Sos: development of CNS Flashcards by Madeline Wing

brain to gut; the ultimate survival machine

autonomic NS

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areas that close last along neural tube where congenital malformations happen

cranial and sacral region

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neural plate to neural _____ to neural ____

plate to crest cells to tube

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sensory and regulated by TGF-beta and BMPs (bone morphogenic proteins)

Alar plate (dorsal)

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motor and induced by SHH

basal plate (ventral)

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neural tube has same ____ as spinal cord

orientation

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neural migration:

ventricular region to pia mater using radial glial cells and hormones

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telencephalon and diencephalon

forebrain

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mesencephalon

midbrain

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pons and cerebellum

metencephalon

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medulla oblongata

myelencephalon

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metencephalon and myelencephalon

hindbrain

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cerebrum, thalamus, hypothalamus

forebrain

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brain stem

midbrain and hindbrain

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comes from neural crest tissue and from mutation in C-MYC gene

neuroblastoma

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causes of neuronal migration defects

failure of neurons to migrate
scattered neuron arrangement
late stage neuronal migration

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abnormal migration of neurons causes abnormal ____ pattern

gyral

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well studied gene associated with neuronal migration defects

RELN/Lis

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With _____ mutation, actin and microtubules don’t form properly and they don’t migrate; don’t migrate to final resting place=cant be fully functional

RELN/Lis

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neuronal migration defects lead to a 4 layer cortex instead of a ____ layer cortex

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stain to look at cell bodies

Nissl stain

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Smooth cortex without gyri and thick cortex with four cell layers

Lissencephaly

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Lissencephaly

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smooth cortex
microcephaly
mental delay/seizures

Lissencephaly

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Lissencephaly

cortex has irregular grooves forming a cobblestone pattern; total disarray of cortex

cobblestone cortex

no cortical layers irregular grooves neurons migrate past cortex

cobblestone cortex

Broad gyri and thick cortex with few layers tissue not compatible with learning and development

Pachygyria

broad gyri

Pachygyria

broad gyri and thick cortex

Pachygyria

Surface of the cortex appears to form microgyri

Polymicrogyria

Often found in vascular territories or watershed areas suggesting a hypoxic/ischemic event; microgyri

Polymicrogyria

normal neurons/glia found in the wrong place

heterotopia

Collections of neurons found under the ependyma of the lateral ventricles Unilateral variants are silent and incidental findings during imaging studies (neurons found in wrong place)

Periventricular (subependymal) Heterotopia

Periventricular (subependymal) heterotopia

neurons in wrong place (didnt migrate out)

periventricular (subependymal) heterotopia

Collections of neurons in the cerebral white matter diffusely scattered, clustered into nodules, bands or grouped in large masses

Subcortical neuronal (band) heterotopia

associated with seizures

subcortical neuronal (band) hetertopia

used to stain myelin

Luxol fast blue (LFB)

not termed precancerous when talking about the brain

cortical dysplasia

(grey matter) improperly forming Most common form is focal ____

cortical dysplasia

most common cause of epilepsy (often gray matter found in temporal lobe)

cortical dysplasia

top bottom

top: normal bottom: cortical dysplasia

Infected in utero; skull in vault doesn’t form and brain and cortex don’t form (microcephaly)

Zika virus

autism, muscle spasms, mental delay Due to disrupted neuronal migration along with an abnormal neuronoglial differentiation

tuberous sclerosis syndrome

organs involved in tuberous sclerosis syndrome

brain, heart, kidneys, skin

Neocortical tubers Subependymal nodules Subependymal giant cell astrocytomas (SEGAs)

brain involvement of tuberous sclerosis syndrome

rhabdomyomas--- heart angiomyolipomas (hamartomas)-- kidney angiofibromas ----skin

Tuberous sclerosis syndrome

tubers (tuberous sclerosis sydnrome)

heart mass in children

tuberous sclerosis syndrome

heart rhabdomyoma

kidney angiomyolipoma (hamartoma)

tuberous sclerosis syndrome

A single telencephalic ventricle with continuity of the cerebral hemisphere across the midline

Holoprosencephaly

forebrain aka

prosencephalon

Associated craniofacial abnormalities, most severe being cyclopia

holoprosencephaly

holoprosencephalies

alobar holoprosencephalies

most common congenital malformation disorders

neural tube defects

increased AFP in amniotic fluid

neural tube defects

poorly controlled DM valproic acid folic acid and vitamin B12 deficiency

neural tube defects

open brain and lack of skull vault

Anencephaly

herniation of meninges and midbrain

Encephalocele

some of vertebrae are not completely closed; asymptomatic (can sometimes feel tuff of hair there)

Spina bifida occulta

protrusion of meninges and CSF through defect in skull or spine

Meningocele

open spinal cord w/ a meningeal cyst (spinal cord and meninges pushed out)

Myelomeningocele

meningocele

skull defect involves meninges and CSF

meningocele

skull defect involves brain parenchyma, meninges, and CSF

Encephalocele

80% of cephaloceles happen in what region

occipital region

myeloencephalocele or encephalocele

midline of cerebellum

vermis

hindbrain aka

posterior fossa

condition in which brain extends through the foramen magnum

Chiari malformations

minimal brain tissue extends downwards cerebellar tonsils are displaced and Syrinx formation

Type I Chiari malformation

greater amounts of brain tissue extends downward and a myelomeningocele and hydrocephalus 90% of time

type II chiari malformation

kinking of medulla and hydrocephalus

type II chiari malformation

most severe and rare, includes cerebellum and brainstem extending downward

type III chiari malformation

cerebellum pushed up enlarged ventricles hydrocephalus

Dandy-Walker syndrome

cerebellum pushed up

Dandy-Walker syndrome

can't find vermis (vermal aplasia)

Dandy-Walker syndrome

Most likely due to decreased or lack of blood flow and brain development brain still intact almost complete loss of cortical tissue though

Hydranencephaly

hydranencephaly

Brain parenchyma compressed to where it is almost nonexistent/nonfunctioning

Hydranencephaly

MCA stroked and all lateral part of brain necrosed cavitary necrosis of the cerebrum, extending to the surface of the brain

Porencephaly

all tissue looks like lissencephaly a cleft of the cerebrum without ventricular communication

Schizencephaly

blood into ventricles of fetus and causes clot and cardiac arrhythmia and death

germinal matrix bleeds

Immature vessels w/o stroma Hypoxic stress Ischemic endothelial cells

germinal matrix bleeds

germinal matrix bleed

Neurological disorders that appear in infancy or early childhood and permanently affects body movement and muscle coordination but doesn’t worsen over time

cerebral palsy

muscle tone that is either too stiff or too relaxed and appears before child reaches 3 ataxia

Cerebral palsy