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Eugenie Notes > Notes 3b > Flashcards

Notes 3b Flashcards

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1
Q

Progressive muscular atrophy

features

A

spectrum of ALS: only LMN

focal asymmetric weakness hat later involves more proximal regions and the extremities: atrophy, hyporeflexia, and fasciculations

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2
Q

Spinal cord blood supply?

A

one anterior spinal artery and 2 posterior spinal arteries

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3
Q

Anterior spinal artery supplies? what tracts are in the territory?

A

Ant ⅔ of the spinal cord

corticospinal and spinothalamic tracts

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4
Q

Posterior spinal arteries supplies? what tracts are in the territory?

A

posterior ⅓ of the spinal cord

dorsal column

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5
Q

Largest radicular artery?

A

Artery of adamkiewicz

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6
Q

Why do mets from the pelvic region commonly migrate to the CNS?

A

epidural venous plexus is a valveless system

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7
Q

West Nile Virus

features

MRI

A

meningitis, encephalitis

presents with flaccid weakness, areflexia, proximal and asymmetric

MRI can show cauda equina, spinal cord, leptomeningeal enhancement

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8
Q

Adrenoleukodystrophy

inheritance, mutation

caused by

features

MRI

Tx

A

X linked, ABCD1 gene on X chromosome q28 (ATP-binding cassette transporter)

impaired VLCFA causing peroxisome dysfunction

paraparesis, sensory neuropathy, bulbar sx, hypogonadism and cognitive impairment

T2 hyperintensities in WM SPARING U FIBERS affecting predominantly the parieto-occipital regions and posterior corpus callosum (may show a rim of enhancement unlike other leukodystrophy)

steroids, or Lorenzos oil (4:1 glyceryl trioleate-glyceryl trierucate)

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9
Q

Central spinal cord lesions (syringomyelia)

presents with

A

“cape like” sensory loss of pain and temp in the spine

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10
Q

In the spine intramedullary tumors are more likely to be primary/mets?

A

primary if intramedullary

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11
Q

Most common spinal intramedullary tumor in adults? children

A

ependymoma

ependymoma and astrocytoma

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12
Q

Most common nonmetastatic extramedullary tumors

A

neurofibromas, schwannoma, meningiomas

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13
Q

Difference between intra and extramedullary

A
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14
Q

ADEM (acute disseminated encephalomyelitis)

features

caused by

tx

A

inflammation of brain and spine at the same time

monophasic disease caused by immune response to a virus

IV steroids

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15
Q

When ADEM afefcts with spine it presents with

A

transverse myelitis (>3 segments affected)

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16
Q

Copper myelopathy causes a pattern similar to ?

A

B12

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17
Q

Kennedy’s disease (X-linked spinobulbar muscular atrophy)

features

mutation

A

presents in males, 40s: proximal weakness with LMN features, gynecomastia, hypogonadism, DM

expansion of CAG repeat in the androgen receptor on X chromosome

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18
Q

BUZZWORDS: survival motor neuron 1 gene mutation

A

spinal muscular atrophy

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19
Q

BUZZWORDS: motor neuron diseases with only lower motor neuron involvement

A

Progressive muscular atrophy, spinal muscular atrophy, benign focal amyotrophy

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20
Q

BUZZWORDS: post-gastric bypass neurologic syndrome

A

copper def

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21
Q

BUZZWORDS: Devic’s Disease- Aquaporin-4

A

NMO

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22
Q

BUZZWORDS: nitrous oxide toxicity, assoc with B12 depletion

A

Anesthesia paresthetica

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23
Q

BUZZWORDS: ipsilateral loss of motor and sensation to vibration and proprioception below the level of the lesion, with contralateral loss of sensation to pain and temp

A

Brown-Sequard Syndrome

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24
Q

BUZZWORDS: tabes dorsalis is assoc with what infection

A

neurosyphilis

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25
BUZZWORDS: MRI finding seen in patients on chronic steroids?
epidural lipomatosis
26
BUZZWORDS: Gene in adrenoleukodystrophy, X-linked
ABCD1 gene on chromosome Xq28
27
Conversion rate of MCI to dementia is
10-15%/ year
28
routine dementia screening labs
B12, CBC, CMP, TSH, depression screening further testing includes HIV, VDRL, LP, heavy metals
29
genes involved in inherited dementia
Presenilin-2 on chromosome 1 leads to early onset familial AD Presenilin-1 on chromosome 14 accounts for nearly 70-80% of early onset aggressive familial cases of AD APP (amyloid precursor protein) on chromosome 21
30
Which mutation is assoc with 3x risk of Alzheimers
Apo-E4 on chromosome 19
31
Earliest signs of Alzheimers
recent memory loss of events
32
FDG-PET in Alzheimers
parietotemporal hypometabolism
33
FDG-PET in FTD?
hypo-metabolism in frontal and anterior temporal regions
34
FDG-PET in Huntington's
caudate hypometabolism
35
FDG-PET in Lewy Body Dementia
hypometabolism in occipital regions
36
earliest feature of Alzheimer's (pathologically)
Amyloid deposition is first, then plaques and tangles
37
What are Hirano bodies
eosinophilic rod like inclusions in the hippocampal pyramidal neurons seen in Alzheimers
38
Where is loss of cholinergic neurons in Alzheimers
Nucleus basalis of meynert
39
CSF findings in Alzheimers
reduced beta amyloid and increased total and phosphorylated tau
40
BUZZWORDS: NT in locus ceruleus
NE
41
BUZZWORDS: NT in median and dorsal raphe nucleus
serotonin
42
BUZZWORDS: NT in nucleus accumbens, ventral tegmental area
Dopamine
43
BUZZWORDS: NT in substantia nigra pars compacta
gaba
44
Triad in Lewy Body Dementia Best neuroleptic?
parkinsonism fluctuating cognitive impairment visual hallucinations Best neuroleptic is clozapine (hella side effects) and quetiapine
45
Familial FTD chromosome assoc types?
17 1. Behavioral variant (most common): personality change, abulia, apathy, poor hygiene, hyperphagia 2. Progressive confluent aphasia: anomia, word-finding difficulty, impaired object naming and effortful speech with preserved comprehension (similar to Brocca) 3. Semantic dementia (progressive fluent aphasia): normal fluency but impaired comprehension, anomia, and semantic paraphrasias.
46
bilateral globus pallidus interna lesions (and sometimes bilateral ACA Infarcts) can cause?
akinetic mutism: preserved awareness with opened eyes but remains immobile, mute and does not respond to commands.
47
FTD is also assoc with what syndrome?
Kluver-Bucy Syndrome (bilateral lesions of the temporal lobe) * hyperorality, hypermetamorphosis , blunted emotional effects, hyper sexuality, and visual agnosia
48
MOA of TGA
thought to be due to temporary ischemia of the medial temporal lobes/hippocampus
49
What are lewy bodies?
**cytoplasmic** inclusions with anti-ubiquitin and anti-alpha-synuclein immunohistochemistry
50
pathology for FTD?
* present of silver-staining, spherical aggregations of tau protein in neurons
51
Psychogenic amnesia: characteristic findings?
loss of autobiographical memory, sometimes with preserved ability for new learning
52
MOA of memantine
NMDA antagonist
53
MOA of donepezil
Acetylcholinesterase inhibitor
54
MOA of rivastigmine
combined acetylcholinesterase and butyrylcholinesterase antagonist
55
MOA of galantamine
* acetylcholinesterase inhibitor and nicotine modulator
56
What are NF tangles?
intraneuronal collection of hyperphosphorylated tau protein
57
Prion protein gene (PRNP) coding for PrP is located on chrom?
20
58
In NPH, what procedure predicts a better chance of improvement from a shunt?
Large volume tap (30-50cc) with improvement in sx
59
Topographagnosis?
inability to navigate in familiar places, read maps, draw floor maps of familiar places
60
Misoplegia?
severe hatred of a limb, rare form of agnosia seen in hemiparetic or hemiplegic patients after strokes
61
Balint's syndome: location and triad
BL parieto-occipital lobes 1. Optic ataxia (deficit of reaching for objects under visual guidance) 2. Oculomotor apraxia (gaze apraxia, inability to voluntarily move the eyes to a new point of visual fixation despite normal EOM) 3. Simultagnosia (inability to visually perceive more than one object at a time)
62
Anton's Syndrome: location and sx
cortical blindness and visual agnosia from bilateral lesions to **medial occipital lobes.** Denial of visual loss and confabulation (patient is blind but completely unaware)
63
Charles Bonnet Syndrome: cause and sx
vivid hallucinations that occur in people with severe visual impairment due to a variety of reasons, most commonly ophthalmic (macular degen) Patients are aware they are hallucinating.
64
Alexia without agraphia: location and sx
Due to a lesion in the dominant PCA affecting the medial and inferior occipito-temporal region **and splenium of the corpus callosum** alexia: loss of reading comprehension writing and language comprehension are normal. Patient will also have contralateral homonymous hemianopsia.
65
Pure word deafness (verbal auditory agnosia): location and sx
Lesion in the bilateral middle portion of the superior temporal gyri, disrupting connection between Wernicke's and Heschl's gyrus * impaired auditory comprehension though hearing is intact (normal audiogram) * normal comprehension of written language (distinguishes it from Wernicke)~
66
Aphemia: location and sx
Lesion in dominant frontal operculum pure word mutism or verbal apraxia: inability to speak fluently, impaired repetitions and intact auditory comprehension (retained ability to write and comprehend written language)
67
Foix-Chavany-Marie Syndrome: location and sx
AKA anterior operculum syndrome, **lesions to bilateral anterior operculums** (frequently in setting of multiple infarcts) * severe dysarthria * bilateral voluntary paralysis of the lower CN with **preserved involuntary and emotional innervation.** Typically, it is described as a loss of voluntary control of palatal, glossal, pharyngeal, masticatory and oral muscles, but with preservation of reflex movements.
68
Amelodia/Affective motor aprosodia: location and sx
Lesion in non-dominant hemispheres analog to Broca's area inability of a person to properly convey or interpret emotional prosody.
69
Receptive aprosodia: location and sx
Lesion in nondominant analog to Wernicke's area inability to perceive and understand the emotional context of other's speech
70
Pseudo-bulbar affect: location and sx
condition that's characterized by episodes of sudden uncontrollable and inappropriate laughing or crying ## Footnote **bilateral lesions that disconnect the corticobulbar tracts from the brainstem CN nuclei, seen in ALS (most common) MS, TBI**
71
Treatment option for pseudobulbar affect ?
Dextromethorphan/quinidine
72
Apraxia: location and sx
dominant parietal lobe impaired ability to execute a previously known task, in the absence of motor weakness, incoordination or deficit in comprehension.
73
Ideomotor apraxia? location and sx
dominant parietal cortex: superior marginal/angular gyrus use of a body part as an object during pantomime (using finger as a toothbrush when asked to pantomime brushing teeth vs pantomiming holding the toothbrush)
74
Ideational apraxia? location and sx
bifrontal or biparietal cortex impairment in the sequence of motions needed to carry out a specific movement. (ex: ask to pour water in a glass and drink, the patient would drink from the glass before pouring water in)
75
Conduction apraxia?
impairment in imitation of movements
76
dissociation apraxia?
cannot execute a movement command but can imitate.
77
orbitofrontal lesions are assoc with changes in?
personality
78
medial frontal cortex lesions are associated with?
amotivation
79
lateral frontal lobe lesions are associated with ?
executive difficulties
80
Gerstmann's Syndrome: location and tetrad
dominant inferior parietal lobe: **angular gyrus** 1. finger agnosia 2. R-L confusion 3. dyscalculia 4. dysgraphia (inabilty to write)
81
Gaze deviation from pontine lesions vs frontal eye field lesions?
Frontal eye field: overcome by oculocephalic maneuver pontine lesions: not overcome by maneuver
82
Kluver Bucy Syndrome: location and sx
**bilateral anterior temporal lobe lesions** (usually following HSV encephalitis, bilateral temporal lobectomy or neurodegenerative disease such as FTD) hyperorality, visual agnosia, hypersexuality, blunted emotional affect, and hypermetamorphosis (excessive attentiveness to visual stimuli with a tendency to touch every such stimulus)
83
Alien Limb Syndrome: location and sx?
lesion to the contralateral ACA, involving the corpus callosum or SMA movement of a limb not under voluntary control Can also be seen in corticobasal syndrome
84
BUZZWORDS: Chromosome for Presenilin 2 in Alzheimers?
1
85
BUZZWORDS: chromosome for Presenilin 1 in Alzheimers
14
86
BUZZWORDS: chromosome for Apo-E4 in Alzheimers
19
87
BUZZWORDS: chromosome for APP (amyloid precursor protein) in Alzheimers
21
88
BUZZWORDS assoc with Alzheimers
neuritic plaques neurofibrillary tangles amyloid plaques amyloid angiopathy granulovacuolar degeneration Hirano bodies
89
BUZZWORDS assoc with Lewy Body Dementia
Fluctuating cognition, visual hallucinations, parkinsonism
90
BUZZWORDS: CAG repeat, Autosomal dom
Huntington's Disease
91
BUZZWORDS: list the synucleinopathies
MSA Parkinsons Dementia with Lewy Bodies neuroaxonal dystrophy
92
BUZZWORDS: List the tauopathies
AD corticobasal ganglionic degeneration (CBGD) PSP FTD
93
BUZZWORDS: globose neurofibrillary tangles and tufted astrocytes
PSP
94
BUZZWORDS: spongiform encephalopathy
CJD
95
BUZZWORDS: pathways assoc with “Where?” (where is an object in space)
parieto-occipital pathways
96
BUZZWORDS: pathways assoc with “What?” (what is an object)
parieto-temporal pathways
97
BUZZWORDS: dressing apraxia
nondominant parietal
98
BUZZWORDS: hemisensory neglect
nondominant parietal
99
BUZZWORDS: expressive aphasia with intact repetition
Transcortical motor aphasia MCA-ACA watershed infarct that disconnects the supplementary motor area from Broca
100
BUZZWORDS: receptive aphasia with intact repitition
Transcortical sensory aphasia MCA-PCA watershed or thalamic infarct

Eugenie Notes (10 decks)

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