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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?

schwann cells

these cells are destroyed in Multiple sclerosis? guillain barre syndrome

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?


what part of the nerve must be rejoined in microsurgery for limb reattachment


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
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

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

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

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

which artery has been affected:
pt has contralateral paralysis and loss of sensation of UL and face
aphasia / hemineglect


which artery has been affected:
contralateral paralysis and loss of sensation in LL


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

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

lateral meduallary syndrome

which artery has been affected:
contralateral hemianopia w/ macular sparing


common sites of berry aneurysm


Berry aneurysm is associated with what conditions

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


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


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
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

IgG oligoclonal bands in the CSF


MRI presents w/ periventricular plaques


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

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 of the CNS and skin
Adenoma Sebaceum
Mitral regurg
Ash leaf sports
Tuberous sclerosis
Mental retardation
Renal Angiomyolipoma

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?

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

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?

- cleft lip
mental retardation
congenital heart disease
Renal defects

deletion of 11p13

wilms tumor, anirdia, genital anomalies, mental retardation

ring enhacing lesions w/ surrounding edema and mass effect + seizure + chorioretinitis


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