Neuro Pathology Flashcards
(128 cards)
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
2
Q
spina bifida
A
failure of closure of the caudal neuropore
vertebral defect
3
Q
spind bifida occulta
A
no herniation
tuft of hair
4
Q
meningocele
A
herniation of the meninges
5
Q
meningomyelocele
A
herniation of meninges and spinal cord
6
Q
cerebral aqueduct stenosis
A
increased ICP
enlarged head circumference due to dilation of the ventricles
7
Q
Dandy-Walker Malformation
A
congenital failure of cerebellar vermis to develop
massively dilated 4th ventricle with absent cerebellum; accompanied by hydrocephalus
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
9
Q
Arnold-Chiari Malformation
Type 2
A
More rare; 12% die within first year
death result of compression of cranial nerves -> respiratory compromise
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)
11
Q
Poliomyelitis
A
fecal-oral transmission
oropharynx > small bowel > blood > CNS
damage to ventral motor horn
FLACCID paralysis
12
Q
Werdnig-Hoffman Disease
A
Inherited degeneration of ventral motor horn
autosomal recessive
FLOPPY BABY
death occurs within a few years after birth
13
Q
ALS
A
degenerative disorder of UMN + LMN
- > flaccid paralysis
- > muscle atrophy
- > fasciculations
- > weakness
- > decreased tone
- > impaired reflexes
- > neg. Babinski
14
Q
ALS vs. Syringomyelia
A
ALS spares pain and temp. sensation
15
Q
How does one get ALS?
A
- sporadic
2. familial mutation ex. zinc-copper superoxide dismutase causing free radical injury
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
17
Q
types of meningitis
A
bacterial (septic)
viral (aseptic)
fungal
18
Q
most common sources of meningitis in newborns
A
GBS (test in 3rd trimester of pregnant women)
E. coli
L. monocytogenes
19
Q
most common source of meningitis in children/teenagers
A
N. meningitidis
20
Q
most common source of meningitis in adults
A
S. pneumo
21
Q
most common source of meningitis in old people
A
L. monocytogenes
22
Q
most common source of meningitis in non vaccinated infants
A
H. influenza
23
Q
most common viral source of meningitis
A
Coxsackievirus
24
Q
most common source of meningitis in immunocompromised
A
fungal
25
meningitis presentation
```
headache
nuchal rigidity
fever
photophobia
vomiting
altered mental status (may be present)
```
26
test for meningitis
LP for CSF profile
27
Bacterial meningitis ( findings)
high PMNs
| low glucose
28
Viral meningitis
| LP findings
high lymphocytes
| normal glucose
29
Fungal meningitis
| LP findings
high lymphocytes
| low glucose
30
complications of bacterial meningitis
massive amts of exudate and pus
cerebral edema > herniation > death
massive tissue damage > fibrosis > hydrocephalus, hearing loss, seizures
31
Cerebrovascular Disease (basics)
Neurologic deficits due to CV compromise
1) ischemia (85%)
2) hemorrhage (15%)
32
Hemorrhagic CVD
intracerebral and subarachnoid
33
Etiologies of Global Ischemia (CVD)
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)
34
mild global ischemic CVD
transient confusion
35
moderate global ischemic CVD
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
36
severe global ischemic CVD
diffuse necrosis of the brain > death or vegetative state
37
ischemic CVD
focal or global
38
TIA
focal neurologic deficits
| symptoms < 24hours
39
ischemic stroke
focal neurologic deficits > 24hours
40
ischemic stroke subtypes
1. thrombotic
2. embolic
3. lacunar
41
thrombotic stroke
due to rupture of atherosclerotic plaque
usually develops at branch points
-> pale infarction; cannot lyse thrombus; cannot get blood to region
42
embolic stroke
due to thromboemboli
most common site is the left side of the heart (a.fib)
-> red infarct; hemorrhagic infarction; can easily be lysed
43
lacunar stroke
risk factors: HTN, diabetes, smoking, advanced age
-> lake-like areas of infarct
thickened wall + narrowed lumen > decreased blood going through small vessels
44
intracerebral hemorrhage
bleeding into brain parenchyma
classically due to rupture of Charcot-Bouchard microaneurysms
basal ganglia is the MOST COMMON SITE
45
subarachnoid hemorrhage
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
46
epidural hematoma
lens-shaped lesion
classically rupture of middle meningeal artery (high pressure circuit)
"talk and die"
lucid interval precede neuro symptoms
47
subdural hematoma
crescent-shaped lesion
tearing of bridging veins
progressive neuro signs
48
tonsillar herniation
cerebellar tonsils into foramen magnum
compression of brainstem (medulla) -> coma, cardiopulmonary arrest, death
49
subfalcine herniation
displacement of cingulate gyrus under the falx cerebri
most common type of brain herniation
compression of ACA > infarction
clinical presentation: headache, contralateral leg weakness
50
uncal herniation
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)
51
oligodendrocytes
myelinate CNS
| multiple
52
schwann cells
myeline PNS
| one-to-one
53
Inherited Leukodystrophies
1. metachromatic leukodystrophy
2. krabbe disease
3. adrenoleukodystrophy
54
metachromatic leukodystrophy
deficiency of arylsulfatase
- > myelin cannot be degraded
- > accumulates in the lysosome
MOST COMMON
AUTOSOMAL RECESSIVE
55
krabbe disease
deficiency of galactocerebroside
-> build up of unmetabolized lipids
AUTOSOMAL RECESSIVE
most common in infants
56
adrenoleukodystrophy
impaired addition of coenzyme A to long-chain fatty acids
X-linked defect
57
Multiple Sclerosis (MS)
autoimmune destruction of CNS myelin and oligodendrocytes
```
young adults (20-30)
women
```
relapsing-remitting
58
MS presentation
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
How is MS diagnosis made?
MRI: plaques
LP: increased lymphocytes, increased immunoglobulins with oligoclonal IgG bands, myelin basic protein
60
MS treatment
acute attacks: high-dose steroids
long-term: INF-beta (slows progression)
61
subacute sclerosing panencephalitis
progressive, debilitating encephalitis
persistent infection of the brain by measles virus
viral inclusions within neurons (gray matter) and oligodendrocytes (white matter)
62
progressive multifocal leukoencephalopathy
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
central pontine myelinolysis
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
dementia
degeneration of the cortex
memory + cognitive dysfunction w/o loss of consciousness
65
movement disorders
degeneration of the brainstem and basal ganglia
66
Alzheimer Disease
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
Early-onset AD
```
familial cases w/ presilin 1 and 2
down syndrome (APP found on chromosome 21)
```
68
Allele Assoc. Risk with AD
increased (epsilon 4 allele of apolipoprotein E)
| decreased (epsilon 2 allele of apolipoprotein E)
69
AD Morphology
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
Vascular Dementia
2nd most common cause of dementia
| multifocal infarction and injury due to atherosclerosis or vasculitis
71
Pick Disease
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
Parkinson's Disease
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
PD Histology
Loss of pigmented neurons in the substantia nigra and round, eosinophilic inclusions of alpha-synuclein (Lewy bodies)
74
Huntington Disease
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
chorea
random movement of muscle loss of inhibitory hold on the cortex
76
anticipation
early onset with early generation
expansion of repeats
occurs during spermatogenesis
77
akathisia
motor restlessness (an inability to sit still)
78
athetosis
slow, writhing movements which may resemble dystonia
79
ballism
proximal, large-amplitude chorea
unilateral=hemiballism
80
chorea
non-rhythmic unpredictable movements; involuntary, randomly flowing jerks
81
dystonia
an abnormal posture; the result of co-contraction of agonist and antagonist muscle contractions may also result in twisting repetitive movements
82
myoclonus
shock-like movements due either to muscle contraction (+) or abrupt loss of muscle tone (-)
83
sterotypy
a set of movements that last for a time; purposeless repetition of the motor set
84
tic
patterned simple or complex paroxysmal movements ("sensory tic")
85
tremor
rhythmic movement, with amplitude and frequency as in a sine wave, involving one or more body parts
86
akinesia
lack of purposeful movement
87
bradykinesia
slowness of movement
88
normal pressure hydrocephalus
wacky
wet
wobbly
89
spongiform encephalopathy
```
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
Creutzfeldt-Jakob Disease
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
CNS tumors
- > metastatic (50%)-commonly from lung, brain and kidney
- > primary (50%)
- > adults above tentorium
- > kids below tentorium
92
Primary tumor cell types
1. astrocytes
2. oligodendrocytes
3. ependymal cells
4. meningothelial cells
5. neuroectoderm
93
Astrocyte Primary Tumors
```
Polycytic Astrocytoma (children)
Glioblastoma Multiforme (adults)
```
94
Polycytic Astrocytoma
benign tumor of astrocytes
most common in children
cystic lesion with mural nodule
95
Glioblastoma Multiforme
```
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
Oligodendroglioma
malignant tumor of oligodendrocytes
97
pseudopalisading
region of necrosis surrounded by tumor cells
98
ependymoma
children
malignant tumor of ependymal cells
most commonly arises in 4th ventricle
perivascular pseudorosettes
99
meningioma
most common benign tumor in adults
more common in women
round mass attached to dura
tumor expresses estrogen receptor
100
schwannoma
benign tumor of Schwann cells
commonly involves CN VIII -> hearing and tinnitus
101
medulloblastoma
malignant tumor derived from the granular cells of the cerebellum (neuroectoderm)
children
drop metastasis to cauda equina
histology > small, round blue cells
102
craniopharyngioma
- > 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
amyotrophy
describes clinically evident muscle atrophy
104
lateral sclerosis
describes the gross pathology findings of sclerosis of the lateral CST in the spinal cord
105
sclerosis
scarring
106
dysmetria
abnormal measure; finger-nose-finger
107
dysdiadochokinesia
abnormal rapid alternating movements
108
past-pointing
overshoot; mirroring
109
dysarthria
scanning speech; impaired articulation and prosody (rhythm)
110
encephalocele
brain herniates through a defect in the skull bones
| most common site occipital bone
111
metastatic CNS tumor
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
parinaud syndrome
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
allodynia
pain from a stimulus that does not normally evoke pain
114
hyperalgesia
exaggerated response to a normally painful stimulus (e.g. hot shower after sunburn)
115
taxonomy of pain
nociceptive
inflammatory
neuropathic
dysfunctional
116
nociceptive pain
no pathology
requires an ongoing noxious stimulus
a high threshold protective alarm system
117
inflammatory pain
tissue injury with inflammation
allodynia, hyperalgesia, spontaneous pain
a low threshold protective system that promotes healing/repair
118
neuropathic pain
PNS or CNS lesions
allodynia, hyperalgesia, spontaneous pain, negative symptoms
low threshold-pathologic/maladaptive
119
dysfunctional pain
altered CNS function--no known lesion/no peripheral pathology
allodynia, hyperalgesia, spontaneous pain, no negative symptoms
low threshold-pathological and maladaptive
120
resting state (pain)
nociceptive
121
sensitized (pain)
inflammatory
neuropathic
dysfunctional
122
physiological pain
nociceptive
| inflammatory
123
pathologic pain
neuropathic
| dysfunctional
124
adaptation
with maintained stimuli, response declines, so cells report stimulus change
125
receptive field
receptor cells report stimuli in one area (space, orientation, color, frequency, odor etc)
126
Synucleinopathies (Lewy Body Diseases)
```
Parkinson's Disease
Multiple Systems Atrophy
Dementia with Lewy Bodies
Essential tremor (occasional)
Neurodegeneration with brain iron accumulation (occasional)
```
127
Multiple System Atrophy (MSA)
progressive neurodegenerative disorder affecting autonomic nerves and can affect the basal ganglia--orthostatic hypotension (differentiates from PD)
lewy bodies present in oligodendrocytes
128
Lewy Bodies
cytoplasmic inclusion containing alpha-synuclein, poly-ubiquitin, neurofilament and other proteins
relationship to pathogenesis unclear
