Movement Disorders Flashcards
Principal location of morbid anatomy with
Unilateral plastic rigidity with rest tremor (Parkinsons Disease)
Contralateral substantia nigra
plus (?) other mesencephalic structures
Principal location of morbid anatomy with
Unilateral hemiballismus and hemichorea
contralateral subthalamic nucleus of Luys or luysial pallidal connections
Principal location of morbid anatomy with
Chronic chorea of Huntington Type
Caudate nucleus and putamen
Principal location of morbid anatomy with
athetosis and dystonia
Contralateral striatum (pathology of dystonia musculorum deformans unknown)
Principal location of morbid anatomy with
cerebellar incoordination, intention tremor and hypotonia
IPSILATERAL cerebellar hemisphere;
ipisilateral middle or inferior cerebellar peduncle
brachium conjunctivum, (ipsilateral if below decussation, contralateral if above)
Principal location of morbid anatomy with
Decerebrate rigidity
ie extension of arms and legs, opisthotonos
usually bilateral in tegmentum of upper brainstem at the level of red nucleus or between red and vestibular nuclei
Principal location of morbid anatomy with
Palatal and facial myoclonus (rhythmic)
ipsilateral central tegmental tract with denervagtion of inferior olivary nucleus and nucleus ambiguus
Principal location of morbid anatomy with
diffuse myoclonus
neuronal degeneration, usually diffuse or predominating in cerebral or cerebellar cortex and dentate nuclei
Most common initial symptom of Parkinsons Disease
a. Stiffness
b. Tremor
c. gait disturbance
d. slowness
b. tremor
Which medication for Parkinsons Disease is associate with the side effect?
urine discoloration
COMT inhibitors - ENTACAPONE
urine discoloration - entacapone
Which medication for parkinsons Disease is associated with hypertensive crisis with intake of tyramine rich foods and sympathomimetics?
MAO inhibitors
Rasagiline
hypertensive crisis
Which of the following medication for parkinsons disease is associated with atropinic effects: dry mouth, urinary outlet obstruction, confusion, psychosis?
a. Levodopa
b. Ropinorole
c. Amantadine
d. Benztropine
D. Benztropine. (anticholinergic)
same as trihexyphenidyl
A parkinsons Disease medication that may cause chronic livedo reticularis
Amantadine
another term for Negro sign
cogwheel phenomenon
rigidity and its cogwheel component are elicited or enhanced by having the patient engage the opposite limb in a motor task requiring some degree of concentration.
what sign is this?
FROMENT SIGN
True or false.
there should be no pyramidal signs in parkinsons disease, unless they are due to an alternate disorder
TRUE
inability to inhibit blinking in response to a tap over the bridge of the nose or glabella
sign?
MYERSON sign
The following are considerations for rapid onset parkinsonism, except:
a. neuroleptic medications
b. variant of Creutzfeldt Jakob disease
c. use of metoclopramide
d. none of the above
D. none of the above
Pathological finding in both idiopathic and postencephalitic parkinsons Disease?
Loss of pigmented cells in the substantia nigra and other pigmented nuclei
(locus ceruleus, dorsal motor nucleus of the vagus)
Rate limiting enzyme for the synthesis of dopamine?
Tyrosine hydroxylase
Protein which is the main component of Lewy Bodies
a-synuclein
a ubiquitin protein ligase that participates in the removal of unnecessary proteins form cells through the proteasomal system.
PARKIN
\
What gene is implicated in Parkinsons Disease that is usually seen in Ashkenazic Jews?
LRRK2 (leucine rich repeat kinase 2)
PARK8
Gene implicated in Parkinsons Disease that accounts for 50% of early onset inherited PD and 20% of sporadic early onset cases
PARK 2
parkin - protein
PARK 1 and PARK 4 is associated with this gene (and protein)
SCNA (a-synuclein)
In parkinsons Disease, mutation in this gene codes for a mitochondrial kinase
PINK
Park 6
Results of ADAGIO trial for rasagiline in Parkinsons Disease? *
no significant change in 2mg
MRI findings in MSA
hot cross bun sign
atrophy of the pontocerebellar fibers -> high T2 signal intensity in an atrophic pons
Most common early complaint of PSP?
unsteadiness of gait and unexplained falling without loss of consciousness
MRI finding in PSP
humming bird sign,
micky mouse ears
due to midbrain atrophy
Drug reported to ameliorate the akinesia and rigidity of PSP
zolpidem
Most common initial symptom of Corticobasal ganglionic degeneration
asymmetrical clumsiness of limbs
(seen in half of the patients)
in one study.
Progressive parkinsonism and dementia are combined with upper or lower motor neuron disease, predominently in Men and occurring in the indigenous Chamorro people of Guam and the Mariana islands
Guamanian Parkinsons-dementia-ALS complex
page 1122
A disorder with acanthocytosis and gradual development of chorea in the middle to late life characterized by degeneration of caudate and putamen and myopathy.
McLeod disease
A 7 year old female presenting with intermittent stiffening of the left lower extremity, frequent falls, peculiar posturing, eventually associated with torticollis and mild parkinsonian features. She had a trial of levodopa at 20mg/ day which eliminated the involuntary movements.
what is the gene mutation implicated in this case?
GCH1 mutation
case: hereditary dystonia-parkinsonism
Segawa Syndrome
Juvenile Dopamine Responsive Dystonia
Gene mutation in Friedreich Ataxia
FNX mutation
frataxin
Genetic defect in DRPLA
Dentatorubropallidoluysian Atrophy
ATN1
CAG trinucleotide repeat in ATN1
codes for atrophin 1
In DRPLA, clinical manifestations are associated with _____ number of CAG repeat triplets
48-93 repeat triplets
normal population: 7-35
Which of the following is inherited as an AR pattern?
a. DRPLA
b. Friedreich Ataxia
c. Machado-Joseph
d. SCA1
b. Friedreich Ataxia
- rest are AD
mutation in episodic ataxia, Type 2?
calcium channel gene on chromosome 19
mutation in paroxysmal ataxia (EA-1)
potassium channel gene on Chromosome 12
The following statements are true of episodic ataxia, type 2, EXCEPT:
a. episodes of ataxia lasts several hours
b. associated with mutation in calcium channel gene on chr 19
c. vertigo is a prominent feature of the attacks
d. does not respond to acetazolamide
D.
-> strikingly responds to administration of acetazolamide
Match the following episodic ataxias with the genetic mutation:
- Episodic ataxia with myokymia (EA1, EAM)
- Paroxysmal Episodic ataxia (EA2)
- Episodic ataxia (EA5)
a. CACNB4
b. KCNA1
c. CACNA1A
- B. KCNA1
- C. CACNA1A
- A. CACNB4
This is the ebb and flow of parkinsonian signs experienced by patients with PD, wherein they alternate between periods of good and poor motor response to dopaminergic therapy
A. Motor fluctuation
B. Wearing off
C. Delayed On
D. Dyskinesias
A. Motor fluctuations
The ‘hot cross bun sign’ in MRI of patients with MSA is due to the degeneration of which fibers?
a. corticospinal fibers
b. pontocerebellar fibers
c. olivocerebellar fibers
d. corticobulbar fibers
b. pontocerebellar fibers
What is the pathological protein that links CBGD, corticospinal degeneration, PSP and FTD?
a. tau
b. TDP-43
c. alpha synuclein
d. amyloid
a. tau
Which medication was found to have possible neuroprotective benefits based on the ADAGIO trial?
a. entacapone
b. rasagiline
c. pramipexole
d. selegeline
b. rasagiline
Case of a male patient taking an antiparkinsonian drug who was admitted to the ER due to hypertensive crisis after intake of large amounts of cheese. Which is likely the drug precipitating this condition?
a. levodopa/carbidopa
b. entacapone
c. pramipexole
d. selegeline
d. selegeline
MAO inhibitors
rasagiline
selegeline
can cause hypertensive crisis
Which of the following symptoms is usually the earliest feature in NPH?
a. impairment of mental function
b. headache
c. sphincter incontinence
d. slowly progressive gait disorder
d. slowly progressive gait disorder
which of the following does not use glutamate as the neurotransmitter?
a. cortex to corpus striatum
b. corpus striatum to subthalamic nucleus
c. subthalamic nucleus to pallidum
d. substantia nigra to corpus striatum
d. substantia nigra to corpus striatum
Which of the following has been proven effective for the akinesia and rigidity in PSP?
a. benztropine
b. levodopa/carbidopa
c. botulinum toxin
d. zolpidem
d. zolpidem
A 32/M with parkinsons disease was found to have PARK 7 (DJ1) mutation. which of the following PD phenotypic expressions is explained by this mutation?
a. rapid progression
b. slow progression
c. severe symptoms
d. mild symptoms
b. slow progression
PARK 7
(DJ-1)
AR
occurs in 30s
slow progression, gene plays role in cellular response to oxidative stress
which of the following structures is NOT affected in MSA?
a. striatum
b. lateral horn cells of the spinal cord
c. globose-emboliform nuclei
d. substantia nigra
c. globose emboliform nuclei
which of the following is not an initial manifestation of corticobasal degeneration?
a. rest tremor
b. clumsiness
c. myoclonus
d. non fluent aphasia
a. rest tremor
What is the least likely genetic mutation in a young onset PD who has a cousin with young onset PD?
a. PARK 1
b. PARK 2
c. PARK 3
d. PARK 4
b. PARK 2
LEAST LIKELY - PARK 2 is AR
PARK 1, 3 and 4 are AD.
which of the following PD medication is incorrectly paired with its mechanism of action
a. peribidil - dopamine agonist
b. rasagiline - MAO B inhibitor
c. benztropine - GABA agonist
d. Entacapone - COMT inhibitor
C.
Benztropine is an anticholinergic
Which of the following antiparkinsonism drugs is D3 receptor selective?
a. bromocriptine
b. pramipexole
c. ropinirole
d. pergolide
b. pramipexole
Band of nerve fibers that separates the caudate nucleus and the lentiform nucleus
internal capsule
Principal output of the basal ganglia
a. putamen
b. caudate nucleus
c. globus pallidus
d. subthalamic nucleus
c. globus pallidus
type of neurons that make up 80-95% of cells in the striatum.
medium spiny neurons
white matter fibers that separates the claustrum from the insular cortex
a. internal capsule
b. external medullary lamina
c. external capsule
d. extreme capsule
d. extreme capsule
white matter fibers that is lateral to the lentiform nucleus and separates the LN from the claustrum
a. internal capsule
b. external medullary lamina
c. external capsule
d. extreme capsule
c. external capsule
divides the lentiform nucleus into the putamen and the globus pallidus
a. internal capsule
b. external medullary lamina
c. external capsule
d. extreme capsule
b. external medullary lamina
divides the globus pallidus into GP externa and interna
a. internal / medial medullary lamina
b. external medullary lamina
c. external capsule
d. extreme capsule
a. internal / medial medullary lamina
Main source of dopamine in the basal ganglia
substantia nigra pars compacta
principal output of the basal ganglia
paleostriatum/
globus pallidus
receptive component of the basal ganglia
neostriatum/
caudate nucleus and putamen
(1) main projection neurons of the striatum
(2) what neurotransmitter do they utilize?
(1) medium spiny neurons (MSNs)
(2) GABA/inhibitory output
T/F
the subthalamic nucleus contains glutaminergic neurons and are excitatory
True
pramipexole acts as a direct agonist at the ____ receptor
a. D1
b. D2
c. D3
d. D4
a. D1
