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Flashcards in movement disorders Deck (29):
1

parkinson's results from..



idiopathic?

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

2

parkinson's features

2 out of 3:
rest tremor
rigidity
bradykinesia

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

3

balance btw glutamate, ACh and dopamine
in Parkinson's

less dopamine

4

ddx parkinsonism

*Wilson’s*
Huntington’s
Multisystem Atrophy

5

Multisystem Atrophy (formerly Shy Drager Syndrome)

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

6

Huntington's

fam hx and dementia

tx: tetrabenazine for
dyskinesia – interferes with storage of amines

7

*Wilson's*

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

8

parkinson's motor features

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

9

lead pipe vs cogwheel rigidity

stiff vs jerky

10

parkinson's non-motor features

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

11

park: Neuropsychiatric Symptoms

Lewy body dementia
Depression – 50% of patients

12

park: patho: atrophy where?

degeneration of ?? with presence of ??

Frontal atrophy


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

13

park tx: dopaminamimetic tx

-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

14

park tx: selegilline

Monoamine oxidase inhibitor (MAO-I)
Added to help with tremor

15

part tx: Entacapone

Catechol O-methyltransferase (COMT) inhibitors
Blocks enzyme that degrades levodopa and dopamine, allows for more sustained levels of response

16

park tx considerations

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)

17

combo Carbidopa/Levodopa brand name

Sinemet

18

another tx for parkinson's

deep brain stimulation

19

essential tremor

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

May be worse with stress/emotions, sleep deprived

Autosomal dominant
Generally bilateral

20

essential tremor improved with

with alcohol, certain beta-blockers (propranolol)

21

Hemiballismus/hemichorea

Dramatic "flailing"
Violent form of chorea
Supportive care

Tx: haloperidol, propranolol, phenytoin, clonazepam, baclofen

22

Huntington's
genetic inheritance?




onset?

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”

23

dystonia




tx?

Syndrome of involuntary contractions of muscles, twisting and repetitive movements

Treatment is symptomatic
Anticholinergic medications (trihexyphenidyl)

24

If dystonia is due to Wilson’s disease ??

copper chelation therapy

25

Tourette Syndrome


age of onset?? how often?

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

26

Tourettes tx: atypical antipsychotic

CBT (cog. behav. therapy)

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

27

Tarditive dyskenisia:

med-induced hyperkinetic disorder

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

28

Myerson sign

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

29

Neuroleptic Malignant Syndrome (NMS):

med-induced hyperkinetic disorder

Serious complication, mortality as high as 20%
Muscle rigidity

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