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132 terms

Neurology

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elevated AFP, elevated ACHE
Neural tube defects
spina bifida occulata vs meningocele vs meningomyelocele
Spina bifida : failure of bony spinal canal to form
meningocele: meninges herniate
meningomyelocele: meninges and SC herniate
what disease is characterized by:
inc AFP and polyhydraminoes ?
Anencephaly : absence of skull and brain (disruption at the cranial end of neural tube)

missing the brain --> no swallowing center --> polyhydraminos
patient has cyclopia and a mutation in sonic hedgehog sigaling pathway?
holoprosencephaly - failure of L and R hemispheres to separate
cerebellar tonsillar and vermian herniation through the foramen magnum
chiari II
agenesis of cerebellar vermis:
1. what disease
2. what will you see on XR?
Dandy walker disease
cystic enlargement of 4th ventricle
cystic enlargement of canal of SC:
1. what is this disease
2. is most common at what location
3. what tract do they affect
4. what is lost in the patient with this disease
1. syringomyelia
2. C8/T1
3. Spinothalamic tract
4. loss of bilateral pain and temperature of upper extremity
1. what forms the anterior 2/3 of the tongue
2. what forms the posterior 1/3 of the tongue
3. who gives motor to the tongue
4. who gives rise to the muscles of the tongue
1. 1st branchial arch
2. 3 and 4 branchial arches
3. CN12
4. occipital myotomes
Name that cranial nerve
1. taste
2. pain
3. motor
1. 7, 9, 10
2. V3, 9, 10
3. 12
what happens to an injured axon?
wallerian degeneration - degeneration distal to injury and axonal retraction proximally --> allows for potential regeneration
+ GFAP refers to the presence of what cell? + nissl substance?
astrocyte marker
cell bodies of neurons
myelin producers of CNS? PNS?
oligodendrocytes
schwann cells
these cells are destroyed in Multiple sclerosis? guillain barre syndrome
oligodenderoglia
schwann cells
acoustic neuroma is a type of ______ that is located in the internal acoustic meatus CN VIII
Schwann cells - schwannoma
what senses do these receptors convey?
1. meissner's corpuscles
2. Pcinian corpuscles
3. Merkel's discs
1. fine and light touch, position sense
2. vibration and pressure
3. pressure, deep static touch, position sense
what receptor does this description portray?
1. large, myelinated fibers, adapt quickly
2. large myelinated fibers
3. large myelinated fibers, adapt slowly
1. Meissners corpuscles
2. pacinian corpuscles
3. merkels discs
where does the inflammatory infiltrate in guillain barre go?
endoneurium
what part of the nerve must be rejoined in microsurgery for limb reattachment
perineurium
what happens when you zap this nucleus of the HT?
1. lateral area
2. ventromedial
3. anterior
4. posterior
1. anorexia
2. hyperphagia
3. warm
4. poikilotherm, cold blooded
Name the thalamic nucleus
1. spinothalamic and dorsal column input
2. face sensation and taste info
3. motor cortex destination
4. Vision info
5. destination is the primary somatosensory cortex
6. basal ganglia input
7. pain and temperature, pressure, touch, vibration, proprioception information
8. superior olive and inferior colliculus of tectum input
9. trigeminal and gustatory pathway input
10. CN II input
11. Calcarine sulcus destination
12. hearing info
1. VpL
2. VPM
3. VL
4. LGN
5. VPL and VPM
6. VL
7. VPL
8. MGN
9. VPM
10. LGN
11. LGN
12. MGN
what are the 3 layers of the bbb
tight junctions between non fenestrated cap endo cells
BM
astrocyte foot processes
what materials can cross the BBB
glucose and aa slowly via carrier mediated transport mech
non polar/lipid soluble substances rapidly via diffusion
what causes vasogenic edema?
infarction or neoplasm destroys endocell tight junctions
where are these NT released from?
1. NE
2. Da
3. 5HT
4. Ach
5. GABA
1. locus ceruleus
2. ventral tegmentum, SN pars compacta
3. raphe nucleus
4. basal nucleus of meynert
5. nucleus accumbens
what are the symptoms of parkinson's disease
TRAP!!!
Tremor (resting, "pill rolling")
Rigidity - less facial expression
Akinesia / bradykinesia
Postural instability
explain the pathophysiology of parkinson's disease
loss of dopaminergic neurons in the substantia nigra pars compacta --> loss of ability initiate movement and inc movements --> akinesia, rigidity
lewy bodies composed of alpha synuclein + hallucinations + freq falling w/ parkinsonism + cognitive defect
lewy body dementia
loss of dopaminergic neurons (depigmentation of SN pars compacta
parkinsons disease
symptoms of huntingtons disease
chorea, athetosis, aggression, depression, dementia
pathophysiology of huntingtons
CAG trinucleotide repeats --> degneration of GABA-nergic neurons in the caudate nucleus --> loss of gaba causes inc activation of the thal --> inc activation of the cortex --> chorea
what is the term that refers to this description. RMR to recognize the one word that differentiates them:

1. sudden flailing of ONE ARM +/- IPSILAT LEG

2. sudden, jerky, purposeless movements of WHOLE BODY

3. SLOW, writhing movements in FINGERS
1. hemiballismus in contralateral subthalamic nucleus
2. chorea in Huntingtons
3. Athetosis in Huntingtons
what is the difference between myoclonus and dystonia
myoclonus : sudden, brief muscle contraction (hiccup)
dystonia : sustained involuntary muscle contraction (writers cramp, blepharospasm)
What type of tremor?
1. slow zig zag motion when pointing extending to a target
2. see in parkinsons
3. cerebellar dysfunction
4. alleviated by intentional movement
5. action tremor
1. intention tremor
2. resting tremor
3. intention tremor
4. resting tremor
5. essential tremor (postural tremor)
lesion in the amygdala leads to?
kluver bucy syndrome
what is the triad of Kluver Bucy syndrome
Hyperorality
hypersexuality
disinhibited behavior
this brain lesion is associated w/ HSV-1
amygdala, Kluver Bucy syndrome
patient has deficits in concenrtation, orientation, judgement, reemergence of primitive reflexes
frontal lobe
patient presents with spacial neglect syndrome, where is the lesion?
R parietal lobe
patient has reduced levels of arousal and wakefulness aka COMA, where is this lesion?
Reticular Activating System
an alcoholic comes to the ER with confusion, ophthalmoplegia, and ataxia. What disease do they have and where is the lesion
B1 deficiency in wernickes korsakoff syndrome in the mammillary bodies
a patient with B1 deficiency was given glocuse with out B1. what condition will they develop?
Wernicke's korsakoff sydnrome
patient presents with intention tremor, limb ataxia, and keeps fallling to the right side. where is their lesion?
R sided cerebellar hemisphere lesion
patient presents with truncal ataxia and dysarthria. where is the lesion
cerebellar vermis
patient presents with locked in syndrome, where is the myelinolysis occurring
central pontine
patient presents with hyponatremia and you corrected it too fast
central pontine myelinolysis
what is the difference between aphasia and dysarthria?
aphasia: higherorder inability to speak
dysarthria : motor inability to speak
name that aphasia:
1. non affluent aphasia w/ impaired comprehension
2. fluent aphasia but impaired comprehension
3. nonfluent aphasia but intact comprehension
4. poor repetition but fluent speech and intact compreshension
5. damage to arcuate fasciuculus
1. Global aphasia (W and B)
2. Wernickes
3. Brocas
4. Conduction
5. Conduction
a dec in O2 (hypoxemia) has what changes in the brain?
PO2 > 50 ==> NO CHANGE
PO2 < 50 ==> O2 is low so vasodialation of the vasculature of the brain --> inc blood flow (aka cerebral blood flow)
an inc in CO2 has what changes in the brain?
PCO2 inc till 90 mmHG ==> inc in cerebral blood flow
PCO2 > 90 ==> NO CHANGE IN BLOOD FLOW
which artery has been affected:
pt has contralateral paralysis and loss of sensation of UL and face
aphasia / hemineglect
MCA
which artery has been affected:
contralateral paralysis and loss of sensation in LL
ACA
which artery has been affected:
contralateral hemiparesis/hemiplegia
lateral striate artery
which artery has been affected:
contralateral hemiparesis of LL
dec contralateral proprioception
tongue deviates ipsilaterally
ASA
medial medullary syndrome
which artery has been affected:
vomitting, vertigo, nystagmus
dec pain and temp
loss of sensation to limbs and face
dysphagia and hoarseness
ipsilateral horners syndrome
ataxia and dysmetria
PICA
lateral meduallary syndrome
which artery has been affected:
contralateral hemianopia w/ macular sparing
PCA
common sites of berry aneurysm
ACOM and PCOM
Berry aneurysm is associated with what conditions
1. ADPKD
2. ehlers danlos
3. Marfans syndrome
Name that intercranial hemorrhage:
1. rupture of middle meningeal aa due to a high impact brain fracture
2. cannot cross falx
3. lucid interval
4. ass w/ aneurysms and AVMs
5. typically in basal ganglia and internal capsule
6. not crossing suture lines, lens shaped, biconvex
7. ass w/ vasospasm that u can treat with nimodipine
8. Can cross falx, tentorium
9. crescent shaped hemorrhage that crosses suture lines
10. midline shift
11. due to sys htn
1. epidural hematoma
2. subdural
3. epidural hematoma
4. subarachnoid hemorrhage
5. intraparenchymal
6. epidural hematoma
7. subarachnoid
8. epidural hematoma
9. subdural
10. subdural
11. intraparenchymal
after an ischemic brain disease/stroke when do these things show up?
1. red neurons
2. neutrophils
3. macrophages
4. reactive gliosis (granulation tissue(
5. glial scar
1. 12 - 48 hours
2. 1-3 days
3. 3-5 days
4. 1-2 weeks
5. >2 weeks
After looking at a non-contrast CT, bright areas are seen. what is contraindicated in this patient?
this patient has an hemorrhagic stroke and should not be given TPA
why can cancer cause a hemorrhagic stroke?
angiogenesis leads to formation of abnormal vessels which can bleed
what can cause an ischemic stroke?
1. Atrial fibrillation (cause stasis of blood, allows clots to form and embolize)
2. carotid dissection
3. patent foramen ovale (DVT and systemic clots and embolize to brain)
4. endocarditis
what are the conditions the patient must match before administering tPA
w/ in 4.5 horus, pt presents w/in 3 horus of onset of symptoms
no risk of hemorrhage
what is it called when there is a reversible episode of focal neuro dysf that lasts <1 hour w/ out acute infarction
TIA
what is the difference between communicating and non communicating hydrocephalus
communicating refers to a non obstructive lesion
non communicating refers to having an obstruction
Name that hydrocephalus:
1. due to atrophy of neural tissue that causes ventricles to expand
2. dec CSF absorption by arachnoid granulations
3. seen in Alzeimers, advanced HIV, Picks disease
4. urinary incontinence, ataxia, cognitive dysfunction
5. expansion of the ventricles impinge on the corona radiata
1. hydrocephalus ex vacuo
2. communicating hydrocephalus
3. hydrocephalus ex vacuo
4. Normal pressure hydrocephalus
5. Normal pressure hydrocephalus
what happens in a vertebral disc herniation
where is teh most common location
nucleus pulposus herniates through the anulus fibrosus posteriolaterally
L4/5 or L5/S1
which column is incharge of this function?
1. pressure
2. voluntary motor
3. sympathetics
4. light touch
5. crude touch
6. pain
7. proprioception
8. temperature
1. dorsal columns, anterior spinothalamic tract
2. corticospinal tract
3. intermediate horn
4. dorsal columns
5. anterior spinothalamic tract
6. lateral spinothalamic tract
7. dorsal columns
8. lateral spinothalamic tract
run through the dorsal column pathway
sensory nerve ending --> DRG --> ascends in the dorsal columns --> synapse in the gracile and cuneatus nucleus --> decussate in the medulla (CONTRALAT) --> Ascend in the medial lemniscus --> VPL of thalamus --> sensory cortex
run through the spinothalamic tract
sensory n ending --> DRG --> enter SC --> go to the ipsilateral gray matter --> decussate in the anterior white comissure --> asc CONTRALAT --> VPL --> sensory cortex
run through the lateral corticospinal tract
motor cortex --> desc internal capsule --> decussate in medually at pyramids --> desc CONTRALAT --> anterior horn --> leave SC --> NMJ
weakness, hyperreflexia, inc tone, up going babinski sign, spastic paralysis, clasp knife spasticity
UMN lesion
weakness, atrophy, fasiculations, hyporeflexia, dec tone, no BAB, flaccid paralysis
LMN lesion
lesion in the anterior horns
polio or wednig hoffman disease
lesion of white matter of cervical region of SC due to demyelination
multiple sclerosis
lesion of lateral corticospinal tract and anterior horns
ALS
loss of posterior columns
tabes dorsalis aka 3 syphilis
bilateral loss of pain and temperature
syrinx causing syringomyelia
loss of dorsal columns, lateral corticospinal tracts, spinocerebellar tracts
vit B12 def
where do the lesions that cause horners syndrome lie? and what are some examples of conditions that present with horners?
pancoast tumor, brown sequard syndrome, synringomyelia
Any lesion of SC above T1
symptoms of horners syndrome
miosis (contriction of pupils)
anhydrosis (no sweating)
rubor of face
ptosis (facial droop)
landmark dermatomes
1. C2
2. C3
3. C4
4. T4
5. T7
6. T10
7. L1
8. L4
9, S2,3,4
1. posterior half of the skull
2. high turtleneck shirt
3. low collar shirt
4. nipple
5.xiphoid
6. umbilicus
7. inguinal ligament
8. knee caps
9. erection and sensation of penile and anal zones
clinical reflexes
1. biceps
2. triceps
3. patella
4. achilles
1. C5,6
2. C7,8
3. L3,4
4. S1,2
name that reflex
1. hangin gon for life, abd and ext limbs when startled
2. curling of fingers if palm is stroked
3. movement of head to one side if cheek is stroked
4. sucking response when roof of mouth is touched
5. fanning of toes
6. stroking spine on one side, lateral flexion of lower body to that side
1. moro
2. palmar reflex
3. rooting
4. suckin g
5. plantar
6. galant
role of the pineal gland?
secrete melatonin and circaidian rhythem
paralysis of conjugate upward gaze
lesion in the superior colliculi
name the afferent and effferent CN of each reflex
1. corneal
2. lacrimation
3. jaw jerk
4. pupillary
5. Gag
1. V1, VII
2. V1, VII
3. V3, V3
4. II, III
5. IX, X
Name the function of these nuclei
1. nucleus solitarius
2. nucleus ambiguous
3. dorsal motor nuc
1. sensory information : taste, baroreceptors, gut distention
2. motor of pharynx, larynx, upper esophagus (swallowing, palate elevation)
3. parasymp fibers to the heart, lungs, upper GI
name the structures located in the cavernous sinus
1. CN 3,4,V1,V2,6
2. internal carotid aa
3. sympathetic fibers
patient has opthalmoplegia, and dec corneal and maxillary sensation with normal vision
cavernous sinus syndrome
does the organ go towards or away from the lesion?
1. CNv lesion
2. CN X lesion
3. CN 11 lesion
4. CN 12 lesion
1. toward
2. away
3. shoulder droop on the same side, rotate neck to opposite side
4. toward
what mediates conductive and sensorineural hearing?
conductive --> mediated by middle ear composed of ossicles
sensorineural --> inner ear with cochlea and hairs, CN 8
how do you perform rhine and weber tests
rhine test: vibrating tuning fork on the mastoid and the air
weber test: vibrating tuning fork on the forehead
what type of hearing loss does this patient have
1. bone conducts sound better than the air, vibrations localize to affected ear
2. air conducts sound better than the bone, vibrations localize to unaffected ear
1. conductive
2. sensorineural
damage to stereocilliated cells in organ of corti
noise induced hearing loss that leads to tympanic membrane rupture
what is charcot's classic triad of MS?
Scanning speech
Intention tremor, incontinence, Internuclear Ophthalmoplegia
Nystagmus
IgG oligoclonal bands in the CSF
MS
MRI presents w/ periventricular plaques
MS
how would you treat multiple sclerosis?
1. B interferon - dec the inflammation
2. Natalizumab --> monoclonal ab that inhibits migration of leukocytes
3. anticholinergics --> neurogenic bladder
4. Baclocfen --> activate gaba receptors to dec firing and dec spasticity
5. opiods --> pain
what is the pathophysiology of guillain barre syndrome
infection w/ campylobacter/CMV --> molecular mimicry --> Attack of schwann cells --> inflammation and demyelination --> symmetric asc weakness
attack of schwann cells
guillain barre syndrome
inc CSF protien w/ a normal cell count
guillain barre syndrome
how do you treat guillain barre syndrome
plasmapheresis
IV immune globulins
name that demyelinating disease:
1. ass w/ HIV
2. ass w/ measles
3. def in arylsulfatase A
4. def in galactocerebrosidase
5. ass w/ JC virus
6. ass w/ getting vaccinations of rabies or small pox
7. def in the production of proteins involved in the struc and func of peripheral n
1. pml
2. acute disseminated encephalomyelitis
3. metachormatic leukodystrophy
4. Krabbes disease
5. PML
6. Acute disseminated encephalomyelitis
7. Charcot marie tooth disease
what is status epilepticus
cont sx for > 30 min
recurrent sz without regaining consciousness between sx for > 30 min
name that seizure
1. blank stare
2. impaired consciousness
3. stiffening
4. drop seizure
5. consciousness intact, middle temporal lobe, aura
6. stiffening and movement
7. quick repetitive jerks
1. Absence
2. complex partial
3. tonic
4. atonic
5. simple partial
6. tonic-clonic
7. myoclonic
patient presents with a steady, bilateral headache.

why type of headache is this?
tension headache
patient with a headache presents with an aura and is highly sensitive to light and sound.
1. what type of headache is this
2. what caused this headache?
1. migraine
2. CNV, meninges, blood vessel irritation
patient presents with a brief headache >15 min that hurts only on one side.
1. what type of HA is this?
2. what symptoms are associated with this headache?
3. how do you treat?
4. what if this head ache lasted < 1 min
1. cluster
2. rhinorrhea, lacrimation
3. O2 and sumatriptan
4. trigeminal neuralgia
patient is very dizzy and has a delayed horizontal nystagmus during the positional testing
Peripheral vertigo
patient is very dizzy and has an immediate nystagmus
1. what condition
2. where is the lesion?
central vertigo
brain stem and cerebellar lesion (stroke or tumor)
port wine stain
strug weber syndrome
ipsilateral leptomeningeal angiomas, pheochromocytoma
strug weber syndrome
what conditions are ass w/ tuberous sclerosis?
HAMARTOMAS
Hamartomas of the CNS and skin
Adenoma Sebaceum
Mitral regurg
Ash leaf sports
Rhabdomyoma
Tuberous sclerosis
AD
Mental retardation
Renal Angiomyolipoma
Sz
patient has :
1. Cafe Au Lait spots
2. lisch nodules
3. neurogibromas of hte skin
4. optic glioma
5. pheochromocytomas
what disease and why?
neurofibromatosis type 1
defect in ch 17, (-) reg of Ras signalling
Name that adult primary tumor:
1. S100 positive
2. leads to bitemporal hemianopia
3. fried egg appearance
4. loc in the cerebral hemisphere
5. often localized to CN8
6. pseudopalisading tumors
7. butterfly glioma, crosses corpus calloseum
8. most commonly located in frontal lobes
9. located in the cerebellopontine angle
10. arises from arachnoid cells
11. ass w/ neurofibromatosis type 2
12. whorled pattern with psammoma bodies
1. Schwannoma
2. pituitary adenoma, craniopharyngioma
3. oligodendroglioma
4. GBM
5. Schwanoma
6. GBM
7. GBM
8. oligodendroglioma
9. schwannoma
10. meningioma
11. schwannoma
12. Meningioma
Name that child primary tumor
1. remanents of rathke's pouch
2. homer wright rosettes
3. GFAP +
4. Foamy cells + high vascularity
5. small blue cells on histology
6. inc EPO, ass w/ VHL
7. rosenthal fibers, eosinophilic, corkscrew fibers
8. perivascular pseudorosettes, rod shaped blepharoplasts
9. ass w/ 4th ventricle and leads to hydrocephalus
1. craniophyarngioma
2. meduloblastoma
3. pilocytic astrocytoma
4. hemangioblastoma
5. medulloblastoma
6. hemangioblastoma
7. pilocytic astrocytoma
8. ependymoma
9. medulloblastoma and ependymoma
vertigo lasting for Hours + low freq sensorineural hearing loss + tinnitus
1. what disease
2. why is it caused
Menieres disease
2. malfunctioning of endolymphatic sac --> inc volume of endolymph system
patient with narcolepsy has a def of which NT? what is the function of this NT?
Orexin
promotes wakefulness and prevents inappropriate entry into rapid eye movement
pt presents with falling to the ground with emotions, sees crazy things, paralyzed for mins after he wakes up
narcolepsy
lack of orexin
the region between T4-T8 is a watershed region between which BV? what deficiencies would you see if it was blocked?
vertebral and lumbar blood supply
leads to loss in pain and temperature (spinothalamic)
voluntary motor control (corticospinal)
ANS (bladder and bowel)
visual distubrances + myoclonus + eeg shows burst suppression and periodic complexes
Creutzfeldt Jakob disease
patient with a history of cardiovascular risk factrs : HTN, hyperlipidemia, DM, smoking w/ a step wise decline in cognitive func
vascular dementia
function of astrocytes
provide structural support to brain parenchyma, potassium metabolism, maintain BBB
these are the cells that form glial scars and gliosis to wall off an abscess
peripheral nerve biopsy shows brown granules in schwann cells and macrophages when stained with toluidine blue
metachromatic leukodystrophy
arylsulfatase A
name that organism
1. causes keratitis via contaminated contact lens, see spiky trophozoites
2. maltese cross appearing parasite
3. cysts in teh water and tear drop shaped trophozoites
4. amastigoes inside macrophages
1. Acanthamoeba
2. Babesia
3. Giardia Lambia
4. leishmania donovani
what are the symptoms of Gerstmanns syndrome? and where is the lesion
1. inability to dist R from L
2. acalculia
3. agraphia
4. cant identify fingers
lesion loc: angular gyrus
patient presents with: cessation of feeding, periumbilical infection, convulsions, arched back
what disease?
what organism
presentation of neonatal tetanus
C. tetani
Neurofibromatosis Type 2
Bilateral schwannoma
decreased hearing, tinnitis, impairments w/ balance if the vestibular system
ch 22
Trisomy 13 is what disease?
Patau's
- cleft lip
microphthalmia
mental retardation
polydactyly
congenital heart disease
Renal defects
deletion of 11p13
WAGR
wilms tumor, anirdia, genital anomalies, mental retardation
ring enhacing lesions w/ surrounding edema and mass effect + seizure + chorioretinitis
toxoplasmosis