Neurology - Hyperkinetic Movement Disorders Flashcards
chronic traumatic encephalopathy is …
a.hypokinetic
b.hyperkinetic
a.hypokinetic
its an akinetic rigid syndrome
MSA, PSP and CBD are…
a.hypokinetic
b.hyperkinetic
a.hypokinetic
Parkinsons is ..
a.hyperkinetic
b.hypokinetic
b.hypokinetic
lewy body dementia is …
a.hyperkinetic
b.hypokinetic
b.hypokinetic
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
involuntary rhythmic oscillatory movement of a body part
a. tremor
b.dystonia
c.chorea
d.hemiballismus
e.tics
a. tremor
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
a tremor that occurs with voluntary muscle contraction
a.resting tremor
b.action tremor
b.action tremor
what are the 3 types of action tremor
postural
isometric
kinetic
possible causes of tremor
essential tremor
genetic tremor disorders
degenrative disorders
metabolic diseases
peripheral neuropathies
drug induced
toxins
functional
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
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
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
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
how is dystonia classified
area affected - focal/generalised
age of onset- juvenille/late
secondary to other degenerative disease ?
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
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
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
b.weak and breathy voice
cords fold open and do not vibrate
how is larangeal dystonia diagnosed
a.clinical diagnosis
b.ENT laynoscopy
c.laproscopy
d. muscle biospy
b.ENT laynoscopy
what is the treatment for laryngeal dystonia
a.methrotrexate
b. botox injections
c. steroid injections
d.surgery
b. botox injections
the onset age for generalised dystonia is usually..
a.over 25 yrs
b.under 25 years
b.under 25 years
typically where does generalised dystonia onset
a.upper limbs
b.lower limbs
c.trunk
d.neck
e.larynx
b.lower limbs
50% of those with generalised dystonia have which mutation
a.DYT1
b.DYT2
c.DYT3
d.DYT4
e.DYT5
a.DYT1
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
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
