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Flashcards in Movement Disorders Deck (16):
1

Huntingtons Disease
-how do you acquire this?
-age of onset
-characterized by
-MOA

Acquire: autosomal dominant

Age: 40yo

Characterized by: chronic progressive chorea, psychological changes, dementia

MOA:
-brain cells waste away
-Decrease in GABA and GABA receptors in basal ganglia. There is an imbalance of dopamine and Ach that cause disease manifestations.
(GABA and Ach reduced & dopamine normal)

*GABA and Ach are inhibitory NT
*Dopamine is excitatory NT

2

Huntington disease
-Sx in adult/child
-early psychological manifestations
-other early/late physical signs

Sx Adult:
-chorea affects the limbs and trunk
-dystonia
-rigidity
-postural instability
-myoclonus
-nystagmus

Juvenile Onset: RARE
-bradykinesia
-rigidity
-quicker progression

Psychological manifestations:
-Depression* (lack of initiative, spontaneity, inability to concentrate)
-personality changes*
-Memory Loss
-Impulsive behavior (almost like manic bipolar)
-antisocial behavior
-moodiness
-emotional outburst

Physical signs:
Early: fidgeting, restlessness

Late: chorea, dystonic posturing, progressive rigidity, akinesia, dementia

3

Huntington Dz
-Diagnostic Studies

Dx:
-MRI - caudate atrophy (caudate: one of the structures that make up the basal ganglia)

-PET: abnormal metabolic changes in the caudate

-Genetic Testing: sensitive and specific

4

Huntington Dz
-Tx
-SE

Tx: can only treat sx
-downregulate dopaminergic neurotransmission

-suppress chorea (Tetrabenazine; breaks down dopamine & Neuroleptics/antipsychotics; deplete cerebral dopamine)

Depression: Prozac, Zoloft, Aventyl(Nortriptyline; TCA)

Postural rigidity and instability: Klonopin(clonazepam), Depakote (Valproic Acid)

Antipsychotics: Resperdal(Resperidone) & Olanzapine (Zyprexa)

5

Essential Tremor
-how do you acquire?
-describe the tremor
-where can tremor occur?
-highest incidence at what age?
-what makes the tremor worse?

Acquire: inherited

Describe: affects both sides of the body symmetrically, more prominant with ACTION than rest, frequency of tremor is constant. Amplitude may vary.

Tremor may occur in hands, head, hands and head, or voice if laryngeal muscles affected.

Highest incidence in 60+, but may begin at any age.

Aggravated by:
-stress, sleep deprivation, stimulants
*ETOH may relieve essential tremor

6

Essential Tremor Tx

Propranolol***

Atenolol- beta blocker of choice for those w/ asthma or bronchospasm

Mysoline (PRimidone)- anticonvulsant

Gabapentin (Neurontin) - anticonvulsant

7

Parkinsons Dz
-Characterized by?
-age at onset
-pathophysiology
-risk factors

Characterized:
TRAP
T- resting/posture tremor
R- Rigidity
A-akinesia (bradykinesia)
P- postural instability

Age:
generally dx between 45-65, but can occur at earlier stages.

Pathophys:
-loss of dopamine in the substantia nigra
-defective alpha-synuclein and lewy bodies
-Increase in cholinergic interneuron activity d/t degeneration of dopamine pathways (contributing to tremor)

Risk Factors:
-genetics
-Viral/environmental exposures
-aging

8

What is the leading cause of neurologic dz in pts over 65?

Parkinsons Dz

9

Parkinson Dz:
-cardinal manifestations:

Manifestations:
-Tremor; mainly hands and feet, pill rolling, usually unilateral, appears at rest and disappears with movement and sleep

-Rigidity: cog-wheel, ratchet-like movement, resistance to movement

-Akinesia (Bradykinesia); slowness in initiating and performing movements, difficult turning, frozen in place esp. when moving through doorway or preparing to turn.

-Postural Instability: lean forward to maintain center of gravity, shuffling steps w/o arm swing, prone to falls

10

Parkinsons Dz:
-other sx

Other sx:
-dull facies
-loss of blinking
-drooling
-sweating
-micrographia
-hypophonia
-depression
-ortho hypotension
-constipation
-impotence
-urinary incontinence
-dementia (late stage)

11

Parkinson Dz:
-Dx

Dx: clinical
-gold standard is neuropathologic exam
-2 of 3 cardinal manifestations must be present
-other confirmation is autopsy
-...heather says some docs give carbadopa/levodopa to see if pts respond.

12

Parkinsons Dz:
-Tx of sx
-Tx of complications:
--Depression
--Hallucination
--Ortho Hypotension
--Sexual disturbance
--Constipation
--Freezing/Difficulty initiating
--akinesia/rigidity
--dysarthria/hypophonia

Tx:
-Levadopa/Carbidopa
-MOA-B inhibitors
-Dopamine agonists
-COMT inhibitors
-Amantidine
-Ach-blocking agents
*max out on these drugs before moving on to deep brain stimulation

Tx of Complications:
-Depression: SSRI

-Hallucinations: decrease levadopa/dopamine agonists or Zyprexa

-Orthostatic Hypotension: TED hose, slow rising

-Sexual Disturbance: Viagra/Dopamine agonist

-Constipation; stop offending medication, bulking agents, no regalan (dopamine receptor antagonist making their sx worse by blocking dopamine)

-Freezing/DIfficulting initiating: tape doorway, mark floor at turning point

-akinesia/rigidity: exercise and PT

-Dysarthria/hypophonia: Speech Therapy

13

Is DBS more effective than L-dopa?

No, though DBS is like electrical dopamine, PO Levodopa at highest dosage has better effects than DBS at highest dosage.

14

Restless Leg Syndrome
-aka
-what is this?
-causes
-Pt description of sensation

aka: wittmaack-Ekboms Syndrome

What: uncontrollable urges to move limbs in order to stop uncomfortable, painful, or odd sensation in the body, most commonly the legs

Cause:
-unknown; something in substantia nigra
-reading, plane ride, movie, napping
-iron deficiency
*may be worse in evening and early in the night

Description:
-uncomfortable, antsy, electrical, creeping, painful, itching, pins and needles

15

RLS:
-may be associated with?

Associations:
-pregnancy
-obesity
-smoking
-anemia
-iron deficiency
-polyneuropathy
-DM
-varicose veins
-Kidney failure
-Medications
-Foods (diet soda; aspartame. ETOH)

16

RLS:
-Tx

Correct underlying cause

Take pain relievers: OTC ibuprogen

Baths/massages

warm or cool packs

relaxation techniques (yoga/meditation)

good sleep

avoid caffeine

exercise

Medications for parkinsons:
-Pramipexole (Mirapex)
-Ropinirole (requip)
-Carbidopa/levodopa (Sinemet)
-Lyrica

-Opiods: codeine, oxycodone & acetaminophen (percocet), hydrocodone and acetominophen (Vicodin, lortab)

-Epilepsy Meds: Gabapentin (Neurontin)