Chapter 17 - CNS Flashcards
What is associated with Neural Tube Defects?
Low folate levels PRIOR to conception.
Symptoms of anencephaly?
Polyhydramnios…
Cause of Dandy-Walker malformation and presentation?
Due to congenital failure of the cerebellar vermis. Results in massively dilated 4th ventricle, with absent cerebellum…
What other defects are associated with Arnold-Chiari Malformation Type II?
Meningomyelocele and syringomyelia
What is syringomelia and where does it most often occur?
Cystic degeneration of the spinal cord. Most often occurs at C8-T1, spares dorsal columns but compresses spinothalamic tract in the anterior white commissure.
“Floppy Baby” Syndrome?
Werdnig-Hoffman Disease (inherited degeration of the anterior motor horn.)
ALS involves the degeneration of what structures?
UMN AND LMN…
Cause of familial ALS?
Superoxide dismutase (SOD1) mutation…
Cause of Friedreich Ataxia?
Expansion of trinucleotide repeat (GAA) in frataxin gene.
Cardiac association with Friedreich Ataxia?
Hypertrophic Cardiomyopathy
Most common cause of bacterial meningitis in newborn?
Group B strep, E coli, and Listeria
Most common cause of bacterial meningitis in children/teenagers?
N meningitidis
Most common cause of bacterial meningitis in adults and elderly?
Strep pneumonia
Most common cause of bacterial meningitis in non vaccinated kids?
H influenza
CNS defect seen with DM, or insulinomas…?
Global Cerebral Ischemia, due to repeated episodes of hypoglycemia.
How does moderate global cerebral ischemia present hystologically?
Cortical Laminar Necrosis (triangular appearance) from damage to the pyramidal nuerons of layers 3, 5, and 6.
Red neurons are seen how long after an ischemic stroke?
12 hours post infarction.
Coagulative necrosis is seen how long after ischemic stroke? And is followed by what?
24 hours, followed by neutrophils (1-3 days), nicroglial cells (4-7 days), and granulation tissue (2-3 weeks)
What are high risk groups for berry aneurisms?
Marfan syndrome, ADPKD, and Ehlers-Danlos
Classic cause and symptoms of an epidural hematoma
Fracture of temporal bone leading to a rupture of middle meningeal artery. Presents with Lens-shaped lesion on CT. Lucid interval may precede signs.
Classic cause and symptoms of subdural hematoma
Due to tearing of bridging veins. Presents with a crescent-shaped lesion of CT and progressive neurologic signs.
What artery is often compressed with a subfalcine herniation?
The ACA!
Metachromatic Leukodystrophy
Due to deficiency in arylsulfatase (AR), which is necessary for the degradation of myelin. Accumulates in the lysosomes of oligodedrocytes…
Krabbe disease
Due to deficiency of galactocerebroside b-galactosidase (AR). Galactocerebroside accumulates in macrophages. Also necessary for production/maintenance of myelin.