movement disorders - dr. navarro, 9-17-12 and 9-19-12
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8/10/2019 Movement Disorders - Dr. Navarro, 9-17-12 and 9-19-12
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NEURO – MOVEMENT DISORDERS: DR. NAVARRO, 9-17-12 AND 9-19-12
NIGROSTRIATAL
- Effect of dopamine on pathways
• Direct pathway: stimulates
MOVEMENT DISORDERS
-
*Doc says to know Parkinsonism, chorea,
athetosis, ballism, and dystonia*
HYPOKINETIC DISEASES
PARKINSON’S DISEASE
- Progressive neurodegenerative disorder
associated with a loss of dopaminergicnigrostriatal neurons
-
1817: James Parkinson, “An Essay on the
Shaking Palsy”
-
Second most common degenerative disorder
- Average age of onset: 55
-
Men have a 1.5 times greater risk of developing
Parkinson’s disease
-
Young onset: 21-40 years
- Juvenile onset: before 21 years of age
Remember the mnemonic!
T remor
R igidity
A kinesia
P overty of movement
-
Tremors
• “Pill-rolling”: When you ask a patient
with Parkinson’s to make a fist, this
hand motion occurs
: hyperkinetic movement
• Pill-rolling is like the hand motion that
a healthy individual makes after he
picks his nose (gross!). Once he
obtains a booger, he makes a small ball
with it by rubbing his fingers together.
-
Rigidity
• Parkinson’s usually starts on one side
of the body as ‘hemiparkinsons’
– stiffness
• Patient drops his legs, no arm swinging
= patient walks very slowly
• Parkinson’s ultimately becomes
bilateral, and the patient’s posture is
stooped-
•
Bradykinesia
Poverty of Movement
•
Righting reflex affected, patient has
difficulty turning around while walking
• “Mask fascies” – no emotion on
patient’s face
• “Reptilian looks” – reptiles hardly ever
close their eyes, much like a
Parkinson’s patient
• Difficulty getting up from a chair
• Difficulty in initiating movement.
Patient rocks to and fro in an attempt to
walk
• “Festinating gait” – patient’s first few
steps are fast. Small, hurried steps.
•
“Myerson Sign” –
a medical condition
where a patient is unable to
resist blinking when tapped on
the glabella, the area above the nose
and between the eyebrows. It is often
referred to as the “glabellar reflex”. It is
also an early symptom of Parkinson's
disease.
• Difficutly putting spoon to mouth
- Mainstay of treatment = levodopa
• Brain needs dopamine to stimulate
caudate nucleus
- Basal ganglia disease
• Brain is not able to produce dopamine
MOVEMENT DISORDERS PT 2
PARKINSON’S DISEASE
-
Depletion of dopamine
- Degenerates substantia nigra (we are not sure
why this occurs)
THEORIES
- Vascular
-
Drug induced (anti-psychotic drugs)
-
Toxins (MPTP – Drug addicts in California
reportedly attempted to convert meperidine into
MPTP = contracted Parkinson’s Disease)
• Von Conome’s (?) Disease: destroys
midbrain, especially substantia nigra
-
Heavy metals
-
Carbon monoxide
-
Infection
CLINICAL FEATURES
T remor
R igidity
A kinesia
P overty of movement
- Asymmetry
-
Bradykinesia
• Loss of arm swing
• Micrographia
•
Hypomimia•
Difficulty with fine movements
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NEURO – MOVEMENT DISORDERS: DR. NAVARRO, 9-17-12 AND 9-19-12
-
Rigidity
•
•
'Cogwheel' rigidity is a combination ofleadpipe rigidity and tremor which
presents as a jerky resistance to passive
movement as muscles tense and relax.
'Leadpipe' rigidity results when an
increase in muscle tone causes a
sustained resistance to passive
movement throughout the whole range
of motion, with no fluctuations
COGWHEEL
• Cogwheel: if you take out the rim of a
stagecoach wheel, you can get a sense
of cogwheel rigidity
STAGECOACH WHEEL
-
Rest tremor
- Postural instability
• Pull back test
DRUGS FOR PARKINSON’S DISEASE
LEVODOPA
- Natural amino acid precursor of dopamine
-
Raises brain dopamine concentrations after being
taken up by the surviving nigrostriatal
dopaminergic terminals
-
Most efficacious drug for symptomatic treatmentof PD
- Side effects: intolerable, nausea and postural
hypotension
• Peripheral metabolism to dopamine
-
Dopa gets to brain substantia nigra
(converts dopa into) dopamine
CARBIDOPA-LEVODOPA
-
Combined levodopa and carbidopa (peripheral
decarboxylase inhibitor) dramatically alleviated
adverse peripheral dopaminergic effects
-
Remains the gold standard for treatment of parkinsonian symptoms
-
Dosing is typically initiated at one 25/100 mg
tablet TID and increased as necessary until
response is achieved
-
Peripheral enzymes convert dopa into dopamine
- Levodopa (via enzyme) dopa
-
Dopa is the only substance that can enter the
brain
-
One needs a substance that can convert dopa into
dopamine
-
Give high amount of dopa in the blood so it
enters into the brain (substantia nigra)
-
Need something to prevent conversion of dopa
into dopamine in peripheral circulation =
CARBIDOPA – peripheral decarboxylase
inhibitor
DOPAMINE AGONIST THERAPY
-
Directly stimulates dopamine receptors- Do not require enzymatic conversion or specific
transport mechanisms
- Dopamine agonists used in PD:
• Bromocriptine
• Pergolide
• Pramipexole
• Ropinirole
• Apomorphine
• Rotigotine
-
Substantia nigra degenerates in PD, does not
stimulate caudate nucleus
-
If you give the patient dopa in PD, the caudatenucleus can be stimulated
-
Dopamine agonist: mimics the action of
dopamine
-
The time will come when the substantia nigra
becomes exhausted, and continuous addition of
dopa becomes fruitless
• Therefore give a dopamine agonist
instead
FAMOUS PEOPLE, PAST AND PRESENT, WITH
PARKINSON’S DISEASE- Hitler, Pope John Paul II, Michael J. Fox,
Muhammad Ali, Mao Zedong
- Involuntary, irregular, purposeless, nonrhythmic,
abrupt, rapid, unsustained movements that seem
to flow from one body part to another
CHOREA
-
involves extremities usually, but also the tongue
-
jerking movement is sometimes incorporated into
some kind of purposeful movement
CHOREAS CAN BE - Primary (hereditary/familial)
-
Secondary (sporadic)
•
Infectious
•
Vascular
• Drug-induced
• Metabolic
SYDENHAM’S CHOREA
-
Autoimmune-mediated
-
Female predominance (F:M ratio 2:1)
-
Age at onset: 3 to 17 years (mean 9-10 years)
- Associated with group A beta-hemolytic
streptococcal infection
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NEURO – MOVEMENT DISORDERS: DR. NAVARRO, 9-17-12 AND 9-19-12
• Chorea usually occurs about 4-8 weeks
after streptococcal pharyngitis, not skin
infection
-
A.k.a St. Vitus Dance
TREATMENT
-
No specific treatment for the disease
-
Symptomatic treatment when severity ofmovements interferes with activities
• Benzodiazepines
• Valproate
• Corticosteroid
-
Prophylactic administration of penicillin for at
least 10 years is recommended to prevent other
manifestations of rheumatic fever, of which
Sydenham chorea may be its sole manifestation
- Back in the day, etiology was possession by
satan!!!
Syndham’s Chorea
-
Major criteria for rheumatic heart disease -
Tx: sedation, antipsychotics
- Found in children
- Found in adults
Huntington’s Chorea
-
Sydenham’s = sudden, jerky, irregular, non-
stereotype
ATHETOSIS
-
Athetosis = almost the same except SLOW in
movement
•
Parang ahas, the patient writhes
-
Like chorea but occurs suddenly in ½ of the body
BALLISMUS
• Hemiballismus
-
In Lubag = “Dystonia of Panay”
•
Very progressive disease with no
treatment
-
Bad posturing
DYSTONIA
-
Tremors: alternating movements of agonist and
antagonist muscles
- Tics: abnormal movement of group of muscles
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