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ABCN Exam August 2021 > ABCN Deck 3 > Flashcards

ABCN Deck 3 Flashcards

1
Q

__ to __% of patients with neurodegenerative dementia have comorbid AD and ___ pathology.

A

20-30%, LBD

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2
Q

The majority of patients with ? display a mild form of cognitive impairment reflecting frontal-subcortical dys- function. Findings include disturbances in attention, mild problems with memory, visuospatial dysfunction, and execu- tive dysfunction. Severe dementia, as often seen in the tauopathies, is uncommon [46] and some consider it an exclusion criterion.

A

MSA

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3
Q

which FTD language variant? agrammatism, effortful, halting speech, speech-sound errors, impaired comprehension of complex syntax

A

nonfluent/agrammatic

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4
Q

FTD is known to occur in 50 to 60% of patients with ___.

A

ALS (amyotrophic lateral sclerosis)

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5
Q

Behavior changes associated with __ include irritability, depression, anxiety, OCD features, psychosis, apathy

A

HD

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6
Q

mvFTD type initial presentation: clumsy, rigid, slow movements of L arm and leg, followed by impairment of R limbs, extreme impairment of voluntary movements, resting tremor. impaired visuospatial skills, calculations, apraxia, alien hand syndrome.

A

CBS

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7
Q

The 3 types of FTD motor variant are___. ___ is the most common motor variant.

A

progressive supranuclear palsy (PSP), corticobasal syndrome (CBS), and FTD with motor neuron disease (FTD_MND). PSP.

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8
Q

Mean onset for diffuse lewy body disease (DLBD) is late ___s whereas onset of Lewy body variant of AD (LBV) varies from __s to __s.

A

50, 50s to 80s

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9
Q

In PD, Lewy bodies are found in the ____.

A

brainstem

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10
Q

Mean age of onset for HD is between __ to __ years, with a range of __ to ___ years.

A

30, 50, 2, 85

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11
Q

heart failure occurs in __% of patients with HD

A

30

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12
Q

___ can treat dyskinesia in younger PD patients.

A

amantadine

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13
Q

___ is the 1st most common form of dementia. ____ is the 2nd most common.

A

AD, LBD

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14
Q

in PD, dopaminergic degeneration of the brain is ____. Typical tremor progression is from a single hand to the _____ leg, then the ____ limbs.

A

asymmetric, ipsilateral, contralateral

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15
Q

mvFTD characterized by ubiquitin-based pathology involving frontal and temporal lobes

A

FTD-MND

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16
Q

Which movement disorder is associated with slow processing speed, impaired complex attention and WM, reduced speech output, micrographia, impaired learning, reduced mental flexibility

A

PD

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17
Q

Which movement disorder is associated with impaired visual attention, impaired processing speed, slowed speech that can progress to mutism, impaired visuospatial skills, impaired learning with intact recognition, impaired WM, impaired motor programming

A

Huntington’s Disease

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18
Q

Alpha-synucleinopathy characterized by parkinsonism, neuronal loss in the substantia nigra, dopamine depletion in the striatum, response to dopaminergic therapy, presence of Lewy bodies, and frontal-subcortical circuitry dysfunction.

A

Parkinson’s Disease

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19
Q

____ drugs are useful in PD patients under 70 with tremor and no significant bradykinesia or gait disturbance.

A

anticholinergic

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20
Q

disease duration for HD is __ to __ years

A

15 to 20

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21
Q

tizanidine and baclofen are medications that reduce ___ and ___ in HD.

A

rigidity, spasticity

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22
Q

Risk factors for PD dementia (10)

A

> 70 years, severe motor symptoms, dx of PD MCI, h/o depression, levodopa side effects (mania, agitation, disorientation, psychosis), sensitivity to meds (hallucinations), daytime sleepiness or REM disorder, posture/gait px, cerebrovascular disease, low SES

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23
Q

About ___ of patients with FTD dementia have a family history of dementia, indicating a genetic component.

A

40%

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24
Q

Which mvFTD is characterized by astrocytic lesions, tau positive neurofibrillary tangles, and neuropil threads in the brainstem and basal ganglia?

A

PSP

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25
Q

Neuroleptic sensitivity can result in development or worsening of _____ symptoms.

A

extrapyramidal motor

26
Q

The average survival time for LBD is ___ years.

A

8

27
Q

botox injections can treat _____ in HD.

A

muscle spasms

28
Q

Side effects of ______ include nausea, vomiting, low BP, dyskinesias, vivid dreaming, visual hallucinations, impulse control problems.

A

dopamine agonists

29
Q

Tetrabenazine (TBZ) is a _____-depleting agent used to treat HD.

A

dopamine

30
Q

Although 40% of all FTD’s are caused by an abnormal accumulation of ___, more than half of FTD’s are tau negative and are caused by an abnormal accumulation of__.

A

tau, TDP-43

31
Q

9 absolute exclusion criteria for PD

A

cerebellar abnormalities, downward vertical supranuclear gaze palsy, bvFTD or primary progressive aphasia within 1st 5 years, Parkinsonism restricted to lower limbs for more than 3 years, drug induced parkinsonism, no response to levodopa, cortical sensory loss, normal imaging of presynaptic dopaminergic system, alternative condition that produces parkinsonism

32
Q

In FTD, cognitive and behavioral changes can begin between the ages of

A

mid-40s to mid 50s.

33
Q

Criteria for possible _____ dementia include: early behavioral disinhibition, apathy, loss of sympathy/empathy, perseverative behavior, hyperorality, EF deficits with sparing of memory and visual-spatial function

A

bvFTD

34
Q

Which mvFTD? vertical gaze palsy, bradykinesia, rigidity, swallowing px, frequent falls, behavioral/cognitive changes (EF), onset in 60s

A

PSP

35
Q

which mvFTD? limb apraxia, alien limb syndrome, cortical reflex myoclonus, cortical sensory impairment, asymmetric ideomotor & lim kinetic apraxia, executive dysfunction, onset from 50s to 70s.

A

CBS

36
Q

which FTD language variant? impaired single-word retrieval, repetition, phonologic errors,

A

logopenic

37
Q

mvFTD characterized by asymmetrical atrophy of the bilateral premotor cortex, superior parietal lobes, striatum

A

CBD

38
Q

First signs of juvenile __ are often behavior disturbances and learning difficulties. Frequent epileptic seizures are observed. In __% of cases, the father is the affected parent.

A

HD, 75

39
Q

which FTD language variant? impaired confrontation naming, single word comprehension, object knowledge, surface dyslexia or dysgraphia

A

semantic

40
Q

Which motor disorder is characterized by the following in its early stages: mild parkinsonism, functional with cognitive deficits, mild confusion, largely preserved insight, benign hallucinations

A

LBD

41
Q

Imaging of patients with behavioral variant FTD shows atrophy of the __, __, and ___ cortices, and hypoperfusion and hypometabolism in the ___ area.

A

orbitofrontal, mesial frontal, anterior insular; frontal

42
Q

which FTD language variant is associated with deterioration of the L posterior frontal/insular regions?

A

nonfluent/agrammatic

43
Q

9 environmental risk factors for PD or parkinsonism

A

viral encephalitis, dopamine antagonists (neuroleptics, toxic substances, MPTP), herbicides, pesticides, heavy metals, elevated iron levels, contaminated well water.

44
Q

Depression has __% prevalence in HD and high suicide rate.

A

40

45
Q

The _____ system includes the basal ganglia, subthalamic nucleus, substantia nigra, thalamic nuclei, and the cortex.

A

extrapyramidal

46
Q

mvFTD type which initially presents with difficulty coordinating eye movements, blurred vision, slow saccades, bradykinesia, rigidity, falls, motor skills involving speech, forced laughing, prolonged crying; fatality within 5 years

A

PSP

47
Q

FTD language variant associated with deterioration of the left temporal-parietal regions

A

logopenic

48
Q

Which mvFTD? dementia (memory and EF), speech production deficits, significant disinhibition, personality changes, muscle atrophy, weak respiratory muscles, hyperflexia, fasciculations, dysphagia, dysarthria.

A

FTD-MND

49
Q

Core clinical features of LBD include (4 criteria):

A

fluctuating cognition, visual hallucinations that are well formed, REM sleep disorder, 1-2 features of parkinsonism

50
Q

Which movement disorder is associated with mild nonverbal IQ impairment, impaired WM and vigilance, deficits in word list generation and confrontation naming, impaired visuo-spatial/constructional abilities, reduced learning and memory, impaired EF, micrographia, visual hallucinations, cognitive fluctuations

A

Lewy Body Dementia

51
Q

With primary LBD, dementia occurs concurrently or within 1 year of ___ symptoms.

A

parkinsonian

52
Q

Causes of death in HD include (3 things)

A

pneumonia, heart disease, suicide

53
Q

In HD, cognitive changes can be detected up to ___ years prior to clinical onset. Measures that appear most sensitive in the prodromal period are _____ performance and _____ measures.

A

15, psychomotor, attention/working memory

54
Q

Ataxia of the limbs usually occurs first [47]. Other cerebellar signs include dysarthria, myoclonus and nystagmus. Dysphagia is seen later in the illness [37]. Autonomic symptoms usually precede or occur within 2 years of the onset of the motor symptoms and can include urinary incon- tinence, postural hypotension, impotence, recurrent syncope, and fecal incontinence.

A

MSA

55
Q

supportive clinical features of ___ include: neuroleptic sensitivity, postural instability, repeated fall, syncope, severe autonomic dysfunction, hallucinations, delusions, apathy, anxiety, depression

A

LBD

56
Q

In LBD, dementia can be treated with donepezil (Aricept) and rivastigmine (Exelon), which can help with ___ and ____.

A

apathy and visual hallucinations

57
Q

The reported median survival rates from diagnosis to death for bvFTD are from _ to _ years.

A

3, 8.

58
Q

Which movement disorder is associated with diffuse global hypometabolism and low dopamine transporter uptake in the basal ganglia?

A

LBD

59
Q

FTD language variant associated with deterioration in the bilateral anterior temporal region

A

semantic

60
Q

positive family history of PD is found in __% of PD patients.

A

10

61
Q

The ___ variant of FTD is caused by progressive, focal degeneration of the frontal and temporal brain regions and/or the presence of argyrophilic globular inclusions and swollen, achromatic neurons.

A

behavioral/frontal/Pick’s disease

62
Q

Glial Cytoplasmc Inclusions (GCIs) are inclusions found in cells (usually oligodendrocytes) that are characteristic of ?

A

Multisystem atrophy

ABCN Exam August 2021 (19 decks)

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