behavior and memory Flashcards
1
Q
what does injury to the mamillary bodies cause?
A
psychosis, memory deficits and confabulation.
2
Q
what does injury to bilateral amygdala cause?
A
hypersexuality, hyperorality and hyperphagia (kluver-Bucy)
3
Q
injury to what part of the thalamus can cause language deficits?
A
medial thalamus
4
Q
injury to what part of the thalamus can cause problems with sleep-wake cycle?
A
anterior and medial thalamus
5
Q
what are AAN recommendations for initial workup of dementia/
A
B12, CBC, CMP, Thyroid function, depression screening.
6
Q
what makes up the circuit of papez?
A
entorhinal cortex–>hippocampus–>fornix–>mamillary bodies–>anterior nuc of thal–> cingulate gyrus
7
Q
What chromosome is Presenilin-1 on? What percentage of familial AD is associated with this gene?
A
14, 80%
8
Q
what chromosome is Presenillin 2 on? What percentage of familial AD is associated with this gene?
A
1, 5%
9
Q
what chromosome is Apolipoprotein E4 on? What percentage of familial AD is associated with this gene?
A
- None Triples risk of AD as a modifier but does not cause AD per se.
10
Q
what chromosome is Amyloid precursor protein (APP) on?
A
21
11
Q
what are common early features of AD?
A
deficits in recent memory, visuospatial skills, verbal dysfluency, anomia.
12
Q
in what regions is PET hypometabolism in diffuse lewy body disease?
A
occipital regions
13
Q
what are the 3 cardinal aspects of diffuse lewy body disease? What are three additional common features?
A
Parkinsonism, fluctuating cognition, visual hallucinations. Also can have dysautonomia, neuroleptic sensitivity, sleep disorder.
14
Q
which class of medications should be avoided in diffuse lewy body disease?
A
typical neuroleptics.
15
Q
Lesion to what area causes personality change, perseveration, apathy and depression?
A
dorsolateral frontal lobe
16
Q
lesion to what area causes OCD traits, disinhibition, hypersexuality, impulsiveness and anti-social behavior?
A
orbitofrontal lobe
17
Q
lesion to what area causes psychosis and memory disturbance?
A
temporal lobe
18
Q
what chromosomal locus is the most commonly associated with familial FTD? what gene?
A
17q21, progranulin
19
Q
in what age range is the mean onset of FTD?
A
55-60
20
Q
what are the three main variants of FTD?
A
- Behavioral- Primarily right frontal degeneration 2. progressive nonfluent aphasia- anomia, word finding difficulty, object naming deficits, effortful speech with impaired comprehension. primarily left insula degeneration 3. semantic Dementia - normal fluency but with semantic paraphasias, anomia and impaired comprehension. unilateral or bilateral temporal lobe.
21
Q
where is hypometabolism on PET in FTD
A
Frontal and anterior temporal regions.
22
Q
what are typical deficits in lesions of dominant temporal lobe?
A
amnesia for verbal information, wernike’s aphasia
23
Q
what are typical deficits in lesions of non-dominant temporal lobe?
A
amnesia for non-verbal and temporal-spatial info, amusia.
24
Q
what is the role of the circuit of papez?
A
memory formation
25
which thalamic nucleus, if injured, can result in abulia, anterograde amnesia, social disinhibition and loss of motivation?
dorsomedial nucleus
26
which thalamic nucleus is involved in processing visual information and sensory integration?
pulvinar
27
which thalamic nucleus is a sensory relay from the body?
ventral posteriorlateral
28
which thalamic nucleus is a sensory relay from the face?
ventral posteriormedial
29
lesions to what two areas can cause akinetic mutism?
bilateral GPi, bilateral ACA territory
30
what is the trinucleotide repeat in HD? What is the inheritance? what chromosome?
CAG, AD, 4p
31
what are the features of Kluver-bucy syndrome?
hyperorality, hypersexuality, hypermetamorphosis (preocupation with minute environmental stimuli), blunted affect (tameness), visual agnosia.
32
lesion to what brain region causes Kluver bucy syndrome?
bilateral anterior temporal lobe/amygdala
33
which dementia is associated with kluver bucy?
Pick's disease
34
what type of "opathy" is diffuse lewy body disease?
alpha-syneuclopathy. Lewy bodies are made up of intracellular inclusions of ubiquitin and alpha syneuclein.
35
what type of "opathy" is pick's disease?
tau Pick bodies are silver staining spherical aggregations of tau.
36
what are the histophathologic features of PSP?
globose neurofibrillary tangles in neurons in subcortical nuclei, tufted astrocytes.
37
what type of memory is impaired in TGA?
recent
38
what is the triad in Wernike's encephalopathy?
ataxia, confusion and opthalmoplegia
39
what is the mechanism of action of Donepazil?
pure acetylcholinesterase inhibitor
40
what is the mechanism of action of Rivastigmine?
acetylcholinesterase inhibitor and butyrylcholinesterase inhibitor.
41
what is the mechanism of action of galantamine?
acetylcholinesterase inhibitor and alosteric nicotinic modulator.
42
what is the mechanism of action of memantine?
NMDA antagonist
43
what type of memory is primarily impaired in major depression?
immediate recall.
44
What type of "opathy" is MSA?
syneuclopathy
45
What type of "opathy" is Corticobasal ganglionic degeneration?
tau
46
what are the 5 main histopathologic features of alzheimer's disease?
1. amyloid (neuritic) plaques. 2. intraneuronal neurofibrillary tangles (made up of tau) 3. granulovacuolar degeneration 4. Amyloid angiopathy. 5. Hirano bodies (made up of actin)
47
What structural change occurs in the PrP protein to cause CJD?
changes from alpha-helical structure to beta-pleated sheets.
48
What gene is associated with familial CJD and what chromosome is it on?
PRNP, 20p
49
what is prosopagnosia?
inability to recognize faces
50
where does prosopagnosia localize to?
bilateral occipito-temporal regions (fusiform gyri). Can occur with bilateral PCA infarcts and AD.
51
what is topographagnosia? Where does it localize?
spatial orientation deficit. Posterior parahippocampal region, nondominant dorso-lateral parietal-occipital lobe.
52
What is asomatognosia? Where does it localize?
indifferent inability to recognize your own limb. contralateral (non-dominant) superior parietal lobule or supramarginal gyrus.
53
What is somatoparaphrenia?
denies ownership of a limb. Often claims limb has been stolen.
54
what is the triad of symptoms in Balint's syndrome?
1. optic ataxia 2. oculomotor apraxia 3 simultagnosia
55
where does Balint Syndrome localize?
bilateral parieto-occipital region. an occur in bilatera MCA-PCA watershed infarcts.
56
What is Anton's syndrome, where does it localize?
cortical blindness with denial of blindness and confabulation. bilateral lesions to medial occipital lobe.
57
what is Charles Bonnet syndrome?
visual hallucinations due to opthalmic disease. Patient has insight that hallucinations are not real.
58
what is pure word deafness (aka verbal auditory agnosia)? Where does it localize?
Inability to understand spoken language but intact comprehension of written language. Localizes to bilateral superior temporal gyri (disrupts connections between wernike's area and auditory cortex) or Dominant auditory area extending into subcortical white matter.
59
What is aphemia (aka pure word mtutism)? where does it localize?
inability to speak fluently, inability to repeat, intact comprehension, intact writing (aka speech apraxia). small lesion in dominant Posterior-inferior frontal gyrus (broca's area).
60
What is Foix-Chavany Marie syndrome? Where does it localize?
bilateral voluntary lower motor neuron facial palsy with preserved involuntary emotional innervation. bilateral anterior opercular lesions.
61
What is transcortical sensory aphasia? Where does it localize?
Wernike's aphasia (fluent speech, impaired comprehansion) with intact repetition. posterior to wernike's area (shown in purple)
62
What is transcortical motor aphasia? Where does it localize?
Broca's aphasia (non-fluent speech, intact comprehension) with intact repitition. Occurs in Dominant ACA-MCA watershed infarcts.
63
What is conduction aphasia? Where does it localize?
impaired repetition, but other aspects of language are intact. Dominant internal arcuate fassiculus connecting Broca's and Wernike's areas.
64
what is amelodia? Where does it localize?
lack of speech prosody. non-dominant posterioinferior frontal gyrus (non-dominant Broca's area).
65
what is receptive aprosodia? Where does it localize?
inability to understand other's speech emotional content. Non-dominant posteriorsuperior temporal gyrus (non-dominant wernike's area)
66
lesions in what brain areas cause pseudobulbar affect?
lesions that disconnect cortico-bulbar tracts from brain stem cranial nerve nuclei
67
what is the treatment for pseudobulbar affect?
dextropormorphan-quinidine
68
what is mixed transcortical aphasia and in what types of lesions can it occur?
non-fluent speech with impaired comprehension but normal repeating. Dominant MCA-ACA and MCA-PCA watershed infarcts.
69
Where does alexia without agraphia localize? what kind of lesion can it be seen in?
dominant occipital cortex with extension into posterior corpus callosum. Can be seen in PCA infarcts.
70
What is Gertzman's syndrome? Where does it localize?
1. agraphia 2. acalculia 3. left-right disorientation 4. finger agnosia. dominant inferior parietal lobule in the region of the angular gyrus.
71
what is apraxia?
inability to perform motor tasks with verbal instruction.
72
what type of lesions can cause left hand apraxia?
left superior MCA infarct. Disconnects language areas from right motor area.
73
what type of neglect do lesions to the right hemisphere cause?
severe left hemi-neglect
74
what type of neglect do lesions to the left hemisphere cause?
mild or absent right neglect.
75
what type of neglect do biparietal lesions cause?
severe right hemi-neglect.
76
what area is important in immediate and working memory?
dorsolateral prefrontal cortex
77
what is achromatopsia? Where does it localize?
inability to name colors presented visually. Intact ability to name the color of an object presented verbally (ie what color is a lemon?) inferior occipito-temporal cortex (unilateral or bilateral)
78
what is color agnosia? where does it localize?
inability to name or point to colors presented visually, but intact ability to perceive colors demonstrated by ability to match colors presented visually. Dominant primary visual cortex extending into corpus callosum.
79
what brain area is particularly important in learning habits?
Head of the Caudate
80
what is ideational apraxia? Where does it localize?
inability to determine the sequence of motor tasks. due to bi-parietal or bi-frontal dysfunction.
81
lesions to what area cause apathy, abulia, indifference, loss of initiative, reduced movement, and urinary incontinence?
bilateral dorsomedial prefrontal cortex.
82
lesions to what area cause imparied judgment, difficulty planning and multi-tasking, problem solving impariment and anhedonia?
dorsolateral prefrontal cortex.
83
What is Capgar's syndrome?
belief that a familiar person is an identical looking imposter.
84
What is Fregoli's syndrome?
belief that the same person exists in several disguises.
85
what is intermetamorphosis?
belief that two people have swapped identities.
86
What is Cotard's syndrome?
belief that one is dead or dying.
87
what is Dejerine-Roussy syndrome? Where does it localize?
thalamic pain. Contralateral posteriolateral thalamus.
88
in what type of dementia does alien limb syndrome occur?
cortico-basal ganglionic degeneration.
89
what are pick bodies made up of? what regions do they accumulate in in pick's disease?
tau. Limbic system. (note most patient's won't have pick bodies on autopsy).
90
CSF findings in AD
increased total and phosphorylated tau, decrease in A-beta protein 42
91
what chromosome is associated with familial FTD with ALS?
19q21-22
92
what are early deficits in HIV Dementia?
1. verbal and nonverbal memory 2. retrieval and processing speed 3. manipulation of acquired knowledge
93
lesions to what area cause palinopsia (persistentce of an image after it is gone), visual allesthesia (transposition of an object to the other half of the visual field) and impaired ipsillateral scanning?
Lateral occipital lobe
94
what is lack of kinisthetic transfer? What kinds of lesions can cause this?
inability of one hand to mimick the other. Corpus callosum lesions.
95
what kind of lesions cause prolonged latency of visual evoked saccades and paucity of eye movements?
contralateral pulvinar
