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Path CNS Malformation Trauma
KYCOM Block 13
57
Pathology
Graduate
01/27/2014

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Cards

Term
define neural tube defect
Definition
reopening or failure to close the neural tube
Term
explain normal neural tube development and differentiation
Definition
neural plate invaginates forming 2 neural crests which grow cranial to caudal
neural crest becomes PNS
neural tube wall becomes CNS
neural tube lumen becomes ventricles and spinal canal
Term
3 risk factors for neural tube defects
Definition
folate deficiency during initial gestation
A-fetoprotein in amniotic fluid signals a neural tube problem
4-5% reoccurrence in subsequent pregnancies
Term
what are the two categories of neural tube defects, which is the most common
Definition
caudal (spinal cord) malformations - most common
rostral (brain) malformations
Term
what are the 3 caudal malformations and their categories
Definition
spina bifidia occulta
spina bifidia: meningocele, myelomeningocele
Term
spina bifidia occulta: cause, 2 signs
Definition
vertebral arch defecit (cord and meninges in tact)
patch of hair or dimple over area
Term
cause of meningocele
Definition
vertebral arch defect, meninges protrusion
Term
cause of myelomeningocele
Definition
vertebral arch defect, meninges protrusion, spinal cord protrusion
Term
2 complications of myelomeningocele
Definition
lower limb and sensory issues: loss of bowel and bladder control
infection risk: thin ulcerated overlying skin
Term
what are the two rostral malformations
Definition
anencephaly
enecephalocele
Term
cause of anencephaly
Definition
absence of brain and calvarium development
Term
2 signs of anencephaly
Definition
maternal polyhydraminos: no brain so CNS controlled swallowing stops and amniotic fluid increases

eyes protrude above top of head due to lack of brain and calvarium (frog fetus)
Term
enecephalocele: cause, location
Definition
diverticulum of malformed CNS tissue extending through cranium
posterior more common than anterior
Term
forebrain malformations 6
Definition
microcephaly
lissencephaly
pachygyria
polymicrogyria
holoproencephaly
dysplastic cortex
Term
causes of microencephaly 3
Definition
chromosome abnormalities
fetal alcohol syndrome
in utero HIV
Term
why does microcephaly occur
Definition
there needs to be a balance between progenitor cells proliferating in subependymal zone and the backup progenitor cell population
if too many cells leave the back up pool premaurley then it causes decreased generation of mature neurons and a small brain
Term
lissencephaly: define, cause, sign
Definition
agyria or smooth brain
disrupts neuonal migration and differentiation due to defect in genes controlling neuron migration
cortex is abnormally thick and only 4 layers
Term
define pachygyria
Definition
less wide spread form of lissencephaly
Term
polymicrogyria: define, signs
Definition
increases and irregular gyri form cobblestone surface in focal or widespread area
altered cortical architecture (fusion of superificial molecular layer)
Term
holoproencephaly: cause, difference in signs between severities
Definition
disruption in normal midline patterning due to SHH mutation

mild: olfactory bulbs (arrhinecephaly)
severe: brain may not have lobes or hemispheres, one large ventricle, mimdline facial defects
Term
dysplastic cortex ause
Definition
focal disortion of cortex
neurons stranded beneath cortex in nodules and bands
Term
what are the 3 posterior fossal malformations, which is most common
Definition
arnold chiari- most common
dandy walker malformation
robert syndrome
Term
arnold chiari type i: symptoms, cause
Definition
NO SYMPTOMS- headache and CN defects may arise as adult

low lying cerebellar tonsils extend through foramen magnum obstructing CSF flow and compressing medulla
Term
arnold chiari type 2: symptoms and cause
Definition
SYMPTOMATIC- hydrocephalus

small posterior fossa causes misshaped cerebellum whose vermis extends through the foramen magnum causing obstruction of flow
Term
what condition is arnold chiari type 2 associated with 2
Definition
lumbar myelomeningocele
syringomyelgia
Term
dandy walker malformation: cause 2, effect
Definition
enlarged posterior fossa with absent or rudimentary cerebellar vermis

large midline cyst (4th ventricle dilation): lined with ependymal cells and contigous with leptomeninges

causes dysplasia of brainstem nuclei and hydrocephalus
Term
robert syndrome: cause, signs
Definition
AR lesionsin primary cilium

looks like dandy walker without brainstem or vermis issies

extra structures in space leads to eye and respiratory problems
Term
where are spinal cord malformations usually located
Definition
cervical
Term
what is the pathological processes normally causing spinal cord malformations
Definition
destruction of gray and white matter causing reactive gliosis
Term
what are the 2 spinal cord formations
Definition
hydromyelia
syringomyelia (syrinx)
Term
define hydromyelia
Definition
expansion of the central canal
Term
2 causes of syringomyelia and how, cellular changes, symptoms and why
Definition
arnold chiara malformation or trauma damages anterior white comissure C8-T1 causing a fluid filled cavity in inner portion of spinal cord surrounded by reactive GLIOSIS AND ROSENTHAL FIBERS

comissure and spinothalamic tracts cross over before they take pain/temp to the thalamus so they get loss of pain and temp in the UPPER LIMBS
Term
if a syringomyelia expands, where does it expand to 2, what symptoms for each
Definition
anterior horns: carry motor to muscles: muscle weakness and atrophy, impaired tone, decreased reflexes

lateral horns: carry sympathetic info to the face: horner syndrom
Term
3 signs of horner syndrome
Definition
ptosis
miosis
anhydrosis on half of face
Term
what are the 4 types of parenchymal injury
Definition
contusion
concussion
coup injury
diffuse axonal injury
Term
contusion: two types, define
Definition
contusion
penetration
brain impacts side of skull
Term
contusion: cause, 4 effects
Definition
rapid tissue displacement, disruption of vessels, hemorrhages, tissue injury, edema
Term
penetration: cause, 3 effects
Definition
object/skull fragment causes laceration
tissue tearing
vascular disruption
hemorrhage
linear path
Term
define concussion
Definition
reversible altered conciousness from head injury in absence of contision
Term
2 signs of concussion
Definition
transient neurological dysfunction: LOC, temp respiratory arrest, loss of reflexes

complete neurological recovery: amnesia of event
Term
3 common locations of parenchymal injury
Definition
orbital gyri of frontal lobe
temporal lobe esp tips
crests of gyri
Term
parenchymal injury: what does a new and old one look like, what cells
Definition
new: wedge shaped wide at point of impact, effects superificial layers first
old: depressed, retracted, yellow brown, mostly on gyri crest

24h: nuclear pyknosis, eosinophils, cellular degeneration
Term
what is a complication of parenchymal injury what does it look like
Definition
extensive hemorrhaging: cavity lesions, like infarct
Term
cause of diffuse axonal injury
Definition
movement of one region of the brain relative to another, twisting and breaking axons
angular acceleration without impact
Term
location of diffuse axonal injury 2
Definition
angle of lateral ventricles or brain stem
asymmetric
Term
effect of diffuse axonal injury 3
Definition
axon dysruption and hemorrhage
50% get coma
Term
2 types of traumatic vessel injury
Definition
epidural hematoma
subdural hematoma
Term
epidural hematoma: cause, effect, location, presentation, progression
Definition
trauma causes little vessels that run through bone (ESP MIDDLE MENINGEAL A) to rip and bleed into SUBDURAL SPACE

this causes DURA TO SEPERATE making SMOOTH CONTOUR STOPPING AT SUTURE LINES

as it spreads it compresses brain and causes death WITHIN HOURS
Term
signs of epidural hematoma
Definition
patient is lucid for hours then rapidly develops neurological signs leading to death
Term
subdural hematoma: cause, progression, effect
Definition
trauma, retraction of brain (atrophy in elderly) or fragile vessels (babies) causes tearing of BRIDGING VEINS running through subdural space into dural sinuses

beacause this is NOT LIMITED BY SUTURES it can spead laterally over the hemisphers as blood builds up (10% become bilateral)
Term
signs of subdural hematoma
Definition
48h: headache, confusion, some localizing signs, neurological deterioration
Term
prognosis of subdural hematoma
Definition
bleed is usually self limited but frequently re-bleeds (chronic subdural hematoma)
Term
morphology of subdural hematoma in 1wk, 2wk, 1-3mo
Definition
1wk: lysis of clot
2wk: growth of granulation tissue from dura
1-3mo: fibrosis
Term
3 types of perinatal brain injuries, which is most common
Definition
cerebral palsy
intraparenchymal hemorrhage and infarct - most common
preiventricular lekomalaca
Term
cerebral palsy: cause, 5 signs
Definition
prenatal and perinatal brain injury causes non-progressive neurological motor disorder

spasticity, dystonia, ataxia, athhetosis, paresis
Term
intraparenchymal hemorrhage and infarct: cause, 1 sign
Definition
germinal matrix of premature infants extends into ventricles and subarachnoid
hydrocephalus
Term
periventricular leukemia: cause, 1 sign
Definition
infarct in supratentoral periventricular white matter (esp when premature)develops large cystic lesion in gray matter (multicystic encephalopathy)

cysts: large, chalky plaque in white matter with calcifications
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