary
movement, difficult to initiate, masked face,
hypophonia, micrographia.

Abnormal gait and posture: flexed posture; difficult to
get up, start walking, turn or stop. Shuffling gait and
absence of the arm swing. Festinating gait(late sign).
Parkinson disease’s--Clinical findings

Other features: Myerson’s sign, oily face,
intractable constipation, postural
hypotension, cognitive disturbance,
hallucination, depression
Parkinson’s disease---Differential diagnosis


Parkinsonism: postencephalitic parkinsonism,
drug or toxin-induced parkinsonism(CO, Mn),
arteriosclerotic parkinsonism.
Depression: a trial of antidepressant drug
treatment may be helpful.
Parkinson’s disease---Differential diagnosis


Essential(benign, familial) tremor: family
history, early onset, nod or head shake.
Parkinsonism associated with other
neurologic diseases: Diffus Lewy body
disease(hallucination), Wilson disease,
Huntington chorea, multiple system
atrophy(MSA), PSP, cortical basal
ganglionic degeneration(CBGD).
Parkinson’s disease---Treatment
Begin from low dose and individualized.
Anticholinergic drugs: helpful in alleviating
tremor and rigidity. Artane(1~2mg, tid).
• Amantadine: for mild parkinsonism. 50mg, bid~tid.
• Levodopa: L-Dopa/DCI(4:1)---compound;
side effects: nausea, hypotension(peripheral)
dyskinesia and motor fluction(central)
wearing off or on-off phenomenon
Parkinson’s disease---Treatment
•
•
•
•
DA-R agonist: bromocriptine
MAOB/COMT inhibitor
Surgery: pallidotomy and deep brain stimulation(high
frequency thalamic stimulation)
Physical therapy and aids for daily living
Prognosis dead from complications
Sydenham Chorea--- Conception
Also called rheumatic chorea.
A usual manifestation of rheumatic fever in
nervous system.
Presents in childhood or young adult life.
Clinical findings
involuntary chorea
dystonia
weakness
automatic action
disturbance
emotional changes
Sydenham Chorea--- Etiology



Related with the infection of Hemolytic
Streptococcus A.
Anti-neuron antibodies can react with
the neurons in caudate or subthalamic
nucleus.
Maybe relate with endocrine changes.
Sydenham Chorea--- Clinical findings




5~15ys.
G/B=3:1
Subacute or insidious onset
Early symptom: irritability, inattention,
unsteady gait, easily dropping objects, et al.
Sydenham Chorea--- Clinical findings

Chorea: rapid, irregular, involuntarily,
unpredictably
obvious on face( facial grimacing and
tongue movements)
prominent when nervous and
disappeared during sleep.
Sydenham Chorea--- Clinical findings
Muscle tone and power: reduced.
Triad: limb weakness, chorea and ataxia.
milkmaid grasp, wax-waning sign, dancing
gait.
• Psychiatric symptom: agitation,
hallucination
• Rheumatic fever findings.
Self-limitation
•
Sydenham Chorea--- Testing
1.Serum test: rapid erythrocyte
sedimentation, increased wbc.
2. EEG: unspecific.
3. Image test: 29~85% patients present
low density focus in caudate nucleus on
CT.
Sydenham Chorea--Diagnosis&Differential diagnosis



Onset age, triad , rheumatic fever findings
1. Habit spasm: stereotyped action on the
same muscle
2. Congenital chorea: earlier onset
3. Tourette syndrome: tics on face
accompanied by strange voice and dirty
words.
Sydenham Chorea--Diagnosis&Differential diagnosis
4.Huntington Chorea: onset after middle age,
dementia, familial history
5. Torsion spasm: continuous,
hypermyotonia when torsion happens
Sydenham Chorea--Treatment & Prognosis



rest, keep quiet, avoid stimulation
penicillin or other antibiotics(10~14 days) and
deltacortone(until the symptoms disapper)
apozepam(5mg, bid/tid), haloperidol(0.5~1mg,
bid/tid).
side effects of extrapyramidal system
Self-limited 3~6 months after the onset
Wiloson Disease--Conception & Etiology
Also called hepatolenticular degeneration(HLD)
A kind of inherited disease
• extrapyramidal sign
Autosomal recessive
• cirrhosis
inheritance disease
• psychiatric symptom
of copper metabolism
• kidney impairment
disturbance---Etiology
• K-F circle on cornea
Wiloson Disease--- Clinical findings


childhood/young adult life
reflect the disproportionate involvement
of the caudate nucleus, putamen, cerebral
cortex and cerebellum.
resting/postural tremor, rigidity, facial
grimacing, ataxia, choreiform movements of
limbs, disorders of affect , behavior , personality
or psychology
Wiloson Disease--- Clinical findings


May progress rapaidly(younger); often
gradually progress with periods of
remission and exacerbation
Nonneurologic findings:
ocular and hepatic abnormalities
Wiloson Disease--- Differential diagnosis
Differential diagnosis:
• multiple sclerosis
• juvenile-onset Huntington disease
Wiloson Disease--- Investigative studies

Serum copper and ceruloplasmin(CP) are low,
and 24h urinary copper excretion is generally
increased.

Abnormal liver function blood test and
aminoaciduria

Image test show cerebrocortical atrophy and
abnormalities in the basal ganglia.
Wiloson Disease--- Treatment



D-Penicillamine(1~1.5g/d): allergic test
Restriction of dietary copper
zinc sulfate(200mg/d) decrease
absorbance
Treatment must be continued for life. Early
treatment can give a better recovery.