movement disorders Flashcards

1
Q

what disease is progressive depletion of dopamine secreting cells

A

parkinsons

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2
Q

accumulation of what in parkinsons causes lewy bodies ?

A

a-synuclein

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3
Q

70 yr old male presents with tremor describes as pill rolling at rest better w movement worse when anxious affected unilateral upper limb then ipsilateral lower limb then bilateral

A

parkinsons

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4
Q

how can u describe the rigidity of parkinsons

A

lead pipe or cogwheel when combined w tremor

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5
Q

what disease presents with shuffling gait stooped posture with poor arm swing

A

parkinsons

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6
Q

parkinson patient most likely all develop

A

dementia

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7
Q

commonest cause of death in parkinsons

A

bronchopneumonia

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8
Q

tx of parkinsons

A

levodopa or carbidopa

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9
Q

secondary causes of hypokinetic movement disorders

A

drug induced: dopamine receptor blocker
post encephalitic
wilsons disease

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10
Q

what type of disorder is autoimmune HLA-DR2

A

multiple sclerosis

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11
Q

what factors cause multiple sclerosis

A

EBV
HHV6
smoking
low vit D
sunlight exposure

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12
Q

what is the most common pattern in multiple sclerosis progression

A

relapsing-remitting

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13
Q

multiple sclerosis is combination of two types of hypersensitivity reactions

A

type 4( oligodendrocyte dysfunction)
type 2 ( antibody and complement mediated damage)

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14
Q

type 4 pathology in ms

A

oligodendrocyte dystrophy , oligo r the ones that produce myelin so if no myelin then degeneration occurs

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15
Q

type 2 pathology in ms explain

A

antibody and complement activated , complement deposit in myelin sheath lead to inflam and destruction

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16
Q

common sites for plaques to deposit in ms

A

optic nerve
perivent white matter
bs and cerebellar connections
cervical sc

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17
Q

two signs found in ms patients

A

Lhermitte sign: tingling electric shock like sensation that radiates to the arm and back or in legs on flexion of pt neck

uhthoffs sign: inc symptoms w hot weather and bath

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18
Q

what do u see on mri of ms pt

A

periventricular lesions

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19
Q

in csf of ms pt you see what

A

igG oliglonal bands
high protein
and cd4 T cells

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20
Q

cerebellar disease affects contralateral side
(true or false)

A

false. ipsilateral side bcz of decussation

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21
Q

signs of cerebellar disease or spinocerebellar ataxia

A

DANISH
disdiadokinesa
ataxia
nystagmus
intention tremor
slurred speech
hypotonia

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22
Q

pancerebellar syndrome causes bilateral signs what are differentials

A

toxins
metabolic
infections
autoimmune

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23
Q

what happens if (vestibulocerebellum) flocculonodular lobe is affected

A

problems in balance and eye movements
(truncal ataxia, vertigo, nystagmus, vestibular ocular reflex)

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24
Q

what is the function of spinocerebellum (vermis and intermediate hemisphere)

A

proprioception

regulate muscle tone and coordinate limb movements

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25
Q

what happens if vermis or intermediate zone are affected

A

gait ataxia
dysmetria
disdadokinesia

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26
Q

how can u differ if person has lesion in dorsal column or vermis / intermediate zone ?

A

by romberg sign , if cerebellar pt will sway and lose balance with eyes open

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27
Q

what happens if rostral (anterior) vermis is affected ?

A

signs: gait and trunk ataxia
differentials: alcoholism, thiamine deficient

28
Q

caudal (posterior) vermis function?

A

coordinates balance and eye movements
postural stability during voluntary movements
associated w vestibular system

29
Q

if caudal posterior vermis affected what are the signs ?

A

nystagmus
vertigo
balance disturbance
truncal ataxia

30
Q

differentials of caudal vermis is

A

medulloblastoma - often in posterior vermis in children

31
Q

drug induced cerebellar degeneration from what drugs

A

anti-epileptics (phenytoin)

32
Q

what is paraneoplastic cerebellar degen ?

A

autoimmune reaction where immune system in response to a tumor mistakenly attack the cerebellum w

33
Q

what do u see in serum csf in paraneoplastic cerebellum degeneration?

A

paraneoplastic antibodies

34
Q

what is the trinucleotide repeat disorder in friedreich’s ataxia?

A

> /= 200 GAA repeats

35
Q

friedriech ataxia mutation in what gene

A

FXN gene on chrom 9 encodes protein frataxin

36
Q

pathophysiology of FA

A

frataxin defeciency impair mitochondrial function causing oxidative stress and energy production deficit in cns cardio pancreas

37
Q

to confirm multiple sclerosis what do u see in fundoscopy

A

optic disc swelling in one eye and in advanced stage optic atrophy

38
Q

if color test is done w pts with ms what will they see

A

red desaturation

39
Q

what is a pnemonic to remember features of idiopathic intracranial hemorrhage( pseudotumor cerebri)

A

FATHEAD

female
abnormal wt
tinnutis
headache
elevated icp
absence of tumor
double vision (CN6)

40
Q

first line tx of ms

A

steroids

41
Q

define increased intracranial pressure

A

sustained elevation in pressure above 20 (normal btwn 8-18)

42
Q

what is progressive decline in memory and at least one other cognitive area such as attention, orientation, judgment

A

dementia

43
Q

what are causes of dementia or risk factors

A

age (elderly)
gender female
down syndrome
Apo E4

44
Q

reversible causes of dementia

A

hypoglycemia
hypothyroidism
vit b12 deficiency
normal pressure hydrocephalus

45
Q

what is acute disorder of attention and global cognition and dusturbance in consciousness that develops rapidly

A

delerium

46
Q

how can u differ between dementia and delirium

A

alertness fluctuates in delirium whereas alertness is normal in dementia

47
Q

what are differentials of dementia

A

alzheimers disease
vascular disease (repeated strokes–> cognitive decline)
drugs, depression, delirium
ethanol (korsakoff wernicke encephalopathy)
endocrine, ears, eye, environ
amnesia, autoimmune, apnea

48
Q

causes of delerium include

A

D dementia
E electrolyte disorder
L lung, liver, heart, kidney, brain
I infection (UTI)
R Rx drugs
I injury, pain, stress
U unfamiliar environment
M metabolic

49
Q

what disease includes extracellular amyloid plaques and intracellular tangles of hyperphosphoralated tau protein (neurofibrillary tangles )

A

alzheimers disease

50
Q

what is the pathology in alzhemiers what accumulates?

A

defective processing of APP( precursor of AB) result in accumulation of AB42 with increase b-secretase activity

51
Q

what may cause death in alzheimers disease?

A

aspiration pneumonia and infections

52
Q

what is an intermediate zone btwn normal and AD w presence of new memory complains with lack of impairment in other cog functions

A

mild cognitive impairment

53
Q

what neurodegenerative disease is associated with parkinsons

A

lewy body dementia

54
Q

what type of dementia is due to stroke

A

vascular dementia

55
Q

what do u see in vascular dementia

A

multi-infarct, strokes, hemorrhages (cystic spaces seen on autopsy)

56
Q

vascular dementia shows signs of upper motor neuron or lower

A

UMNL

57
Q

what type of dementia shows early change in personality, loss of empathy, and socially inapropriate behavior?

A

frontotemporal dementia (pick’s disease)

58
Q

what kind of dementia that drugs of AD dont help with

A

picks disease (frontotemporal dementia )

59
Q

what is the early onset or hallmark of lewy body dementia

A

visual hallucinations

60
Q

what is an autosomal dominant disease characterized by progressive chorea, personality change, dementia, death

A

huntingtons disease

61
Q

what is the cause of huntington disease depletion of what hormones ?

A

Gaba (inhibitory neuron) and acetylcholine sparing dopamine

62
Q

how is dopamine spared in huntington?

A

the main part degenerated in HD is striatum which includes gaba and ach and dopamine remain intact and elevated bcz of loss of ihibitory gaba input

63
Q

what type of chorea occurs due to post-strep in children and history of enlarged tonsils followed by fidgety movements

A

sydenhams chorea

64
Q

what are sudden electric jerk like movements of a part of the body or whole body

A

myoclonus

65
Q

sudden vigorous forceful movements usually involving a whole limb due to stroke in subthalamic nucleus

A

ballismus