movement disorders

Question 1

parkinson’s results from..

idiopathic?

Answer 1

reduced dopaminergic transmission at basal ganglia level

Idiopathic in ~75% of cases

Mutations in several genes may contribute

~1,000,000 affected
Peak onset 60 years of age

Question 2

parkinson’s features

Answer 2

2 out of 3:
rest tremor
rigidity
bradykinesia

May include masked facies, decreased eye blinking, stooped posture, decreased arm swing

Question 3

balance btw glutamate, ACh and dopamine

in Parkinson’s

Answer 3

less dopamine

Question 4

ddx parkinsonism

Answer 4

Wilson’s
Huntington’s
Multisystem Atrophy

Question 5

Multisystem Atrophy (formerly Shy Drager Syndrome)

Answer 5

ANS insufficiency (blood pools in legs), parkinsonism, LMN signs, cerebellar dysfunction
-think tests?

Question 6

Huntington’s

Answer 6

fam hx and dementia

tx: tetrabenazine for
dyskinesia – interferes with storage of amines

Question 7

Wilson’s

Answer 7

don’t want to miss
Early age onset,
Kayser Fleischer rings (eyes), increased copper in tissues

Question 8

parkinson’s motor features

Answer 8

Bradykinesia
Micrographia
Hypophonia 
Rest Tremor, 4 to 6 Hz, “Pill rolling” 
Rigidity, “Plastic” quality, “Cogwheel” sensation 
Short shuffling steps 
Myerson Sign

Question 9

lead pipe vs cogwheel rigidity

Answer 9

stiff vs jerky

Question 10

parkinson’s non-motor features

Answer 10

Depression, Anxiety 
Cognitive impairment 
Sleep disturbances 
Anosmia 
Orthostatic hypotension – Syncope

Question 11

park: Neuropsychiatric Symptoms

Answer 11

Lewy body dementia

Depression – 50% of patients

Question 12

park: patho: atrophy where?

degeneration of ?? with presence of ??

Answer 12

Frontal atrophy

Degeneration of dopaminergic cells with presence of Lewy bodies that have high concentration of alpha-synuclein

Question 13

park tx: dopaminamimetic tx

Answer 13

-Dopamine agonists cross blood-brain barrier acting on dopamine receptors

-Levadopa converted to dopamine in body
Combined with carbidopa, which inhibits enzyme that breaks down levodopa to dopamine, but does NOT cross blood brain barrier
levels can go up and down
take at sp. times

Question 14

park tx: selegilline

Answer 14

Monoamine oxidase inhibitor (MAO-I)

Added to help with tremor

Question 15

part tx: Entacapone

Answer 15

Catechol O-methyltransferase (COMT) inhibitors

Blocks enzyme that degrades levodopa and dopamine, allows for more sustained levels of response

Question 16

park tx considerations

Answer 16

Time of medications very important to prevent symptom return/withdrawal

Don’t give Levodopa if patient has narrow angle glaucoma, or is on MAOI within 2 weeks of stopping the drug (HTN crisis)

Question 17

combo Carbidopa/Levodopa brand name

Answer 17

Sinemet

Question 18

another tx for parkinson’s

Answer 18

deep brain stimulation

Question 19

essential tremor

Answer 19

Most common movement disorder (hands, head, or voice)

May be worse with stress/emotions, sleep deprived

Autosomal dominant
Generally bilateral

Question 20

essential tremor improved with

Answer 20

with alcohol, certain beta-blockers (propranolol)

Question 21

Hemiballismus/hemichorea

Answer 21

Dramatic “flailing”
Violent form of chorea
Supportive care

Tx: haloperidol, propranolol, phenytoin, clonazepam, baclofen

Question 22

Huntington’s
genetic inheritance?

onset?

Answer 22

Autosomal Dominant –
CAG mutation on Chromosome 4
genetic testing confirms diagnosis

Fatal
Onset 30-50 years of age (50% chance of your children have it)
Chorea “dance”

Question 23

dystonia

tx?

Answer 23

Syndrome of involuntary contractions of muscles, twisting and repetitive movements

Treatment is symptomatic
Anticholinergic medications (trihexyphenidyl)

Question 24

If dystonia is due to Wilson’s disease ??

Answer 24

copper chelation therapy

Question 25

Tourette Syndrome

age of onset?? how often?

Answer 25

Tic
Neurologic and behavioral
Symptoms before age 21
criteria: tics occur for at least 1 year, tics very in number frequency and nature over time

Question 26

Tourettes tx: atypical antipsychotic

Answer 26

CBT (cog. behav. therapy)

Clonidine, Botox options
Risperidone, olanzapine, ziprasidone in more severe cases

Question 27

Tarditive dyskenisia:

Answer 27

med-induced hyperkinetic disorder

Usually from exposure to central dopamine blockers
1/3 temporary, but can be perminant

Question 28

Myerson sign

Answer 28

glabellar tap sign is 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

Question 29

Neuroleptic Malignant Syndrome (NMS):

Answer 29

med-induced hyperkinetic disorder

Serious complication, mortality as high as 20%
Muscle rigidity

Treatment is dantrolene, cooling body
tx, problem is recognition, fam hx