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Neurology > Neuro Pathology > Flashcards

Neuro Pathology Flashcards

1
Q

anencephaly

A

failure of closure of the rostral neuropore
absence of skull and brain
frog-like appearance
absence of CNS controlled swallowing > maternal polyhydraminos

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2
Q

spina bifida

A

failure of closure of the caudal neuropore

vertebral defect

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3
Q

spind bifida occulta

A

no herniation

tuft of hair

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4
Q

meningocele

A

herniation of the meninges

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5
Q

meningomyelocele

A

herniation of meninges and spinal cord

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6
Q

cerebral aqueduct stenosis

A

increased ICP

enlarged head circumference due to dilation of the ventricles

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7
Q

Dandy-Walker Malformation

A

congenital failure of cerebellar vermis to develop

massively dilated 4th ventricle with absent cerebellum; accompanied by hydrocephalus

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8
Q

Arnold-Chiari Malformation

Type 1

A

Congenital extension of the cerebellar tonsils through foramen magnum
obstruction of CSF -> meningomyelocele or syringomyelia
presentation @ adolescence or young adulthood

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9
Q

Arnold-Chiari Malformation

Type 2

A

More rare; 12% die within first year

death result of compression of cranial nerves -> respiratory compromise

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10
Q

syringomyelia

A

cyst forms within the spinal cord (syrinx)
usually C8-T1
1) effects anterolateral spinothalamic tract; knocks out anterior commissure

CAPE-LIKE distribution

spares DCML

2) expansion -> knocks out ventral horn (LMN) -> flaccid
3) expansion -> knocks out lateral (iml) horn -> sympathetics lost to face (hypothalamic spinal tract)

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11
Q

Poliomyelitis

A

fecal-oral transmission
oropharynx > small bowel > blood > CNS

damage to ventral motor horn
FLACCID paralysis

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12
Q

Werdnig-Hoffman Disease

A

Inherited degeneration of ventral motor horn
autosomal recessive
FLOPPY BABY
death occurs within a few years after birth

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13
Q

ALS

A

degenerative disorder of UMN + LMN

  • > flaccid paralysis
  • > muscle atrophy
  • > fasciculations
  • > weakness
  • > decreased tone
  • > impaired reflexes
  • > neg. Babinski
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14
Q

ALS vs. Syringomyelia

A

ALS spares pain and temp. sensation

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15
Q

How does one get ALS?

A
  1. sporadic

2. familial mutation ex. zinc-copper superoxide dismutase causing free radical injury

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16
Q

Fredreich Ataxia

A

degenerative disorder of the cerebellar and spinal cord
progressive limb and gait ataxia, dysarthria, loss of proprioception and vibration sense
AUTOSOMAL RECESSIVE
expansion of unstable trinucleotide repeat (GAA) frataxin gene chromosome 9

iron buildup with free radical damage

presents early childhood (8-15yrs)
assoc. hypertrophic cardiomyopathy

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17
Q

types of meningitis

A

bacterial (septic)
viral (aseptic)
fungal

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18
Q

most common sources of meningitis in newborns

A

GBS (test in 3rd trimester of pregnant women)
E. coli
L. monocytogenes

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19
Q

most common source of meningitis in children/teenagers

A

N. meningitidis

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20
Q

most common source of meningitis in adults

A

S. pneumo

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21
Q

most common source of meningitis in old people

A

L. monocytogenes

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22
Q

most common source of meningitis in non vaccinated infants

A

H. influenza

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23
Q

most common viral source of meningitis

A

Coxsackievirus

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24
Q

most common source of meningitis in immunocompromised

A

fungal

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25
Q

meningitis presentation

A 
headache
nuchal rigidity 
fever 
photophobia 
vomiting 
altered mental status (may be present)
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26
Q

test for meningitis

A

LP for CSF profile

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27
Q

Bacterial meningitis ( findings)

A

high PMNs

low glucose

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28
Q

Viral meningitis

LP findings

A

high lymphocytes

normal glucose

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29
Q

Fungal meningitis

LP findings

A

high lymphocytes

low glucose

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30
Q

complications of bacterial meningitis

A

massive amts of exudate and pus

cerebral edema > herniation > death

massive tissue damage > fibrosis > hydrocephalus, hearing loss, seizures

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31
Q

Cerebrovascular Disease (basics)

A

Neurologic deficits due to CV compromise

1) ischemia (85%)
2) hemorrhage (15%)

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32
Q

Hemorrhagic CVD

A

intracerebral and subarachnoid

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33
Q

Etiologies of Global Ischemia (CVD)

A
  1. low perfusion (e.g. atherosclerosis)
  2. acute disease in blood flow (e.g. cardiogenic shock)
  3. chronic hypoxia (e..g anemia)
  4. repeated episode of hypoglycemia (e.g. insulinoma)
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34
Q

mild global ischemic CVD

A

transient confusion

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35
Q

moderate global ischemic CVD

A

infarct in “watershed” areas (areas supplied by end of a circulation)

  1. pyramidal neurons of the cerebral cortex (layers 3, 5, 6) leads to laminar necrosis
  2. pyramidal neurons of the hippocampus (temporal lobe)-impt long term memory
  3. purkinje layer of the cerebellum-integrates sensory perception with motor control
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36
Q

severe global ischemic CVD

A

diffuse necrosis of the brain > death or vegetative state

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37
Q

ischemic CVD

A

focal or global

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38
Q

TIA

A

focal neurologic deficits

symptoms < 24hours

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39
Q

ischemic stroke

A

focal neurologic deficits > 24hours

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40
Q

ischemic stroke subtypes

A
  1. thrombotic
  2. embolic
  3. lacunar
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41
Q

thrombotic stroke

A

due to rupture of atherosclerotic plaque

usually develops at branch points

-> pale infarction; cannot lyse thrombus; cannot get blood to region

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42
Q

embolic stroke

A

due to thromboemboli

most common site is the left side of the heart (a.fib)

-> red infarct; hemorrhagic infarction; can easily be lysed

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43
Q

lacunar stroke

A

risk factors: HTN, diabetes, smoking, advanced age

-> lake-like areas of infarct

thickened wall + narrowed lumen > decreased blood going through small vessels

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44
Q

intracerebral hemorrhage

A

bleeding into brain parenchyma

classically due to rupture of Charcot-Bouchard microaneurysms

basal ganglia is the MOST COMMON SITE

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45
Q

subarachnoid hemorrhage

A

bleeding into the subarachnoid space

  1. sudden headache
  2. “worst headache of my life”
  3. nuchal rigidity

most commonly (85%) due to berry aneurysm rupture

lack of media layer at the branch point, weak wall, saccular outpouching

assoc. w/ Marfan syndrome, Ehlers-Danlos syndrome, and autosomal dominant polycystic kidney disease

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46
Q

epidural hematoma

A

lens-shaped lesion
classically rupture of middle meningeal artery (high pressure circuit)

“talk and die”
lucid interval precede neuro symptoms

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47
Q

subdural hematoma

A

crescent-shaped lesion
tearing of bridging veins

progressive neuro signs

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48
Q

tonsillar herniation

A

cerebellar tonsils into foramen magnum

compression of brainstem (medulla) -> coma, cardiopulmonary arrest, death

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49
Q

subfalcine herniation

A

displacement of cingulate gyrus under the falx cerebri
most common type of brain herniation
compression of ACA > infarction

clinical presentation: headache, contralateral leg weakness

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50
Q

uncal herniation

A

displacement of the temporal lobe uncus under the tentoriumcerebelli

  1. compression of CN III
    down and out
    dilated pupil
    ptosis
  2. compression PCA -> infarction of occipital lobe (contralateral homonymous hemianopsia)
  3. rupture of paramedian artery -> brainstem hemorrhage (duret)
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51
Q

oligodendrocytes

A

myelinate CNS

multiple

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52
Q

schwann cells

A

myeline PNS

one-to-one

53
Q

Inherited Leukodystrophies

A
  1. metachromatic leukodystrophy
  2. krabbe disease
  3. adrenoleukodystrophy
54
Q

metachromatic leukodystrophy

A

deficiency of arylsulfatase

  • > myelin cannot be degraded
  • > accumulates in the lysosome

MOST COMMON
AUTOSOMAL RECESSIVE

55
Q

krabbe disease

A

deficiency of galactocerebroside
-> build up of unmetabolized lipids

AUTOSOMAL RECESSIVE

most common in infants

56
Q

adrenoleukodystrophy

A

impaired addition of coenzyme A to long-chain fatty acids

X-linked defect

57
Q

Multiple Sclerosis (MS)

A

autoimmune destruction of CNS myelin and oligodendrocytes

young adults (20-30) 
women

relapsing-remitting

58
Q

MS presentation

A
  1. blurred vision in one eye (optic nerve)
  2. vertigo and scanning speech
  3. iNO (MLF damage)
  4. hemiparesis or unilateral loss of sensation
  5. lower extremity loss of sensation and weakness
  6. bowel, bladder, and sexual dysfunction (ANS)
59
Q

How is MS diagnosis made?

A

MRI: plaques
LP: increased lymphocytes, increased immunoglobulins with oligoclonal IgG bands, myelin basic protein

60
Q

MS treatment

A

acute attacks: high-dose steroids

long-term: INF-beta (slows progression)

61
Q

subacute sclerosing panencephalitis

A

progressive, debilitating encephalitis

persistent infection of the brain by measles virus

viral inclusions within neurons (gray matter) and oligodendrocytes (white matter)

62
Q

progressive multifocal leukoencephalopathy

A

rapid progressive neuro signs leading to death

  1. visual loss
  2. weakness
  3. dementia

immunosuppression leads to reactivation of the latent virus

JC virus

63
Q

central pontine myelinolysis

A

focal demyelination of the pons -> “locked-in” syndrome (acute bilateral paralysis)

due to rapid intravenous correction of hyponatremia

predisposed patients: malnourished, alcoholics, liver disease

64
Q

dementia

A

degeneration of the cortex

memory + cognitive dysfunction w/o loss of consciousness

65
Q

movement disorders

A

degeneration of the brainstem and basal ganglia

66
Q

Alzheimer Disease

A

most common cause of dementia

presentation

  1. slow onset memory loss
  2. progressive disorientation
  3. loss of learned motor skills and language
  4. changes in behavior and personality
  5. patients become mute and bedridden
  • > APP cleaved to A-beta amyloid (as opposed to A-alpha)
  • > A-beta amyloid cannot be broken down
  • > deposits in the brain
67
Q

Early-onset AD

A 
familial cases w/ presilin 1 and 2 
down syndrome (APP found on chromosome 21)
68
Q

Allele Assoc. Risk with AD

A

increased (epsilon 4 allele of apolipoprotein E)

decreased (epsilon 2 allele of apolipoprotein E)

69
Q

AD Morphology

A
  1. Cerebral atrophy with narrowing gyri; sulci widening
  2. Neuritic plaques
  3. Neurofibillary tangles (intracellular aggregates of fibers composed of hyperphosphylated tau)
  4. Loss of cholinergic neurons in the nucleus basalis of Meynert
70
Q

Vascular Dementia

A

2nd most common cause of dementia

multifocal infarction and injury due to atherosclerosis or vasculitis

71
Q

Pick Disease

A

degenerative disease of the frontal and temporal cortex and spares the parietal and occipital lobes

round aggregates of tau protein (pick bodies) in the neurons of the cortex

behavior and language symptoms (first)
dementia (eventually)

72
Q

Parkinson’s Disease

A

Degenerative loss of dopaminergic neurons in the substantia nigra of the basal ganglia

TRAP
Tremor 
Rigidity 
Akinesia/Bradykinesia 
Postural instability and shuffling gait

MPTP can cause PD

73
Q

PD Histology

A

Loss of pigmented neurons in the substantia nigra and round, eosinophilic inclusions of alpha-synuclein (Lewy bodies)

74
Q

Huntington Disease

A

Degeneration of GABAergic neurons in the caudate nucleus of the basal ganglia

AUTOSOMAL DOMINANT
Expanded CAG repeats

chorea that can progress to dementia and depression

avg age onset 40

75
Q

chorea

A

random movement of muscle loss of inhibitory hold on the cortex

76
Q

anticipation

A

early onset with early generation
expansion of repeats
occurs during spermatogenesis

77
Q

akathisia

A

motor restlessness (an inability to sit still)

78
Q

athetosis

A

slow, writhing movements which may resemble dystonia

79
Q

ballism

A

proximal, large-amplitude chorea

unilateral=hemiballism

80
Q

chorea

A

non-rhythmic unpredictable movements; involuntary, randomly flowing jerks

81
Q

dystonia

A

an abnormal posture; the result of co-contraction of agonist and antagonist muscle contractions may also result in twisting repetitive movements

82
Q

myoclonus

A

shock-like movements due either to muscle contraction (+) or abrupt loss of muscle tone (-)

83
Q

sterotypy

A

a set of movements that last for a time; purposeless repetition of the motor set

84
Q

tic

A

patterned simple or complex paroxysmal movements (“sensory tic”)

85
Q

tremor

A

rhythmic movement, with amplitude and frequency as in a sine wave, involving one or more body parts

86
Q

akinesia

A

lack of purposeful movement

87
Q

bradykinesia

A

slowness of movement

88
Q

normal pressure hydrocephalus

A

wacky
wet
wobbly

89
Q

spongiform encephalopathy

A 
degenerative disease due to prion protein 
PrPc (normal; alpha-helical)
PrPSc (pathologic; beta-helical)
conversion 
1. sporadic 
2. inherited 
3. transmitted

damage to neurons and glial cells -> intracellular vacuoles

MOST COMMON = Creutzfeldt-Jakob Disease (CJD)

90
Q

Creutzfeldt-Jakob Disease

A

sporadic
exposure to prion-infected human tissue (ex. hGH or corneal transplant)

rapidly progressive dementia assoc. with ataxia and startle myoclonus

death < 1 year

91
Q

CNS tumors

A
  • > metastatic (50%)-commonly from lung, brain and kidney
  • > primary (50%)
  • > adults above tentorium
  • > kids below tentorium
92
Q

Primary tumor cell types

A
  1. astrocytes
  2. oligodendrocytes
  3. ependymal cells
  4. meningothelial cells
  5. neuroectoderm
93
Q

Astrocyte Primary Tumors

A 
Polycytic Astrocytoma (children) 
Glioblastoma Multiforme (adults)
94
Q

Polycytic Astrocytoma

A

benign tumor of astrocytes
most common in children
cystic lesion with mural nodule

95
Q

Glioblastoma Multiforme

A 
malignant high-grade tumor of astrocytes 
most primary malignant tumor in adults 
arises in cerebral hemisphere
crosses corpus callosum 
"butterfly" lesions 
pseudopalisading 
GFAP +
poor prognosis
96
Q

Oligodendroglioma

A

malignant tumor of oligodendrocytes

97
Q

pseudopalisading

A

region of necrosis surrounded by tumor cells

98
Q

ependymoma

A

children
malignant tumor of ependymal cells
most commonly arises in 4th ventricle
perivascular pseudorosettes

99
Q

meningioma

A

most common benign tumor in adults
more common in women
round mass attached to dura
tumor expresses estrogen receptor

100
Q

schwannoma

A

benign tumor of Schwann cells

commonly involves CN VIII -> hearing and tinnitus

101
Q

medulloblastoma

A

malignant tumor derived from the granular cells of the cerebellum (neuroectoderm)

children

drop metastasis to cauda equina

histology > small, round blue cells

102
Q

craniopharyngioma

A
  • > tumor that arises from epithelial remnants of Rathke’s pouch
  • > supratentorial mass in a child/young adult
  • > calcifications on imaging

may compromise optic chiasm > BITEMPORAL HEMIANOPSIA

don’t confuse with pituitary tumor

103
Q

amyotrophy

A

describes clinically evident muscle atrophy

104
Q

lateral sclerosis

A

describes the gross pathology findings of sclerosis of the lateral CST in the spinal cord

105
Q

sclerosis

A

scarring

106
Q

dysmetria

A

abnormal measure; finger-nose-finger

107
Q

dysdiadochokinesia

A

abnormal rapid alternating movements

108
Q

past-pointing

A

overshoot; mirroring

109
Q

dysarthria

A

scanning speech; impaired articulation and prosody (rhythm)

110
Q

encephalocele

A

brain herniates through a defect in the skull bones

most common site occipital bone

111
Q

metastatic CNS tumor

A

most common type of CNS tumor
indication: located at the junction of cortical gray and white matter, round shape

tend to metastasize from lung, breast, kidney, colorectal cancer, melanoma

112
Q

parinaud syndrome

A

lesion of the dorsal tectum of the midbrain (eg pineal gland tumor) that compresses

  • superior colliculus
  • pretectal area
  • and cerebral aqueduct (obstructive hydrocephalus)

interrupts melatonin production -> disrupts circadian rhythm -> insomnia

palsy of upward gaze
dissociation of light and accommodation
failure of convergence

113
Q

allodynia

A

pain from a stimulus that does not normally evoke pain

114
Q

hyperalgesia

A

exaggerated response to a normally painful stimulus (e.g. hot shower after sunburn)

115
Q

taxonomy of pain

A

nociceptive
inflammatory
neuropathic
dysfunctional

116
Q

nociceptive pain

A

no pathology
requires an ongoing noxious stimulus
a high threshold protective alarm system

117
Q

inflammatory pain

A

tissue injury with inflammation
allodynia, hyperalgesia, spontaneous pain
a low threshold protective system that promotes healing/repair

118
Q

neuropathic pain

A

PNS or CNS lesions
allodynia, hyperalgesia, spontaneous pain, negative symptoms
low threshold-pathologic/maladaptive

119
Q

dysfunctional pain

A

altered CNS function–no known lesion/no peripheral pathology
allodynia, hyperalgesia, spontaneous pain, no negative symptoms
low threshold-pathological and maladaptive

120
Q

resting state (pain)

A

nociceptive

121
Q

sensitized (pain)

A

inflammatory
neuropathic
dysfunctional

122
Q

physiological pain

A

nociceptive

inflammatory

123
Q

pathologic pain

A

neuropathic

dysfunctional

124
Q

adaptation

A

with maintained stimuli, response declines, so cells report stimulus change

125
Q

receptive field

A

receptor cells report stimuli in one area (space, orientation, color, frequency, odor etc)

126
Q

Synucleinopathies (Lewy Body Diseases)

A 
Parkinson's Disease 
Multiple Systems Atrophy 
Dementia with Lewy Bodies 
Essential tremor (occasional) 
Neurodegeneration with brain iron accumulation (occasional)
127
Q

Multiple System Atrophy (MSA)

A

progressive neurodegenerative disorder affecting autonomic nerves and can affect the basal ganglia–orthostatic hypotension (differentiates from PD)

lewy bodies present in oligodendrocytes

128
Q

Lewy Bodies

A

cytoplasmic inclusion containing alpha-synuclein, poly-ubiquitin, neurofilament and other proteins

relationship to pathogenesis unclear

Neurology (4 decks)

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