Movement Disorders - Cohen

Question 1

parkinsons disease

Answer 1

4 main features

1 - resting tremor
2 - bradykinesia - slow movement - initiating movement
3 - dysequilibrium - instability of gait and posture
4 - rigidity - later in illness

diagnosis - should have at least 3 of 4

Question 2

tremor

Answer 2

regular oscillatory movement

usually always present

poor control agonist/antagonist muscles

worse with fatigue, stress, drugs, age

Question 3

chorea

Answer 3

dance like movement

Question 4

myoclonus

Answer 4

brief jerk

Question 5

athetosis

Answer 5

writhing movement

snake/worms

Question 6

dystonic movement

Answer 6

slow uncomfortable movement

Question 7

pathologic tremor

Answer 7

disturb normal functioning

Question 8

resting tremor

Answer 8

parkinsons

often asymmetric

first in hand
-to head, jaw, leg

can be only feature of parkinsons

Question 9

parkinsons bradykinesia

Answer 9

slow movement

patient like statue when standing - no fidgeting when sitting

drool - hard to swallow

can overcome - under stress - kinesis paradoxica

Question 10

kinesis paradoxica

Answer 10

in parkinsons bradykinesia

-pt can overcome it

Question 11

dysequilibrium parkinson

Answer 11

hard to become steady

don’t swing arms well

dangerous sign and least responsive to meds

Question 12

parkinsons rigidity

Answer 12

later sign
-cramps - shoulders

problem when medication wears off

Question 13

other symptoms of PD

Answer 13

sleep disorder
loss semll
autonomic problem
cramps, pain
depression
small handwriting
loss of power of voice - monotonous and hurried

Question 14

pathology of PD

Answer 14

lewy bodys - substantia nigra

loss of dopaminergic neurons

loss of cells in BS, thalamus, cerebarl hemisphers, and basal ganglia

Question 15

lewy bodies

Answer 15

alpha synuclein

eosinophilic structures in cytoplasm of neurons - especially in substantia nigra

Question 16

genetics of PD

Answer 16

rare in twins

genes - PARK 1-11

markin protein

Question 17

epidemiology of PD

Answer 17

age of incidence 55-59yo

older - rapid deterioration

majority men

Question 18

tx of PD

Answer 18

best tx - L-dopa

dopamine does not cross BBB - but L-dopa does

Question 19

dopamine synthesis

Answer 19

from tyrosine

rate limiting - tyrosine hydroxylase

Question 20

carbidopa

Answer 20

inhibit metabolism of L - dopamine in blood

-more L-dopa to brain

Question 21

sinemet

Answer 21

levodopa/carbidopa

tx - can wear off after 5-10 years

can have dyskinesia - too much movement
also possible psychosis

Question 22

other dopaminergic drugs

Answer 22

bind DA receptors - especially D2

less wearing off effect

not as effective as sinemet

psychosis may occur

Question 23

psych problems in PD

Answer 23

dementia - later in disease

depression common

psychosis - troubling, violent/aggression

Question 24

50% PD patient

Answer 24

die within 15 years

Question 25

surgery for PD

Answer 25

deep brain stimulation - very effective - advanced PD disease

also - small strokes in lower basal ganglia - thalamus or subthalamic nucleus

Question 26

drug induced parkinsons

Answer 26

with drugs that block dopamine

nausea drugs - phenothiazines and butyrophenones

Question 27

progressive supranuclear palsy

Answer 27

PSP

• severe dysequilibrium
• falls early - as is choking

NO tremors

puzzled look on face

progressive loss of extraocular movements
-cannot move eyes

often die - aspiration pneumonia

Question 28

PSP treatment

Answer 28

levodopa works for some

but no known tx

Question 29

essential tremor

Answer 29

4-8 Hz

symmetric - arms, head, voice, legs

action tremor or familial

“Is there when I do things.”

Question 30

DDx for essential tremor

Answer 30

hyperthyroid
antidepressant drugs
cerebellar disease
parkinsonian features

Question 31

tx of essential tremor

Answer 31

weight arms and wrists

alcohol helps

meds - propranolol and primidone

Question 32

tics

Answer 32

mostly disease of kids
-quick random simple movements

usually head

may fade with time

never serious pathology

Question 33

tourettes syndrome

Answer 33

motor and vocal tics
-start before age 21

minority use obscenities

does not fade away with adolescence

many also have OCD and depression

Question 34

pathology of tourette

Answer 34

unknown

possible dopamine derangement

Question 35

Tx of tourette

Answer 35

haloperidol or other D2 blockers

Question 36

chorea

Answer 36

dance like disorder

irregular and variable movement

no clear pattern

Question 37

st vitus dance

Answer 37

untreated group A strep infection
-syndeham chorea

delayed autoAbs for that affect brain

Question 38

huntington disease

Answer 38

inherited disease - auto dom

typically begin 40-50yo

trinucleotide repeat chromosome 4
-CAG

movement disorder - chorea, tremor, jerk
cognitive loss - dementia
psychiatric - agitation, depression, psychosis

often suicidal

Question 39

Tx of huntington

Answer 39

dopamine blockers

new drug - tetrabenazine

Question 40

diagnosis of HD

Answer 40

fam hx

symptoms in middle age

genetic testing available - huntingtin protein

Question 41

restless leg syndrome

Answer 41

cannot stop moving

sleep disorder as well

bad when get into bed

Question 42

pathology of RLS

Answer 42

reduction of brain iron

reduced dopamine activity in BS and basal ganglia

tx - dietary iron

tyrosine hydroxylase - iron cofactor

L-dopa helps

best tx - dopamine agonists - pramipexole