ABCN Deck 3

Question 1

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

Answer 1

20-30%, LBD

Question 2

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.

Answer 2

MSA

Question 3

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

Answer 3

nonfluent/agrammatic

Question 4

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

Answer 4

ALS (amyotrophic lateral sclerosis)

Question 5

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

Answer 5

HD

Question 6

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.

Answer 6

CBS

Question 7

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

Answer 7

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

Question 8

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.

Answer 8

50, 50s to 80s

Question 9

In PD, Lewy bodies are found in the ____.

Answer 9

brainstem

Question 10

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

Answer 10

30, 50, 2, 85

Question 11

heart failure occurs in __% of patients with HD

Answer 11

30

Question 12

___ can treat dyskinesia in younger PD patients.

Answer 12

amantadine

Question 13

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

Answer 13

AD, LBD

Question 14

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

Answer 14

asymmetric, ipsilateral, contralateral

Question 15

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

Answer 15

FTD-MND

Question 16

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

Answer 16

PD

Question 17

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

Answer 17

Huntington’s Disease

Question 18

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.

Answer 18

Parkinson’s Disease

Question 19

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

Answer 19

anticholinergic

Question 20

disease duration for HD is __ to __ years

Answer 20

15 to 20

Question 21

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

Answer 21

rigidity, spasticity

Question 22

Risk factors for PD dementia (10)

Answer 22

> 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

Question 23

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

Answer 23

40%

Question 24

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

Answer 24

PSP

Question 25

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

Answer 25

extrapyramidal motor

Question 26

The average survival time for LBD is ___ years.

Answer 26

8

Question 27

botox injections can treat _____ in HD.

Answer 27

muscle spasms

Question 28

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

Answer 28

dopamine agonists

Question 29

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

Answer 29

dopamine

Question 30

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__.

Answer 30

tau, TDP-43

Question 31

9 absolute exclusion criteria for PD

Answer 31

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

Question 32

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

Answer 32

mid-40s to mid 50s.

Question 33

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

Answer 33

bvFTD

Question 34

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

Answer 34

PSP

Question 35

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.

Answer 35

CBS

Question 36

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

Answer 36

logopenic

Question 37

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

Answer 37

CBD

Question 38

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.

Answer 38

HD, 75

Question 39

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

Answer 39

semantic

Question 40

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

Answer 40

LBD

Question 41

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

Answer 41

orbitofrontal, mesial frontal, anterior insular; frontal

Question 42

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

Answer 42

nonfluent/agrammatic

Question 43

9 environmental risk factors for PD or parkinsonism

Answer 43

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

Question 44

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

Answer 44

40

Question 45

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

Answer 45

extrapyramidal

Question 46

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

Answer 46

PSP

Question 47

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

Answer 47

logopenic

Question 48

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

Answer 48

FTD-MND

Question 49

Core clinical features of LBD include (4 criteria):

Answer 49

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

Question 50

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

Answer 50

Lewy Body Dementia

Question 51

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

Answer 51

parkinsonian

Question 52

Causes of death in HD include (3 things)

Answer 52

pneumonia, heart disease, suicide

Question 53

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.

Answer 53

15, psychomotor, attention/working memory

Question 54

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.

Answer 54

MSA

Question 55

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

Answer 55

LBD

Question 56

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

Answer 56

apathy and visual hallucinations

Question 57

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

Answer 57

3, 8.

Question 58

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

Answer 58

LBD

Question 59

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

Answer 59

semantic

Question 60

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

Answer 60

10

Question 61

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.

Answer 61

behavioral/frontal/Pick's disease

Question 62

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

Answer 62

Multisystem atrophy