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34 Cards in this Set
- Front
- Back
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Craniosynostosis (CSO):
Saggital -Most common -80% males -Results in ... (boat shaped skull) (elongated cranium) -Frontal bossing, prominent occiput, palpable keel-like sagittal ridge |
scaphocephaly
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Craniosynostosis (CSO)
Coronal -Accounts for about 20% of craniosynostoses -More common in ... -Can be unilateral or bilateral -Unilateral produces ... (The forehead on the affected side is flattened or may be concave above the eye) -Bilateral results in ... (broad flattened forehead) |
females
plagiocephaly brachycephaly |
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Coronal CSO with abnormalities of the sphenoid, orbital, and facial bones = ... disease
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Crouzon’s
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Multiple CSOs with syndactyly = ...
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Apert’s Syndrome
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...:
-Maldevelopment of cerebral convolutions (gyri and sulci) -Likely due to arrest of cortical development at an early fetal age |
Lissencephaly
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...:
Surface of the brain is smooth -None or very few gyri and sulci |
Agyria
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...:
Thickened gyri Reduced in number |
Pachygyria
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...:
Small gyri Increased in number |
Polymicrogyria
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...:
Islands (blobs) of neuronal tissue where they don’t belong Commonly associated with epilepsy White matter with islands of gray matter |
Heterotopia
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...:
-Due to failure of fusion of the anterior neuropore -Neither the cranial vault nor the scalp covers the malformed brain tissue -Universally fatal |
Anancephaly
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...:
Herniation of meningeal and central nervous system tissue through a defect in the skull -herniation full of fluids and possibly parts of the brain |
Encephalocele
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Spina Bifida:
The term spina bifida covers a range of vertebral and neural tube defects Result from failure of the ... vertebral arch to fuse Most commonly occur in ... region |
posterior
lumbosacral |
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Spina Bifida:
If meninges bulge through, defect can result in a ... (meninges) or ... (meninges and spinal cord) |
meningocele
myelomeningocele |
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Spina Bifida:
-... does not contain spinal cord elements -... contains spinal cord and nerve roots -May be associated with ... displacement of medulla and cerebellum -Can result in ... |
Meningocele
Myelomeningocele caudal hydrocephalus |
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What type of spina bifida is most common and how does it usually present?
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spina bifida occulta
usually has tuft of hairs over the site of defect, may present with subtle neurological abnormalities such as enuresis or incontinence, neurological deficit is rare |
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Spina Bifida:
-can be detected prenatally by increased serum ... (must correlate with woman’s age and how far along she is) |
alpha-fetoprotein
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Of patients with a meningomyelocele
-One-third have complete paralysis and loss of sensation below the level of the defect -One-third have preservation of distal segments below the level of the defect -One-third have an incomplete lesion -90% of children develop ... problems |
urinary
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Prevent spina bifida by increased ... consumption
... is very impt in assisting with the development of proteins Very impt for pregnant women |
folic acid
Folic acid |
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Arachnoid Cyst:
Often referred to as ... cyst Arise during development from splitting of ... membrane Two histological types -“...”: lined with cells that are capable of secreting cerebral spinal fluid -Cysts with more complex lining (neuroglia, ependyma, etc.) Commonly found incidentally on CT or MRI |
leptomeningeal
arachnoid simple |
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... cyst:
Cystic lesion lined with glial or connective tissue that communicates with the ventricular system -Usually due to trauma or vascular event |
porencephalic
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...:
-Large portions of the cerebrum replaced by cerebrospinal fluid -Due to destructive process such as ischemic infarcts or infection (gets replaced with fluid) |
Hydranencephaly
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...:
Due to accumulation of cerebrospinal fluid (CSF) -Obstruction of CSF flow -Decreased absorption -In infants and children, monitor head circumference -If due to obstruction, rapid deterioration may occur -Treatment is ... |
Hydrocephalus
shunt placement |
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What is the most common type of chiari malformation we will see?
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type 1
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Chiari Malformation:
Type I -AKA ... -Caudal displacement of the ... with herniation of the tonsils below the foramen magnum -Can be associated with ... |
primary cerebellar ectopia
cerebellum syringomyelia |
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Chiari Malformation:
Clinical presentation (Type I) -Average age at presentation about age ... -Most common symptom is ... (70%) *Usually in the suboccipital region -Other signs/symptoms *Weakness *... sign (if patient flexes head forward, they get a lightning bolt sensation down their back) *Upper motor neuron signs *Spasticity |
40
pain Lhermitte’s |
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Chiari Malformation:
Type 2 -... dislocated cervicomedullary junction, pons, fourth ventricle, and medulla -Brainstem and lower cranial nerve dysfunction -Possible hydrocephalus -Rarely presents in ... -In neonates, there can be rapid neurological deterioration -Apnea, swallowing difficulties, stridor, aspiration, pooling of secretions, limb weakness |
Caudally
adulthood |
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Chiari Malformation:
Treatment -... decompression -... for hydrocephalus |
Suboccipital
Shunt |
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... Malformation:
Due to atresia of foramina of Magendie and Luschka -Results in agenesis of the cerebellar vermis with a large posterior fossa cyst that communicates with an enlarged fourth ventricle -Hydrocephalus occurs in 90% of cases -Usually associated with some degree of mental retardation, poor fine motor skills, ataxia, and spasticity |
Dandy-Walker
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Agenesis of the ...:
-Forms from rostrum (genu) to splenium -May be complete or incomplete -May be found incidentally and may be of no clinical significance -Can be part of complex of malformations (for example, ... Syndrome: agenesis of CC, seizures, retardation, retinal pigment abnormalities) |
Corpus Callosum
Aicardi |
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... - A cystic cavitation of the spinal cord
If it extends into the brainstem it is called a ... |
Syringomyelia
syringobulbia |
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Syringomyelia:
...: primary dilitation of the central canal of the spinal cord (enlargement of the central part of the cord) -Most common type associated with Chiari malformation -Can be dramatic or very small. Many levels ...: arises in the cord substance and does not communicate with the central canal of the spinal cord. |
Communicating
Noncommunicating |
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...:
-Abnormally low conus medullaris associated with a thickened filum terminale -May also be associated with an intradural ... -Commonly seen with myelomeningocele |
Tethered Cord
lipoma |
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Tethered Cord:
Most cases present in ... -Progressive scoliosis -Foot deformities (club foot) -Gait problems (regression) -Urinary problems (dribbling, incontinence) -Cutaneous stigmata of dysraphism (tuft of hair,dimple) -Back and lower extremity pain ... presentation is very rare -Significant perianal and perineal shock-like pain -Urinary problems |
childhood
Adulthood |
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Split cord:
Type 1(...) -Two hemicords in their own dural tube separated by a ... medium septum -Associated with abnormalities at the level of the split *Absent ..., tuft of hair Type 2 (...) – (very unusual) -Two hemicords within a single dural tube separated by a ... median septum -Usually no abnormalities at the level of the split, but most affected individuals have ... in the lumbosacral region |
diastematomyelia
bony disc diplomyelia fibrous spina bifida occulta |
