Pathology - S2B4 - Neuro 5

Front 1

Spinal Cord & Brain

Vertebral Column & muscles

Back 1

What structures does the Neural Tube give rise to?

What structures do Somites give rise to?

Front 2

List the 3 Neural Tube Defects

Back 2

1. Anencephaly
2. Encephalocele
3. Spina Bifida
-Occulta
-Meningocele
-Meningomyelocele

Front 3

Define Anencephaly

Back 3

Failure of the ANTERIOR end of the Neural Tube to close
-absence of Forebrain
-absence of Calvarium

Front 4

Anencephaly
-failure of anterior NT to close

Back 4

What NTD is this?

Front 5

Define "Encephalocele

Back 5

NTD in the cranium that allows for out-pouching of the brain thru the skull
- most commonly in the Posterior Fossa = area at the base of the skull attached to the spinal cord
-diverticulum of the CNS

Front 6

Encephalocele

Back 6

What NTD is this?

Front 7

Encephalocele
-failure of Mesoderm to close
-out-pouching of brain thru skull

Back 7

What NTD is this?

Front 8

This is the most common NTD

Back 8

Spina Bifida

Front 9

What is Spina Bifida due to?

Back 9

failure of the Posterior end of the NT to close

Front 10

What is Spina Bifida Occulta?

Back 10

failure of the bony spinal canal to close, but no structural herniation
-commonly have a tuft of hair on lower back

Front 11

What is Meningocele?

Back 11

Spina bifida in which the Meninges herniates thru the Spinal Canal defect

Front 12

What is Meningomyelocele?

Back 12

Spina Bifida in which the Meninges & Spinal Cord herniate thru spinal canal defect
-usually occurs in Lumbrosacral area

Front 13

List the 5 Forebrain Anomalies

Back 13

1. Polymicrogyria
2. Lissencephaly
3. Neuronal Heterotopias
4. Holoprosencephaly
5. Agenesis of Corpus Callosum

Front 14

Developmental malformation of the human brain characterized by an excessive number of small folds (gyri) on the surface of the brain

Back 14

Polymicrogyris

Front 15

-4 or few layers of Cortex
-Entrapment of Meninges at points of fusion
-Localized injury during neuronal migration

What is the Forebrain Anomaly?

Back 15

Polymicrogyria

Front 16

Polymicrogyria

Back 16

What forebrain anomaly is this showing?

Front 17

Pachygyria = congenital malformation of the cerebral hemisphere that results in unusually thick convolutions of the cerebral cortex

Back 17

What forebrain anomaly is this showing?

Front 18

Neurological disorder of early brain development that leads to the gross appearance of a smooth brain

Back 18

Lissencephaly

Front 19

What is Miller-Dieker Syndrome?

Back 19

Characterized by:
1. Lissencephaly = agyria
2. 90% has a deletion on 17p
3. LIS1 gene absence
4. Seizures & Mental Retardation

Front 20

What are Neuronal Heterotopias?

Back 20

Displacement of Gray Matter (perikaryon) into the cerebral White Matter (axons) or ventricles due to migrational defect

Almost always have seizures

Front 21

Neuronal Heterotopia
-Gray Matter is displaced in White Matter
-due to migrational defect

Back 21

What is seen here?

Front 22

What is Holoprosencephaly?

Back 22

Incomplete separation of Cerebral Hemispheres

Front 23

Holoprosencephaly

Back 23

What congenital CNS anomaly is this?

Front 24

What is Holoprosencephaly associated with?

Back 24

Trisomy 13 (Patau Syndrome)

Children of Diabetic mothers

Front 25

What bodily malformation may accompany Holoprosencephaly?

Back 25

Cyclopia = single median eye

Arrhinencephaly = part or all of the rhinencephalon is absent and the nose is malformed

Front 26

What mutation is Holoprosencephaly associated with?

Back 26

Sonic Hedgehog mutation

Front 27

What is the Forebrain anomaly in which radiologic studies show a "Bat-wing" deformity?

Back 27

Agenesis of Corpus Callosum

Front 28

Agenesis of Corpus Callosum

Back 28

What forebrain anomaly is seen here?

Front 29

What may accompany a partial Agenesis of Corpus Callosum?

Back 29

Lipoma in the posterior part

Front 30

List the 3 Posterior Fossa Abnormalities

Back 30

1. Chiari I malformation
2. Arnold-Chiari Malformation
3. Dandy-Walker Malformation

Front 31

What is Arnold-Chiari Malformation?

Back 31

Small Posterior Fossa, resulting in displacement of Cerebellum & Medulla thru the Foramen Magnum

Front 32

What are the complications of Arnold-Chiari Malformation?

Back 32

1. Non-communicating Hydrocephalus = obstructed flow of CSF from the Ventricles to the Subarachnoid space

2. Lumbar Meningomyelocele = spinal cord + meninges herniation

Front 33

Arnold-Chiari Malformation
-small Posterior Fossa
-downward displacement of Cerebellar tonsils & Medulla

Back 33

What posterior fossa defect is this?

Front 34

What is Dandy-Walker Malformation?

Back 34

Enlarged Posterior Fossa with replacement of the Cerebellar Vermis with a large Cyst
-brainstem nuclei dysplasias

Front 35

Dandy-Walker Malformation
-hypoplasia of the Cerebellar Vermis
-cystic dilation of the 4th ventricle

Back 35

What Posterior Fossa abnormality is this?

Front 36

What is Syringomyelia?

Back 36

formation of fluid-filled cavity often extending from the central canal of the Spinal Cord usually in the cervical region of the cord

Results in destruction of adjacent gray & white matter

Front 37

What is Syringomyelia associated with?

Back 37

Chiari I, tumors, trauma

Front 38

What are the symptoms of Syringomyelia?

Back 38

loss of pain & temperature in the upper extremities with preservation of touch & propioception

Front 39

List 2 Perinatal Brain Injuries

Back 39

1. Intraparenchymal Hemorrhage = Germinal Matrix Hemorrhage

2. White Matter Infarcts = Periventricular Leukomalacia

Front 40

Bleeding into the subependymal germinal matrix with or without subsequent rupture into the lateral ventricle. The microcirculation in this particular area is extremely sensitive to hypoxia and changes in perfusion pressure. It is most frequent before 35 weeks gestation and is typically seen in low birth-weight (<1500g) premature infants

Back 40

Germinal Matrix Hemorrhage = Intraparenchymal Hemorrhage

Front 41

What may result from Intraparenchymal Hemorrhage?

Back 41

Hydrocephalus

Front 42

What may Periventricular Leukomalacia lead to?

Back 42

Cerebral Palsy

Front 43

Germinal Matrix Hemorrhage = Intraparenchymal Hemorrhage

Back 43

What is seen here?

Front 44

White Matter Infarcts = Periventricular Leukomalacia

Back 44

What is seen here?

Front 45

What is Periventricular Leukomalacia (White Matter Infarct)?

Back 45

Characterized by the death of the white matter of the brain due to softening of the brain tissue. It can affect fetuses or newborns; premature babies are at the greatest risk of the disorder. PVL is caused by a lack of oxygen or blood flow to the periventricular area of the brain, which results in the death or loss of brain tissue