Extrapyramidal Disease

Question 0

oxidative stress

Answer 0

adverse effects on tissues of reactive oxygen species (free radicals)

Question 1

extrapyramidal

Answer 1

those parts of the motor system that are not directly pyramidal; that is not the corticospinal tracts (upper motor neurons); always includes basal ganglia– often includes cerebellar system

Question 2

parkinson disease

Answer 2

dopamine deficiency syndrome characterized by bradykinesia, poor postural responses, rigidity, tremor

Question 3

parkinson disease

Answer 3

dopamine deficiency syndrome characterized by bradykinesia, poor postural responses, rigidity, and tremor

Question 4

bradykinesia

Answer 4

pathological slowness of movement

Question 5

delayed postural reflexes

Answer 5

refers to a delay in the normal righting responses that permit stance and gait

Question 6

resting tremor

Answer 6

tremor that occurs with the hands resting in the lap or at the sides when walking or standing

Question 7

cogwheel

Answer 7

referring to an irregular, ratchety resistance to passive movement which is superimposition of the tremor rigidity

Question 8

festination

Answer 8

shortening of stride such that the patient takes many short steps to cover a distance; they may become propulsive if they cannot catch up with their center of gravity

Question 9

on-off

Answer 9

sudden and unpredictable loss of effectiveness of parkinson’s medication

Question 10

hypophonia

Answer 10

pathological loss of power in the voice

Question 11

corpus striatum

Answer 11

caudate nucleus

lenticular nucleus- putamen, globus pallidus

Question 12

subthalamic nucleus of luys

Answer 12

target for movement disorders because overactive in many disorders such as parkinson disease

Question 13

where do you do DBS?

Answer 13

subthalamic nucleus of luys

Question 14

substantia nigra

Answer 14

striatum
caudate
putamen

Question 15

pars compacta

Answer 15

produces dopamine and axons of dopaminergic neurons that project to
striatum–>globus pallidus–>thalamus–>cortex–>back to red nucleus/brainstem/cerebellum/spinal cord
caudate
puatmen

Question 16

basal ganglia functions

Answer 16

unconsciously; helps to correct pattern organized movements nad inhibit unwanted ones

Question 17

slowest postural reactions

Answer 17

delayed reactions to being knocked off balance

Question 18

mask facies

Answer 18

decreased facial expressiveness; bradykinesia of facial muscles

Question 19

rigitidy + tremor

Answer 19

cogwheeling

Question 20

differential dx of choreoathetosis

Answer 20

autoimmune-sydenham's (post strep)
encephalitis
metabolic-thyrotoxicosis, hyper and hypothyroidism, hypocalcemia, hypoglycemia
drugs- neuroleptics
poisioning- CO, mercury
genetic disease- huntington, wilsons, other
pregnancy
senie, 
cerebrovascular
perinatnal injury

Question 21

huntington disease genetics

Answer 21

autosomal dominant with complete penetrance

Question 22

epi of disease

Answer 22

CAG repeats- number of repeats anywhere from 35-40

Question 23

mean onset huntingtons

Answer 23

35-40; mean duration 13 years

Question 24

clinical symptoms

Answer 24

``` chorea athetosis severe and disabling dementia behavior changes (not nec in that order) ```

Question 25

symptoms of huntington's made worse by

Answer 25

levodopa

Question 26

pharm treatment huntingtons

Answer 26

neuroleptic agents: most effective dopamine blocking properties--> haloperido, perphenazine neurotransmitter depletors: reserpine (potentiates depression), tetrabenazine

Question 27

chronic use of neuroleptics

Answer 27

sensitizes system-->reverse effect-->tardive dyskinesia

Question 28

essential treatment genetics

Answer 28

sporadic OR autosomal dominant with incomplete penetrance

Question 29

essential tremors worsen

Answer 29

with age

Question 30

what type of tremor is an essential tremor?

Answer 30

postural and intentional | symmetric

Question 31

postural tremor

Answer 31

hands shake while held in sustained position

Question 32

action tremor

Answer 32

worsens with finger to nose testing or bringing a spoon to the mouth

Question 33

suppresses essential tremor

Answer 33

ETOH

Question 34

treatment of essential tremor

Answer 34

``` beta blockers Primidone Neurotonin, Topiramate- anti epileptic ethanol surgery-thalamotomy, DBS (ventral intermediate nucleus of thalamus) botulinum toxin ```

Question 35

what drug do you not use for essential tremor

Answer 35

valproate- high tremor side effect

Question 36

parkinson tremor

Answer 36

static tremor | usually unilateral in beginning-->progresses quickly with noticeable worsening when seen at six month intervals

Question 37

parkinson vs essential area of body

Answer 37

park- hand/leg | essential-hand/head/voice

Question 38

progress parkinson vs essential

Answer 38

parkinson- quickly | essential- slowly; over years

Question 39

diagnosis of parkinsonism requires 2/3 of the cardinal signals

Answer 39

bradykinesia rigidity tremor

Question 40

things that cause parkinsonism

Answer 40

genetics | other things like drugs

Question 41

Parkinson DIsease

Answer 41

``` marked depigmentation of substantia nigra dopamine loss (50% asymptomatic, 70% first symptom, 90% death) ```

Question 42

parkinson pathological hallmark

Answer 42

lewy body inclusions

Question 43

pathology correlates best with

Answer 43

bradykinesia

Question 44

survival with parkinsons

Answer 44

15 years after onset longer in non-demented PD cases longer with L-Dopa use

Question 45

cause of death is usually

Answer 45

pulmonary infection/aspiration | urinary tract infection

Question 46

MPTP toxicicty

Answer 46

reproduces all major clinicopathological elements of PD (including lewy bodies) MPTP is in astrocytes-->converted to MPP+ by MAO-B MPP+= toxic metabolite; free radical that destroys mito fx and kills sells by inhibiting complex 1 of resp pathway-->ATP, cell death

Question 47

corner stone of parkinson's treatment

Answer 47

promoting DA | antagonizing acetylcholine

Question 48

carbidopa

Answer 48

taken with levodopa to prevent conversion of levodopa in the blood before it gets to the brain, therefore use a lot less levodopa with fewer systemic effects, such as nausea too much DA too soon produces oxidative metabolites-->damages R

Question 49

other pharm of parkinson

Answer 49

COMT inhibitors MAO-B inhibitors anticholinergics

Question 50

amantadine

Answer 50

suppresses dyskinesias that people get as a side effect of too much dopamine

Question 51

THalmotomy

Answer 51

treat tremor by ablating globus pallidus

Question 52

palidotomy

Answer 52

useful for bradykinesia or rigidity but not tremor

Question 53

DBS for parkinson

Answer 53

most effective in internal segment of gp and esp subthalamic nucleus

Question 54

Tics

Answer 54

fleeting, purposeful actions that may be simple (appearing as a muscle twitch) or complex (repetitive behavior)

Question 55

suppression

Answer 55

not voluntary, but tics can be suppressed for a perioid of time through force of will

Question 56

Tourette syndrome

Answer 56

motor and repetitive coval tics-throat clearing, snorting, sniffinf

Question 57

tics begin

Answer 57

age 5-18

Question 58

what worsens tics?

Answer 58

psychostimulants used to treat adhd

Question 59

medication for tics

Answer 59

neuroleptic

Question 60

hemibalism leasion

Answer 60

contralateral subthalamic nucleus