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131 Cards in this Set
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- Back
- 3rd side (hint)
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In patients over age 75, what are the most common neurodegenerative dementias?
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1. AD
2. DLB |
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True or False
Life threatening reactions to neuroleptic (antipsychotic) medications can occur in patients with DLB. |
True
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Core features of DLB include:
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- Fluctuations in attention
- Visual hallucinations - Parkinsonism -Also, autonomic symptoms |
Behavioral Neurology of Dementia-2
p.10 |
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Supportive features of DLB include:
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- 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
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When demetia occurs in the context of well-established parkinsonism (>1 yr), what dx should be made?
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PDD
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Behavioral Neurology of Dementia-2
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Accurate dx of DLB can be difficulty due to:
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Concomitant AD pathology;
Perceived similarity to other motor disorders such as PD |
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MRI findings for AD
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Hippocampal & Posterior Temporal/Parietal Atrophy
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Behavioral Neurology of Dementia
Ch.1 |
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Pathologically, DLB is associated with:
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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.
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In patients with DLB, what likely contributes to the development of visual hallucinations and fluctuations in attention.
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Cholinergic deficits
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Frequent falls are characteristic of:
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PSP
Vascular Dementia |
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If first symptoms are progressive problems with speech or language, think:
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FTD-related conditions (NFAV or svPPA)
or LPA |
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If first symptoms are prominent behavioral or personality changes, think:
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bvFTD
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If early features are visual deficits, think:
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PCA
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If early features are visual hallucinations or delusional misidentification syndromes, think:
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DLB
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Early movement abnormalities suggest the possibility of:
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PD syndromes such as DLB, PSP, or CBD.
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Vascular or CBD can begin with:
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Early asymmetric motor findings.
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What disorder(s) are consistent with symptoms that vary from moment to moment or day to day?
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DLB and Vascular
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Behavioral Neurology of Dementia
Ch.1 |
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What disorder(s) are consistent with symptoms that progress rapidly?
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CJD
Neoplastic Encephalitis Vasculitis Metabolic Disorders |
Behavioral Neurology of Dementia
Ch.1 |
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Patient's with this disorder are delirium prone:
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DLB
Consequently, the disorder can begin in either an acute or subacute fashion. |
Behavioral Neurology of Dementia
Ch.1 |
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Symptoms of depression, psychosis, and anxiety are typical of:
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Subcortical disorders, though they do occur to a limited degree in all ND disorders
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Behavioral Neurology of Dementia
Ch.1 |
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REM-behavior syndrome is a characteristic of which disorder(s)
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Synuclein-related disorders:
DLB PD MSA |
Behavioral Neurology of Dementia
Ch.1 |
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What types of disorder(s) should be considered when there is an ACUTE change in cognitive status?
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Toxic
Metabolic Infectious |
Behavioral Neurology of Dementia
Ch.1 |
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Difficulty navigating NEW places could be a result of deficits in what domain(s)?
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Visuospatial or Frontal (attn, WM)
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my notes
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Difficulty navigating FAMILIAR places is due to deficits in what domain(s)?
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Visuospatial (Benson copy will probably be bad)
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my notes
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What neurological disorder(s) begin with autonomic dysfx?
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Parkinson related disorders, in particular, MSA, but also PSP
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Miller Case conference
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Neglect (at least on drawings) is a sign of which disorder(s)
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Vascular Dementia or
CBD |
Behavioral Neurology of Dementia
Ch.1 |
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MMSE & Bedside deficits for FTD
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"world" backwards
WM "d" Generation Alternating Sequences Emotion Recognition |
Behavioral Neurology of Dementia
Ch.1 |
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MMSE & Bedside deficits for DLB
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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 |
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Bedside deficits for svPPA
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Naming (no benefit from cues)
Episodic verbal memory Animal generation |
Behavioral Neurology of Dementia
Ch.1 |
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Neuro exam findings for AD
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Normal till late
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Behavioral Neurology of Dementia
Ch.1 |
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Neuro exam findings for DLB can include:
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Facial bradykinesia
Soft festinating voice Cogwheel rigidity Tremor Slowing of movement Micrographia Stooped posture with festinating gait |
Behavioral Neurology of Dementia
Ch.1 |
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Neuro exam findings for PSP:
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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 |
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Neuro exam findings for CBD:
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Asymmetric parkinsonism (like PD)
Ocular apraxia Dystonia Alien Limb Ideomotor apraxia |
Ideomotor apraxia: ncbi.nlm.nih.gov/pmc/articles/PMC3340570/ |
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Prominent autonomic symptoms can occur with:
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DLB (Moderate)
MSA (Severe) PD (Mild) |
Behavioral Neurology of Dementia
Ch.1 p. 10 |
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Pseudobulbar affect, uncontrollable laughter and crying associated with a brisk jaw jerk is seen in:
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ALS
PSP Vascular dementia |
Behavioral Neurology of Dementia
Ch.1 |
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Rapidly progressive dementia with Parkinsonism brings up the possibility of:
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CJD
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Behavioral Neurology of Dementia
Ch.1 |
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What percentage of PD patients progress to PDD?
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80%
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MRI of MSA is notable for
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Hot Cross Bun sign on pons
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What symptoms do you expect with Vascular Dementia?
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Motor Impairment
Executive Symptoms Mental Slowing cause vasc usually hits subfrontal WM |
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PSP Molecule
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Tau
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Behavioral Neurology of Dementia
Ch.1 |
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Bedside pattern for NFAV
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Remarkably normal except for:
Word fluency "d" generation, & sometimes Alternating sequence speed. Nonfluent, dysarthic, apractic speech |
Behavioral Neurology of Dementia
Ch.1 |
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MMSE & Bedside deficits for AD
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Orientation, Item recall, sometimes pentagons
Episodic memory Semantic fluency & sometimes: Drawing |
Behavioral Neurology of Dementia
Ch.1 |
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MRI findings for bvFTD
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Atrophy to:
Anterior cingulate, Orbitofrontal, Insular, & usually DLPF (later in course) Basal ganglia |
Behavioral Neurology of Dementia
Ch.1 |
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Pseudobulbar affect, uncontrollable laughter and crying associated with a brisk jaw jerk is seen in what disorder(s)?
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ALS
PSP Vascular dementia |
Behavioral Neurology of Dementia
Ch.1 |
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Asymmetric pyramidal deficits are seen with what disorder(s)?
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CVD
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Behavioral Neurology of Dementia
Ch.1 |
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EPS occur as a result of:
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Pathology in the basal ganglia
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Pyramidal signs occur as a result of:
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Upper motor neuron lesions
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Features of Parkinsonism in patients with mild dementia should suggest other disorders like:
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DLB
PSP CBD MSA-P has early Parkinsonism, but not much cognitive. |
Behavioral Neurology of Dementia
Ch.1 p.143 |
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Motor features of
Parkinson's disease include: |
TRAP
Tremor Rigidity Akinesia/hypo/bradykinesia Postural Deficits |
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Molecule(s)
bvFTD |
50% Tau
50% TDP-43 |
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Molecule(s)
AD |
ABeta-42
Tau |
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Molecule(s)
MSA |
a-synuclein
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Molecule(s)
DLB |
a-synuclein
Often comorbid ABeta-42 |
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Ideational apraxia
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Loss of ability to carry out learned tasks in the proper order.
ie: socks on before shoes |
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MRI findings of CBD
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Frontally predominant, basal ganglia and sometimes parietal
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Behavioral Neurology of Dementia
Ch.1 |
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MRI findings of PSP
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Midbrain atrophy (variable)
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Behavioral Neurology of Dementia
Ch.1 |
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MRI findings for DLB
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Variable, but atrophy tends to be more posterior than in AD.
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Behavioral Neurology of Dementia
Ch.1 |
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MRI findings for CJD
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Cortical ribboning and basal ganglia hyperintensities on FLAIR that demonstrate decrease diffusion of water molecules on diffusion-weighted imaging.
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Behavioral Neurology of Dementia
Ch.1 |
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Imaging svPPA
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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 |
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Imaging NFAV
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Left frontoinsular, Basal ganglia
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Behavioral Neurology of Dementia
Ch.1 |
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Molecule(s)
NFAV |
Tau
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Behavioral Neurology of Dementia
Ch.1 |
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Molecule(s)
svPPA |
TDP-43
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Behavioral Neurology of Dementia
Ch.1 |
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Molecule(s)
CBD |
Tau
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Behavioral Neurology of Dementia
Ch.1 |
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Molecule(s)
PSP |
Tau
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Behavioral Neurology of Dementia
Ch.1 |
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Molecule(s)
CJD |
Prion
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Behavioral Neurology of Dementia
Ch.1 |
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Bedside pattern for CBD
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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 |
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MMSE & Bedside pattern for PSP
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"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 |
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How do parkinsonian symptoms of DLB differ from those of PD?
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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
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3 anatomies of word finding problems
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L Anterior temporal
L Angular gyrus L Frontoinsular |
Bruce
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Which affects reading the most?
svPPA NFAV LPA |
LPA
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Bruce
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Which should be excluded if there are phonemic paraphasias?
LPA NFAV svPPA |
svPPA
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Bruce
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"I can find my word but I get it out and it's wrong" is consistent with what PPA?
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LPA
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Bruce
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Acalculia could be do to dysfx where?
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Frontal (WM)
Parietal |
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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 |
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Dysautonomic symptoms include:
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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. |
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Bedside pattern for MSA
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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
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bvFTD criteria
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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 |
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NFAV-PPA criteria
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1. Agrammatism in L production
2. Effortful, halting speech 3. Impaired syntax comp 4. Spared single word comp 5. Spared object knowledge |
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svPPA criteria
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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 |
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LPA criteria
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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 |
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Of the genetic forms of FTD, which is symmetrical and which is asymetrical?
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Tau is incredibly symmetrical.
Progranulin is very asymmetrical (probably because there's and inflammatory component where inflammation spreads along the hemisphere) |
Bruce case conference
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Asymmetric extra-pyramidal signs occur in which disorder(s)?
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CBD
PD (ideopathic) NFAV (sometimes) also FTD, tho not sure if it's asymmetric |
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Symmetric extra-pyramidal signs occur in which disorder(s)
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MSA
PSP |
also FTD, tho not sure if it's symmetric
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CBD begins with:
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Asymmetric akinesia-rigidity
Dystonia Apraxia Exec, beh, or motor speech imp |
Beh Neuro of Dementia p. 143
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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 |
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Alien Hand
Common Classifications |
Posterior or sensory form
Anterior or motor form |
dwz.psych.ucla.edu/AlienHandReprint.pdf
Neurol Sci (2003) 24:252–257 |
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The presence of complex, well formed hallucinations (often people & animals) in the Central Field of vision in DLBs suggests:
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--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
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Pathologically, RBD is associated with:
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A complex interaction of Lewy Bodies in the brainstem & Neurotransmitter deficits, esp ACh.
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Beh Neuro of Dementia p.10
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Autonomic dysfx in PD is:
Mild Moderate Severe |
Mild
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Beh Neuro of Dementia p. 10
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In addition to complex visual hallucinations, DLBs may also experience:
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--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
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Capgras is hypothesized to reflect:
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A disconnection of visual information (occipital lobe) from emotional processing (amygdala)
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BNofD p 13
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Neuropsychiatric symptoms of DLB include:
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Visual Hallucination
Delusions Anxiety (65%) Depression (62%) Apathy (58%) Agitation (55%) Sleep disorders (55%) Psychosis (50%) |
BNofD p 13
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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
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Compare memory performance between AD and DLB
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Both show impaired Learning and DR.
But, DLB has intact recognition, suggesting less-severe consolidation deficit. |
BN of N p 13
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Compared to ADs, DLBs do worse on tasks of:
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Attention
EF VS |
BN of N p 13
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What test(s) on the MMSE or Bedside is particularly suggestive of DLB in the context of otherwise preserved cognitive abilities.
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Pentagons
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BN of D p.14
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Synucleinopathies include:
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DLB
PD MSA |
BN of D p.14
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a-synuclein is:
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An intracellular protein involved in axonal transport
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BN of D p.14
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How does Lewy Body pathology differ between PD and DLB?
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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
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The most common pathology associated with altered a-synuclein processing is:
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the Lewy Body
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BN of D p.14
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Neuropsychiatric features of CBD:
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Depression (50%)
Sleep disturbance, including RBD & OSA |
Continuum Dementia p 71
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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
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What are the expected pathological outcomes for PNFA?
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CBD or PSP
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BN of D p 47
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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
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True or false
bvFTDs present with asymmetric right but bilateral frontal involvement. |
Treu
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BN of D p 47
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FTLD subtypes overlap with what other disorders
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CBD
PSP FTD-MND |
BN of D p 47
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FTD-MNDs typically live ____ after diagnosis and die from ________.
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1.4 years
Respiratory complications of bulbar palsy |
BN of D p 47
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What is Binswanger's Disease?
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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
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Paroxysms are:
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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/
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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
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What percentage of bvFTDs develop ALS?
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15%
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Beh Neur of Dem p 48
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Rates of progression & Age of onset for the different FTLDs.
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bvFTD (3.4 yrs: ~58)
PNFA (4.3 yrs) svFTD (5.2 yrs: ~59) |
Beh Neur of Dem p 48
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Do left-sided SD patients have more trouble with nouns or verbs?
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Nouns
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Beh Neur of Dem p 48
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In left-sided SD, does knowledge about tools or animals go first?
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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
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True or False
Extrapyramidal symptoms are common in bvFTD. |
True
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Beh Neur of Dem p 48
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What is lost first and what other features follow when SD begins on the Right?
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Familiar Face Recognition goes first.
Also, Psychiatric: Loss of empathy, Atypical depression, Affect Recognition in faces |
Beh Neur of Dem p 48
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Speech Apraxia is:
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A deficit in articulatory planning in which the patient is unable to direct speech musculature to produce sounds in a proper sequence.
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Beh Neur of Dem p 49
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Compulsive interests in visually appealing objects emerge in which disorder?
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SD.
May lead to compulsive card-playing, coin collecting, or stealing. |
Beh Neur of Dem p 48
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Memory
Patients with fronto-subcortical atrophy have problems with________, but relatively intact ___________. |
Encoding and initial learning.
Retention |
Beh Neu of Dem p75
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Patients who have difficulty multitasking likely have _________ deficits.
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Working memory
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Beh Neu of Dem p76
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WM tasks include:
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Serial 7s
Recite months of year backward |
Beh Neu of Dem p76
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Hallucinations and delusions EACH occur in over ___% of DLBs.
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>50%
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Beh Neu of Dem p 88
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RBD is reported in over _____ of DLB and PD patients.
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> 50%
It is rarely seen in other dementias |
Beh Neu Dem p. 89
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True or false
SDs eventually develop associative visual agnosia. |
True
|
Beh Neu Dem p. 91
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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 |
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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 |
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PPA subtypes
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PNFA
svFTD LPA |
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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 |
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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 |
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Patients with surface dyslexia have difficulty reading irregular words because they attempt to read the words by converting _________ into _____________.
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Graphemes into Phonemes
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True or False
ALS, CBD, or PSP may accompany all FTD subtypes |
True
Continuum p 203 |
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