Movement Disorders Flashcards by Jadda Sajo

Principal location of morbid anatomy with

Unilateral plastic rigidity with rest tremor (Parkinsons Disease)

Contralateral substantia nigra

plus (?) other mesencephalic structures

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Principal location of morbid anatomy with

Unilateral hemiballismus and hemichorea

contralateral subthalamic nucleus of Luys or luysial pallidal connections

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Principal location of morbid anatomy with

Chronic chorea of Huntington Type

Caudate nucleus and putamen

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Principal location of morbid anatomy with

athetosis and dystonia

Contralateral striatum 
(pathology of dystonia musculorum deformans unknown)

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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)

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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

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Principal location of morbid anatomy with

Palatal and facial myoclonus (rhythmic)

ipsilateral central tegmental tract with denervagtion of inferior olivary nucleus and nucleus ambiguus

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Principal location of morbid anatomy with

diffuse myoclonus

neuronal degeneration, usually diffuse or predominating in cerebral or cerebellar cortex and dentate nuclei

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Most common initial symptom of Parkinsons Disease

a. Stiffness
b. Tremor
c. gait disturbance
d. slowness

b. tremor

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Which medication for Parkinsons Disease is associate with the side effect?

urine discoloration

COMT inhibitors - ENTACAPONE

urine discoloration - entacapone

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Which medication for parkinsons Disease is associated with hypertensive crisis with intake of tyramine rich foods and sympathomimetics?

MAO inhibitors
Rasagiline

hypertensive crisis

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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

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A parkinsons Disease medication that may cause chronic livedo reticularis

Amantadine

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another term for Negro sign

cogwheel phenomenon

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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

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True or false.

there should be no pyramidal signs in parkinsons disease, unless they are due to an alternate disorder

TRUE

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inability to inhibit blinking in response to a tap over the bridge of the nose or glabella

sign?

MYERSON sign

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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

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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)

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Rate limiting enzyme for the synthesis of dopamine?

Tyrosine hydroxylase

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Protein which is the main component of Lewy Bodies

a-synuclein

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a ubiquitin protein ligase that participates in the removal of unnecessary proteins form cells through the proteasomal system.

PARKIN

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What gene is implicated in Parkinsons Disease that is usually seen in Ashkenazic Jews?

LRRK2 (leucine rich repeat kinase 2)

PARK8

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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

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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: 1. Episodic ataxia with myokymia (EA1, EAM) 2. Paroxysmal Episodic ataxia (EA2) 3. Episodic ataxia (EA5) a. CACNB4 b. KCNA1 c. CACNA1A

1. B. KCNA1 2. C. CACNA1A 3. 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