Extrapyramidal Disease Flashcards

0
Q

oxidative stress

A

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

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1
Q

extrapyramidal

A

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

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2
Q

parkinson disease

A

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

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3
Q

parkinson disease

A

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

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4
Q

bradykinesia

A

pathological slowness of movement

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5
Q

delayed postural reflexes

A

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

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6
Q

resting tremor

A

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

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7
Q

cogwheel

A

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

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8
Q

festination

A

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

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9
Q

on-off

A

sudden and unpredictable loss of effectiveness of parkinson’s medication

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10
Q

hypophonia

A

pathological loss of power in the voice

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11
Q

corpus striatum

A

caudate nucleus

lenticular nucleus- putamen, globus pallidus

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12
Q

subthalamic nucleus of luys

A

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

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13
Q

where do you do DBS?

A

subthalamic nucleus of luys

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14
Q

substantia nigra

A

striatum
caudate
putamen

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15
Q

pars compacta

A

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

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16
Q

basal ganglia functions

A

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

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17
Q

slowest postural reactions

A

delayed reactions to being knocked off balance

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18
Q

mask facies

A

decreased facial expressiveness; bradykinesia of facial muscles

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19
Q

rigitidy + tremor

A

cogwheeling

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20
Q

differential dx of choreoathetosis

A
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
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21
Q

huntington disease genetics

A

autosomal dominant with complete penetrance

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22
Q

epi of disease

A

CAG repeats- number of repeats anywhere from 35-40

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23
Q

mean onset huntingtons

A

35-40; mean duration 13 years

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24
Q

clinical symptoms

A
chorea
athetosis
severe and disabling dementia
behavior changes
(not nec in that order)
25
Q

symptoms of huntington’s made worse by

A

levodopa

26
Q

pharm treatment huntingtons

A

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

27
Q

chronic use of neuroleptics

A

sensitizes system–>reverse effect–>tardive dyskinesia

28
Q

essential treatment genetics

A

sporadic
OR
autosomal dominant with incomplete penetrance

29
Q

essential tremors worsen

A

with age

30
Q

what type of tremor is an essential tremor?

A

postural and intentional

symmetric

31
Q

postural tremor

A

hands shake while held in sustained position

32
Q

action tremor

A

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

33
Q

suppresses essential tremor

A

ETOH

34
Q

treatment of essential tremor

A
beta blockers
Primidone
Neurotonin, Topiramate- anti epileptic
ethanol
surgery-thalamotomy, DBS (ventral intermediate nucleus of thalamus)
botulinum toxin
35
Q

what drug do you not use for essential tremor

A

valproate- high tremor side effect

36
Q

parkinson tremor

A

static tremor

usually unilateral in beginning–>progresses quickly with noticeable worsening when seen at six month intervals

37
Q

parkinson vs essential area of body

A

park- hand/leg

essential-hand/head/voice

38
Q

progress parkinson vs essential

A

parkinson- quickly

essential- slowly; over years

39
Q

diagnosis of parkinsonism requires 2/3 of the cardinal signals

A

bradykinesia
rigidity
tremor

40
Q

things that cause parkinsonism

A

genetics

other things like drugs

41
Q

Parkinson DIsease

A
marked depigmentation of substantia nigra
dopamine loss (50% asymptomatic, 70% first symptom, 90% death)
42
Q

parkinson pathological hallmark

A

lewy body inclusions

43
Q

pathology correlates best with

A

bradykinesia

44
Q

survival with parkinsons

A

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

45
Q

cause of death is usually

A

pulmonary infection/aspiration

urinary tract infection

46
Q

MPTP toxicicty

A

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

47
Q

corner stone of parkinson’s treatment

A

promoting DA

antagonizing acetylcholine

48
Q

carbidopa

A

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

49
Q

other pharm of parkinson

A

COMT inhibitors
MAO-B inhibitors
anticholinergics

50
Q

amantadine

A

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

51
Q

THalmotomy

A

treat tremor by ablating globus pallidus

52
Q

palidotomy

A

useful for bradykinesia or rigidity but not tremor

53
Q

DBS for parkinson

A

most effective in internal segment of gp and esp subthalamic nucleus

54
Q

Tics

A

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

55
Q

suppression

A

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

56
Q

Tourette syndrome

A

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

57
Q

tics begin

A

age 5-18

58
Q

what worsens tics?

A

psychostimulants used to treat adhd

59
Q

medication for tics

A

neuroleptic

60
Q

hemibalism leasion

A

contralateral subthalamic nucleus