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Neurological Pathology 2


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Patients with myelomeningoceles above what level are usually wheelchair-dependent?
Lumbar meningomyeloceles are almost always accompanied by what malformation?
Chiari II (Arnold-Chiari)
(causes hydrocephalus and sometimes brainstem dysfunction)
Chiari I malformation is characterized by what?
Chronic cerebellar tonsillar herniation
(associated with syrinx)
What is craniorachisisis totalis?
Total failure of neural tube closure
Failure of anterior neural tube to close results in what malformation?
Restricted failure of the posterior neural tube to close results in what malformation?
Are meningoceles usually associated with neurological defects?
What is the constellation of abnormalities in Chiari II malformation? (5)
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
Is Chiari I malformation related to a neural tube defect?
Occult spinal dysraphism results from failure of what to develop correctly?
Caudal cell mass
Diseases due to maldevelopment of the prosencephalic structures include what two entities?
1. Holoprosencephaly
2. Callosal agenesis
Is holoprosencephaly more or less common than the neural tube defects?
Much less
Holoprosencephaly is often associated with what other defects?
Midline facial defects
What is schizencephaly?
Complete agenesis of a localized portion of the wall of the cerebral hemisphere resulting in a cleft in the wall of the hemisphere
Pachygyria and agyria are marked by what clinically?
Severe mental retardation and seizure activity
What are pachygyria and agyria (lissencephaly)?
Smooth brain with no cerebral gyri (lissencephaly) or only a few, broad flat gyri (pachygyria)
Neuronal heterotopia results from what?
Groups of neurons (gray matter) that did not complete migration from the periventricular germinal matrix to the cerebral cortex
What is polymicrogyria?
An area of cerebral cortex with an increased number of very small gyri with very small sulci
What is the most vulnerable area to ischemia in the brain of a perinatal fetus/infant?
Where are brain hemorrhages localized in the perinatal brain?
Subendymal germinal matrix
What are the common causes of congenital encephalitis?

Toxoplasmosis (congenital)
Other congenital infections (syphilis, HIV)
Rubella (congenital)
CMV (congenital)
HSV (neonatal)
What is porencephaly?
Localized cavity in the cerebral hemisphere that extends from the surface of the brain to the ventricle
What is hydranencephaly?
Massive hemispheric necrosis, presumably due to severe ischemia in utero, at approximately week 22–24 of brain development
Question about periventricular leukomalacia
What is the major componenet of white matter in the CNS?
What is the definition of a demyelinating disease?
Loss of myelin with relative preservation of the axons
What are three immune-mediated or inflammatory demyelinating diseases?
1. Multiple sclerosis (MS)
2. Acute disseminated encephalomyelitis
3. Acute hemorrhagic leukocephalitis
What are two viral demyelinating diseases?
1. Progressive multifocal leukoencephalopathy (PML)
What are two toxic demyelinating diseases?
1. Osmotic myelinolysis
2. Solvent vapor abuse leukoencephalopathy
What is the cause of porencephaly?
Remote, cavitary infarct that occured in utero around week 22-24 of brain development
What areas of the brain are involved in hydranencephaly?
Those within the territories of the left and right internal carotid arteries
Which areas of the cortex are most vulnerable to ischemia in the preterm and term infant?
Preterm = white matter
Term = gray matter
Is periventricular leukomalacia more common in term or preterm infants?
Preterm (24-32 weeks gestation)
Clinically, periventricular leukomalacia may be associated with what?
Spastic cerebral palsy
Why is subependymal germinal matrix hemorrhage usually seen in the premature and not full term infant?
The SGM involutes during the third trimester
How soon after birth does hemorrhage usually develop in an infant with subependymal germinal matrix hemorrhage?
Withing first three days
Where does IVH in the term infant usually arise?
From the glomus of the chroid plexus

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