Lecture 49 - Neuropathology of Pediatric Disorders

Question 1

_______ are the most common Perinatal brain lesions, especially in the ______ Matrix (aka subventricular zone). ______ (pre-term or full-term?) are at highest risk, especially those born before week _________.

Answer 1

Hemorrhage

Germinal Matrix (aka Subventricular zone)

Pre-term

30

Question 2

If the child survives the hemorrhage, it is likely to develop post-hemorrhagic ________ due to ________ stenosis.

Answer 2

Hydrocephalus

Aqueductal stenosis

Question 3

White matter lesions also most often occur in pre-term babies, and they are thought to be due to poor _______ (ischemia). Look for yellow/white spots in the ________ white matter. In chronic cases, the presentation could be Cystic.

Answer 3

Perfusion

Periventricular white matter

Question 4

One of the associated findings in babies with Anencephaly is _____ _____ Hypoplasia due to absence of Hypothalamus.

Answer 4

Adrenal Cortical Hypoplasia

Question 5

Holoprosencephaly is usually diagnosed with ultrasound revealing a single _______.

Answer 5

Ventricle

Question 6

______ ______ Dysplasia is a focal, abnormal arrangement of neurons in the cerebral cortex. It’s most commonly associated with seizures in the _____ lobe.

Answer 6

Focal Cortical Dysplasia

Temporal lobe (Temporal Lobe Epilepsy - TLE)

Question 7

The two most common causes of Bacterial Meningitis are _______ and Group ____ _______.

Answer 7

E. coli

Group B Strep

Question 8

Is Cerebral Palsy progressive?

When is it most likely for a Cerebral Palsy causing event to occur: Antenatal, Perinatal, or Postnatal?

Keep in mind ________ is the most important risk factor for Cerebral Palsy.

Answer 8

NO! Level of deficit remains pretty constant.

Antenatal (so before birth)

Multiple births (twinning)

Question 9

Pilocytic Astrocytoma is characterized histologically by _______ Astrocytes and ______ Fibers.

Answer 9

Bipolar Astrocytes

Rosenthal Fibers

Question 10

Ependymomas most commonly occur within the ________ ventricle in children, rather than in the _______ ventricles in adults.

Answer 10

4th ventricle

Lateral Ventricles

Question 11

Kernicterus (Bilirubin Encephalopathy) occurs in children but NOT adults because children have an immature ______.

This results in a deposition of ________ (conjugated or unconjugated?) bilirubin most often in the _______ nucleus, Globus Pallidus, _________, and Lateral _________. Look for yellowing (due to the bilirubin) of these structures.

Answer 11

BBB

Unconjugated

Subthalamic nucleus, Globus Pallidus, Hippocampus, and Lateral Thalamus

Question 12

Aqueduct Stenosis can be spontaneous, ____-______, or rarely Autosomal Recessive.

Answer 12

X-linked