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219 Cards in this Set

  • Front
  • Back
What neural cells come from:

Neuroectoderm?
Neural crest?
Mesoderm
Neuroectoderm: CNS neurons, ependymal cells, oligodendroglia, astrocytes

Neural creset: Schwann cells, PNS neurons

Mesoderm (M's): microglia (like macrophages)
what cells are destroyed in MS?

What cells are destroyed in Guillain-Barre?
oligodendroglia

Schwann cells
How are the levels of the following NTs changed in disease?

1. NE
2. Dopamine
3. 5-HT
4. Ach
5. GABA
1. NE: high in anxiety, low in depression

2. Dopamine: high in schizophrenia, low in Parkinson's and depression

3. 5-HT: low in depression, anxiety

4. Ach: low in Alzheimer's, Huntington's, and REM sleep

5. GABA: low in anxiety, Huntington's
Which receptor type is carried by C fibers?

fxn of the receptors?
free nerve endings (pain and temperature)
where are these NT synthesized?
NE
Dopamine
5-HT
ACh
GABA
NE: locus ceruleus
Dopamine: Ventral tegmentum and SNc
5-HT: Raphe nucleus
ACh: basal nucleus of meynert
GABA: Nucleus accumbens
what part of brain is involved in stress and panic?

what part of brain is involved in reward center, pleasure, addiction?
Locus ceruleus: stress and panic

Nucleus accumbens and septal nucleus: reward center, pleasure, addiction
What 3 structures make up the Blood-brain barrier?
tight junctions (b/w non-fenestrated capillary endothelial cells)
basement membrane
astrocyte processes
List 2 sensory receptor types involved in position sense and touch.

How are they similar? different?
Meissner's corpuscles: dynamic fine touch, adapts fast
Merkel's disks: static touch, adapts slow

both sense position sense and carried by large, myelinated fibers (fast conduction)
What are the few specialized brain regions w/NO blood-brain barrier
area postrema (vomiting after chemo)
neurohypophysis (ADH release; also other neurosecretory products to enter circulation)
What are the few specialized brain regions w/NO blood-brain barrier
area postrema (vomiting after chemo)
OVLT (osmotic sensing)
neurohypophysis (ADH release; also other neurosecretory products to enter circulation)
What are the functions of the hypothalamus?
TAN HATS
T: Thirst and water balance
A: Adenohypophysis control
N: neurohypophysis (release hormones from hypothalamus)
H: Hunger
A: Autonomic regulation
T: Temp regulation
S: sexual urges
Where is ADH made
Supraoptic nucleus of hypothalamus
Where is oxytocin made
paraventricular nucleus of hypothalamus
what controls cooling
anterior hypothalamus (parasympathetic)
(A/C: Anterior Cools)
what controls heating
posterior hypothalamus
(sympathetic)
if zap Posterior hypothal, become a Poikilotherm (cold-blooded like a snake)
what controls hunger
lateral area of hypothalamus
destruction leads to anorexia and failure to thrive in infants
inhibited by leptin
what controls satiety
ventromedial area of hypothalamus
destruction (cranipharyngioma) -> hyperphagia
stimulated by leptin
where is visual information in the thalamus
lateral geniculate nucleus
(Lateral for Light)
what control sexual urges
septal nucleus hypothalamus
Where is auditory information in thalamus
Medium geniculate nucleus
(Medial for Music)
what controls circadian rhythm
suprachiasmatic nucleus of hypothalamus
What information is sent to the Ventral Posterior nucleus, lateral (VPL)?
body sensation (proprioception, pressure, pain, touch, vibration via dorsal column, spinothalamic tract)
What information is sent to the Ventral posterior nucleus, medial (VPM)?
facial sensation via CN 5 (Makeup on face, so vpM)
What information if sent to Ventral anterior/lateral nuclei (VA/VL)
motor
What is the blood supply to the thalamus?
Pcomm (posterior communicating),
PCA (posterior cerebral),
ICA (anterior choroidal arteries)
Input to cerebellum?
contralateral cortical input via MCP
ipsilateral proprioceptive info via ICP

input nerves are climbing and mossy fibers
Output from cerebellum?
stimulatory feedback to contralateral cortex to modulate movement

output nerves are Purkinje fibers to deep nuclei of cerebellum, which output to cortex via SCP
What are the deep nuclei of the cerebellum, lateral to medial?
Don't Eat Greasy Foods

Dentate, Emboliform, Globose, Fastigial
What does the lateral cerebellum control
voluntary movement of extremities
What does the medial cerebellum control?
truncal coordination, balance, ataxia,
propensity to fall toward injured (ipsi) side
What is the excitatory pathway in the basal ganglia?
SNc dopamine bind to D1 receptors in excitatory pathway -> stimulate excitatory pathway -> increase motion

cortex -> striatum -> GPi/SNr -> thalamus -> cortex
What is the inhibitory pathway in the basal ganglia?
SNc dopamine bind to D2 receptors in inhibitory pathway -> inhibit inhibitory pathway -> increase motion

cortex -> striatum -> GPe -> subthalamic nucleus -> GPi -> thalamus -> cortex
How does Parkinson's dz affect the excitatory/inhibitory pathway in the basal ganglia?
loss of dopamine inhibits the excitatory pathway

and excites inhibitory pathway (disinhibits)

both -> decrease in motion
Typical characteristics of Parkinson's dz
TRAP
T: Tremor (at rest, pill-rolling)
R: cogwheel Rigidity
A: Akinesia
P: Postural instability

TRAPped in body
What is Hemiballismus?
sudden, wild flailing of 1 arm +/- leg

contralateral subthalamic nucleus lesion (lacunar stroke in pt w/Hx of HTN)

get loss of inhibition of thalamus through globus pallidus
Huntington's dz:
What is the mechanism of neuronal death?
What are the gross pathology?
associated sxs?
Mechanism: NMDA-receptor binding and glutamate toxicity

Gross: atrophy of caudate nucleus (loss of GABA and Ach), enlarged lateral ventricles, atrophy of putamen and defined sulci

chorea, athetosis, oculmotor abnormalities, depression, aggression
what are the main components of the basal ganglia?
striatum, pallidum (globus pallidus), substantia nigra, and subthalamic nucleus

striatum: caudate nucleus (cognitive) and putamen (motor)
What is an essential/postural tremor?
How do you treat it?
Autosomal dominant,
action tremor (worsens when holding posture),
pts often self-medicate w/EtOH, which decreases tremor

Trx: B-blockers
What is Kluver-Bucy syndrome?
Amygdala (bilateral) lesion

hyperorality, hypersexuality, disinhibited behavior
What is an essential/postural tremor?
How do you treat it?
Autosomal dominant,
action tremor (worsens when holding posture),
pts often self-medicate w/EtOH, which decreases tremor

Trx: B-blockers (propranolol)
what happens with a lesion in the reticular activating system (RAS)?

where is the RAS located?
reduced levels of arousal and wakefulness (e.g., coma)

located in the midbrain
Name the characteristics of Wernicke-Korsakoff syndrome.

What lesion is it associated with?
Wernicke: confusion, ophthalmoplegia, ataxia
Korsakoff: confabulation, memory loss, personality changes

assoc w/mammillary bodies lesion (bilateral)
What happens with a cerebellar hemisphere lesion?

w/a cerebellar vermis lesion?
hemispheres are lateral -> affect lateral limbs
vermis is central -> affect central body

hemisphere: intention tremor, limb ataxis, IPSIlateral deficits, fall toward side of lesion (cerebellum -> SCP -> contralateral cortex -> corticospinal decussation = ipsilateral)

vermis: truncal ataxia, dysarthria
What happens w/a lesion in the PPRF (paramedian pontine reticular formation)?

w/a frontal eye fields (FEF) lesion?
PPRF: eyes look AWAY from side of lesion

FEF: eyes look TOWARD side of lesion
Where is Broca's area located?

Wernicke's area?
Broca's: inferior frontal gyrus

Wernicke's: superior temporal gyrus (associative auditory cortex)

both in the dominant hemisphere
What is a conduction aphasia?
Poor repetition but fluent speech and intact comprehension

affects arcuate fasciculus, that connect broca's and wernicke's areas
What happens w/a Right parietal lobe lesion?
spatial neglect syndrome (agnosia of contralateral side of world)
Central pontine myelinolysis

sxs?
cause?
caused by very rapid correction of hyponatremia

acute paralysis, dysarthria, dysphagia, diplopia, and loss of consciousness
Lateral medullary syndrome?
Wallenberg's syndrome, PICA (post. inf. cerebellar a.)

1. Spinothalamic tract: contralateral loss of pain and temp
2. CN V nucleus/tract: Ipsi loss of pain/temp on face
3. Nucleus ambiguus (CN IX, X): ipisilateral dysphagia, hoarseness, decrease gag reflex
4. Descending sympathetic: Horner's syndrome, diplopia
5. vestibular nucleus/tract: vertigo, nystagmus
6. Cerebellum: ipsi ataxia
what happens w/a posterior cerebral artery lesion?
contralateral homonymous hemianopia w/macular sparing,
supplies the occipital cortex
what happens w/an MCA lesion?
contralateral face and arm paralysis and sensory loss, aphasia (dominant sphere), left-sided neglect
lateral inferior pontine syndrome?
AICA

1. CN VII nucleus/tract: ipsi facial paralysis
2. CN VIII nucleus/trct: ipsi cochlear nucleus, vestibular (nystagmus),
3. Middle/inf cerebellar penducle: ipsi dystaxia
4. CN V nucleus/tract: temp, pain loss of ipsi face
What is the most common site of circle of Willis anuerysms
Anterior communicating artery

lesion may cause visual field deficit
What artery is a common area of aneursyms?

what palsy can it cause?
Posterior communicating artery,

can cause CN 3 palsy
What areas do lateral striate arteries supply?

what is the consequence of an assoc. lesion?
supply internal capsule, caudate, putamen, globus pallidus,

are "arteries of stroke." infarct of the posterior limb of internal capsule causes pure motor hemiparesis
An infarct in which artery causes "locked-in"syndrome?
Basilar artery (CN 3 is typically intact)
What artery is a common area of aneurysm that can cause CN III palsy?
Posterior communicating artery,
What happens with a watershed zone infarct?

When do these infarcts clinically happen?
upper leg/upper arm weakness, defects in higher-order visual processing

happen from damage in severe HYPOtension

located b/w ACA/MCA, MCA/PCA
What is the risk of subarachnoid hemorrhage 2-3 days after?
vasospasm due to blood breakdown products which irritate vessels. Trx w/Ca-channel blockers
Berry aneurysm: most common site? risk factors

Charcot-bouchard aneurysm: affected sites? the risk factor?
Berry aneurysm:
Anterior communicating cerebral artery
Marfan's, Ehlers-Danlos, ADPKD

Charcot-Bouchard microaneurysm:
small vessels (e.g. in basal ganglia and thalamus)
associated with chronic HTN
what are the etiologies of ischemic stroke?
AFib, carotid dissection, PFO, endocarditis

trx: is tPA w/in 3 hours
describe flow of ventricular system?
Lateral ventricle -> 3rd ventricle (via foramen of monro) -> 4th ventricle (via cerebral aqueduct) -> subarachnoid space (via foramina of Luschka and Magendie)

Luschka is Lateral; Magendie is Medial
what are the most vulnerable parts of the brain to ischemia?
hippocampus (pyramidal cells)
neocortex (pyrmidal cells)
cerebellum (purkinje cells)
watershed areas
What are the characteristics of Normal pressure hydrocephalus?
Wet, Wobbly, Wacky

Ventricle expansion distorts fibers of corona radiata -> urinary incontinence, ataxia, and dementia (reversible cause of dementia in elderly)

NO increase in subarachnoid space volume
List order of structures pierced in a lumbar puncture

At what level? landmark for finding the level?
contraindication? why?
1. Skin/superficial fascia
2. Ligaments (supraspinous, interspinous, ligamentum flavum)
3. Epidural space
4. Dura mater
5. Subdural space
6. Arachnoid
7. subarachnoid space - CSF

Pia is NOT pierced

LP in L3/L4 or L4/L5 at the level of cauda equina (at the level of iliac crest)

contraindicated in papilledema bc LP can cause tonsilar herniation
Poliomyelitis

affected site? lost fxns?
mode of transmission?
anterior horn cell destruction -> LMN destruction (atrophy/weakness, fasciculation, fibrillation, hyporeflexia)

fecal-oral transmission and virus replicates in oropharynx and small instestine before spreading through blood to CNS

CSF has lymphocytic pleocytosis w/slight elevation of protein (NO glucose change)

virus recovered from stool and throat
Werdnig-Hoffman dz
infantile spinal muscular atrophy (SMA)

auto-recessive; presents at birth as "floppy baby," tongue fasciculations, median age of death 7 months

anterior horn degeneration. only LMN involvement
Tabes dorsalis: characteristics
Charcot's joints, shooting (lightning) pain, Argyll-Robertson pupils, ABSENCE OF DTRs, positive Rhomberg, sensory ataxia at night
Friedreich's ataxia: characteristics
staggering gait, frequent falling, nystagmus, dysarthria, pes cavus, hammer toes, HYPERTROPHIC CARDIOMYOPATHY (cause of death). presents in childhood w/kyphoscoliosis

is the FRatastic (frataxin gene GAA repeat, chrom 9) frat brother, always falling, staggering
Loss of both LMN and UMN

dx?
causes?
common presentation?
Amyotrophic lateral sclerosis (AML or Lou Gehrig's disease)
caused by defect in superoxide dismutase 1 (SOD1) , betel nut ingestion

commonly presents as fasciculations
What are the findings in Brown-Sequard syndrome?
Ipsilateral: UMN signs below lesion (corticospinal), loss of tactile/vibration/proprioception sense below lesion (dorsal columns), loss of all sensation AT level of column

Contralateral: pain and temp loss below lesion (spinothalamic)

LMN signs AT level of lesion
What if a Brown-Sequard syndrome lesion occurs above T1?
pt will present with Horners's syndrome
Causes of Horner's syndrome?
Pancoast tumor, Brown-Sequard, late-stage syringomyelia

assoc w/lesions of cord above T1
What are the dermatome landmarks?
T4
T7
T10
L1
L4
S2 - S4
T4: nipple
T7: xiphoid process
T10: umbilicus (important for appendicitis pain referral)
L1: inguinal ligament
L4: kneecaps
S2- S4: erection and sensation of penile anal zone
Moro reflex

what is it?
When is it observed? (2)
"hang on for life": abduct/extend limbs when startled, and then draw together

in the first year of life. may reemerge following frontal lobe lesion
What cranial nerves lie medially in the brain stem?

where is the pineal gland? fxn?
CN 3, 6, 12 (motor = medial)

pineal gland is located dorsally superior to the colliculi.
fxn: melatonin secretion, cracadian rhythm
What is parinaud syndrome?
paralysis of conjugate vertical gaze due to lesion in superior colliculi (e.g., pinealoma)
Where are the cranial nerve nuclei located in the brainstem

What nuclei are in the midbrain?
in the pons?
in the medulla?
Tegmentum portion (b/w dorsal and ventral portions)

midbrain: CN 3, 4
pons: CN 5-8
medulla: CN 9-12
What cranial nerve nuclei and information is sent by the Nucleus Solitarius?
Visceral Sensory info (taste, baroreceptor, gut distention)

CN 7, 9, 10
What cranial nerve nuclei and information is sent by the Nucleus Ambiguus?
Motor Innervation of pharynx, larynx, and upper esophagus (swallowing, palate elevation)

CN, 9-11
What info does the dorsal motor nucleus send?
sends autonomic (PARAsympathetic) fibers to heart, lungs and upper GI
What is cavernous sinus syndrome?
(from mass effect): ophthalmoplegia, ophthalmic and maxillary sensory loss

nerves that control extraocular mm (plus V1 and V2) also pass through cavernous sinus
What happens w/a LMN lesion of CN 7

What happens w/an UMN lesion of CN 7?
LMN: ipsilateral paralysis of upper and lower face

UMN: contralateral paralysis of lower face ONLY b/c upper face is innervated bilaterally
What are some causes of Bell's palsy?

Findings?
AIDS, Lyme dz, Herpes zoster, Sarcoidosis, Tumors, Diabetes

(ALexander graHam Bell w/STD)

complete destruction of facial nucleus or peripheral branches --> ipsilateral facial paralysis with inability to close eye on involved side
What innervates palatoglossus?

What innervates tensor veli palatini?
palato: CN X
(every other "glossus" m innervated by CN XII)

tensor veli palatini: CN V (V3, mandibular br.)
(every other "palat" muscle innvervated by CN X)
Epidural hematoma vs Subdural hematoma

rupture vessel?
CT shows?
epidural hematoma: rupture of middle menigeal artery, biconvex disk on CT; can cross falx, tentorium but no suture lines

Subdural hematoma: rupture of bridging veins, Crescent-shaped hemorrhage on CT; crosses suture lines but does not cross falx, tentorium
Subarachnoid hemorrhage

presentation?
cause?
"worst headache of my life"

rupture of a berry aneurysm in Marfan's, Ehlers-Danlos, APCKD, or AVM
parenchymal hematoma

causes?
typical locations?
causes:
chronic HTN (Charcot-Bouchard aneurysm)
cerebral amyloid angiopathy
tumor, DM

typicaly basal ganglia and internal capsule
Describe the timeline of irreversible neuronal injury from first 12 hrs to after 2 weeks.
12-48 hrs: red neurons
24-72 hrs: necrosis + neutrophils
3-5 days: foamy macrophages
1-2 weeks: reactive gliosis + vascular proliferation
> 2weeks: glial scar
Transient ischemic attack (TIA)
brief, reversible episodes of neurologic dysfxn to focal ischemia.

typically, sxs last for < 24 hrs
Stroke imaging

how does it look on CT? MRI?
MRI: bright for 10 days

CT: dark in 24 hrs
Hydrocephalus ex vacuo

pathogenesis?
associated diseases?
how is it compared to normal pressure hydrocephalus?
appearance of increased CSF due to atrophy
seen in Alzheimer's advanced HIV, Pick's disease, Huntington's

normal intracranial pressure but no triad seen (dementia, ataxia, urinary incontinence)
Scanning speech, intention tremor, nystagmus, MLF lesion

dx?
Multiple sclerosis
What 2 nutrition defiencies can have similar presentations as Freidreich's ataxia?
Vitamin B12 neuropathy
vitamin E deficiency

ataxic gait, hyperreflexia, impaired position, vibraiton sense
Muscle spindle vs Golgi Tendon organ

fxn of each?
how is muscle spindle maintain its sensitivity?
Muscle spindle monitors muscle length by stimulating alpha neurons to contract the extrafusal fibers

Golgi tendon organ monitors muscle tendon by inhibit contraction

Muscle spindle's sensitivity is controlled by intrafusal fibers, which are contracted with gamma neuron stimulation (influenced by the brain)
Alpha motor neuron vs gamma motor neuron
alpha: innervates extrafusal fibers for muscle contraction

gamma: innervates intrafusal fibers for maintaining the sensitivity of the muscle spindle
What nerves innervate salivation?
Describe the routes from nuclei to the effect?
CN VII: submandibular, and subligual gland
reaches the effector glands via superior salivatory nucleus -> chorda tympani n. -> lingual n. -> glands

CN IX: parotid gland
Inf salivatory gland -> otic ganglion -> auricotemporal n. (CN V) -> parotid gland
What nerves receive taste informaiton? be specific
CN VII: anterior 2/3 of tongue

CN IX: posterior 1/3 of tongue

CN X: taste from epiglottic region
what nerve innervates the lacrimal glands?
CN VII
Which nerves are involved in the following reflexes?

1. corneal
2. lacrimation
3. gag
1. corneal: V1 (nasociliary branch) -> VII (temporal branch)

2. lacrimation: V1 -> VII; loss of reflex does not preclude emotional tear

3. gag: IX -> IX, X
Nucleus Solitarius

fxn?
what nerves are associated?
location?
one of the vagal nuclei in the pons
located laterally (sensory)
Visceral sensory info: taste, baroreceptors, gut distension

VII, IX, X
What CN nucleus is involved in motor innervation of the viscera?
dorsal motor nuclei (vagus)

sends parasympathetic fibers to heart, lungs, and upper GI
Superior orbital fissure

location?
what goes through it? (6)
in the middle cranial fossa through sphenoid bone

CN III, IV, V1, VI, opthalmic vein, sympathetic fibers
How do trigeminal fibers reach their destination from the brainstem?
through holes in the middle cranial fossa (through sphenoid bone)

Standing Room Only
Superior orbital fissure: V1
Foramen Rotundum: V2
Foramen Ovale: V3
Internal auditory meatus

location?
what goes through it?
posterior cranial fossa

CN VII, VIII
jugular foramen

location?
what goes through it?
posterior cranial fossa
CN IX, X, XI, jugular vein
Cavernous sinus

contents?
which is free floating?
CN III, IV, VI, V1, V2
Internal carotid artery
Sympathetics (postganglionic)

CN VI is free floating
CN X lesion

presentation of uvula?
uvula deviates to the opposite of the lesion (affected side is weakened)
What CNs are tested with "kuh, la, mi" sounds?
kuh: CN IX
la: CN XII
mi: CN VII
What muscle opens the jaw?

what muscles close the jaw?
open: gravity and lateral pterygoids

close: temporalis, masseter, medial pterygoids
What ion is the major content of endolymph?

how about perilymph?
Peri: like ECF; Na+
endo: like ICF: K+
List 3 inner ear structures that senses acceleration.
Utricle: horizontal
saccule: vertical
semicircular canal: angular
what type of sound is detected at the helicotrema end of the cochlea?
high frequency
What type of sound is lost first in the elderly?
high frequency
Weber test lateralizes to the left
Rinne: AC>BC

dx?
Right sided sensorineural hearing loss
Weber test lateralizes to the right
Rinner: BC>AC

dx?
right sided conductive hearing loss
Describe the sensory innervation of the tympanic membrane.
Outer: V3 (auriculotemporal)
Inner: CN IX via tympanic brach
Describe the sensory innervation of external auditory duct.
Most: V3 (auriculotemporal)

posterior part: auricular branch of CN X
Amaurosis fugax vs retinal artery occlusion

compare and contrast
both cause painless monocular vision loss

amaurosis fugax: transient; only lasts a few seconds; due to a small embolus to the opthalmic artery

Retinal artery occlusion: presents with cherry red macula and pale retina
ciliary muscle of the eye

fxn?
receptor?
accomodation
M3
What do you see on the fundoscopic exam in glaucoma pts?

How is this different from pts with papilledema?
glaucoma: due to impaired flow of aqueous humor --> increase intraocular pressure --> optic disk atrophy with cupping

papilledema: due to increased intracranial pressure --> elevated optic disk with blurred margins, bigger blind spot
Open angle glaucoma vs closed angle glaucoma
open angle: due to obstructed outflow

closed/narrow: obstruction of flow b/t iris and lens
What drugs are contraindicated in glaucoma pts?
anti-muscarinics: will inhibit contraction of sphincter constrictor muscle

epinephrine (only in closed): will contract dilator/radial muscle, which will narrow the pathway for the flow
List risk factors for cataracts. (only list risk factors related to sugar metabolism)
classic galactosemia, galactokinase deficiency, diabetes (sorbitol)
ocular presentation of CN III damage
eye looks down and out; ptosis, pupillary dilation, loss of accomodation
ocular presentation of CN IV damage
diplopia with defective downward gaze

adjust by tilting head toward lesion (if lesion is before crossing)
How do you test for inferior oblique muscle of the eye?
IOU

to test Inferior Oblique, have pt look Up
Strabismus
Amblyopia
strabismus: misalignment of eyes.

Amblyopia: reduction of vision from disuse in critical period
What muscle produces mydriasis?
Describe how this muscle is innervated.
radial muscle

T1 pregang sympathetic -> superior cervical ganglion -> postgang -> long ciliary nerve
what muscle produces miosis?
Describe how this muscle is innervated?
pupillary sphincter muscle

CN III form Edinger-Westphal nucleus --> ciliary ganglion
Marcus Gunn Pupil
afferent pupil defect --> decreased bilateral pupillary constriction when light is shown in affected eye
How does diabetes affect CN III?

How does compression affect CN III?
diabetes -> vascular disease -> affects inner layer of CN III -> affects extraocular muscles

compression -> affects the outer layer -> affects parasympathetics (accomodation and miosis)
What 2 layers are separated in retinal detachment?
neurosensory layer of retina from pigment epithelium
Dry ARMD vs wet ARMD
age-related macular degeneration

dry: slow, due to fat deposits and cause gradual loss of vision

wet: rapid, due to neovascularization
Describe the visual defect

1.Right Lesion in dorsal optic radiation
2. Pit tumor
1. dorsal optic radiation: Left lower quadrantic anopia (right parietal lesion, MCA)

2. pit tumor: bitemporal hemianopia
Describe the visual defect

1. right Meyer's loop lesion
2. right PCA occlusion
1. left upper quadrantic anopia

2. left hemianopia with macular sparing b/c macula receives bilateral input
right MLF damage

presentation
when asked to look left

right eye: medial rectus palsy
left eye: right beating nystagmus
explain the caloric test
Caloric test is used to exam vestibular apparatus

COWS: nystagmus with quick phase correction

Cold water: nystagmus toward the lesion with quick phase to Opposite side
Warm water: nysgatmus to opposite side with quick phase to Same side
List genes associated with early onset Alzheimer's disease.

late onset gene?
early onset: APP (chrom 21), presenilin-1 (chrom 14), presenilin-2 (chrom 1)

late onset: ApoE4 (19)
What gene is protective from Alzheimer's disease?
ApoE2 (19)
What is the gross finding of Alzheimer's disease?

List 2 histological findings in Alzheimer's.
widespread cortical atrophy (esp. temporal lobe)

Senile plaques (extracellular beta- amyloid)
Neurofibrillary tangles (abnormally phosphorylated tau protein)
What histological finding correlates with degree of dementia in Alzheimer's?
Neurofibrillary tangle
what is the complication of senile plaques?
amyloid angiopathy --> intracranial hemorrhage
List 2 diseases associated with abnormal tau protein.
Alzheimer's
Pick's disease (frontotemporal dementia)
What lobes are atrophied in Pick's disease? spared?
atrophy of frontotemporal lobe, but spares posterior 2/3 of superior temporal gyrus
Parkinsonism with dementia and hallucinations

dx?
hallmark finding?
Lewy body dementia

lewy body: defective alpha-synuclein
Charcot's triad of MS
SIN

Scanning speech
Intention tremor, Incontinence, Internuclear ophthalmoplegia
Nystagmus
MS

affected population?
diagnostic finding?
histological finding?
white women in 20s and 30s
diagnostic finding: IgG oligoclonal band
histo: periventricular plaques with preservation of axons
Guillain-Barre syndrome

pathogenesis?
lab findings?
autoimmune attack of peripheral myelin due to molecular mimicry (e.g. Campylobacter jejuni or herpesvirus infection)

High CSF protein with normal cell count (albuminocytologic dissociation
increased protein -> papilledema
Symmetric ascending muscle weakness beginnign in distal extremities. 50% are affected facial paralysis. Autonomic fx may be affected.

dx?
Guillain-Barre syndrome (acute inflammatory demyelinating polyradiculopathy)

affects the peripheral nerves and motor fibers of ventral roots
Demyelinating disease associated with JC virus?

affected population?
PML (progressive mutlfocal leukoencephalopathy)

seen in AIDS pts
arylsulfatase A deficiency. Buildup of sulfatides

dx?
Metachromatic leukodystrophy
originates from mesial temporal lobe, one area of brain, and pt is unaware of the seizure

what type of seizure?
partial, complex
alternating stiffening and movement

what type of seizure?
tonic-clonic (grand mal)
"drop" seizures

what type of seizure
atonic
tx for trigeminal neuralgia?
Carbamazepine (inhibit high frequency firing by preventing Na channels to recover from inactivation)
Migraine

presentations?
pathogenesis?
tx?
unilateral; 4-72 hrs of pulsating pain with nausea, photophobia. +/- aura of neurologic sxs before headache, including visual, sensory, speech disturbances

due to irritation of CN V and release of substance P, CGRP, vasoactive peptides

tx: propranolol, NSAIDs, and sumatriptan (acute migraines)
Cluster headache

presentation?
unilateral
repetitive brief headaches characterized by periorbital pain associated with ipsilateral lacrimation, rinorrhea, Horner's syndrome
Meniere's disease

pathogenesis?
sx?
increased endolymph in inner ear and loss of cochlear hairs

peripheral vertigo (illusion of mvmt)
How do you differentiate b/t peripheral vertigo and central vertigo?
use positional testing

Peripheral: delayed horizontal nystagmus
Central: immediate nystagmus in any direction (usually due to PICA or AICA occlusion)
Port-wine stains in V1 opthalmic distribution, ipsilateral leptomeningeal angiomas, pheochromocytoma

dx?
Sturge-Weber syndrome
ash leaf spots, sebeaceous adenoma, hamartomas, cardiac rhabdomyoma, renal angiomyolipoma, subependymal giant cell astrocytoma

dx?
Tuberous sclerosis

TSC1: chrom 9
TSC2: chrom 16
von Recklinghausen's disease

aka?
associated gene and chrom?
presentation?
neurofibromatosis 1
mutated NF-1 gene on chrom 17

Cafe-au-lait spots, Lisch nodules (pigmented iris hemartomas), neurofibromas in skin, optic gliomas, pheochromocytoma
von Hippel-Lindau disease

mutation?
presentation?
mutated tumor suppressor VHL on chrom 3

cavernous hemagiomas in skin, mucosa, organs
bilateral renal cell carcinoma
Hemagioblastoma in retina, brain stem, cerebellum
pheochromocytoma
Glioblastoma multiforme

stain?
histology?
stains astrocytes for GFAP

pseudopalisating pleomorphic tumor cells: border central areas of necrosis and hemorrhage
whorled pattern
psammoma bodies

what brain tumor?
meningioma
S100 positive, bilateral, Antoni A/B pattern cells

what brain tumor?
bilateral Schwannoma found in NF2
Chicken-wire capillary pattern, Fried egg cells

what brain tumor?
Oligodendroglioma
Most common tumor in children?
2nd most common in children?

Most common location of brain tumor in children?
most common: pilocytic astrocytoma
2nd: medulloblastoma

infratentorial
Perivascular pseudorossettes in the brain

ddx? (2)
medulloblastoma

ependymoma
histology of medulloblastoma?
rosettes or perivascular pseudorossette pattern of cells

small blue cells
List 4 types of herniation in herniation syndrome
1. cingulate (subfalcine) herniation under falx cerebri
2. downward transtentorial (central) herniation
3. uncal herniation
4. Cerebellar tonsillar herniation into foramen magum
Most severe complication of brain herniation?
compress brainstem -> coma and death
4 clinical signs of uncal herniation
1. ipsilateral dilated pupil/ptosis: stretching of CN III
2. contralateral homonymous hemianopia with macula sparing: compression of ipsilateral PCA
3. Ipsilateral paresis: compression of contralateral crus cerebri (Kernohan's notch)
4. Duret (pontine) hemorrhage: paramedian artery rupture
ddx for ring-enhancing lesion on CT.
metastases, abscesses, toxoplasmosis, AIDS lymphoma
List 5 classes of drugs used in glaucoma
1. alpha agonists (epi and brimonidine)
2. beta blockers
3. Acetazolamide
4. Cholinomimetics
5. Prostaglandin: Latanoprost
Brimonidine

MOA?
Indication?
alpha2 agonist
decrease aqueous humor synthesis
what drug is indicated in Emergency glaucoma?

MOA?
Pilocarpine (direct cholinomimetic)

contract ciliary muscle and open trabecular meshwork
Methadone

MOA?
Indication? why?
agonist at opioid mu receptor and opens K+ channels and close Cl- channels -> decrease synaptic tranmission

indicated for maintenance programs for addicts b/c it has a long half-life, which suppresses the withdrawal sxs and reduces risks of addiction
Morphine

toxicity?
what sxs do not develop tolerance?
addiction, respiratory depression, additive CNS depression wither other drugs, miosis, constipation

Miosis and constipation do not develop tolerance
Tramadol

class/MOA?
toxicity?
very weak opioid agonist; SNRI

decreases seizure threshold
Phenytoin

MOA?
indication?
toxicity?
increases Na channel inactivation

indicated for seizures; first line for tonic-clonic and status epilepticus prophylaxis

Gingival hyperplasia, megaloblastic anemia, fetal hydantoin syndorme, SLE-like syndrome, induction of cytochrome P450
1st line tx for status elipticus prophylaxis
phenytoin
1st line tx for acute sxs of status epilepticus
Diazepam and lorazepam
1st line tx for absence seizure
MOA?
Ethosuximide

blocks thalamic T type Ca channels
1st line tx for tonic-clonic generalized seizure (3)
Phenytoin
Valproic acid
Carbamazepine
First line tx for trigeminal neuralgia.
carbamazepine
first line tx for seizures in pregnant women and children
phenobarbital
first line tx to prevent seizures of eclampsia
MgSO4
Carbamazepine

class/MOA?
toxicity?
anti-seizure medication; increases inactivation of Na channels

agranulocytosis, aplastic anemia, teratogenesis, induction of P450, SIADH, Stevens-Johnson syndrome
List anti-seizure medications associated with Stevens-Johnson syndrome.

What is Stevens-Johnson syndrome?
Carbamazepine, Ethosuximide, lamotrigine

SJ syndrome: allergic rxn to the drugs; prodrome of malaise and fever followed by rapid onset of erythematous/purpuric macules (oral, ocular, genital). Skin lesions progress to epidermal necrosis and sloughing
Valproic acid

toxicity?
rare but fetal hepatotoxicity, spina bifida in fetus

contraindicated in pregnancy
Barbiturates

MOA?
toxicity?
contraindication?
facilitate GABAa axn by increasing the DURATION of Cl- channel opening, thus decreasing the neuron firing

additive CNS depression with EtOH, respiratory or CV depression, P450 induction

contraindicated in porphyria
Benzodiazepines

MOA?
Advantage over barbiturates?
facilitate GABAa axn by increasing frequency of Cl channel opening. Decreased REM sleep

less risk of respiratory depression and coma than with barbiturates
what drug is used for detoxification, esp EtOH withdrawal?
long acting benzo: diazepam and chlordiazepoxide
list 3 short-acting benzodiazepines.

side effect?
TOM Thumb
Tirazolam
Oxazepam
Midazolam

highly addictive
how do you tx overdose of benzodiazepine?
flumazenil: competitive antagonist at GABA receptor
N2O as inhaled anesthetic

induction rate?
potency?
toxicity?
low blood solubility -> fast induction
low lipid solublity -> low potency

expansion of trapped gas
Halothane as inhaled anesthetic

induction rate?
potency?
toxicity?
high blood solubility -> slow induction
high lipid solubility -> high potency

hepatotoxicity
how is potency measures?

how does AV concentration gradient related to onset of action?
potency = 1/ MAC
MAC: minimum alveolar conc at which 50% of population of anesthesized

high AV conc gradient = high solubility = more gas required to saturate tissue = SLOWER onset of action
Thiopental

class?
indication? why?
barbiturate

used for induction of anesthesia b/c it has high potency, high lipid solubility, rapid entry into brain
what is the most common anesthetic used for endoscopy?
Midazolam (benzodiazepine)
What anesthetic acts as dissociative anesthetics and causes disorientation, hallucination and bad dreams?
Ketamine (arycyclohexylamines)
Propofol

MOA?
indication?
potentiate GABAa

for rapid anesthesia induction and short procedures
List 2 classes of local anesthetics.
Esters: procaine, cocaine, tetracaine

Amides (2 I's in the name): Lidocaine, mepivacaine, bupivavaine
In what situation do you need to apply more local anesthetics to achieve the same result?
in infected (acidic) wound b/c alkaline anesthetics are charged and cannot penetrate membrane effectively
What drug is co-administered to increase the effect of local anesthetics?

exception?
epinephrine to enhance local vasoconstriction

except for cocaine
Which sensation is blocked first with local anesthetics? last?
Pain (first) > temperature > touch > pressure (last)

small-diameter, and myelinated fibers are affected first. Size predominates over myelination
succinycholine

class/MOA?
indications?
toxicity?
depolarizng neuromuscular (nicotinic) blocker

indicated for muscle paralysis in surgery or mechanical ventilation

complications: hypercalcemia and hyperkalemia
irreversible during depolarizing phase
Tubocurarine, pancuronium

class/MOA?
indication?
reversal of action?
nondepolarzing neuromuscular blocker (nicotinic receptor)

indicated for muscle paralysis in surgery or mechanical ventilation

reversal by cholinesterase inhibitors (neostigmine, edrophonium)
Malignant hyperthermia

cause?
tx?
caused by inhalation of anesthetics (except N2O) and succinycholine

treat with dantrolene
List drugs used in Parkinson's. (5)
BALSA
1. Bromocriptine: agonize DA receptors
2. Amatadine: increase DA
3. Levodopa (with carbidopa): act as DA
4. Selegiline: MAO B inhibitor; prevent breakdown of DA
5. Antimuscarinic (Benztropine): curb excess cholinergic activity
List 2 phenomenon observed with long-term Parkinson's therapy.
On-off phenomenon

wearing off phenomenon
Benztropine

MOA
indication
muscarinic antagonist

Used in Parkinson's: improves tremor and rigidity but has little effect on bradykinesia
What is co-administered with L-dopa? why?
carbidopa to prevent peripheral conversion of L-dopa to DA, which can cause arrhythmia and decreased the bioavailability in the brain
Selegiline

MOA?
indication?
MAO-B inhibitor (MAO-B selective degrades DA over NE and 5HT)

adjunctive to L-dopa in tx of Parkinson's
Tx strategy for Huntington's
Huntington's is characterized by increased DA and decreased GABA + Ach

Reserpine + tetrabenazine: amine depleting (decrease DA)

Haloperidol: DA receptor antagonist
Sumatriptan

MOA?
indication?
toxicity?
5-HT1b/1d agonist
causes vasoconstriction, inhibition of CN V activation and vasoactive peptide release

used in acute migraine and cluster headache

toxicity: coronary vasospasm and mild tingling
memantine

MOA?
indication?
NMDA receptor antagonist; helps prevent excitotoxicity

used in Alzheimer's drugs