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Eugenie Notes > Notes 3b > Flashcards

Notes 3b Flashcards

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
Q

BUZZWORDS: MRI finding seen in patients on chronic steroids?

A

epidural lipomatosis

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26
Q

BUZZWORDS: Gene in adrenoleukodystrophy, X-linked

A

ABCD1 gene on chromosome Xq28

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27
Q

Conversion rate of MCI to dementia is

A

10-15%/ year

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28
Q

routine dementia screening labs

A

B12, CBC, CMP, TSH, depression screening

further testing includes HIV, VDRL, LP, heavy metals

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29
Q

genes involved in inherited dementia

A

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

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30
Q

Which mutation is assoc with 3x risk of Alzheimers

A

Apo-E4 on chromosome 19

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31
Q

Earliest signs of Alzheimers

A

recent memory loss of events

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32
Q

FDG-PET in Alzheimers

A

parietotemporal hypometabolism

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33
Q

FDG-PET in FTD?

A

hypo-metabolism in frontal and anterior temporal regions

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34
Q

FDG-PET in Huntington’s

A

caudate hypometabolism

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35
Q

FDG-PET in Lewy Body Dementia

A

hypometabolism in occipital regions

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36
Q

earliest feature of Alzheimer’s (pathologically)

A

Amyloid deposition is first, then plaques and tangles

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37
Q

What are Hirano bodies

A

eosinophilic rod like inclusions in the hippocampal pyramidal neurons seen in Alzheimers

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38
Q

Where is loss of cholinergic neurons in Alzheimers

A

Nucleus basalis of meynert

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39
Q

CSF findings in Alzheimers

A

reduced beta amyloid and increased total and phosphorylated tau

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40
Q

BUZZWORDS: NT in locus ceruleus

A

NE

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41
Q

BUZZWORDS: NT in median and dorsal raphe nucleus

A

serotonin

42
Q

BUZZWORDS: NT in nucleus accumbens, ventral tegmental area

A

Dopamine

43
Q

BUZZWORDS: NT in substantia nigra pars compacta

A

gaba

44
Q

Triad in Lewy Body Dementia

Best neuroleptic?

A

parkinsonism

fluctuating cognitive impairment

visual hallucinations

Best neuroleptic is clozapine (hella side effects) and quetiapine

45
Q

Familial FTD

chromosome assoc

types?

A

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
Q

bilateral globus pallidus interna lesions (and sometimes bilateral ACA Infarcts) can cause?

A

akinetic mutism: preserved awareness with opened eyes but remains immobile, mute and does not respond to commands.

47
Q

FTD is also assoc with what syndrome?

A

Kluver-Bucy Syndrome (bilateral lesions of the temporal lobe)

  • hyperorality, hypermetamorphosis , blunted emotional effects, hyper sexuality, and visual agnosia
48
Q

MOA of TGA

A

thought to be due to temporary ischemia of the medial temporal lobes/hippocampus

49
Q

What are lewy bodies?

A

cytoplasmic inclusions with anti-ubiquitin and anti-alpha-synuclein immunohistochemistry

50
Q

pathology for FTD?

A
  • present of silver-staining, spherical aggregations of tau protein in neurons
51
Q

Psychogenic amnesia: characteristic findings?

A

loss of autobiographical memory, sometimes with preserved ability for new learning

52
Q

MOA of memantine

A

NMDA antagonist

53
Q

MOA of donepezil

A

Acetylcholinesterase inhibitor

54
Q

MOA of rivastigmine

A

combined acetylcholinesterase and butyrylcholinesterase antagonist

55
Q

MOA of galantamine

A
  • acetylcholinesterase inhibitor and nicotine modulator
56
Q

What are NF tangles?

A

intraneuronal collection of hyperphosphorylated tau protein

57
Q

Prion protein gene (PRNP) coding for PrP is located on chrom?

A

20

58
Q

In NPH, what procedure predicts a better chance of improvement from a shunt?

A

Large volume tap (30-50cc) with improvement in sx

59
Q

Topographagnosis?

A

inability to navigate in familiar places, read maps, draw floor maps of familiar places

60
Q

Misoplegia?

A

severe hatred of a limb, rare form of agnosia seen in hemiparetic or hemiplegic patients after strokes

61
Q

Balint’s syndome: location and triad

A

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
Q

Anton’s Syndrome: location and sx

A

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
Q

Charles Bonnet Syndrome: cause and sx

A

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
Q

Alexia without agraphia: location and sx

A

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
Q

Pure word deafness (verbal auditory agnosia): location and sx

A

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
Q

Aphemia: location and sx

A

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
Q

Foix-Chavany-Marie Syndrome: location and sx

A

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
Q

Amelodia/Affective motor aprosodia: location and sx

A

Lesion in non-dominant hemispheres analog to Broca’s area

inability of a person to properly convey or interpret emotional prosody.

69
Q

Receptive aprosodia: location and sx

A

Lesion in nondominant analog to Wernicke’s area

inability to perceive and understand the emotional context of other’s speech

70
Q

Pseudo-bulbar affect: location and sx

A

condition that’s characterized by episodes of sudden uncontrollable and inappropriate laughing or crying

bilateral lesions that disconnect the corticobulbar tracts from the brainstem CN nuclei, seen in ALS (most common) MS, TBI

71
Q

Treatment option for pseudobulbar affect ?

A

Dextromethorphan/quinidine

72
Q

Apraxia: location and sx

A

dominant parietal lobe

impaired ability to execute a previously known task, in the absence of motor weakness, incoordination or deficit in comprehension.

73
Q

Ideomotor apraxia? location and sx

A

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
Q

Ideational apraxia? location and sx

A

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
Q

Conduction apraxia?

A

impairment in imitation of movements

76
Q

dissociation apraxia?

A

cannot execute a movement command but can imitate.

77
Q

orbitofrontal lesions are assoc with changes in?

A

personality

78
Q

medial frontal cortex lesions are associated with?

A

amotivation

79
Q

lateral frontal lobe lesions are associated with ?

A

executive difficulties

80
Q

Gerstmann’s Syndrome: location and tetrad

A

dominant inferior parietal lobe: angular gyrus

  1. finger agnosia
  2. R-L confusion
  3. dyscalculia
  4. dysgraphia (inabilty to write)
81
Q

Gaze deviation from pontine lesions vs frontal eye field lesions?

A

Frontal eye field: overcome by oculocephalic maneuver

pontine lesions: not overcome by maneuver

82
Q

Kluver Bucy Syndrome: location and sx

A

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
Q

Alien Limb Syndrome: location and sx?

A

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
Q

BUZZWORDS: Chromosome for Presenilin 2 in Alzheimers?

A

1

85
Q

BUZZWORDS: chromosome for Presenilin 1 in Alzheimers

A

14

86
Q

BUZZWORDS: chromosome for Apo-E4 in Alzheimers

A

19

87
Q

BUZZWORDS: chromosome for APP (amyloid precursor protein) in Alzheimers

A

21

88
Q

BUZZWORDS assoc with Alzheimers

A

neuritic plaques

neurofibrillary tangles

amyloid plaques

amyloid angiopathy

granulovacuolar degeneration

Hirano bodies

89
Q

BUZZWORDS assoc with Lewy Body Dementia

A

Fluctuating cognition, visual hallucinations, parkinsonism

90
Q

BUZZWORDS: CAG repeat, Autosomal dom

A

Huntington’s Disease

91
Q

BUZZWORDS: list the synucleinopathies

A

MSA

Parkinsons

Dementia with Lewy Bodies

neuroaxonal dystrophy

92
Q

BUZZWORDS: List the tauopathies

A

AD
corticobasal ganglionic degeneration (CBGD)

PSP

FTD

93
Q

BUZZWORDS: globose neurofibrillary tangles and tufted astrocytes

A

PSP

94
Q

BUZZWORDS: spongiform encephalopathy

A

CJD

95
Q

BUZZWORDS: pathways assoc with “Where?” (where is an object in space)

A

parieto-occipital pathways

96
Q

BUZZWORDS: pathways assoc with “What?” (what is an object)

A

parieto-temporal pathways

97
Q

BUZZWORDS: dressing apraxia

A

nondominant parietal

98
Q

BUZZWORDS: hemisensory neglect

A

nondominant parietal

99
Q

BUZZWORDS: expressive aphasia with intact repetition

A

Transcortical motor aphasia

MCA-ACA watershed infarct that disconnects the supplementary motor area from Broca

100
Q

BUZZWORDS: receptive aphasia with intact repitition

A

Transcortical sensory aphasia

MCA-PCA watershed or thalamic infarct

Eugenie Notes (10 decks)

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