neuro Flashcards
(129 cards)
Q
nucleus pulposus
is a remnant of ?
notochord.
failure of the prosencephalon to divide into 2 cerebral hemispheres
results in?
the most likely mechanism of these findings
holoprosencephalon, (fused cerebral hemispheres),
seen in trisomy 13
fetal alcohol syndrome.
mechanism = field defect
Q
pt with hyperthermia.
caused by problem with the center regulating heart.
what is the origin of this center
diencephalon
forms the thalmus and hypothalamus
which chiari malformation is always associated with spina bifida (meningomyelocele)
type 2
bc
type 1 (adult type) is associated with syringomyelia
dandy walker syndrome
is associated with what type of hydrocephalus
Q why hydrocephalus
noncommunicating hydrocephalus.
(malfunction of Luschka and Magendie)
- cystic dilation of the 4th vent –> hypoplasia of the cerebellum and absence of cerebellar vermis
Q on the boards
neonate that survved meningtits (usually grp B strept) and started to develop enlargement of the skull?
whats the cause
congenital hydrocephalus
acquired after bacterial meningitis
Q on boards
congenital hydrocephalus
(aqueduct of sylvius)
can be caused by defect in what embryolonic origin
mesencephalon
also can be caused by failure of the roof of the 4th ventricle to develop
noncommunicating (obstructive hydrocephalus)
arnold chiari
dandy walker
congenital hydrocephalus
arnold - compression of the 4th ventricle
dandy walker syndrome - luschka and magendie malfunction
congenital hydrocephalus - stenosis of cerebral aqueduct
Communicating (nonobstructive) hydrocephalus
kid had meningitis 1 month ago, his head is getting bigger
whats the problem
disoder of the arachnoid granulations
what to know
port wine stain (nevus flammeus - birth mark) cnV1/V2
seizures
episcleral hemangioma –> glaucoma
intellectual disability
sturge weber syndrome
(encephalotrigeminal angiomatosis)
what you need to know
autosomal dominant
multisystem disorder –> tumors in diff organs
brain - (cortical tubers = large white hamartomas nodules)
rhabdomyoma –> mitral regurg
hypopigmented ash leaf spots and angiofibroma
kidney: bilateral renal angioMyolipoma
tuberous sclerosis
most common primary heart tumor in children and infants
rhabdomyoma.
what are neurofibroma composed of
A. neural tissue
B. Schwann cells and fibroblast
schwann cells and fibroblasts
NOT NEURAL TISSUE
WHAT to know
chromo 17 mut in a tumor supressor gene
Cafe au lait macules
pigmented neurofibromas
axillary freckling (crowe sign)
associated with Pheochromocytoma and wilms tumor
optic glioma
NF 1
bilateral acoustic neuroma (tinnitu vertigo hearing loss)
- usually schwannoma located cerebellopontine angle
chromo 22
meningiomas
juvenile cataracts
NF2
cerebellar degeneration –> ataxia (early onset)
telangiectasia (dilated blood vessels) of the eyes and skin
immunodef
low IgA and T cell deficit
recurrent infections
ataxia telangiectasia
VHL gene is a
A. oncogene
B. tumor supressor gene
tumor supressor gene
angiomatosis of the retina –> blindness
(hemangioblastomas (high vascularity w/ hyperchromatic nuclei) in retina, cerebellum, brain stem.
bilateral RCC
Pheochromocytomas
VHL disease
2/3 of sporadic clear renal cell carcinoma
have difinite evidence for
biallelic inactivation of VHL
know this
why is carmustine and lomustine
used to treat brain tumors
(lipophilic)
bc they are small, lipid soluble, nonpolar,
molecules that CAN cross the BBB
what brain tumor will you see mulltiple of in
NF2
meningioma
on the boards
uniformly encircled tumor cells with psammoma bodies
presents with new onset seizures
meningioma
on the boards
pt in a coma after head injury with bradycardia and ST segment elevation on EKG
from increased ICP
cranial nerve palsy (VI common due to long intracranial course) –> binocular horizontal diplopia due to lateral rectus palsy
whats the cause
inc ICP