Neurological Pathology 2

Front 1

Patients with myelomeningoceles above what level are usually wheelchair-dependent?

Back 1

L2

Front 2

Lumbar meningomyeloceles are almost always accompanied by what malformation?

Back 2

Chiari II (Arnold-Chiari)
(causes hydrocephalus and sometimes brainstem dysfunction)

Front 3

Chiari I malformation is characterized by what?

Back 3

Chronic cerebellar tonsillar herniation
(associated with syrinx)

Front 4

What is craniorachisisis totalis?

Back 4

Total failure of neural tube closure

Front 5

Failure of anterior neural tube to close results in what malformation?

Back 5

Anencephaly

Front 6

Restricted failure of the posterior neural tube to close results in what malformation?

Back 6

Myelomeningocele

Front 7

Are meningoceles usually associated with neurological defects?

Back 7

No

Front 8

What is the constellation of abnormalities in Chiari II malformation? (5)

Back 8

1. Downward extension of the cerebellar vervis through the foramen magnum (main malformation)
2. Small and enlongated 4th ventricle
3. "Beaked" collicular plate on the midbrain
4. "Kinked" medulla
5. Small posterior fossa

Front 9

Is Chiari I malformation related to a neural tube defect?

Back 9

No

Front 10

Occult spinal dysraphism results from failure of what to develop correctly?

Back 10

Caudal cell mass

Front 11

Diseases due to maldevelopment of the prosencephalic structures include what two entities?

Back 11

1. Holoprosencephaly
2. Callosal agenesis

Front 12

Is holoprosencephaly more or less common than the neural tube defects?

Back 12

Much less

Front 13

Holoprosencephaly is often associated with what other defects?

Back 13

Midline facial defects

Front 14

What is schizencephaly?

Back 14

Complete agenesis of a localized portion of the wall of the cerebral hemisphere resulting in a cleft in the wall of the hemisphere

Front 15

Pachygyria and agyria are marked by what clinically?

Back 15

Severe mental retardation and seizure activity

Front 16

What are pachygyria and agyria (lissencephaly)?

Back 16

Smooth brain with no cerebral gyri (lissencephaly) or only a few, broad flat gyri (pachygyria)

Front 17

Neuronal heterotopia results from what?

Back 17

Groups of neurons (gray matter) that did not complete migration from the periventricular germinal matrix to the cerebral cortex

Front 18

What is polymicrogyria?

Back 18

An area of cerebral cortex with an increased number of very small gyri with very small sulci

Front 19

What is the most vulnerable area to ischemia in the brain of a perinatal fetus/infant?

Back 19

Periventricular

Front 20

Where are brain hemorrhages localized in the perinatal brain?

Back 20

Subendymal germinal matrix

Front 21

What are the common causes of congenital encephalitis?

Back 21

TORCH

Toxoplasmosis (congenital)
Other congenital infections (syphilis, HIV)
Rubella (congenital)
CMV (congenital)
HSV (neonatal)

Front 22

What is porencephaly?

Back 22

Localized cavity in the cerebral hemisphere that extends from the surface of the brain to the ventricle

Front 23

What is hydranencephaly?

Back 23

Massive hemispheric necrosis, presumably due to severe ischemia in utero, at approximately week 22–24 of brain development

Front 24

Question about periventricular leukomalacia

Back 24

37-38

Front 25

What is the major componenet of white matter in the CNS?

Back 25

Myelin

Front 26

What is the definition of a demyelinating disease?

Back 26

Loss of myelin with relative preservation of the axons

Front 27

What are three immune-mediated or inflammatory demyelinating diseases?

Back 27

1. Multiple sclerosis (MS)
2. Acute disseminated encephalomyelitis
3. Acute hemorrhagic leukocephalitis

Front 28

What are two viral demyelinating diseases?

Back 28

1. Progressive multifocal leukoencephalopathy (PML)
2. AIDS

Front 29

What are two toxic demyelinating diseases?

Back 29

1. Osmotic myelinolysis
2. Solvent vapor abuse leukoencephalopathy

Front 30

What is the cause of porencephaly?

Back 30

Remote, cavitary infarct that occured in utero around week 22-24 of brain development

Front 31

What areas of the brain are involved in hydranencephaly?

Back 31

Those within the territories of the left and right internal carotid arteries

Front 32

Which areas of the cortex are most vulnerable to ischemia in the preterm and term infant?

Back 32

Preterm = white matter
Term = gray matter

Front 33

Is periventricular leukomalacia more common in term or preterm infants?

Back 33

Preterm (24-32 weeks gestation)

Front 34

Clinically, periventricular leukomalacia may be associated with what?

Back 34

Spastic cerebral palsy

Front 35

Why is subependymal germinal matrix hemorrhage usually seen in the premature and not full term infant?

Back 35

The SGM involutes during the third trimester

Front 36

How soon after birth does hemorrhage usually develop in an infant with subependymal germinal matrix hemorrhage?

Back 36

Withing first three days

Front 37

Where does IVH in the term infant usually arise?

Back 37

From the glomus of the chroid plexus