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Neurodegenerative disorders Flashcards

1
Q

If only one cognitive domain is affected without functional impairment, it is more likely to be _ rather than dementia

A

If only one cognitive domain is affected without functional impairment, it is more likely to be mild cognitive impairment rather than dementia

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2
Q

Dementia is an acquired, persistent, progressive impairment in intellectual function that affects _ cognitive domain(s)

A

Dementia is an acquired, persistent, progressive impairment in intellectual function that affects multiple cognitive domain(s)
* One of which is usually memory
* Also see a decline in function
* Interference with work or social life

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3
Q

By definition, neurodegenerative disorders are _

A

By definition, neurodegenerative disorders are loss of neurological function (dementia, loss of movement control, paralysis) due to loss of neurons
* The most common is Alzheimer disease
* All neuronal death is linked to the deposition of abnormal proteins

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4
Q

Lewy body dementia is often associated with [symptoms]

A

Lewy body dementia is often associated with visual hallucinations, parkinson-like symptoms
* Tremor, shuffling gait, mask-like facies

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5
Q

CJD is associated with [neuro exam finding] and [neuro exam finding]

A

CJD is associated with myoclonus and ataxia

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6
Q

Progressive supranuclear palsy is sometimes referred to as _

A

Progressive supranuclear palsy is sometimes referred to as Parkinson plus
* Postural instability, bradykinesia, oculomotor findings

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7
Q

Supranuclear palsy is distinguished from parkinson disease by the presence of _

A

Supranuclear palsy is distinguished from parkinson disease by the presence of oculomotor findings (impaired downward gaze, loss of vertical saccades)
* They often fall backwards

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8
Q

Progressive supranuclear palsy is associated with accumulation of [protein] and formation of [structure]

A

Progressive supranuclear palsy is associated with accumulation of tau and formation of neurofibrillary tangles
* It is another “tauopathy”

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9
Q

The three most important proteinopathies associated with neurodegenerative dementias are:

A

The three most important proteinopathies associated with neurodegenerative dementias are:
1. Tau
2. Amyloid
3. Alpha synuclein

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10
Q

Alzheimer disease specifically affects [locations]

A

Alzheimer disease specifically affects cerebral cortex, hippocampus, amygdala

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11
Q

Plaques seen in Alzheimer disease are composed of [protein]

A

Plaques seen in Alzheimer disease are composed of beta-amyloid

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12
Q

Neurofibrillary tangles seen in Alzheimer disease are composed of [protein]

A

Neurofibrillary tangles seen in Alzheimer disease are composed of hyperphosphorylated tau

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13
Q

Lewy bodies are composed of [protein]

A

Lewy bodies are composed of alpha-synuclein
* Seen in Lewy body dementia and Parkinson disease
* “Lew” “Synu”

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14
Q

Diffuse lewy bodies is suggestive of _

A

Diffuse lewy bodies is suggestive of lewy body dementia
* Parkinson disease has lewy bodies in the basal ganglia

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15
Q

Huntington disease is associated with [protein] and specifically affects [locations]

A

Huntington disease is associated with mutant huntington protein and specifically affects caudate and putamen
* Intranuclear inclusions with polyglutaminated huntingtin due to CAG repeat expansion

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16
Q

CJD is an issue of [proteins]

A

CJD is an issue of prions forming amyloid

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17
Q

Diagnosis

A

Alzheimer disease: neurofibrillary tangles
* Hyperphosphorylated tau protein

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18
Q

Diagnosis

A

Alzheimer disease: amyloid plaques

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19
Q

Diagnosis

A

CJD: spongiform change

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20
Q

Ach gets made in [nucleus]

A

Ach gets made in basal nucleus of meynert

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21
Q

Dopamine is made in [nucleus]

A

Dopamine is made in ventral tegmentum (and the SNc)

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22
Q

GABA is made in [nucleus]

A

GABA is made in nucleus accumbens

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23
Q

NE is made in the [nucleus]

A

NE is made in the locus ceruleus
* In the pons

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24
Q

Serotonin is made in [nucleus]

A

Serotonin is made in raphe nuclei
* In the medulla, pons

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25
Q

Anxiety is associated with an increase in [NTs] and decrease in [NTs]

A

Anxiety is associated with an increase in NE and decrease in GABA, Serotonin

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26
Q

Depression is associated with an increase in [NTs] and decrease in [NTs]

A

Depression is associated with an increase in nothing and decrease in dopamine, NE, serotonin

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27
Q

Schizophrenia is associated with [NT change]

A

Schizophrenia is associated with increased dopamine

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28
Q

Alzheimer disease is associated with [NT change]

A

Alzheimer disease is associated with decreased Ach

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29
Q

Huntington disease is associated with an increase in [NTs] and decrease in [NTs]

A

Huntington disease is associated with an increase in dopamine and decrease in Ach, GABA

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30
Q

Parkinson disease is associated with an increase in [NTs] and decrease in [NTs]

A

Parkinson disease is associated with an increase in Ach and decrease in dopamine, serotonin

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31
Q

Cognitive fluctuation is a feature of [neurodegenerative disease]

A

Cognitive fluctuation is a feature of dementia with lewy bodies

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32
Q

Visual hallucination is a feature of [neurodegenerative disease]

A

Visual hallucination is a feature of dementia with lewy bodies

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33
Q

REM sleep behavior disorder is associated with [neurodegenerative disease]

A

REM sleep behavior disorder is associated with dementia with lewy bodies

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34
Q

Alzheimer disease may be detectable on MRI by the presence of _

A

Alzheimer disease may be detectable on MRI by the presence of hippocampal atrophy
* Also see widened sulci, enlarged ventricles, and shrinkage of cortex

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35
Q

Frontotemporal dementia is associated with _ and _ (cell inclusions)

A

Frontotemporal dementia is associated with Tau and TDP-43 (cell inclusions)

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36
Q

The pattern of atrophy in Alzheimer disease is _

A

The pattern of atrophy in Alzheimer disease is diffuse and symmetric;
* Seen in frontal, temporal, parietal and usually spares the occipital

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37
Q

Individuals with [chromosomal abnormality] are at an increased risk for Alzheimer

A

Individuals with down syndrome are at an increased risk for Alzheimer
* Due to third copy of chromosome 21 –> APP gene
* All patients with downs will eventually develop AD

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38
Q

What is APO-E and how is it related to Alzheimer?

A

APO-E is involved in cholesterol transport to neurons & breakdown of beta-amyloid

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39
Q

_ form of APO-E increases the risk of Alzheimer while _ form decreases risk

A

E4 form of APO-E increases the risk of Alzheimer while E2 form decreases risk

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40
Q

Alpha secretase/ alpha cleavage results in soluble, nontoxic protein whereas beta secretase/beta cleavage results in _

A

Alpha secretase/ alpha cleavage results in soluble, nontoxic protein whereas beta secretase/beta cleavage results in insoluble protein –> A-beta deposits

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41
Q

_ is a functional subdivision of the prefrontal cortex that is involved in moedulation of emotional behavior, decision making, and social recognition

A

Orbitofrontal cortex is a functional subdivision of the prefrontal cortex that is involved in moedulation of emotional behavior, decision making, and social recognition

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42
Q

Grossly, the brain would appear with _ in frontotemporal dementia

A

Grossly, the brain would appear with asymmetric atrophy of frontal and temporal lobe in frontotemporal dementia
* Note that it tends to be asymmetric (unlike Alzheimer)
* It tends to spare the parietal lobes

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43
Q

Frontotemporal dementia presents with [signs]

A

Frontotemporal dementia presents with market personality change > memory loss
* Apathy, disinhibition, loss of insight and emotional control
* Global cognitive decline

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44
Q

Frontotemporal dementia involves tau-positive spherical _ cytoplasmic neuronal inclusions called _

A

Frontotemporal dementia involves tau-positive spherical intracytoplasmic neuronal inclusions called pick bodies

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45
Q

Hereditary frontotemporal dementia (common) tends to be [inheritance]

A

Hereditary frontotemporal dementia (common) tends to be autosomal dominant

46
Q

Three types of primary progressive aphasias include:

A

Three types of primary progressive aphasias include:
* Agrammatic Nonfluent, impaired repetition but preserved naming
* Logopenic Reduced fluency, impaired word retrieval (AD) and impaired repetition
* Semantic fluent speech and repetition but impaired word comprehension (naming)

47
Q

Progressive supranuclear palsy is caused by _

A

Progressive supranuclear palsy is caused by midbrain atrophy, “hummingbird sign”

48
Q

Huntington disease is an autosomal dominant trinucleotide repeat: _

A

Huntington disease is an autosomal dominant trinucleotide repeat: CAG

49
Q

Huntington disease involves a repeat expansion in the [gene] on [chromosome]

A

Huntington disease involves a repeat expansion in the HTT on chromosome 4
* Variable penetrance depending on repeats
* Anticipation results from expansion of CAG

50
Q

Huntington symptoms usually present around age _ and include _

A

Huntington symptoms usually present around age 20-50 and include chorea, athetosis, aggression, depression, dementia

51
Q

In huntington disease we see atrophy of the _ structures with ex vacuo ventriculomegaly

A

In huntington disease we see atrophy of the caudate and putamen with ex vacuo ventriculomegaly

52
Q

Neuronal cell death in huntington occurs via NMDA-R binding and _ excitotoxicity

A

Neuronal cell death in huntington occurs via NMDA-R binding and glutamate excitotoxicity

53
Q

The clinical hallmark of ALS is _ motor neuron findings

A

The clinical hallmark of ALS is mixed upper and lower motor neuron findings
* Muscles will undergo denervation atrophy

54
Q

Degeneration of lower motor neurons in ALS occurs in the _

A

Degeneration of lower motor neurons in ALS occurs in the anterior horn cells

55
Q

Degeneration of upper motor neurons in ALS occurs in the _

A

Degeneration of upper motor neurons in ALS occurs in the corticospinal tract

56
Q

_ are small eosinophilic intraneuronal inclusions in the remaining lower motor neurons of ALS patients

A

Bunina bodies are small eosinophilic intraneuronal inclusions in the remaining lower motor neurons of ALS patients

57
Q

ALS can also affect _ , resulting in bulbar muscle dysfunction (dysphagia, dysarthria, difficulty chewing/breathing)

A

ALS can also affect motor nuclei in brainstem , resulting in bulbar muscle dysfunction (dysphagia, dysarthria, difficulty chewing/breathing)

58
Q

ALS will spare the extraocular muscles and _

A

ALS will spare the extraocular muscles and spincters (no incontence)

59
Q

Many sporadic and familial cases of ALS involve a mutation in [gene]

A

Many sporadic and familial cases of ALS involve a mutation in SOD1

60
Q

Mutations in [gene] is linked to both ALS and FTD

A

Mutations in C9ORF72 is linked to both ALS and FTD

61
Q

The pyramidal system involves _ and _ tracts

A

The pyramidal system involves corticospinal and corticobulbar tracts

62
Q

The extrapyramidal system involves (4 tracts):

A

The extrapyramidal system involves (4 tracts):
1. Rubrospinal
2. Tectospinal
3. Reticulospinal
4. Vestibulospinal

63
Q

Name 5 parts of the basal ganglia:

A

Name 5 parts of the basal ganglia:
1. Caudate nucleus
2. Putamen
3. Globus pallidus
4. Substantia nigra
5. Subthalamic nucleus

64
Q

ID the substantia nigra and the subthalamic nucleus

A
65
Q

All input to the basal ganglia comes through the _

A

All input to the basal ganglia comes through the striatum
* Recall that the striatum is caudate + putamen

66
Q

The [globus pallidus i or e] is the major output from the basal ganglia

A

The globus pallidus internus is the major output from the basal ganglia

67
Q

The direct basal ganglia pathway _ movement

A

The direct basal ganglia pathway facilitates/increases movement

68
Q

The indirect basal ganglia pathway _ movement

A

The indirect basal ganglia pathway inhibits movement

69
Q

The GPi is involved in the (direct/ indirect) pathway

A

The GPi is involved in the direct pathway

70
Q

The substantia nigra pars compacta uses _ to project to the striatum

A

The substantia nigra pars compacta uses dopamine to project to the striatum

71
Q

The substantia nigra reticulata uses _ to project to the globus pallidus

A

The substantia nigra reticulata uses GABA to project to the globus pallidus

72
Q

The subthalamic nucleus uses _ to project to the globus pallidus

A

The subthalamic nucleus uses glutamate to project to the globus pallidus

73
Q

The motor symptoms of parkinson disease can be remembered with the mneumonic:

A

The motor symptoms of parkinson disease can be remembered with the mneumonic: TRAP
* Tremor
* Rigidity
* Akinesia
* Postural instability

74
Q

Benign essential tremors are characterized by occuring with (rest/action)

A

Benign essential tremors are characterized by occuring with action
* Includes both postural and intention tremors
* Difficulty with eating, drinking, fine motor tasks
* Worse with emotional distress and relieved by alcohol

75
Q

Treatment for benign essential tremor is _

A

Treatment for benign essential tremor is beta-blockers (propranolol)

76
Q

Cerebellar tremors are coarse and are often worse during _

A

Cerebellar tremors are coarse and are often worse during end of purposeful movement

76
Q

Parkinsonism-associated tremor is activated by (rest/action)

A

Parkinsonism-associated tremor is activated by rest

77
Q

Differential for conditions that cause chorea:

A

Differential for conditions that cause chorea:
* Huntington’s
* Parkinson’s meds
* Antipsychotics
* Post-strep infection (sydenham’s chorea)
* Lupus associated
* Chorea gravidarum

78
Q

Antipsychotic drugs can cause a chorea movement called _

A

Antipsychotic drugs can cause a chorea movement called tardive dyskinesia

79
Q

When extension of the wrists causes “flapping” motion we call it _

A

When extension of the wrists causes “flapping” motion we call it asterixis
* Can be associated with Wilson disease

80
Q

Restlessness or intense urge to move is called _ ; can be seen as a side effect of Parkinson treatment

A

Restlessness or intense urge to move is called akathisia; can be seen as a side effect of Parkinson treatment

81
Q

Slow, snake-like, writhing movements (especially in the fingers) is called _ and is a feature of _ disease

A

Slow, snake-like, writhing movements (especially in the fingers) is called athetosis and is a feature of Huntington disease
* Suggests issue of basal ganglia

82
Q

_ are sudden, jerky, purposeless movements

A

Chorea are sudden, jerky, purposeless movements
* Seen in Huntington

83
Q

Dystonia is _

A

Dystonia is sustained, involuntary muscle contractions
* Ex: writher’s cramp, blepharospasm, torticollis
* Treat with botox

84
Q

Sudden, wild flailing of one side of the body is called _

A

Sudden, wild flailing of one side of the body is called hemiballismus
* Caused by contralateral subthalamic nucleus lesion (lacunar stroke)

85
Q

The goal of most parkinson drugs is to _

A

The goal of most parkinson drugs is to increase or enhance dopamine signaling

86
Q

Levodopa works as [mechanism of action]

A

Levodopa works as a precursor of dopamine synthesis
* It does cross the blood brain barrier, which is good because we need dopamine in CNS

87
Q

The issue with giving levodopa alone is that _ breaks it down in the periphery

A

The issue with giving levodopa alone is that DDC (DOPA decarboxylase) breaks it down in the periphery
* Side effect is dopamine in periphery = vasoconstriction

88
Q

Carbidopa mechanism is _

A

Carbidopa mechanism is blockage of peripheral DDC
* It does not cross the BBB so it cannot block central DDC which is a good thing –> we want conversion to dopamine in CNS

89
Q

_ is another drug which blocks peripheral degradation of levodopa by blocking COMT

A

Entacapone is another drug which blocks peripheral degradation of levodopa by blocking COMT

90
Q

_ and _ are two dopamine agonists sometimes used to treat Parkinson disease

A

Pramipexole and Ropinirole are two dopamine agonists sometimes used to treat Parkinson disease

91
Q

_ and _ are two drugs that can be used for Parkinson disease but also alternate treatments for restless leg syndrome

A

Pramipexole and Ropinirole are two drugs that can be used for Parkinson disease but also alternate treatments for restless leg syndrome

92
Q

Toxicities of dopamine agonists like pramipexole and ropinirole:

A

Toxicities of dopamine agonists like pramipexole and ropinirole:
* Inhibits prolactin secretion
* Sleep attacks
* OCD/ICD

93
Q

_ and _ are selective MAO-B inhibitors that slow the degradation of dopamine (and all other monoamine neurotransmitters)

A

Selegiline and Rasagiline are selective MAO-B inhibitors that slow the degradation of dopamine (and all other monoamine neurotransmitters)
* They also increase the release of dopamine

94
Q

Selegiline and Rasagiline are _ type drugs

A

Selegiline and Rasagiline are MAO-B inhibitors and also increase release

95
Q

An off-target toxicity of selegiline and rasagiline is _

A

An off-target toxicity of selegiline and rasagiline is inhibition of MAO-A in the gut –> hypertensive crisis
* This is rare because the drugs are selective for MAO-B
* However, tyramine containing foods can cause hypertensive crisis

96
Q

Selegiline and rasagiline should not be combined with other [type drugs]

A

Selegiline and rasagiline should not be combined with other serotonergic drugs
* Can cause serotonin syndrome

97
Q

All Parkinson drugs can have _ as a side effect due to enhanced signaling via the mesocortical and mesolimbic dopaminergic pathways

A

All Parkinson drugs can have hallucinations as a side effect due to enhanced signaling via the mesocortical and mesolimbic dopaminergic pathways

98
Q

Amantadine can be used to treat Parkinson disease; it works by _

A

Amantadine can be used to treat Parkinson disease; it works by increasing dopamine release and inhibiting reuptake

99
Q

Benztropine and trihexyphenidyl are two [category of drugs] that help Parkinson’s patients by _

A

Benztropine and trihexyphenidyl are two anti-cholinergics that help Parkinson’s patients by rebalancing Ach & DA in the basal ganglia

100
Q

Benztropine and trihexyphenidyl act in the [location] and they only help to improve _ symptoms

A

Benztropine and trihexyphenidyl act in the striatum and they only help to improve tremor and rigidity
* Does not improve bradykinesia or gait instability

101
Q

Benztropine and trihexyphenidyl have _ toxicities

A

Benztropine and trihexyphenidyl have anti-parasympathetic toxicities

102
Q

“-Benazine” drugs are [drug type] that can be used for Huntington’s

A

“-Benazine” drugs are vMAT inhibitors that can be used for Huntington’s
* They deplete the DA from vesicles
* Ex: deutetrabenazine & tetrabenazine

103
Q

_ , _ , and _ are three anti-psychotic drugs that treat Huntington’s by antagonizing dopamine receptors

A

Aripiprazole , Olanzapine , and Risperidone are three anti-psychotic drugs that treat Huntington’s by antagonizing dopamine receptors

104
Q

_ , _ , and _ are three AchE inhibitors that improve Alzheimer disease by slowing the breakdown of Ach

A

Donepezil , Galantamine , and Rivastigmine are three AchE inhibitors that improve Alzheimer disease by slowing the breakdown of Ach

105
Q

Donepezil , Galantamine , and Rivastigmine are AD drugs that have _ toxicities

A

Donepezil , Galantamine , and Rivastigmine are AD drugs that have parasympathetic toxicities

106
Q

Abeta plaques in Alzheimer disease can affect the reuptake of _ , causing an increase in synaptic levels, spilling over into extrasynaptic receptors and causing excitotoxicity

A

Abeta plaques in Alzheimer disease can affect the reuptake of glutamate , causing an increase in synaptic levels, spilling over into extrasynaptic receptors and causing excitotoxicity

107
Q

_ is an AD drug that inhibits NMDA receptors in the postsynaptic neuron to prevent glutamate excitotoxicity

A

Memantine is an AD drug that inhibits NMDA receptors in the postsynaptic neuron to prevent glutamate excitotoxicity

108
Q

Lecanemab and Aducanumab are two AD drugs that work by _

A

Lecanemab and Aducanumab are two AD drugs that work by clearing amyloid beta plaques
* However, they do not improve neurofibrillary tangles

109
Q

_ is an ALS drug that inhibits glutamate excitotoxicity and cell death

A

Riluzole is an ALS drug that inhibits glutamate excitotoxicity and cell death

110
Q

Edaravone is an ALS drug with [MoA]

A

Edaravone is an ALS drug that scavenges O2-radicals

Neuro (18 decks)

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