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85 Cards in this Set
- Front
- Back
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Spina bifida
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failure of posterior vertebral arches to close
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spina bifida occulta
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failure of post vert arch to close in 1-2 vertebrae, no clinical abnormalities
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spina bifida cystica
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failure of post vertebral arches to close, with herniation of meninges through defect
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meningocoele
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herniated membranes of meninges
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meningomyelocele
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portion of spinal cord included in herniated tissue
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hydrocephalus ex vacuo
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decreased cerebral mass --> increased CSF in brain
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different types of hydrocephalus
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internal hydrocephalus
external hydrocephalus communicating hydrocephalus noncommunicating hydrocephalus |
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internal hydrocephalus
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increased CSF confined w/i ventricles
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external hydrocephalus
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increased CSF in subarachnoid space
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communicating hydrocephalus
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CSF between ventricles and subarachnoid space
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noncommunicating hydrocephalus
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obstructed flow of csf from ventricles to subarachnoid space
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arnold chiari malformation
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downward displacement of cerebellar tonsils and medulla through foramen magnum
--> pressure atrophy of displaced brain tissue and hydrocephalus from CSF outflow obstruction |
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what is characteristically seen in arnold chiari malformation
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thoracolumbar meingomyelocele
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sx of agenesis of corpus callosum
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none, but associated with other abnormalities
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tuburous sclerosis syndrome
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nodular proliferation of multinucleated atypical astrocytes forming tubers (small white nodules scattered across cerebral cortex and periventricular areas)
adenoma sebaceum of skin and angiomyolipoma of kidney seizures and MR seen in infancy |
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which type fo cerebrovascular dz is most common
pathology |
infarction
liquefactive necrosis --> cyst formation |
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causes of cerebral infarction
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thrombosis*
embolism |
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where is the most common site of thrombotic obstruction
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carotid bifurcation and MCA
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where is hte most common site of embolic occlusion?
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MCA
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clinical presentation of arterial occlusion of MCA
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contralateral paralysis and motor/sensory defects, aphasia
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what is a lacunar stroke
clinical features |
when infarction is caused by arteriolar occlusion
lacunae form --> purely sensory or motor deficits |
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where would a stroke occur if there were purely motor deficits?
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internal capsule
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where would a stroke occur if there were purely sensory deficits?
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thalamus
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intracerebral hemorrhage
cause? |
bleeding into brain
HTN minute dilations at small artery bifurcations |
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Charcot Bouchard aneurysms
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intracerebral hemorrhage at site of small artery bifurcation
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what parts of the brain does intracerebral hemorrhage most often occur
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basal ganglia/thalamus
pons cerebellum frontal lobe white matter |
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what is associated with subarachnoid hemorrhage
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berry aneurysm in circle of Willis
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what is a TIA
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temproary disturbance of cerebral circulation
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what is a common cause of epidural hematoma?
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skulll fractures --> laceration of middle meningeal artery
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clinical features of epidural hematoma?
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short period of consciouness (lucid interval) --> rapidly developing signs of cerebral compression
amenable to emergency surgery |
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Waterhouse-Friderichsen syndrome
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hemorrhagic destruction of adrenal cortex, acute hypocorticism with circ collapse, and DIC
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where are the calcifications that result from toxo in neonates?
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periventricular
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how does toxo present in adults?
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lymphadenitis
CNS involvement may appear in immunosuppressed |
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CSF of viral meningitis
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increased lymphos
moderately increased protein normal glucose |
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CSF of bacterial meningitis
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increased polys
increased protein decreased glucose ** (differs from viral) |
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morphological changs seen in meningoencephalitis and encephalitis
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perivascular cuffing (mononuclear infiltrate within Virchow-Robin spaces)
inclusion bodies in neural/glial cells glial nodules (from prolif of microglia) |
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what are the arbovirus encephalitides
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St. Louis Encephalitis
Eastern equine Western equine |
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which is the most common cause of severe viral encephalitis
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Herpes simplex
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clinical presentation of a person infected with rabies
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increased CNS excitability
violent muscle contractions and convulsions after minimal stimuli fatal once clinical signs develop |
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how can the d/o from rabies be prevented
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give active immuniczation btwn the interval of the bite and onset of clinical manifestations
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histo of rabies
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neuronal degeneration
perivascular accumulation of mononuclear cells in brain stem and spinal cord Negri bodies in hippocampus and Purkinje cells of cbl |
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what are negri bodies
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eosinophilic intracytoplasmic inclusions
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clinical manifestations of CMV in adults
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encephalomyelitis
lesions in kidney, liver, lungs, salivary glands |
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CMV in infants
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MR
microcephaly chorioretinitis hepatosplenomegaly periventricular calcifications |
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histo of CMV
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giant cells with eosinophilic inclusions involving nucleus and cytoplasm
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morph of spongiform encephalopathy
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small cysts in CNS gray matter, aong with absence of inflammation
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morph features of kuru
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loss of neurons
gliosis spongiosis in cerebrum, bl, spinal cord cbl atrophy |
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clinical features of kuru
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tremor
ataxia slurred speech mental deficiency death w/i a few months |
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whch virus causes subacute sclerosing panencephalitis
clinical course |
altered measles virus
infx during infancy, but then asx --> neuro manifestations in late childhood or early teens usually fatal |
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micro of subacute sclerosing panencephalitis
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lack of M component of measles virsu (required for extracellular spread of viruS)
CSF contains oligoclonal Igs against viral proteins, but lack anti-M |
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Cause of progressive multifocal leukoencephalopathy
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papovavirus, infects oligodendrocytes --> demyelination
and leukemia/lymphoma |
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cliincal features of progressive multifocal leukoencephalopathy
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rapidly progressive multiple foci of demylelination in brain
associated with abnormal oligodendrocytes and astrocytes |
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what is progressive multifocal leukoencephalopathy usually associated with?
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leukemia or lymphoma
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what is seen in the CSF of pts with MS
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multiple oligoclonal bands, seen on electrophoresis
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what HLA haplotypes are associated with MS
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A3
B7 DR2 DW2 |
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morph of MS
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depletion of myelin producing oligodendrocytes, w multiple focal areas of demyelination (plaques) that are scattered in brain and spinal cord
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where are the plaques normally found in MS
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optic nerve
brain stem paraventricular areas |
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clinical features of MS
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weakness of lower extremities
visual disturbances retrobulbar pain sensory probs loss of bladder control Charcot triad |
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what is charcot triad?
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nystagmus
intention tremor scanning speech |
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another name for acute disseminated encephalomyelitis
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postinfectious encephalitis
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morphologic abnormalities associated with Alzheimers
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neurofibrillary tangles
neuritic plaques granulovacuolar degeneration amyloid angiopathy hirano bodies cerebral atrophy with neruonal loss choline acetyltransferase deficiency alterations in the nucelus basalis of meynert |
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what are neurofibrillary tangles
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intracytoplasmic bundles of filaments dervide from MT adn neurofilaments
they are found w/i the neurons of cerebral cortex |
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what is granulovacuolar degeneration
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intraneuronal cytoplasmic granule containing vacuoles w/i pyramidal cells of hippocampus
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amyloid angiopathy
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amyloid deposition in and abou tthe BV
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hirano bodies
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intracytoplasmic proximal dendritic eoisinophilic inclusions cosisting of actin
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where are neuritic (senile) plaques
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cortex
hippocampus amygdala |
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where are neurofibrillary tangles seen
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postencephaliti parkinson disease
alzheimer dz |
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etiology of alzheimers
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aggregates of A-beta-40 amyloidogenic peptide
APP gene on chromosome 21 --> amyloid precursor protein E4 allele of apoprotein E mutations mutations in genes for presenilins choline acetyltransferase deficiency |
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what is the 2nd most common cause of dementia
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multi-infarction dementia (caused by cerebral atherosclerosis)
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binswanger disease
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subcortical leukoencephalopathy
associated with HTN multiple lacunar infarcts and progressive demyelination |
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where does binswanger disease affect
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subcortical area
cortex is spared |
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morph of pick dz
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cortical atrophy in temporal and frontal lobes
swollen neurons Pcik bodies |
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Pick bodies
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round, intracytoplasmic inclusions consisting of neurofilaments
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what regions of the brain are affected by huntington
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striatum (caudate nuceus and putamen)
frontal cortex wiht neuronal depletion and gliosis |
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where is the huntingtin gene
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4p
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what is bdnf
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prosurvival factor for neurons in the striatum
may be related to huntington dz it is decreased in presence of huntingtin gene w increased CAG repeats |
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morph of parkinson disease
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depigmentation of substantia nigra and locus ceruleus
Lewy bodies |
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what are lewy bodies
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eosinophilic intracytoplasmic inclusions
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causes of parkinson
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von economo encephalitis
trauma drugs/toxins shy-drager syndrome |
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what is von economo encephalitis
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infectious do seen after flue pandemic --> postencephalic parkinsonism
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what drugs/toxins are associated with parkinsons
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DA antagonists (MPTP), a component street drugs
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what is shy drager syndrome
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parkinsonism with autonomic dysfxn and orthostatic hypotension
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clinical manifestations of ALS
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atrophy and fasciculation (LMN)
hyper-reflexia, spasticity, pathologic reflexes (UMN) death in 1-6 yrs |
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progressive bulbar palsy
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brain stem and cranial nerve probs
--> dysphagia, dysarthria respiratory failure --> death |
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werdig-hoffmann syndrome
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LMN dz of infancy
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