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85 Cards in this Set

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