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Neuro > 18: movement disorders > Flashcards

18: movement disorders Flashcards

1
Q

Hypokinetic movement disorders

A

parkinson’s
progressive supranuclear palsy
mutliple system atrophy

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2
Q

neurological features of PD

A

Asymmetric onset-later bilateral
Primary Extrapyramidal Features

Rigidity
resting tremor
bradykinesia
postural instability -late

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3
Q

PD- REM sleep behavior disorder

A

ndividuals retain the ability to move during dreaming and, thus, may “act out” their dreams with hitting, kicking, hollering, and even jumping out of bed.

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4
Q

gross pathological features of PD

A

degeneration of pigmented neurons

  1. substantia nigra pars compact
  2. locus ceruleus
  3. dorsal vagal nucleus
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5
Q

microscopic pathological features of PD

A

Lewy body formation

  1. CNS-pigmented
  2. ENS-non-pigmented
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6
Q

microscopic inclusions found in surviving pigmented neurons

A

lewy bodies

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7
Q

primary component of lewy bodies

A

synuclein

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8
Q

primary NT deficit in PD

A

dopamine

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9
Q

parkinsonism plus syndrome

A

progressive supranuclear palsy

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10
Q

clinical features of PSp

A

extrapyramidal features
rigidity-especially axial
neck hyperextension

bradykinesia
astoniched facies
dysarthria
life expectancy 10 years

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11
Q

neuro features of PSP

A

gait disturbances-early
postural; instability-falling
unexplained falling
tremor is unusual

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12
Q

distinguishing feature of PSP that seprates it from PD

A

development of characteristic eye movement

supranuclear gaze palsy

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13
Q

desribe supranuclear gaze palsy

A

impairment of volitional downgaze
(PD has difficulty with upgaze)

PSP then gets difficulty with upgaze and horizaontal gaze late

apraxia-unable to open closed eyes
-oculocephalic meneuver in tact

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14
Q

gross anatomic pathologic features of PSP

A

midbrain ad cerebral cortical atrophy

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15
Q

microscopic PSP features

A

neuronal loss and gliosis of SN , and peduncolopontine of rostral midbrain, also GPE and GPI,

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16
Q

pathological hallmark of PSP

A

Neurofibrillary tangles, composed of unpaired straight filaments that contain abnormally phosphorylated tau protein are a pathological hallmark of PSP.

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17
Q

tauopathy

A

PSP

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18
Q

synucleinopathy

A

PD

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19
Q

Most prominent nrueochemical abnormality fo PSP

A

Nigrostriatal Dopa deficiency

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20
Q

another Parkansonism plsu syndrome

A

Multiple System atrophy

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21
Q

three presentations of MSA (originally thought to be three diseases)

A

clinical picture of parkinsonism,

some with progressive autonomic failure

some with cerebellar syndrome

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22
Q

clinical features of MSA

A

RIGIDITY
BRADYKINESIA
POSTURAL INSTABILITY AND FALLING-EARLY
TREMOR UNUSUAL

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23
Q

DOES MSA RESPONE TO LEVODOPA

A

SOME

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24
Q

CLINICAL HALLMARK OF MSA THAT SEPARATES IT

-78%

A

PROGRESSIVE AUTONOMIC FAILURE
-78%

URINARY DYSFUNCTION
ORTHOSTATIC HYPOTENSION
IMPOTENCE
GI DYSFUNCTION
THERMOREGULATORY DYSFUNCTION
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25
Q

MOST COMMON AUTONOMIC DYSFUNCTION SEEN IN MSA

A

ORTHOSTATIC HYPOTENSION

26
Q

CEREBELLAR DYSFUNCTION ON MSA LESS COMMON

-55%

A

ATAXIA, DYSARTHRIA, OCULOMOTOR ABNORMALITIES, EXAGGERATED REBOUND

27
Q

PYRAMIDAL SIGNS IN MSA-61%

A

HYPERREFLEXIA
BABINSKI
SPASTICITY
PESUOBULBAR PALSY

28
Q

DOES DEMENTIA DEVELOP WITH MSA

A

NOT TYPICALLY
-SOME CONSIDER IT AN EXCLUSION CRITERIA FOR MSA
BUT FRONTAL LOBE EXECTIVE FUNCTION DECLINES

29
Q

OTHER UNIQUE FEATURES IN SOME MSA PATIENTS

A

Respiratory (laryngeal) stridor, probably produced in most instances by vocal cord abductor weakness, may develop in approximately one-third of patients with MSA and confers an increased risk of sudden nocturnal death. Involuntary sighing, Raynaud’s phenomenon, and postural myoclonus of the hand

30
Q

MICROSCOPIC HALLMARK OF MSA

A

GLIAL CYTOPLASMIC INCLUSION BODIES THAT STAIN FOR ALPHA SYNUCLEIN

31
Q

SYNUCLEINOPATHIES

A

PD AND MSA

32
Q

MSA CHARATERIZED BY CELL LOFF AND GLIOSIS OF

A

BASAL GANGLA
BRAINSTEM,
CEREBELLUM
SPINAL CORD

CORTEX USUALLY UNAFFECTED**`

33
Q

HYPERKINETIC MOVEMENT DISORDERS

A

HUNTINGTON’S
TOURRETTE’S
PRIMARY (IDIOPATHIC) DYSTONIA

34
Q

INHERITANCE PATTER OF HD

A 
autosomal dominant
shorp arm of 4
-expanded trinucleotide CAG repeats
-codes for huntingtin 
protein
-too much causes neuronal cell damage
35
Q

hallmark of HD

A

chorea-random, rapid jerky movements, that flow form one movement into tanother and impart a restless wiggly or dancingb appearance to the affected patient

36
Q

neuro features of HD

A 
chorea early
dystonia with progression
hyperkinetic dysarthria
Parkinsonism in juvenile form
Parkinsonism in advanced disease
37
Q

other dominant clinical features of HD

*may be the initial disease symptom

A

behavioral changes

impulsiveness
irritability
obsessive behavior
aggression
depression
dementia
38
Q

eye dysfunction in HD

A

cant initiate saccades, slowed sccades, gaze impersistence

39
Q

path features of HD

especially in the caudate/putamen and cortex

A

Striatum–> caudate
neuron and glial closs
atrophy of GPallidus

Cortex-< atrophy neuronal loss

40
Q

defining feature of TS

A

tics

sudden sterotypes non-rhythmic movements or vocalizations

41
Q

motor tics

A

simple or complex-preceded by premonition or urge that is satiated by making the movement

42
Q

hallmark of TS progression

A

compliment of tics changes-some dissappear to be replaced by new ones
*frequency and severity of tics waxes and wanes over time

43
Q

formal Dx of tourrettes

A

multple motor and at least one vocal tic must be present at some point during the course, though not necessarily concurrenlty
must occyre many times a day, almost every day, or intermittently over the course of more than abyear, with no more than 3 consecutive free months
onset before 18
not explained by anything else

44
Q

incoluntary gestures and words respectively

A

copropraxia

coprolalia

45
Q

other conditions seen in a high prevalence with TS

A

ADHD 90% and OCD 50%

46
Q

dopa disturbance with TS?

A

TOO MUCH-responds somewhat to dopa inhibition

47
Q

dystonia clinical picture

A

sustained muscle contraction producing sustained anc repetitive twisting movements resulting in abnormal psotures

48
Q

inheritance pattern of dystonia

A 
onset during childhood
autosomal dominant
DYT1 gene mutation on chromosome 9
glutamate deletion in torsin A
penetrance is 40%
worse in askinazi jews
49
Q

pathological changes of dystonia

A

no consistent abnormality

basal ganglia and cerebellum are proposed

50
Q

neurchemical abnormality in dystonia

A

no constitent chemical found

some suggest dopa., noradrenergic, GABAergic

51
Q

focal dystonia arises in

A

adulthood and appears to have no genetic basis-unlike generalized dystonia

52
Q

as dystonia (gen and focal) progresses it geenerally becomes

A

static

53
Q

Mixed hypokinetic and hyperkinetic movement disease

A

wilsons dz

54
Q

mutation in copper transporting ATPase

A

wilsons disease

55
Q

inheritance pattern of wilsons dz

A

autosomal recessive
long arm of 13
gene product is ATPB7-copper transporting ATPase

56
Q

ATP7b utation

A

within liver,cannot transport Cu to apocerulopasmin forming ceruloplasmin

  • under conditions of elevated copper in the plasma-ATp7B causes biliary excretion of copper
  • copper accumulates in the liver
57
Q

copper accumulation in the liver

A

wilson’s disease

58
Q

three areas affected by wilsons disease

A
  1. hepatic is most common and in 12-15 yo
  2. neurologic
  3. psychiatric-19 year old
59
Q

hypo/hyper neuro disorders of wilsons

A 
parkinsonism
chorea
dystonia
kindetic intention tremor (defining)
dysrthria
incoordination
60
Q

opthalmologic features of Wilsons Disease

A 
Kayser fleicher (descemet's_ rings
suflower catarcts (lens accumulation)
61
Q

in wilsons dz the copper in a KF ring accumulates in the

A

outer rim of cornea in Descemets membrane

62
Q

neuro path features of wilsons

A

putamen, thalamus, cerebral cortex neuronal loss and gliosis
OPALSKI CELL FORMATION

*FROM DEGENERATING ASTROCYTES

Neuro (19 decks)

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