Neurology - Hyperkinetic Movement Disorders Flashcards by Jenna Dymond

chronic traumatic encephalopathy is …

a.hypokinetic
b.hyperkinetic

a.hypokinetic

its an akinetic rigid syndrome

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MSA, PSP and CBD are…

a.hypokinetic
b.hyperkinetic

a.hypokinetic

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Parkinsons is ..

a.hyperkinetic
b.hypokinetic

b.hypokinetic

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lewy body dementia is …

a.hyperkinetic
b.hypokinetic

b.hypokinetic

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MSA, PSP, CBD are all examples of what

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

b.atypical parkinsonism

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involuntary rhythmic oscillatory movement of a body part

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

a. tremor

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

a.resting tremor
b.action tremor

a.resting tremor

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a tremor that occurs with voluntary muscle contraction

a.resting tremor
b.action tremor

b.action tremor

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what are the 3 types of action tremor

postural
isometric
kinetic

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possible causes of tremor

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

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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. essential tremor

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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. essential tremor

patients also often have a family history

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

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

b.dystonia

caused by genetics/ birth trauma

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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

d.putamen / thalamus

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how is dystonia classified

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

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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

b.dystonia

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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.strangled speech sounds, words cut off

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

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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

cords fold open and do not vibrate

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how is larangeal dystonia diagnosed

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

b.ENT laynoscopy

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what is the treatment for laryngeal dystonia

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

b. botox injections

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the onset age for generalised dystonia is usually..

a.over 25 yrs
b.under 25 years

b.under 25 years

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typically where does generalised dystonia onset

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

b.lower limbs

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50% of those with generalised dystonia have which mutation

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

a.DYT1

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

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

c.chorea

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

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what condition leads to sydenhams chorea a.scarlett fever b.rheumatic fever c.yellow fever d.HIV e.Parvovirus B19

b.rheumatic fever

which of these is a hyperkinetic , psychiatric and cognitive neurodegenerative disorder a.chronic traumatic encephalopathy b. MSA c. PSP d. huntingtons e.parkinsons

d. huntingtons chorea

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

d. huntingtons

how is the huntingtons gene inherited a.autosomal dominant b.autosomal recessive c.x linked

a.autosomal dominant

how many offspring of an affected parent will inherit huntigtons disease a.25% b.50% c.75% d.100%

b.50%

when does huntigtons usually onset a.below 25 years b. at adolescence c.at 40 years d.60+ years

c.at 40 years

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

d. huntingtons

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.caudate nucleus/ putamen

which neurotransmitter is reduced in huntingtons a.dopamine b.GABA c.ACh d.Serotonin e.glutamate

b.GABA

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

d. huntingtons

what is used to reduce chorea in huntingtons a.botox injections b.tetrabenzine c.chloramazepine d. antipsychotics e.tramadol

b.tetrabenzine

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

b.10-20 yrs

severe proximal chorea, with violent flinging movements of ONE side of the body a. tremor b.dystonia c.chorea d.hemiballismus e.tics

d.hemiballismus

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

d.subthalmic nucleus

in extreme cases of hemiballismus what is given a.botox injections b.tetrabenzine c.chloramazepine d. dopamine blockers e.tramadol

d. dopamine blockers or surgery

sudden repetitive , non rhythmic , stereotyped movement or vocalsation , with a preceeding urge a. tremor b.dystonia c.chorea d.hemiballismus e.tics

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

kicking ,jumping and hopping are examples of.. a.simple motor tics b.complex motor tics

b.complex motor tics

blinking, pouting and grimacing are examples of.. a.simple motor tics b.complex motor tics

a.simple motor tics

repeating words spoken by someone else

echolalia complex vocal tic

spontaneous utterance of obscene words

coprolalia complex vocal tic

grunting, sniffing and coughing are all... a.simple vocal tics b. complex vocal tics

a.simple vocal tics

primary causes of tics

simple transient tics of childhood giles de la tourettes syndrome

secondary causes of tics

neurodegenerative disease eg huntingtons/wilsons learning disabilities - autism, down syndrome, retts syndrome infection eg syndenhams chorea - rheum, PANDAS (strep)

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

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

what is the usual treatment for tourettes syndrome a.botox injections b.tetrabenzine c.psychobehavioural therapy d. dopamine blockers e.tramadol

c.psychobehavioural therapy

what is the prognosis for tourettes

improves with maturity normal life expectancy and intelligence

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. myoclonus contractions - positive myoclonus relaxation - negative myoclonus

causes of myoclonus

epilepsy CJD uraemia liver failures drug induced often

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

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.guillian mollaret triangle ipsilateral red nucleus and inferior olivary nucelus and contralateral

dyskinesia

involuntary, erratic writhing movements of face arms legs trunk

salbutomol , lihtium and valproate can all cause what a.tremor b.chorea c.dystonia d.hemiballismus

a.tremor

chorea is commonly due to which drug in parkinsons patients a. lithium b.valproate c.levodopa d. salbutomol e.chloromazepine

c.levodopa

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

d.tardive dyskinesia

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%

C.50%

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

c.acute dystonic reaction can occur with a single dose

acute dystonic reactions to neuroleptics/ antipsychotics are treated with what a.botox injections b.tetrabenzine c.psychobehavioural therapy d. dopamine blockers e.IV anticholinergic

e.IV anticholinergic benztropine or procyclidine

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

athetosis

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

d.striatum and globus pallidus

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

akathisia

wilsons disease and huntingtons are both examples of what...

mixed moevement disorders

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

d.wilsons disease

accumulation of what substance in the brain, liver and cornea is seen in wilsons disease a.iron b.copper c.magnesium d.bilirubin

b.copper

what is the inehritance pattern of wilsons disease a.autosomal dominant b.autosomal recessive c.x linked

b.autosomal recessive

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

c.wilsons

what is the treatment for wilsons a.botox injections b.tetrabenzine c.penicillamine/trietene d. dopamine blockers e.benxtrycline/procyclidine

c.penicillamine/trietene copper chelating drugs

pencillamine and trietine are examples of ............. chelating drugs used to treat wilsons a.iron b.copper c.magnesium d.bilirubin

b.copper

lack of coordination of voluntary motor acts impairing their smooth performance

ataxia

causes of ataxia

cerebellar, sensory, optic and frontal dysfunction

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

sensory ataxia

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

b.rombergs

involuntary slow writhing movements resulting from loss of proprioception

pseudoathetosis

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

d.cerebellar ataxia

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

d.cerebellar ataxia

lesions in which part of the cerebellum cause ipsilateral limb ataxia a.hemisphere b.midline

a.hemisphere

lesions in which part of the cerebellum cause truncal and gait ataxia a.hemisphere b.midline

b.midline

causes of cerebellar ataxia

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