NEURO MOVEMENT DISORDERS: DR.

NAVARRO, 9-17-12 AND 9-19-12

Dr. Navarro was less into slides and more into
extemporaneous discussion. Ill include what he said in
lecture in this handout

MOVEMENT DISORDERS PT 1
- Changes in movement disorders are biochemical,
one cannot see problems w/such disorders on an
autopsy
:
- Within therapeutic trials: if the patient responds
to medication, he may have Parkinsons Disease
- Majority of movement disorders have no cure
- Medication can simply help a patient control
his/her movement

- Defined as neurological syndromes in which
there is either an excess of movement
(hyperkinetic movements) or paucity of voluntary
or automatic movements (hypokinetic
movements)

ANATOMIC CONSIDERATIONS
- The activities of basal ganglia and the cerebellum
are blended with and modulate the corticospinal
and cortical brainstemspinal systems

ANATOMY OF THE BASAL GANGLIA



- Ganglia

group of neurons outside the CNS (i.e.
sympathetic ganglia, Gasserian ganglion
connects to CN V)
BASAL GANGLIA
- Inhibitory (GABAergic)
Striatum(caudate, putamen)
Globus pallidus
- Excitatory (Glutamate)
Cortex
Thalamus
Subthalamic nucleus
- Lack of stimulation: poverty of movement
- Overstimulation: hyperkinetic movement




- Substantia nigra is found in the midbrain, neurons
are pigmented black

FUNCTIONS OF BASAL GANGLIA
Non-Motor Loops
- Executive/prefrontal loop
- Limbic loop
- Oculomotor loop
- Basal ganglia plans movement execution i.e. if
you plan to move your hands
- Main function of the limbic loop: emotion,
intimately related to CN I (not much movement
needed here)
Motor loop
- Regulation of upper motor neurons
- Necessary for normal initiation

PATHWAYS OF MOTOR LOOP
Direct pathway
- Overall excitatory
- Kung nasira yung excitatory pway =poverty of
movement
Indirect pathway
- Overall inhibitory





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
PARKINSONS DISEASE
- Progressive neurodegenerative disorder
associated with a loss of dopaminergic
nigrostriatal neurons
- 1817: J ames 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
Parkinsons disease
- Young onset: 21-40 years
- J uvenile 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 Parkinsons 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
Parkinsons usually starts on one side
of the body as hemiparkinsons
stiffness
Patient drops his legs, no armswinging
=patient walks very slowly
Parkinsons ultimately becomes
bilateral, and the patients posture is
stooped
-
Bradykinesia
Poverty of Movement
Righting reflex affected, patient has
difficulty turning around while walking
Mask fascies no emotion on
patients face
Reptilian looks reptiles hardly ever
close their eyes, much like a
Parkinsons patient
Difficulty getting up froma chair
Difficulty in initiating movement.
Patient rocks to and fro in an attempt to
walk
Festinating gait patients 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
PARKINSONS 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 Parkinsons Disease)
Von Conomes (?) 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 armswing
Micrographia
Hypomimia
Difficulty with fine movements
NEURO MOVEMENT DISORDERS: DR. NAVARRO, 9-17-12 AND 9-19-12

- Rigidity

'Cogwheel' rigidity is a combination of
leadpipe 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 rimof a
stagecoach wheel, you can get a sense
of cogwheel rigidity



STAGECOACH WHEEL

- Rest tremor
- Postural instability
Pull back test

DRUGS FOR PARKINSONS 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 treatment
of PD
- Side effects: intolerable, nausea and postural
hypotension
Peripheral metabolismto 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 caudate
nucleus 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
PARKINSONS DISEASE
- Hitler, Pope J ohn Paul II, Michael J . Fox,
Muhammad Ali, Mao Zedong

- Involuntary, irregular, purposeless, nonrhythmic,
abrupt, rapid, unsustained movements that seem
to flow fromone 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

SYDENHAMS 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
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 of
movements 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
Sydenhamchorea may be its sole manifestation

- Back in the day, etiology was possession by
satan!!!
Syndhams Chorea
- Major criteria for rheumatic heart disease
- Tx: sedation, antipsychotics
- Found in children
- Found in adults
Huntingtons Chorea

- Sydenhams = 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 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