neuro Flashcards
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
if someone is on a ventilator and a patient is suffering from increased ICP
what can you do to help
hyperventilation
–> dec co2 –> vasoconstriction –> dec cerebral blood flow –> dec ICP
Aids pt with CD4 count <50
using natalizumab
for tx of MS or crohns disease
has deficits in speech, memory, coordination
PML
oligodendrocytes –> intranuclear inclusions –> symptoms
on the boards
12-15 yo hx of measles
infection early in life (before age of 2)
presents with personility changes, seizures, myoclonus, atxia, and photosensitivity
Subacute sclerosing pancencephalitis
on the boards
pt with hx of long term corticosteroid use who develops
pulmonary infection initially then brain abscess
nocardia asteroides
on the boards
hiv infection pt who develops signs of
encephalitis or brain abscess with ring enhancement and calcification on mri.
diagnosis
protozoa
(Toxoplasma gondii)
trick question
is CFJ prion spongiform encepholpathy
effect patchy or whole cerebral cortex
whole cerebral cortex
Q on board. 30 yo mexican immigrant healthy person visiting area from South america. has been to ER for new onset seizures
ct of brain shows cystic mass
what is the treatment
how did he acquire this infection?
albendazole and corticosteriods (for the edema)
through eating pork meat
taenia solium (cysticercosis)
when the pts thigh is flexed at the hip and knee at 90 degree angles
you see ressitance in extending the knee (painful)
what sign is this and what is it positive in
kernig sign
+ in SAH, meningitis
healthy middle age 30yo pt
all of sudden have increase ICP. on biopsy you see intracerebral hemorrhage formed of numerous tortuous vessels
what is the casue
av malformation rupture
stroke queston on boards!!!
pt with
vertigo, N/V, nystagmus, ipsilateral limb ataxia.
what kind of stroke?
what artery
** if brainstem compression you need to do what?
cerebellar stroke
Superior cerebellar A. or
AICA or PICA
must consult neurosurgery for decompression
what stroke causes
dysphagia, hoarseness
dec gag refelx
lateral medullary wallenberg syndrome
PICA
which stroke causes facial paralysis (lmn lesion
(facial nucleus))
dec lacrimation
dec salivation
dec taste from anterior 2/3 tongue
AICA = lateral pons
which stroke
ipsilateral tongue paralysis
contralateral upper and lower limbs paralysis
loss of contralateral proprioception
ASA
medial medullary syndrome
on boards
conscious quadrplegic pt who is unable to move the face, mouth, and the tongue and has loss of horizontal eye movement (NOT the VERTICLE)
locked in syndrome,
involving the pons, medulla, and the midbrain.
spares the reticular activating system (RAS)
dysarthria meaning
inability to move the tongue and mouth muscles.
on boards
pt with conjuctival injection, edema of the eyelids with
paralysis of the cranial nerves passing through the cavernous sinus, after a dental procedure
diagnosis
and most likely organsim
cavernous sinus thrombosis
peptostreptococcus
what is the most common extraocular muscle involved in wernickes encephalopathy
lateral rectus
on boards
what is korsakoff psychosis due to ?
necrosis and perminent damage to the mamillary bodies
what will happen if you glucose before thiamine if pt has wernicke encephalopathy
inc anion ap metabolic acidosis
inc anaerobic metabolism of glucose and accumulation of lactic acid.
for the boards
pts with metabolic encephalopathy (including alcohol intoxication) will have?
whats the one exception
equal reactive pupils
even in the terminal conditions (cheyne-stokes breathing) except
opioid induced encephalopathy –> pinpoint pupils
on the boards
pt who presents with drooping eye lids, slurred speech, vomiting , and muscle weakness 6 hr.
after ingestion of contamnated food
cause?
botulism.
(miller-fisher syndrome)
Descending paralyses, ocular muscles first, ataxia and areflexia
it blocks release of acetylcholine from NMJ –> respiratory failure
C4 dermatome
shoulder tip
C6 dermatome
thumb
C5 dermatome
outer part of the upper arm
L1 dermatome
inguinal ligament
C2 occiptal and parietal area
pain in occipital area after a hit in the rear (whiplash)
diffuse hyperreflexia
generalized weakness and atrophy and fasciculations of limbs
compression of the cervical cord.
cant toe walk
L4-L5
pain and sensory loss over the back of the thigh, lateral posterior calf
L5-S1
cant TOE WALK
S1
Hair follicle ending
hair displacement
krause corpuscle
located at lip tongue and genitals
respond to pressure
ruffini ending
located dermis of skin
pressure on skin
meissner corpuscle
dermis of skin
light touch
pacinian corpuscle
located deep dermis
vibration - slow and pressure
Free nerve ending
mechanical , thermal
on the boards
macrocytic anemia, smooth tongue ,
achlorhydria with hx of vitamin B12 deficiency - (prenicious anemia)
posterior column degeration -> bilateral loss of vibration and tactile sense
corticospinal tract –> bilateral spastic paresis bleow the lesions
progressive degen disease of posterior roots of the spinal cord –> bladder incontinence
subacute combined degeneration
AIDS demential complex
look for pt with Subacute combined degeneration
dementia
long hx of AIDs
Bilateral horners if above T2
flaccid paralysis (LMN)
spastic paresis (corticospinal treact)
pain and temp (lateral spinothalamic tract)
all impaired
anterior spinal artery infarction
on the boards
fasciculations (usually tongue)
preserved ocular muscle movement
ALS
mutation in superoxide dismutase 1
(SOD1 gene)
ALS inherited form
what is the only nerve in nerve that regenerates in adults
olfactory (CN2)
which cn has the slowest fibers in conducting action potential
olfactory
fracture of the cribriform plate leads to
loos of smell (anosmia) due to injury of the olfactory bulb.
however, why can the pt still smell/have a response to ammonia.
noxious stimuli from the olfactory mucosa is carried by the trigeminal nerve
on the boards
injury to geniculocalcarine tract in the temporal lobe will cause what visual field defect
upper visual field defect
(meyers loop)
Q
pt with
acute glaucoma and orthostatic hypotension in a pt with DEPRESSION
what do you think the cause is
imipramine
drug to give someone that has narrow angle glaucoma
(red painFUL EYE)
PILOCARPINE
in the treatment of open angle glaucoma
what are the 2 main side effects of prostaglandin (latanoprost)
“PGF2)
darkening of the iris color (browning)
and eye lash growth
Q
what nerve was injured when a pt had their mandibular molar extraction now causing numbness in the tip of the tongue?
lingual nerve ( a branch of the madibular division) passes close to the third molar
(anterior 2/3 sensation)
what supplies innervation to the posterior scalp and the neck
C2-C3
NOT TRIGEMINAL NERVE
trigeminal neurlagia
what branch
maxillary
on boards
pt with levator palpebrae superioris problem
intracranial aneursym
in pt with polycystic kidney disease
can somehow effect what nerve.
oculomotor nerve
most likely in the PCOM
also could have
the facial nerve course
from the pons –> internal auditory meatus–> facial canal of the petrous portion of the temporal bone through the _____ foramen
stylomastoid foramen.
** the facial canal is Z shpaed located in temporal bone extending from internal acoustic meatus to styloidmastoid foramen.
Q on boards
pt has hyperacusis (inc sensitivity to everyday sound)
where is the damage causing this
injury to the facial canal where the nerve to the stapedius originates.
stapedius n is a branch of the facial n.
pt with bells palsy and cant close the eye lid all the way when they sleep
what nerve and muscle
facial nerve.
orbicularis oculi
chorda tympani
is a branch of what nerve that supplies
taste sensation to anterior 2/3 of tongue
and parasympathetic to (submandibular and sublingual)
facial nerve.
pt has eye dryness (weakness in orbicularis oculi) and increased sensitivity ot noise (hyperacusis)- know (injury in facial canal, injury to stapedius)
bells palsy
horner syndrome
ipsilateral ptosis due to paresis of what muscle
muller muscle
smooth muscle supplied by sympathetic fibers from superior cervical ganglion help to raise upper eyelid
Q
pt with bells palsy and acoustic neuroma
where is the injury
facial canal
internal auditory meatus in temporal bone
Q
pt has difficulty of hearing low frequency sounds
think of damaged?
inner ear hair cells
or
menieres disease
Q
autosomal dominant defect w/ type 1 collagen deficiency
SENSORY HEARING LOSS Is tdue to atrophy of the cochlear hair cells and abnormal bone formation in the cochlea.
what disease?
osteogenesis imperfecta
ipsilateral ataxia
and
intention tremor
are due to?
cerebellar lesions with loss of purkinje cells
in the HEMISPHERES
not the vermis
treatment for
essential tremor
B - Blocker
if b blocker is contraindicated
primidone
asterixis
hepatic encephalopathy
alcohol withdraw and
side effect of lithium
hyperthyroidism
HYPOglycemia
tremor
pt bitten by a black widow spider
presents as ?
muscle twitching
NOT TREMOR
abdominal and chest pain
supraoptic and paraventricular nuclei
ADH and oxytocin formation
effect of lesion
diabetes insipidus
what is ghrelin
gut brain peptide produced by GI mucosa mainly the stomach.
acts on teh hypothalamus –> inc hunger –> wt gain – FATASS
on the boards
a retrochiasmatic mass
or
multiple sclerosis
wiill displace the optic tract –> ?
contralateral homonymous hemianopsia
in parkinsons disease you see an overproduction in ACH?
why
bc dopamine normally has an inhibitory effect on cholinergic neurons
no dopamine = no inhibitory effect
the tremor in parkinsons pt is due to
excess ACH in the stratum
rigidity for boards
___ is a form of increased tone –> inc muscle stiffness, fatigue, weakness
lead pipe rigidity
rigidity for baords
lead pipe rigidity + tremor –> a stop and go effect during a range of motion
cogwheel rigidity
rigidity for board
is seen in UMNL starts with a higher resistance to flexion ollowed by a loss of resistance
clasp knife response
in huntingtons
you get atrophy of?
which leads to enlargement of the lateral ventricles (ex vacuo ventriculomegaly)
caudate
on the boards
middle aged women started on a medicine for psychosis and developed dyskinesia, galactorrhea and amenorrhea.
what is the most likely cause of her symptoms
low potency antipyschotic drugs – phenothiazines (chlorpromazine)
cause blockade of dopamine receptors
boards
unsteady wide based gait (staggering side to side)
whats the cause
cerebellar vermis lesion
long hx of alcohol abuse –> ataxia
waxing and waning breathing followed by apnea
look for pt with
CHF, Immature newborn, or a person who moved recently to high altitude area
cheyne stokes respiration.
kussmaul sign
increased jugular venous distention during inspiration in pt with constrictive pericarditis
Erb palsy
most comm birth related neurapraxia
upper arm paralysis C5/ C6
will they have intact or lost grasp
intact grasp
in erB palsy
what two nerves are involved
Axillary and musculocutaneous
klumpke palsy
lower arm paralysis C8/T1
grasp
lost grasp
boards
common presentation
what nerve of the arm is commonly effected in cycylist
ulnar
what nerve is commonly affect in these scenarios
elbow injury
hematoma in the cubital fossa (after IV infusion)
median
thoracic outlet syndrome
pain, numbness and or tingling of the upper extremity increased by overhead work caused by compression of what?
seen in painters
brachial plexus and subclavian vessels
high yield on boards
S2-4 of the sacral plexus
innervates the structures of the perineum and sensation of the genitalia
pudendal nerve
Q
vaginal tear due to instrument delivery
can cause pudendal nerve injury that can cause
urinary incontinense
loss of sexual sensation
triptans
like sumatriptan moa
selective serotonin receptor agonist
triptans (sumatriptan)
the most serious side effect is ?
coronary artery vasospasm
microscopically see
fragmentation of the internal elastic lamina and multinucleated gaint cells.
temporal arteritis
on the boards
old pt admitted to the hospital w/ acute illness
acute onset memory problem, disorganized speech, altered consciousness w/ fluctuating course
delirium
what is the highest risk for developing delirium
dementia
Q
why do alzheimers pts have early memory loss
atrophy of hippocampus
HY on the boards
alzheimer pt has dec ACH why?
loss of cholinergic neurons (acetyltransferase activity) in nucleus basalis of meynert
on boards
hyperpohosphorylated tau protein
is from a mutation on chromosome?
chromosome 14***
leads to neurofibrillary tangles - inracellular aggregates of fibers (coreelates with the degree of dementia
boards
in wilsons disease
they have neuroological symptoms including psychosis and dementia!!!!
inc LFTs and jaundice
what iwll there alkaline phosphatase level be
low alkaline phosphatase
picks disease (frontotemporal dementia)
pt has personality changes (behavioral disturbances) and inappropriate/aggressive.
what will be seen on biopsy
intracellular tau protein
rounded aggregates inside cerebral neurons
alheimers tx
1st line
donepezil
Q
tacrine (anticholinergic) used for alheimers tx
sideeffect
hepatotoxicity
Q on boards
pt diagnosed with alzhiemers is started on a medication .
pt is getting better for 3-4 months, then her physician increases the close.
one week later pt is in ER with syncope why?
donepezil is a central ach esterase inhibitor that causes
increased vagal tone –> bradycardia –> atrioventricular block
alzheimers tx
for boards
know that ginkgo biloba
does or does not imporove the global function in pts with alzheimers
does NOT
inclusions bodies and pigments for the boards
alzheimers disease
Neurofibrillary tangles
- = degenerated neurofilaments
hirano bodies
- eosinophilic, rod like inclusion bodies in the neurons of the hippocampus
inclusions bodies and pigments for the boards
dark pigments
neuromelanin
- black pigments found in the cytoplasm of the neural cells of substantia nigra
- def of neuromelanin is seen in pts with parkinsons disease
negri bodies
- eosinophilic, inclusion bodies, found in the pyramidal cells of the hippocampus and purkinje cells of the cerebellum
- know that they are pathognomonic for rabies infection
