Study your flashcards anywhere!

Download the official Cram app for free >

  • Shuffle
    Toggle On
    Toggle Off
  • Alphabetize
    Toggle On
    Toggle Off
  • Front First
    Toggle On
    Toggle Off
  • Both Sides
    Toggle On
    Toggle Off
  • Read
    Toggle On
    Toggle Off
Reading...
Front

How to study your flashcards.

Right/Left arrow keys: Navigate between flashcards.right arrow keyleft arrow key

Up/Down arrow keys: Flip the card between the front and back.down keyup key

H key: Show hint (3rd side).h key

A key: Read text to speech.a key

image

Play button

image

Play button

image

Progress

1/131

Click to flip

131 Cards in this Set

  • Front
  • Back
  • 3rd side (hint)
In patients over age 75, what are the most common neurodegenerative dementias?
1. AD
2. DLB
True or False

Life threatening reactions to neuroleptic (antipsychotic) medications can occur in patients with DLB.
True
Core features of DLB include:
- Fluctuations in attention
- Visual hallucinations
- Parkinsonism

-Also, autonomic symptoms
Behavioral Neurology of Dementia-2

p.10
Supportive features of DLB include:
- Falls/Syncope
-Transient unexplained LOC
- Severe autonomic dysfx
- Neuroleptic sensitivity
- RBD (REM sleep beh disorder)
- Depression
- Delusions/Hallucinations in other modalities
- Relative preservation of MTL structure on CT/MRI
Behavioral Neurology of Dementia-2
When demetia occurs in the context of well-established parkinsonism (>1 yr), what dx should be made?
PDD
Behavioral Neurology of Dementia-2
Accurate dx of DLB can be difficulty due to:
Concomitant AD pathology;
Perceived similarity to other motor disorders such as PD
MRI findings for AD
Hippocampal & Posterior Temporal/Parietal Atrophy
Behavioral Neurology of Dementia
Ch.1
Pathologically, DLB is associated with:
Alterations in a-synuclein, with Lewy body pathology that begins in the brainstem, progresses to the amygdala & limbic cortex, and finally extends into the neocortex.
In patients with DLB, what likely contributes to the development of visual hallucinations and fluctuations in attention.
Cholinergic deficits
Frequent falls are characteristic of:
PSP
Vascular Dementia
If first symptoms are progressive problems with speech or language, think:
FTD-related conditions (NFAV or svPPA)
or
LPA
If first symptoms are prominent behavioral or personality changes, think:
bvFTD
If early features are visual deficits, think:
PCA
If early features are visual hallucinations or delusional misidentification syndromes, think:
DLB
Early movement abnormalities suggest the possibility of:
PD syndromes such as DLB, PSP, or CBD.
Vascular or CBD can begin with:
Early asymmetric motor findings.
What disorder(s) are consistent with symptoms that vary from moment to moment or day to day?
DLB and Vascular
Behavioral Neurology of Dementia
Ch.1
What disorder(s) are consistent with symptoms that progress rapidly?
CJD
Neoplastic
Encephalitis
Vasculitis
Metabolic Disorders
Behavioral Neurology of Dementia
Ch.1
Patient's with this disorder are delirium prone:
DLB
Consequently, the disorder can begin in either an acute or subacute fashion.
Behavioral Neurology of Dementia
Ch.1
Symptoms of depression, psychosis, and anxiety are typical of:
Subcortical disorders, though they do occur to a limited degree in all ND disorders
Behavioral Neurology of Dementia
Ch.1
REM-behavior syndrome is a characteristic of which disorder(s)
Synuclein-related disorders:

DLB
PD
MSA
Behavioral Neurology of Dementia
Ch.1
What types of disorder(s) should be considered when there is an ACUTE change in cognitive status?
Toxic
Metabolic
Infectious
Behavioral Neurology of Dementia
Ch.1
Difficulty navigating NEW places could be a result of deficits in what domain(s)?
Visuospatial or Frontal (attn, WM)
my notes
Difficulty navigating FAMILIAR places is due to deficits in what domain(s)?
Visuospatial (Benson copy will probably be bad)
my notes
What neurological disorder(s) begin with autonomic dysfx?
Parkinson related disorders, in particular, MSA, but also PSP
Miller Case conference
Neglect (at least on drawings) is a sign of which disorder(s)
Vascular Dementia or
CBD
Behavioral Neurology of Dementia
Ch.1
MMSE & Bedside deficits for FTD
"world" backwards

WM
"d" Generation
Alternating Sequences
Emotion Recognition
Behavioral Neurology of Dementia
Ch.1
MMSE & Bedside deficits for DLB
Pentagons (may be particularly predictive of underlying Lewy Body pathology; PDDs also do poorly on this)
"world" backward

Drawing and, sometimes, EF (Howie says D words?)

Chart says Visuospatial, attentional
Behavioral Neurology of Dementia
Ch.1
Bedside deficits for svPPA
Naming (no benefit from cues)
Episodic verbal memory
Animal generation
Behavioral Neurology of Dementia
Ch.1
Neuro exam findings for AD
Normal till late
Behavioral Neurology of Dementia
Ch.1
Neuro exam findings for DLB can include:
Facial bradykinesia
Soft festinating voice
Cogwheel rigidity
Tremor
Slowing of movement
Micrographia
Stooped posture with festinating gait
Behavioral Neurology of Dementia
Ch.1
Neuro exam findings for PSP:
Wide-eyed stare
Vertical gaze disturbance
Square wave jerks
Parkinsonism (Axial rigidity, bradykinesia, masked facies)
Loss of balance and gait instability resulting in frequent falls
Dysarthria
Dysphagia
Pseudobulbar affect
Behavioral Neurology and Blumenfeld (p715)
http://www.theaftd.org/frontotemporal-degeneration/disorders/psp
Neuro exam findings for CBD:
Asymmetric parkinsonism (like PD)
Ocular apraxia
Dystonia
Alien Limb
Ideomotor apraxia


Ideomotor apraxia: ncbi.nlm.nih.gov/pmc/articles/PMC3340570/
Prominent autonomic symptoms can occur with:
DLB (Moderate)
MSA (Severe)

PD (Mild)
Behavioral Neurology of Dementia
Ch.1

p. 10
Pseudobulbar affect, uncontrollable laughter and crying associated with a brisk jaw jerk is seen in:
ALS
PSP
Vascular dementia
Behavioral Neurology of Dementia
Ch.1
Rapidly progressive dementia with Parkinsonism brings up the possibility of:
CJD
Behavioral Neurology of Dementia
Ch.1
What percentage of PD patients progress to PDD?
80%
MRI of MSA is notable for
Hot Cross Bun sign on pons
What symptoms do you expect with Vascular Dementia?
Motor Impairment
Executive Symptoms
Mental Slowing

cause vasc usually hits subfrontal WM
PSP Molecule
Tau
Behavioral Neurology of Dementia
Ch.1
Bedside pattern for NFAV
Remarkably normal except for:

Word fluency
"d" generation, & sometimes Alternating sequence speed.
Nonfluent, dysarthic, apractic speech
Behavioral Neurology of Dementia
Ch.1
MMSE & Bedside deficits for AD
Orientation, Item recall, sometimes pentagons

Episodic memory
Semantic fluency & sometimes:
Drawing
Behavioral Neurology of Dementia
Ch.1
MRI findings for bvFTD
Atrophy to:
Anterior cingulate,
Orbitofrontal,
Insular,
& usually DLPF (later in course)
Basal ganglia
Behavioral Neurology of Dementia
Ch.1
Pseudobulbar affect, uncontrollable laughter and crying associated with a brisk jaw jerk is seen in what disorder(s)?
ALS
PSP
Vascular dementia
Behavioral Neurology of Dementia
Ch.1
Asymmetric pyramidal deficits are seen with what disorder(s)?
CVD
Behavioral Neurology of Dementia
Ch.1
EPS occur as a result of:
Pathology in the basal ganglia
Pyramidal signs occur as a result of:
Upper motor neuron lesions
Features of Parkinsonism in patients with mild dementia should suggest other disorders like:
DLB
PSP
CBD

MSA-P has early Parkinsonism, but not much cognitive.
Behavioral Neurology of Dementia
Ch.1

p.143
Motor features of
Parkinson's disease include:
TRAP
Tremor
Rigidity
Akinesia/hypo/bradykinesia
Postural Deficits
Molecule(s)

bvFTD
50% Tau

50% TDP-43
Molecule(s)

AD
ABeta-42

Tau
Molecule(s)

MSA
a-synuclein
Molecule(s)

DLB
a-synuclein

Often comorbid ABeta-42
Ideational apraxia
Loss of ability to carry out learned tasks in the proper order.

ie: socks on before shoes
MRI findings of CBD
Frontally predominant, basal ganglia and sometimes parietal
Behavioral Neurology of Dementia
Ch.1
MRI findings of PSP
Midbrain atrophy (variable)
Behavioral Neurology of Dementia
Ch.1
MRI findings for DLB
Variable, but atrophy tends to be more posterior than in AD.
Behavioral Neurology of Dementia
Ch.1
MRI findings for CJD
Cortical ribboning and basal ganglia hyperintensities on FLAIR that demonstrate decrease diffusion of water molecules on diffusion-weighted imaging.
Behavioral Neurology of Dementia
Ch.1
Imaging svPPA
Anterior temporal (some start in L temp, others in R, but eventually both)

They can have beh problems too, so insula, amygdala, ant cingulate
Behavioral Neurology of Dementia
Ch.1

& Rankin talk
Imaging NFAV
Left frontoinsular, Basal ganglia
Behavioral Neurology of Dementia
Ch.1
Molecule(s)

NFAV
Tau
Behavioral Neurology of Dementia
Ch.1
Molecule(s)

svPPA
TDP-43
Behavioral Neurology of Dementia
Ch.1
Molecule(s)

CBD
Tau
Behavioral Neurology of Dementia
Ch.1
Molecule(s)

PSP
Tau
Behavioral Neurology of Dementia
Ch.1
Molecule(s)

CJD
Prion
Behavioral Neurology of Dementia
Ch.1
Bedside pattern for CBD
Like FTD (WM,"d" Generation, Alternating Sequences,Emotion Recognition
or NFAV (Semantic fluency, "d" generation, motor speech impairment, & sometimes; Alternating sequence speed.)
Also, Sometimes parietal.
Behavioral Neurology of Dementia
Ch.1
MMSE & Bedside pattern for PSP
"world" backward

Digits Backward,
"d" generation
Drawings may be flat (recall may be better than copy)
Slowed information processing speed.
Slowed alternating sequences

Imitation beh
Apathy is typical
Disinhibition is sometimes reported.
Behavioral Neurology of Dementia
Ch.1& pa 80

Bruce case conference
How do parkinsonian symptoms of DLB differ from those of PD?
DLB has:
Increased gait difficulty & rigidity
More symmetric
Less-frequent resting tremor.

These symptoms are less responsive to dopaminergic agonists & may reflect cholinergic deficits in addition to alterations in dopamine.
Behavioral Neurology of Dementia-2
3 anatomies of word finding problems
L Anterior temporal
L Angular gyrus
L Frontoinsular
Bruce
Which affects reading the most?
svPPA
NFAV
LPA
LPA
Bruce
Which should be excluded if there are phonemic paraphasias?
LPA
NFAV
svPPA
svPPA
Bruce
"I can find my word but I get it out and it's wrong" is consistent with what PPA?
LPA
Bruce
Acalculia could be do to dysfx where?
Frontal (WM)
Parietal
MSA subtypes

Early symptoms of each:
1. Cerebellar:
-Dysautonomis
-Early Ataxia
-Dysarthria
-Oculomotor dysfx

2. Parkinson:
-Dysautonomia
-Early Parkinsonism (w/ absence of resting tremor)
-Evidence of pyramidal tract involvement (+ Babinski, spasticity)
-Beh Neuro of Dementia p.143
-Continuum Dementia p. 69
Dysautonomic symptoms include:
BP (orthostatic hypotension, syncope; lightheadedness, dizziness, vertigo)
Urinary (frequent, finishing, urge)
Impotence, HR, GI, Sweating, fatigue, thirst, anxiety, panic
Sudden drop in libido, think autonomic.
Bedside pattern for MSA
Usually pretty good.
Maybe a hint of EF, but better than DLB and PSP.

So if Digits Reverse is 6, it's more likely to be MSA than PSP.
Bruce case conference
bvFTD criteria
Possible: EARLY
- beh disinhibition
- apathy or inertia
- loss of sympathy or empathy
- perseverative/stereotyped beh
Hyperorality/Dietary changes
NP: Exec/generation deficits w/ relative VS and Mem sparing
NFAV-PPA criteria
1. Agrammatism in L production
2. Effortful, halting speech
3. Impaired syntax comp
4. Spared single word comp
5. Spared object knowledge
svPPA criteria
1. Poor confrontation naming
2. Impaired single word comp
3. Poor object knowledge
4. Surface dyslexia &/or dysgraphia
5. Spared repetition
6. Spared motor speech & grammar
LPA criteria
1. Impaired single-word retrieval in spontaneous speech & confrontation naming
2. Impaired repetition
3. Phonological errors
4. Spared single word comprehension and object knowledge
5. Spared motor speech
6. Absence of frank agrammatism
Of the genetic forms of FTD, which is symmetrical and which is asymetrical?
Tau is incredibly symmetrical.

Progranulin is very asymmetrical (probably because there's and inflammatory component where inflammation spreads along the hemisphere)
Bruce case conference
Asymmetric extra-pyramidal signs occur in which disorder(s)?
CBD
PD (ideopathic)
NFAV (sometimes)
also FTD, tho not sure if it's asymmetric
Symmetric extra-pyramidal signs occur in which disorder(s)
MSA
PSP
also FTD, tho not sure if it's symmetric
CBD begins with:
Asymmetric akinesia-rigidity
Dystonia
Apraxia
Exec, beh, or motor speech imp
Beh Neuro of Dementia p. 143
Alien limb most commonly involves which hand:

Occurs in which disorders?
Usually Left hand, but contralateral to lesion.

TBI (most common cause)
CBD
CJD
dwz.psych.ucla.edu/AlienHandReprint.pdf
Neurol Sci (2003) 24:252–257
Alien Hand

Common Classifications
Posterior or sensory form

Anterior or motor form
dwz.psych.ucla.edu/AlienHandReprint.pdf
Neurol Sci (2003) 24:252–257
The presence of complex, well formed hallucinations (often people & animals) in the Central Field of vision in DLBs suggests:
--Profound brainstem pathology
--Cholinergic deficits
--Altered visual processing (underlying dysfunction in the ventral visual stream; dorsal stream dysfx is also seen, but less frequently (palinopsia)
Beh Neur of Dementia, p.11
Pathologically, RBD is associated with:
A complex interaction of Lewy Bodies in the brainstem & Neurotransmitter deficits, esp ACh.
Beh Neuro of Dementia p.10
Autonomic dysfx in PD is:
Mild
Moderate
Severe
Mild
Beh Neuro of Dementia p. 10
In addition to complex visual hallucinations, DLBs may also experience:
--Visual illusions (tree stump looks like an animal; faces morphing out of wallpaper)
--Extracampion hallucinations (dark shadows in periphery that disappear when looked at; feeling of someone looking over shoulder)
--Delusions (Capgras; Reduplicative paramnesia: "this house is a duplicate of my real house")
BNofD p 12
Capgras is hypothesized to reflect:
A disconnection of visual information (occipital lobe) from emotional processing (amygdala)
BNofD p 13
Neuropsychiatric symptoms of DLB include:
Visual Hallucination
Delusions
Anxiety (65%)
Depression (62%)
Apathy (58%)
Agitation (55%)
Sleep disorders (55%)
Psychosis (50%)
BNofD p 13
True or False

Hallucinations and delusions in DLB respond well to AChEI
True.
So the absence of these symptoms in the context of tx with AChEIs (possibly prescribed after a dx of AD) should not necessarily dissuade one from a potential dx of DLB
BN of D p.13
Compare memory performance between AD and DLB
Both show impaired Learning and DR.
But, DLB has intact recognition, suggesting less-severe consolidation deficit.
BN of N p 13
Compared to ADs, DLBs do worse on tasks of:
Attention
EF
VS
BN of N p 13
What test(s) on the MMSE or Bedside is particularly suggestive of DLB in the context of otherwise preserved cognitive abilities.
Pentagons
BN of D p.14
Synucleinopathies include:
DLB
PD
MSA
BN of D p.14
a-synuclein is:
An intracellular protein involved in axonal transport
BN of D p.14
How does Lewy Body pathology differ between PD and DLB?
PD: Lewy Bodies are typically restricted to the brainstem

DLB: profound LB pathology starts in brainstem but then progresses to amygdala, limbic cortex and, finally, neocortex.
BN of D p.14
The most common pathology associated with altered a-synuclein processing is:
the Lewy Body
BN of D p.14
Neuropsychiatric features of CBD:
Depression (50%)
Sleep disturbance, including RBD & OSA
Continuum Dementia p 71
True or False

There is significant and simultaneous degeneration of basal ganglia structures in FTLD
True.
This often leads to the co-expression of parkinsonian features within all FTLD subtypes. eg: Most PNFA patients demonstrate CBD or PSP at autopsy
BNof D p 47
What are the expected pathological outcomes for PNFA?
CBD or PSP
BN of D p 47
True or false

SD begins in the LT OR RT degeneration
True.
LTs show profound anomia associated with progressive loss of conceptual knowledge of words

RTs show deficits in empathy and knowledge about the emotions of others.
BN of D p 47
True or false

bvFTDs present with asymmetric right but bilateral frontal involvement.
Treu
BN of D p 47
FTLD subtypes overlap with what other disorders
CBD
PSP
FTD-MND
BN of D p 47
FTD-MNDs typically live ____ after diagnosis and die from ________.
1.4 years

Respiratory complications of bulbar palsy
BN of D p 47
What is Binswanger's Disease?
AKA subcortical VascD.
Caused by widespread, microscopic areas of damage to the deep layers of WM resulting from atherosclerosis.
http://www.ninds.nih.gov/disorders/binswangers/binswangers.htm
Paroxysms are:
Sudden recurrence or intensification of symptoms.
In MS, Temporary neurological disturbances, such as muscle spasms (called tonic spasms), paresthesias, slurred speech, imbalance (called ataxia), and so forth. They may mimic symptoms of MS attacks, but the difference is that they are fleeting, from several seconds up to 2 minutes, rather than persistent over several days to weeks as described in MS attacks.
http://www.unitedspinal.org/msscene/2008/11/18/paroxysmal-symptoms-of-multiple-sclerosis-they-come-and-they-go/
True or False

SD can begin either in the L OR R temporal lobe.
True

but 2:1 begin in Left
Beh Neur of Dem p 48
What percentage of bvFTDs develop ALS?
15%
Beh Neur of Dem p 48
Rates of progression & Age of onset for the different FTLDs.
bvFTD (3.4 yrs: ~58)

PNFA (4.3 yrs)

svFTD (5.2 yrs: ~59)
Beh Neur of Dem p 48
Do left-sided SD patients have more trouble with nouns or verbs?
Nouns
Beh Neur of Dem p 48
In left-sided SD, does knowledge about tools or animals go first?
Animals

They'll substitute specific words for superodinate categories. eg: an osprey may become "eagle", then "bird", then "animal", then "thing"
Beh Neur of Dem p 48
True or False

Extrapyramidal symptoms are common in bvFTD.
True
Beh Neur of Dem p 48
What is lost first and what other features follow when SD begins on the Right?
Familiar Face Recognition goes first.
Also, Psychiatric:
Loss of empathy, Atypical depression, Affect Recognition in faces
Beh Neur of Dem p 48
Speech Apraxia is:
A deficit in articulatory planning in which the patient is unable to direct speech musculature to produce sounds in a proper sequence.
Beh Neur of Dem p 49
Compulsive interests in visually appealing objects emerge in which disorder?
SD.
May lead to compulsive card-playing, coin collecting, or stealing.
Beh Neur of Dem p 48
Memory

Patients with fronto-subcortical atrophy have problems with________, but relatively intact ___________.
Encoding and initial learning.

Retention
Beh Neu of Dem p75
Patients who have difficulty multitasking likely have _________ deficits.
Working memory
Beh Neu of Dem p76
WM tasks include:
Serial 7s
Recite months of year backward
Beh Neu of Dem p76
Hallucinations and delusions EACH occur in over ___% of DLBs.
>50%
Beh Neu of Dem p 88
RBD is reported in over _____ of DLB and PD patients.
> 50%

It is rarely seen in other dementias
Beh Neu Dem p. 89
True or false

SDs eventually develop associative visual agnosia.
True
Beh Neu Dem p. 91
Neuro exam for MSA-C
Distinguised primarily by:
-dysarthria
-ataxia
-oculomotor dysfx

Parkinsonian motor signs and autonomic dysfx are present to a variable degree.

Impaired saccadic eye movements & vertical gaze disturbance may also occur.

Continuum Dementia p 69
True or False

Most MSA patients do not meet the criteria for dementia.
True

In face, clinically significant cog imp is viewed as a nonsupportive feature, tho deficits in EF & processing speed are common.

Continuum Dementia p 69
PPA subtypes
PNFA
svFTD
LPA
True or False

The anterior temporal lobes are "transmodal areas"
True

They play an integrative role in bringing together visual, verbal, auditory, and somatosensory information about objects.

Continuum Dementia p200
True or False

Patients with svFTD that starts on the right side may present like bvFTDs.
True

Loss of empathy, lack of warmth, disinhibition, other beh features

Continuum p 201
Patients with surface dyslexia have difficulty reading irregular words because they attempt to read the words by converting _________ into _____________.
Graphemes into Phonemes
True or False

ALS, CBD, or PSP may accompany all FTD subtypes
True

Continuum p 203