Pathology - Cns Malformation And Vascular

Front 1

Two theories of dysraphic disorders?

Back 1

failure of neural tube closure due to primary failure of neuroectoderm or mesoderm OR repoening or rupture of previously closed tube

Front 2

What kinds of prescribed drugs can cause neural tube defects?

Back 2

Valproic acid

Front 3

Two genetic disorders associated with neural tube defects?

Back 3

Patau's and Edwards

Front 4

Often asymptomatic; may be associated with foot abnormalities and gait. What is the lesion?

Back 4

Spina bifida occulta- non-closed vertebral arches with overlying skin, most often lumbosacral and lest severe NT defect

Front 5

What is a meningocele?

Back 5

dura and leptomeninges hernate through vertebral defect but skin is still covering it.

Front 6

Myelomeningocele?

Back 6

meninges and spinal cord herniate through defect. If no skin covering, is myeloschisis which is what usually occurs

Front 7

SC defect with no elecated AFP?

Back 7

spina bifida occulta

Front 8

Anencephaly more common in ?

Back 8

female fetuses

Front 9

What is area cerebrovasculosa?

Back 9

the mass of malformed neural tissue present in the cranial vault seen leftover in anencephaly

Front 10

absent calvaria, baby has rachishisis. What else does the baby have and what is noted during pregnancy?

Back 10

anenchephaly: elevated AFP and polyhydramnios

Front 11

What is encephalocele?

Back 11

When a mass of cerebral tissue protrudes through a cranial defect, usaully occipital, confined to one hemisphere, and covered

Front 12

What is a nasal glioma?

Back 12

When encephalocele is misnamed because of its projection into ethmoid sinus, orbit, or nasal cavity.

Front 13

Disorders of migration of forebrain include?

Back 13

sulcation, cleavage, or genesis

Front 14

What is sulcation induced by?

Back 14

presence of normally migrated neuronal population

Front 15

Smooth brain with many small bumps. Etiology?

Back 15

this is polymicrogyria, caused by intrauterine ischemia/infection which interferes with normal gyral development.

Front 16

Miller Dieker syndrome?

Back 16

Triad: seizures, mental retardation, lissencephaly (Lisa is a miller dieker)

LIS1 gene on chromosome 17 involvement

Front 17

Arrest of neuroblast migration at end of 4th fetal month causes?

Back 17

lissencephaly or pachygyria

Front 18

nodular gray matter heterotopias in ventricular wall, what genetic defect?

Back 18

LIS1 on Ch 17, this is found in agyria

Front 19

mnemonic for agyria?

Back 19

lisa was Ch17 when she discovered miller-DIECKEr

Front 20

Cyclopia and hypotelorism, what malformation is suspected?

Back 20

Arhinencephaly

Front 21

Disturbance that results in arhinencephaly?

Back 21

formation of cranial end of neural tube with lack of formation of olfactory buds and cleavage during 4-6 week!

Front 22

alobar holoprosencephay?

Back 22

no hemispheric fissure

Front 23

lobar holoprosencephaly?

Back 23

hemispheres joined at frontal pole

Front 24

semilobar holoprosencephaly?

Back 24

shallow interhemispheric fissure

Front 25

Sx of corpus callosum agenesis?

Back 25

no symptoms except perceptual and language tests

Front 26

X-linked, lethal in males, bat-wing lateral ventricles?

Back 26

Aicardi syndrome: agenesiss of crpus callosum, chorioretinal defects, and seizures

Front 27

Most common malformation of posterior fossa?

Back 27

Arnold Chiari Type 1, usually asymptomatic chronic tonsillar herniation

Front 28

Z-shaped sagittal brainstem/spinal cord section. What clinical disorder exists?

Back 28

true arnold chiari type 2, with lumbar myelominingocele, syringomyelia, and hydrocephalus

Front 29

Dandy-walker malformation?

Back 29

DWalker is an ACE:
Agenesis of vermis,
Cystic dilation of fourth ventricle
Enlargement of posterior fossa,
hydrocephalus also frequently present.

Front 30

Static motor defecit due to prenatal and perinatal insults?

Back 30

Cerebral palsy, due to ischemia/hemorrhage; classified according to motor impairment

Front 31

Chalky yellow plaques around lateral ventricles? due to ischemia?

Back 31

periventricular leukomalacia,WHITE MATTER hypoxia/ischemia

Front 32

What is multicystic encephalopathy?

Back 32

due to 3rd trimester ischemic insult, it is where white matter and deep cortical layers are replaced by sponge-like glial lined cysts

Front 33

Perinatal cerebral hemorrhage, originates where?

Back 33

this would be a subependymal germinal matrix hemorrhage that originates between caudate and thalamus where the grminal layer is

Front 34

Why is germinal matrix most likely to bleed?

Back 34

it has a rich capillary bed not supported well by capillary tissue.

Front 35

Grading of sybependymal hemorrhaged based upon?

Back 35

confinement of bleed and degree of ventricular dilatation

Front 36

Factors affecting degree of damage caused by ischemia?

Back 36

collateral cirulcation, duration , and severity and rapidity of reduced flow

Front 37

Most vulnerable region in transient global ischemia?

Back 37

HIPPOcampus, Cerebral neocortex (lamina 3,4,5), and verebellum (purkinje cells)

HCC

Front 38

Most vulnerable cells during transient ischemia?

Back 38

Neurons, then their helpers, oligodendrocytes, then astrocytes

Front 39

Acute phase of permanent global ischemia results in?

Back 39

coma

Front 40

Diffuse cortical injury indicated by?

Back 40

FLAT EEG

Front 41

brainstem damage indicated by?

Back 41

absent reflexes, respiratory drive, and cerebrla perfusion

Front 42

What does respirator brain look like?

Back 42

swollen with flattened gyri often herniated with slitlike ventricles

Front 43

Factors affecting degree of damage caused by ischemia?

Back 43

collateral cirulcation, duration , and severity and rapidity of reduced flow

Front 44

Most common site of thrombosis?

Back 44

carotid bifurcation, MCA origin, ends of basilar artery

MCA Basilar, Carotid

Front 45

Most common vessel affectd by emboli?

Back 45

MCA, which is bad because it is not part of the circle of willis

Front 46

Most vulnerable region in transient global ischemia?

Back 46

HIPPOcampus, Cerebral neocortex (lamina 3,4,5), and verebellum (purkinje cells)

HCC

Front 47

hemorrhagic infarct most likely due to?

Back 47

embolism because fibrinolytic events after the occlusion = hemorrhage

Front 48

Most vulnerable cells during transient ischemia?

Back 48

Neurons, then their helpers, oligodendrocytes, then astrocytes

Front 49

Factors affecting degree of damage caused by ischemia?

Back 49

collateral cirulcation, duration , and severity and rapidity of reduced flow

Front 50

Acute phase of permanent global ischemia results in?

Back 50

coma

Front 51

Most vulnerable cells during transient ischemia?

Back 51

Neurons, then their helpers, oligodendrocytes, then astrocytes

Front 52

Acute phase of permanent global ischemia results in?

Back 52

coma

Front 53

What does respirator brain look like?

Back 53

swollen with flattened gyri often herniated with slitlike ventricles

Front 54

Diffuse cortical injury indicated by?

Back 54

FLAT EEG

Front 55

brainstem damage indicated by?

Back 55

absent reflexes, respiratory drive, and cerebrla perfusion

Front 56

What are two microvasculopathy's associated with demtnia?

Back 56

Binswanger's disease and CADASIL

Front 57

What does respirator brain look like?

Back 57

swollen with flattened gyri often herniated with slitlike ventricles

Front 58

What is CADASIL?

Back 58

most common hereditary stroke disorder: mutation of NOTCH 3 on chromosome 19.

Front 59

Most common site of thrombosis?

Back 59

carotid bifurcation, MCA origin, ends of basilar artery

MCA Basilar, Carotid

Front 60

Most common vessel affectd by emboli?

Back 60

MCA, which is bad because it is not part of the circle of willis

Front 61

What causes binswanger's?

Back 61

subcortical infarcts caused by hypertension and resulting dementia due to white matter distruction.

Front 62

hemorrhagic infarct most likely due to?

Back 62

embolism because fibrinolytic events after the occlusion = hemorrhage

Front 63

PAthogenesis of notch 3 mutation?

Back 63

causes intracytoplasmic accumulation in smooth muscle cells in blood vessels which cause infarcts and dementia in CADASIL

Front 64

Incomplete infarct causes what cells to die?

Back 64

neurons, they are first before oligos and astros

Front 65

most common area of cerebral venous thrombosis?

Back 65

superior sagittal and lateral sinuses

Front 66

most common cause of primary intraparenchymal hemorrhage?

Back 66

hypertension

Front 67

aneurysms associated with HTN?

Back 67

charcot bouchard (they are microaneurysms, not saccular like berry)

Front 68

Why does htn cause hemorrhage?

Back 68

hyaline arteriolosclerosis weakens vessels and cause aneuryms that can go BOOM

Front 69

Most common vascular malformation?

Back 69

AV malformation, more common in men 10-30 yrs

Front 70

most common AV malformation where?

Back 70

MCA (seems like all path goes here)

Front 71

Difference between AV malformation an cavernous angioma?

Back 71

AV has intervening brain tissue wherase canvernous has no tissue and usually from prior hemorrhage

Front 72

What can cause a lobarhemorrhage?

Back 72

amyloid angiopathies that weaken small/medium arterioles

Front 73

Subarachnoid hemorrhages due to?

Back 73

rupture of congenital berry aneurysms (80%)

Front 74

Most berry aneurysms located where?

Back 74

between ACA and anterior communication branch of circle of willis

Front 75

Where are fusiform aneurysms usually found and alongside what disease process?

Back 75

Basilar artery, usually HTN artherosclerosis

Front 76

lacurnar infarcts due to?

Back 76

hyaline arteriosclerosis due to HTN and diabetes.

Front 77

RElationship between lacunar infarcts and Binswanger?

Back 77

If enough white cortical matter is damaged by lacunar infarcts, one can see dementia! It is the dementia due to these infarcts that is termed Binswanger

Front 78

Optic neuritis in a young female. What is elevated in CSF?

Back 78

MS, she has protein with IgG in CSF

Front 79

Multifocal, hyperintsne plaques seen in MRI with bladder dysfunction and CN signs. What HLA type and hypersensitivity?

Back 79

HLA-DR2, Type 4 which is cellular mediated

Front 80

PAthogenesis of MS?

Back 80

cd4_ t cells react against myelin and secrete cytokines that activate macrophages to nom nom nom

Front 81

An acute version of MS?

Back 81

monophasic demyelinating disease activated by viral illness or immunization

Front 82

Demylination common in alcoholics that undergo too rapid correction of hyponatremia?

Back 82

Central Pontine Myelinosis

Front 83

Cause of PML?

Back 83

JC virus infection of oligodendrocytes, casuing rapdily progressing demyelination in HIV pts.

Front 84

Inclusions in PML

Back 84

intraNUCLEAR in oligodendrocytes.

Front 85

Central pontine myelinolysis area affected?

Back 85

upper 2/3 of pons

Front 86

What is central pontine myelinolysis?

Back 86

demylination of pons nerve cells due to too rapid correction of profound hyponatremia

Front 87

What are the leukodystrophies?

Back 87

inherited defects of metabolism that affect myelin synthesis or turnover. NO neuronal storage defects. white matter only.