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61 Cards in this Set
- Front
- Back
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Neural tube defects cause ____ levels of Alpha-fetoprotein in the amniotic fluid and maternal blood
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elevated
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This developmental abnormality leads to a "frog-like" appearance of the fetus
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Anencephaly
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Why does anencephaly cause polyhydraminos?
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Because fetus cannot swallow amniotic fluid
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This occurs when the cerebellar vermis fails to develop
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Dandy-Walker malformation- presents as a massively dilated 4th ventricle and absent cerebellum
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This malformation occurs with congenital extension of cerebellar tonsils through the foramen magnum
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Arnold-Chiari malformation
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What sensory modalities are lost with syringomyelia? What tract?
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pain and temperature in the upper extremities "cape-like" distribution
from the spinothalamic tract |
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If syringomyelia progresses, what 2 things can happen?
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1) lower motor neuron lesion with weakness and atrophy
2) Horners syndrome- due to disruption of the lateral horn of the hypothalamospinal tract which carries sympathetics |
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What part of the spinal cord is damaged in poliomyelitis?
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anterior horn --> lower motor neuron signs (flaccid paralysis, atrophy, weakness, impaired reflexes, neg Babinski)
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This disease presents with inherited degeneration of anterior horn and presents as "floppy baby"
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Werdnig-Hoffman disease
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T/F
ALS presents with both UMN and LMN symptoms |
True!
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How do we differentiate syringomyelia from ALS since they are similar?
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ALS lacks a sensory deficit or pain/temp loss
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What genetic mutation can lead to ALS?
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zinc-copper superoxide dismutase mutation (SOD1) is present in some familial cases
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What is the cause of Fridreich Ataxia?
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auto recessive.
expansion of an unstable trinucleotide repeat (GAA) in the frataxin gene |
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Sx of Fridreich Ataxia?
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degeneration of cerebellum (ataxia)
degeneration of multiple spinal tracts (loss of vibration and propioception, muscle weakness in lower exts, loss of DTRs) |
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What cardiac condition is a/w Fridreich Ataxia?
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hypertrophic cardiomyopathy
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What are the 3 most common causes of meningitis in neonates?
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Group B strep
E coli listeria monocytogenes |
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What are the 3 most common causes of meningitis in children?
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Coxsackie virus
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Triad for meningitis?
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headache, nuchal rigidity, fever
can also have photophobia, vomiting, alt ment status |
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What meningeal layer does the lumbar puncture NOT pierce?
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does NOT pierce the pia
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Explain the important CSF findings in the different types of meningitis (bacterial, viral, fungal)
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Bacterial- Neutrophils, decreased CSF glucose (they eat the glucose)
Viral- lymphocytes, normal CSF glucose (viruses dont eat glucose) Fungal- lymphocytes, decreased CSF glucose |
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Which of the 3 types of meningitis has the worst complications?
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Bacterial
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Thrombotic strokes cause _____ infarcts
Embolic strokes cause ______ infarcts |
pale
hemorrhagic |
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What regions of the brain does a lacunar stroke involve and via what vessels?
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deep regions of the brain via the lenticulostriate vessels
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What is the first finding in an ischemic stroke?
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Red neurons (just like in MI)
Red neurons are an eosinophilic change in the cytoplasm of the neurons |
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Rupture of Charcot-Bouchard microaneurysms of the lenticulostriate vessels typically cause a _________
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intracerebral hemorrhage
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What is the most common site of an intracerebral hemorrhage?
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Basal ganglia since it is fed by lenticulostriate vessels
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sudden headache described as "the worse headache of my life"
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Subarachnoid hemorrhage
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What causes 85% of all subarachnoid hemms?
Why? |
Ruptured berry aneurysm
They are saccular outpouchings that lack a media layer, increasing risk for rupture |
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Where are berry aneurysms most frequently located in the brain?
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anterior circle of willis at branch points of the anterior communicating artery
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This cranial bleed appears lens shaped on CT and it is due to bleeding from ________
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Epidural
Middle meningeal artery |
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In a subdural hematoma, you see a ______shaped lesion on CT and it is caused by bleeding from ______
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crescent shaped
bridging veins |
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What are leukodystrophies?
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inherited mutations in enzymes necessary for production and maintenance of myelin
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What are the 3 types of leukodystrophies?
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1) Metachromatic
2) Krabbe disease 3) Adrenoleukodystrophy |
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This leukodystrophy is due to a deficiency of arylsulfatase and is the most common leukodyst.
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1) Metachromatic
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This leukodystrophy is due to a deficiency of galactocerebroside Beta galactosidase
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2) Krabbe disease
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This leukodystrophy is due to impaired addition of coenzyme A to long chain fatty acids
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3) Adrenoleukodystrophy
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This disease shows increased immunoglobulins with oligoclonal IgG bands in the CSF
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Multiple Sclerosis
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Multiple sclerosis is a/w this HLA type....
Bonus: What two other diseases have the same HLA type? |
HLA-DR2
IDDM and narcolepsy are also DR2 |
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Central Pontine Myelinolysis occurs due to rapid IV correction of this electrolyte imbalance:
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hyponatremia
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Locked in syndrome is more officially known as
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central pontine myelinolysis- can only move eyes! Freaky...
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Degeneration of cortex leads to ______ while degeneration of basal ganglia leads to _________
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dementia; movement disorders
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In Alzheimer Dz, the E4 allele of APOE is a/w ______ed risk while the E2 allele is a/w ______ed risk
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4>2, so E4 has increased risk over E2
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What is deposited in the brain in Alz dz?
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Neuritic plaques of A-Beta Amyloid with entangled neuritic processes
(Amyloid Precursor Protein (APP) is cleaved into A-Beta Amyloid) |
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We see early onset Alz Dz in this congenital disease....
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Trisomy 21- remember that bc APP is coded on Chrom 21.
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Neurofibrillary tangles are seen in _____
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Alz dz
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Where does Pick disease hit in the brain?
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It "Picks" the frontal and temporal lobes
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What is the Pathophys of Parkinsons?
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Loss of dopaminergic neurons in the substantia nigra
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"TRAP" stands for:
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Tremor
Rigidity Akinesia Postural instability |
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alpha-synuclein are a major component of ______
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Lewy bodies
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What does early onset dementia suggest?
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Lewy body demetia, not parkinsons even if there are parkinsonian features
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Pathophys of Huntingtons?
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degeneration of GABAergic neurons in Caudate Nucleus
Autopsy shows obliterated Caud. Nuc AD disorder with expanded CAG trinucleotide repeats |
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Fun fact: When does anticipation in Huntingtons take place?
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During spermatogenesis of course!
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What are the 3 W's of normal pressure hydrocephalus?
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Wet- urinary sx
Wobbly- gait instability Wacky- dementia |
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This disease occurs when pt is exposed to prion-infected human tissue or mad cow and develops rapidly progressing dementia, ataxia, myoclonus
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Creutzfeldt-Jakob disease
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Where in the brain do metastatic tumors usually present?
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multiple, well circumscribed lesions at the gray-white junction
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Most common primary malig CNS tumor in adults?
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glioblastoma multiforme (GBM)
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This CNS tumor has whorled pattern and psammoma bodies on histo slide
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Meningioma
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This CNS tumor is S-100 positive
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Schwannoma
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most common CNS tumor in children (benign)
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Pilocytic astrocytoma
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This CNS tumor ____ presents as a supratentorial mass in a child or young adult and may compress optic nerve causing _______
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Craniopharyngioma; bitemporal hemianopsia
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What does bitemporal hemianopsia look like?
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the outer half of the visual field bilaterally is gone
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