DSA 24 CNS Pathology

Question 1

elongated eosinophilia within astrocytic process in response to injury

Answer 1

rosenthal fibers

Question 2

contain alphabeta-crystallin and hsp27

Answer 2

rosenthal fibers

Question 3

lamellated polyglucosan bodies

Answer 3

corpora amylacea

Question 4

increases with advancing age and represent degenerative change; injury response

Answer 4

corpora amylacea

Question 5

enlarged nucleus with intranuclear glycogen and pale chromatin; response to injury

Answer 5

alzehimer type II astrocyte

Question 6

occur in the setting of hyperammonemia

Answer 6

Alzheimer type II astrocyte

Question 7

central chromatolysis

Answer 7

axonal reaction/regeneration/degeneration

Question 8

compresses the ACA

Answer 8

subfalcine herniation

Question 9

subfalcine herniation

Answer 9

compresses ACA; results in contralateral paralysis of lower limb and loss of sensation

Question 10

presses on PCA

Answer 10

transtentorial (uncinate, mesial tmeporal herniation)

Question 11

transtentorial herniation

Answer 11

CN III compromised, contralateral hemianopsia and macular sparing

Question 12

Duret hemorrhage

Answer 12

due to substantial herniation of the transtentorial causing downward displacement of the brainstem

Question 13

compresses the medulla and compromises cardiac/respiratory centers

Answer 13

tonsillar herniation

Question 14

hydrocephalus due to extensive tissue loss; compensatory expansion of the entire CSF compartment

Answer 14

ex vacuo hydrocephalus

Question 15

malformation of the anterior neural tube

Answer 15

anencephaly

Question 16

no forebrain and an open calvarium

Answer 16

anencephaly

Question 17

increased AFP, polyhydramnios

Answer 17

anencephaly

Question 18

associated with maternal type II diabetes; maternal folate supplementation decreases risk

Answer 18

anencephaly

Question 19

malformed CNS diverticulum extending through a defect in the cranium

Answer 19

encephalocele

Question 20

What are the AFP levels in encephalocele?

Answer 20

normal

Question 21

complete absence of gyri

Answer 21

Lissencephaly

Question 22

abnormal clusters of neurons in inappropriate locations along normal migratory routes

Answer 22

neuronal heterotopias

Question 23

commonly associated with epilepsy

Answer 23

neuronal heterotopias

Question 24

what are the mutations commonly associated with neuronal heterotopias?

Answer 24

cytoskeletal (filamin A) or microtubule associated proteins can be causal

Question 25

What are the mutations associated with Holoprosencephaly?

Answer 25

Trisomy 13 and sonic hedgehog gene mutation

Question 26

failure of the left and right hemispheres to separate resulting in midline facial abnormalities

Answer 26

holoprosencephaly

Question 27

associated with low-lying cerebellar tonsils extending into the vertebral canal

Answer 27

Chiari I

Question 28

significant herniation of the cerebellar tonsils and vermis through foramen magnum with aqueductal stenosis and hydrocephalus

Answer 28

chiari II

Question 29

associated with lumbosacral myelomeningocele

Answer 29

chiari II

Question 30

agenesis of cerebellar vermis with cystic enlargement of 4th ventricle

Answer 30

dandy-walker malformation

Question 31

syrinx

Answer 31

fluid-filled neuroglial cavity within the spinal cord; syringomyelia

Question 32

spinal cord ependymal-lined canal expansion in the cervical cord

Answer 32

hydromyelia

Question 33

intraparenchymal hemorrhage in infants

Answer 33

occurs within the germinal matrix between the thalamus and caudate nucleus and extends into the ventricles

Question 34

infarcts in infants that occur within the periventricular white matter

Answer 34

periventricular leukomalacia

Question 35

ischemic infarcts within the hemispheres

Answer 35

multicystic encephalopathy

Question 36

cortical scarring in the deep regions of the sulcus that leads to distortion of gyri

Answer 36

ulegyria

Question 37

ischemic neuronal loss and gliosis in the basal ganglia and thalamus associated with aberrant and irregular myelin; occurs in infants

Answer 37

status marmoratus

Question 38

coupe and contrecoup

Answer 38

gyral crests are most susceptible to contusion at the site of impact (coupe) or the point in the cranium opposite site of impact (contrecoup)

Question 39

plaque jaune

Answer 39

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

Question 40

Where do intraparenchymal hemorrhages in adults usually occur?

Answer 40

basal ganglia and internal capusle

Question 41

Charcot-Bouchard aneurysm

Answer 41

occurs in the small vessels of the basal ganglia; lenticulostriate vessels ; causes intraparenchymal hemorrhage

Question 42

Damage to what blood vessel is common with an ischemic stroke due to thrombosis?

Answer 42

MCA

Question 43

What does damage to MCA cause?

Answer 43

motor cortex (upper limb and face), sensory cortex (upper limb and fact), temporal lobe (Wernicke area) and frontal lobe (Broca area)

Question 44

Most common site of intracerebral hemorrhage?

Answer 44

basal ganglia

Question 45

when maintained on mechanical ventilation on someone who is brain dead it results in the brain autolyzing

Answer 45

respiratory brain

Question 46

Notch3 receptor mutation

Answer 46

CADASIL

Question 47

low flow without arteriovenous shunting

Answer 47

cavernous hemangiomas

Question 48

tangles of abnormally tortuous and misshapen vessels, shunting arterial blood directly into the venous circulation

Answer 48

arteriovenous malformation

Question 49

associated with Foix\_Alajouanine disease

Answer 49

venous angiomas; venous antiomatous malformation typically seen in the lumbosacral region

Question 50

less than 15 mm cystic infarcts

Answer 50

lacunar infarct

Question 51

accompanied by lipid\_laden macrophages and surrounding gliosis

Answer 51

lacunar infarct

Question 52

small vessel rupture that eventually resorbs and leaves residual hemosiderin\_laden macrophages and associated gliosis

Answer 52

slit hemorrhage

Question 53

psuedobulbar signs

Answer 53

multi\_infarct dementia

Question 54

when the pattern of recurrent ischemic injury preferentially involves subcortical white matter with myelin and axonal loss

Answer 54

binswanger disease

Question 55

what are the two conditions that are a result of chronic hypertensive injury?

Answer 55

multi-infarct dementia, Binswanger disease

Question 56

what is anencephaly? clinical finding?

Answer 56

malformation of anterior neural tube → no forebrain. increased levels of AFP

Question 57

what is holoprosencephaly? what mutation is it related to?

Answer 57

failure of left and right hemispheres to separate. mutations in SHH signaling pathway

Question 58

clinical presentation of holoprosencephaly?

Answer 58

moderate form has cleft lip/palate. severe form results in cyclopia.

Question 59

what is Dandy-Walker associated with?

Answer 59

hydrocephalus and spina bifida

Question 60

what is the difference between syringomyelia and hydromyelia?

Answer 60

syringomyelia is a cystic cavity within spinal cord. hydromyelia involves the central canal.

Question 61

what is the clinical presentation of syringomyelia?

Answer 61

cape-like bilateral loss of pain and temperature sensation in the upper extremities. fine touch sensation is preserved.

Question 62

what is syringomyelia associated with?

Answer 62

Chiari malformation, trauma, and tumors