DSA 24 CNS Pathology Flashcards
elongated eosinophilia within astrocytic process in response to injury
rosenthal fibers
contain alphabeta-crystallin and hsp27
rosenthal fibers
lamellated polyglucosan bodies
corpora amylacea
increases with advancing age and represent degenerative change; injury response
corpora amylacea
enlarged nucleus with intranuclear glycogen and pale chromatin; response to injury
alzehimer type II astrocyte
occur in the setting of hyperammonemia
Alzheimer type II astrocyte
central chromatolysis
axonal reaction/regeneration/degeneration
compresses the ACA
subfalcine herniation
subfalcine herniation
compresses ACA; results in contralateral paralysis of lower limb and loss of sensation
presses on PCA
transtentorial (uncinate, mesial tmeporal herniation)
transtentorial herniation
CN III compromised, contralateral hemianopsia and macular sparing
Duret hemorrhage
due to substantial herniation of the transtentorial causing downward displacement of the brainstem
compresses the medulla and compromises cardiac/respiratory centers
tonsillar herniation
hydrocephalus due to extensive tissue loss; compensatory expansion of the entire CSF compartment
ex vacuo hydrocephalus
malformation of the anterior neural tube
anencephaly
no forebrain and an open calvarium
anencephaly
increased AFP, polyhydramnios
anencephaly
associated with maternal type II diabetes; maternal folate supplementation decreases risk
anencephaly
malformed CNS diverticulum extending through a defect in the cranium
encephalocele
What are the AFP levels in encephalocele?
normal
complete absence of gyri
Lissencephaly
abnormal clusters of neurons in inappropriate locations along normal migratory routes
neuronal heterotopias
commonly associated with epilepsy
neuronal heterotopias
what are the mutations commonly associated with neuronal heterotopias?
cytoskeletal (filamin A) or microtubule associated proteins can be causal
What are the mutations associated with Holoprosencephaly?
Trisomy 13 and sonic hedgehog gene mutation
failure of the left and right hemispheres to separate resulting in midline facial abnormalities
holoprosencephaly
associated with low-lying cerebellar tonsils extending into the vertebral canal
Chiari I
significant herniation of the cerebellar tonsils and vermis through foramen magnum with aqueductal stenosis and hydrocephalus
chiari II
associated with lumbosacral myelomeningocele
chiari II
agenesis of cerebellar vermis with cystic enlargement of 4th ventricle
dandy-walker malformation
syrinx
fluid-filled neuroglial cavity within the spinal cord; syringomyelia
spinal cord ependymal-lined canal expansion in the cervical cord
hydromyelia
intraparenchymal hemorrhage in infants
occurs within the germinal matrix between the thalamus and caudate nucleus and extends into the ventricles
infarcts in infants that occur within the periventricular white matter
periventricular leukomalacia
ischemic infarcts within the hemispheres
multicystic encephalopathy
cortical scarring in the deep regions of the sulcus that leads to distortion of gyri
ulegyria
ischemic neuronal loss and gliosis in the basal ganglia and thalamus associated with aberrant and irregular myelin; occurs in infants
status marmoratus
coupe and contrecoup
gyral crests are most susceptible to contusion at the site of impact (coupe) or the point in the cranium opposite site of impact (contrecoup)
plaque jaune
brain hemorrhage and edema as a result of a contusion and eventually resolves as a depressed, yellow-brown glial scar extending to the pial surface
Where do intraparenchymal hemorrhages in adults usually occur?
basal ganglia and internal capusle
Charcot-Bouchard aneurysm
occurs in the small vessels of the basal ganglia; lenticulostriate vessels ; causes intraparenchymal hemorrhage
Damage to what blood vessel is common with an ischemic stroke due to thrombosis?
MCA
What does damage to MCA cause?
motor cortex (upper limb and face), sensory cortex (upper limb and fact), temporal lobe (Wernicke area) and frontal lobe (Broca area)
Most common site of intracerebral hemorrhage?
basal ganglia
when maintained on mechanical ventilation on someone who is brain dead it results in the brain autolyzing
respiratory brain
Notch3 receptor mutation
CADASIL
low flow without arteriovenous shunting
cavernous hemangiomas
tangles of abnormally tortuous and misshapen vessels, shunting arterial blood directly into the venous circulation
arteriovenous malformation
associated with Foix_Alajouanine disease
venous angiomas; venous antiomatous malformation typically seen in the lumbosacral region
less than 15 mm cystic infarcts
lacunar infarct
accompanied by lipid_laden macrophages and surrounding gliosis
lacunar infarct
small vessel rupture that eventually resorbs and leaves residual hemosiderin_laden macrophages and associated gliosis
slit hemorrhage
psuedobulbar signs
multi_infarct dementia
when the pattern of recurrent ischemic injury preferentially involves subcortical white matter with myelin and axonal loss
binswanger disease
what are the two conditions that are a result of chronic hypertensive injury?
multi-infarct dementia, Binswanger disease
what is anencephaly? clinical finding?
malformation of anterior neural tube → no forebrain. increased levels of AFP
what is holoprosencephaly? what mutation is it related to?
failure of left and right hemispheres to separate. mutations in SHH signaling pathway
clinical presentation of holoprosencephaly?
moderate form has cleft lip/palate. severe form results in cyclopia.
what is Dandy-Walker associated with?
hydrocephalus and spina bifida
what is the difference between syringomyelia and hydromyelia?
syringomyelia is a cystic cavity within spinal cord. hydromyelia involves the central canal.
what is the clinical presentation of syringomyelia?
cape-like bilateral loss of pain and temperature sensation in the upper extremities. fine touch sensation is preserved.
what is syringomyelia associated with?
Chiari malformation, trauma, and tumors
