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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