Neurology - Hyperkinetic Movement Disorders Flashcards

1
Q

chronic traumatic encephalopathy is …

a.hypokinetic
b.hyperkinetic

A

a.hypokinetic

its an akinetic rigid syndrome

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2
Q

MSA, PSP and CBD are…

a.hypokinetic
b.hyperkinetic

A

a.hypokinetic

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3
Q

Parkinsons is ..

a.hyperkinetic
b.hypokinetic

A

b.hypokinetic

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4
Q

lewy body dementia is …

a.hyperkinetic
b.hypokinetic

A

b.hypokinetic

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5
Q

MSA, PSP, CBD are all examples of what

a.parkinsons disease
b.atypical parkinsonism
c. drug induced parkinsonism
d.lewy body dementia
e.alzheimers

A

b.atypical parkinsonism

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6
Q

involuntary rhythmic oscillatory movement of a body part

a. tremor
b.dystonia
c.chorea
d.hemiballismus
e.tics

A

a. tremor

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7
Q

a tremor that occurs in a body part that is not activated and completely supported against gravity

a.resting tremor
b.action tremor

A

a.resting tremor

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8
Q

a tremor that occurs with voluntary muscle contraction

a.resting tremor
b.action tremor

A

b.action tremor

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9
Q

what are the 3 types of action tremor

A

postural
isometric
kinetic

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10
Q

possible causes of tremor

A

essential tremor
genetic tremor disorders
degenrative disorders
metabolic diseases
peripheral neuropathies
drug induced
toxins
functional

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11
Q

what is the most common tremor syndrome in adults

a. essential tremor
b. genetic tremor disorders
c. degenrative disorders
d. metabolic diseases
e. peripheral neuropathies
f. drug induced

A

a. essential tremor

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12
Q

patient with bilateral tremor in hands, arms and speech
frequency : 4-7 hz
no other neuro signs
improves with alcohol
handwriting is shaky , size normal
patient noticed it improved when starting beta blockers
which most likely diagnosed

a. essential tremor
b. genetic tremor disorders
c. degenrative disorders
d. metabolic diseases
e. peripheral neuropathies
f. drug induced

A

a. essential tremor

patients also often have a family history

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13
Q

involuntary sustained contractions of opposing muscle groups causing stereotyped twisting movements or abnormal postures

a. tremor
b.dystonia
c.chorea
d.hemiballismus
e.tics

A

b.dystonia

caused by genetics/ birth trauma

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14
Q

what is the pathological location of dystonia suspected to be

a.caudate nucleus/ putamen

b.putamen/ globus pallidus

c.substancia nigra

d.putamen / thalamus

e.red nucleus / hypothalamus

A

d.putamen / thalamus

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15
Q

how is dystonia classified

A

area affected - focal/generalised
age of onset- juvenille/late
secondary to other degenerative disease ?

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16
Q

patient presenting with abnormal posture, pain, cramps and tremor, appear to have twisiting movements of limbs what diagnosis most likely

a. tremor
b.dystonia
c.chorea
d.hemiballismus
e.tics

A

b.dystonia

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17
Q

what does the voice sound like of the adductor muscles of the larynx are affected by larangeal dystonia

a.strangled speech sounds, words cut off

b.weak and breathy voice

A

a.strangled speech sounds, words cut off

vocal cords slam together and stiffen
difficulty for vibration and therefore voice production

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18
Q

what does the voice sound like of the abductor muscles of the larynx are affected by larangeal dystonia

a.strangled speech sounds, words cut off

b.weak and breathy voice

A

b.weak and breathy voice

cords fold open and do not vibrate

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19
Q

how is larangeal dystonia diagnosed

a.clinical diagnosis
b.ENT laynoscopy
c.laproscopy
d. muscle biospy

A

b.ENT laynoscopy

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20
Q

what is the treatment for laryngeal dystonia

a.methrotrexate
b. botox injections
c. steroid injections
d.surgery

A

b. botox injections

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21
Q

the onset age for generalised dystonia is usually..

a.over 25 yrs
b.under 25 years

A

b.under 25 years

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22
Q

typically where does generalised dystonia onset

a.upper limbs
b.lower limbs
c.trunk
d.neck
e.larynx

A

b.lower limbs

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23
Q

50% of those with generalised dystonia have which mutation

a.DYT1
b.DYT2
c.DYT3
d.DYT4
e.DYT5

A

a.DYT1

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24
Q

rapid jerky movements that flit from one part of the body to another

a. tremor
b.dystonia
c.chorea
d.hemiballismus
e.tics

A

c.chorea

causes..
huntingtons
rheumatic fever
drugs - OCP, Ldopa
pregnancy

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25
Q

what condition leads to sydenhams chorea

a.scarlett fever
b.rheumatic fever
c.yellow fever
d.HIV
e.Parvovirus B19

A

b.rheumatic fever

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26
Q

which of these is a hyperkinetic , psychiatric and cognitive neurodegenerative disorder

a.chronic traumatic encephalopathy
b. MSA
c. PSP
d. huntingtons
e.parkinsons

A

d. huntingtons

chorea

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27
Q

which condition is caused by a triplet repeat disorder of the CAG triplet >40 x producing the huntingtin protein

a.chronic traumatic encephalopathy
b. MSA
c. PSP
d. huntingtons
e.parkinsons

A

d. huntingtons

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28
Q

how is the huntingtons gene inherited

a.autosomal dominant
b.autosomal recessive
c.x linked

A

a.autosomal dominant

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29
Q

how many offspring of an affected parent will inherit huntigtons disease

a.25%
b.50%
c.75%
d.100%

A

b.50%

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30
Q

when does huntigtons usually onset

a.below 25 years
b. at adolescence
c.at 40 years
d.60+ years

A

c.at 40 years

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31
Q

boxcar sign : widening of the frontal horns of the lateral venricles where the caudate nucleus has atrophied is seen in what

a.chronic traumatic encephalopathy
b. MSA
c. PSP
d. huntingtons
e.parkinsons

A

d. huntingtons

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32
Q

loss of cells in which structures leads to boxcar sign on ct in huntigtons

a.caudate nucleus/ putamen

b.putamen/ globus pallidus

c.substancia nigra

d.putamen / thalamus

e.red nucleus / hypothalamus

A

a.caudate nucleus/ putamen

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33
Q

which neurotransmitter is reduced in huntingtons

a.dopamine
b.GABA
c.ACh
d.Serotonin
e.glutamate

A

b.GABA

34
Q

patient presents wit jerky uncontrollable movements , unsteady gait, dysarthria, dysphagia , anxiety and insomnia
ct brain shows boxcar sign
which most likely

a.chronic traumatic encephalopathy
b. MSA
c. PSP
d. huntingtons
e.parkinsons

A

d. huntingtons

35
Q

what is used to reduce chorea in huntingtons

a.botox injections
b.tetrabenzine
c.chloramazepine
d. antipsychotics
e.tramadol

A

b.tetrabenzine

36
Q

how long after onset of huntigtons does death usually occur

a.5-10 yrs
b.10-20 yrs
c.20-30 yrs
e. does not effect life expectancy

A

b.10-20 yrs

37
Q

severe proximal chorea, with violent flinging movements of ONE side of the body

a. tremor
b.dystonia
c.chorea
d.hemiballismus
e.tics

A

d.hemiballismus

38
Q

hemiballismus is usually caused by infarction/ haemorrhage in the opposite …….

a.red nucleus
b.substancia nigra
c.putamen and caudate nucleus
d.subthalmic nucleus
e.putamen and thalamus

A

d.subthalmic nucleus

39
Q

in extreme cases of hemiballismus what is given

a.botox injections
b.tetrabenzine
c.chloramazepine
d. dopamine blockers
e.tramadol

A

d. dopamine blockers

or surgery

40
Q

sudden repetitive , non rhythmic , stereotyped movement or vocalsation , with a preceeding urge

a. tremor
b.dystonia
c.chorea
d.hemiballismus
e.tics

A

e.tics

build up of tension, choose to release and relieve the urge
surpressible yet irresisitible
increasing with stress and excitement
decreasing when concentrating

41
Q

kicking ,jumping and hopping are examples of..

a.simple motor tics
b.complex motor tics

A

b.complex motor tics

42
Q

blinking, pouting and grimacing are examples of..

a.simple motor tics
b.complex motor tics

A

a.simple motor tics

43
Q

repeating words spoken by someone else

A

echolalia

complex vocal tic

44
Q

spontaneous utterance of obscene words

A

coprolalia

complex vocal tic

45
Q

grunting, sniffing and coughing are all…

a.simple vocal tics
b. complex vocal tics

A

a.simple vocal tics

46
Q

primary causes of tics

A

simple transient tics of childhood
giles de la tourettes syndrome

47
Q

secondary causes of tics

A

neurodegenerative disease eg huntingtons/wilsons

learning disabilities - autism, down syndrome, retts syndrome

infection eg syndenhams chorea - rheum, PANDAS (strep)

48
Q

severe expression of a tic disorder, 5 year old boy, hopping and blinking and grunting many times a day almost every day which most likely

a.transient tics of childhood
b.neurodegenerative disease
c.learning difficulty
d.sydenhams chorea
e.gilles de tourettes syndrome

A

e.gilles de tourettes syndrome

onset usually 5-7 years
m>f
multiple motor and at least 1 vocal tic occuring many times daily almost every day

49
Q

what is the usual treatment for tourettes syndrome

a.botox injections
b.tetrabenzine
c.psychobehavioural therapy
d. dopamine blockers
e.tramadol

A

c.psychobehavioural therapy

50
Q

what is the prognosis for tourettes

A

improves with maturity
normal life expectancy and intelligence

51
Q

brief involuntary twitch (lightning fast jerk) of a muscle or group of muscles
may be normal not always pathological

a. myoclonus
b.dystonia
c.chorea
d.hemiballismus
e.tics

A

a. myoclonus

contractions - positive myoclonus
relaxation - negative myoclonus

52
Q

causes of myoclonus

A

epilepsy
CJD
uraemia
liver failures
drug induced often

53
Q

which structures make up the guillian mollaret triangle responsible for modulating spinal cord motor activity

a.ipsilateral red nucleus , inferior olivary nucleus and contralateral dentate nucleus

b.contralteral red nucleus , inferior olivary nucelus and ipsilateral dentate nucelus

A

a.ipsilateral red nucleus , inferior olivary nucleus and contralateral dentate nucleus

54
Q

pathology in myoclonus is located where

a.guillian mollaret triangle
b.substancia nigra
c.putamen and caudate nucleus
d.subthalmic nucleus
e.putamen and thalamus

A

a.guillian mollaret triangle

ipsilateral red nucleus and inferior olivary nucelus and contralateral

55
Q

dyskinesia

A

involuntary, erratic writhing movements of face arms legs trunk

56
Q

salbutomol , lihtium and valproate can all cause what

a.tremor
b.chorea
c.dystonia
d.hemiballismus

A

a.tremor

57
Q

chorea is commonly due to which drug in parkinsons patients

a. lithium
b.valproate
c.levodopa
d. salbutomol
e.chloromazepine

A

c.levodopa

58
Q

mouthing and smacking of lips, grimaces with face and neck contortion occuring several years after treatment with neuroleptics

a.chorea
b.distonia
c.tics
d.tardive dyskinesia
e.acute dystonic reaction

A

d.tardive dyskinesia

59
Q

after stopping a neuroleptic drug causing tardive dyskinesia what is the liklehood that the patients symptoms will improve

a.10%
B.25%
C.50%
d.75%
e.100%

A

C.50%

60
Q

spasmodic torcollis, trismus and oculogyric crises (episodes of sustained upwards gaze) are examples of what effect of neuroleptics/antipsychotics

a.tardive dyskinesia
b.chorea
c.acute dystonic reaction

A

c.acute dystonic reaction

can occur with a single dose

61
Q

acute dystonic reactions to neuroleptics/ antipsychotics are treated with what

a.botox injections
b.tetrabenzine
c.psychobehavioural therapy
d. dopamine blockers
e.IV anticholinergic

A

e.IV anticholinergic
benztropine or procyclidine

62
Q

involuntar , irregular, slow writhing movements of the extremities and exagerrated postures of the limbs

A

athetosis

63
Q

where is the pathology located in athetosis eg cerebral palsy from birth trauma

a.guillian mollaret triangle
b.substancia nigra
c.putamen and caudate nucleus
d.striatum and globus pallidus
e.putamen and thalamus

A

d.striatum and globus pallidus

64
Q

unpleasant sensation of restlessness, manifesting as inability to sit still or remain motionless

A

akathisia

65
Q

wilsons disease and huntingtons are both examples of what…

A

mixed moevement disorders

66
Q

patient with progressive wing beating tremor, dystonia, chorea , rigidity and cognitive impairment , eye examination kaiser fleischer rings are observed which most likely diagnosis

a. myoclonus
b.huntingtons
c.chorea
d.wilsons disease
e.cerebral palsy

A

d.wilsons disease

67
Q

accumulation of what substance in the brain, liver and cornea is seen in wilsons disease

a.iron
b.copper
c.magnesium
d.bilirubin

A

b.copper

68
Q

what is the inehritance pattern of wilsons disease

a.autosomal dominant
b.autosomal recessive
c.x linked

A

b.autosomal recessive

69
Q

what condition is caused by a mutation in the gene for ATP dependent Cu transporting protein

a.parkinsons
b.huntingtons
c.wilsons
d.cerebral palsy
e.tardive dyskinesia

A

c.wilsons

70
Q

what is the treatment for wilsons

a.botox injections
b.tetrabenzine
c.penicillamine/trietene
d. dopamine blockers
e.benxtrycline/procyclidine

A

c.penicillamine/trietene

copper chelating drugs

71
Q

pencillamine and trietine are examples of …………. chelating drugs used to treat wilsons

a.iron
b.copper
c.magnesium
d.bilirubin

A

b.copper

72
Q

lack of coordination of voluntary motor acts impairing their smooth performance

A

ataxia

73
Q

causes of ataxia

A

cerebellar, sensory, optic and frontal dysfunction

74
Q

imapired proprioception due to disease of the dorsal columns of the spinal cord, neuropathies or dorsal root ganglia damage

A

sensory ataxia

75
Q

inability to stand with feet parallel upright with closed eyes describes which sign seen in sensory ataxia

a.trousseaus
b.rombergs
c.tinnels
d.phalens

A

b.rombergs

76
Q

involuntary slow writhing movements resulting from loss of proprioception

A

pseudoathetosis

77
Q

intention tremor, dysarthria , dysdiadochokinesia , head tremor, nystagmus, rebound phenomenon and limb hypotonia is seen in what

a.parkinsons
b.huntingtons
c.wilsons
d.cerebellar ataxia
e.tardive dyskinesia

A

d.cerebellar ataxia

78
Q

rebound phenomenon - patient moves limb against resistance ,when resistance is removed the limb moves a short direction in the desired way then jerks back in opposite direction

is seen in which condition

a.parkinsons
b.huntingtons
c.wilsons
d.cerebellar ataxia
e.tardive dyskinesia

A

d.cerebellar ataxia

79
Q

lesions in which part of the cerebellum cause ipsilateral limb ataxia

a.hemisphere
b.midline

A

a.hemisphere

80
Q

lesions in which part of the cerebellum cause truncal and gait ataxia

a.hemisphere
b.midline

A

b.midline

81
Q

causes of cerebellar ataxia

A

localised
MS
tumour
infarct
haemorrhage

global
infective - kids VZV cerebellitis
endocrine - myxoedema
immune mediated - opsoclonus, miller fisher syndrome

degenerative
MSA
SCA
episodic

toxic
DDT, mercury salts, 5- fluorouracil