neuro Flashcards
1
Q
what kind of pathology would result in such a lesion
A
destruction of LMNs
i.e. polio, Werding-Hoffman Ds (group of inherited diseases that are characterized by progressive weakness of the muscles and reduced muscle tone)
present as flaccid paralysis, muscle atrophy, and fasciculations
2
Q
what kind of pathology would result in such a lesion
A
MS= a demyelinating disorder affecting the white matter of the CNS in random and assymetric pattern
= dif neuro deficits across space and time
3
Q
what kind of pathology would result in such a lesion
A
degeneration of dorsal root columns
the only (SC)manifestion of this is loss of touch, vibration, and proprioception
-tabes dorsalis= tertiary syhphillis = degneration of the dorsal root ganglia and DCML
4
Q
what kind of pathology would result in such a lesion
A
x dorsal columns (touch, vibration, proprioception)
x lateral corticospinal tract (spastic paresis)
this occurs in Vit B12 (cobalamin) deficiency= subacute combined degeneration
=x dorsal columns, lateral corticospinal tract, +spinocerebellar (ataxia)
=will also have atrophic glossitis (big, glossy tongue)
—> ds results from pernicious anemia (autoimmune gastritis w low IF), gastrectomy, ileal resection, diphyllobothrium latum infection (tapeworm)
5
Q
what kind of pathology would result in such a lesion
A
this is an injury to the anterior white commissure of the spinal thalamic tract
=syringomyelia (cape distribution, central cavitation of the cervical cord) –>bilat loss of pain and temp of the UE
-if it expands into the ventral horn –> flaccid paralysis of the hands
6
Q
in the setting of pancoast tumors, what diff structures could be compressed and cause sx
A
- brachial plexus : ipsi shoulder pain, UE parasthesias, areflexic arm wknss
- cervical sympathetic ganglia = horner’s syndrome
=ipsi ptosis, miosis, anhidrosis
7
Q
brain tumor
what is this, how does it present
A
biopsy shows a meningioma
red arrow: whorled grown of (meningeal) cells that are forming syncitial nests
black arrows: nests have calcified into lamellar structures aka psammoma bodies
round masses attached to the dura, commonly found at falcine= present with seizures, w compression of the adjacent structures.
-slow growing: HA, N/V –> inc ICP, (worse w recumbency and sleep) in intracranial volume
8
Q
brain tumor
what is this, how does it present
A
adamantinomatous craniopharyngitis = benign suprasellar tumors in children
cords/nests of pallisading squamous epithelium with internal areas of lamellar “wet” keratin under light microscopy
9
Q
what brain tumor is this
A
ependymoma = paraventricular tumors (i.e. floor of the 4th ventricle)
of the ependymal cells that line ventricles
10
Q
this is the most common primary malignant brain tumor in adult
describe its anatomy and histo findings
A
glioblastomas
often in the cerebral hemisphere and may cross the corpus callosum (butterfly glioma)
histo = hypercellular areas of atypical astrocytes border areas of necrosis
11
Q
malignagnt brain tumor in children composed of small blue cells
what is the other classical histologic finding
A
medulloblastoma
the small blue cells surround neuropil = homer-wright rosettes under lightmicroscopy
12
Q
what brain tumor is this
showing what classical finding below (round nuclei surrounded by halo of clear cytoplasm)
A
oligodendroglioma
in the white matter of cerebral hemispheres, tumors with fried egg appearance
13
Q
what benign brain tumor is this?
showing eosinophilic granular bodies around elongated fibers on histo
clinical presentation
A
pilocytic astrocytoma
the elongated, hair like processes = rosenthal fibers
in cerebellum of children and YAs
14
Q
what brain tumor commonly arises from CN VIII at the cerebellopontine angle?
what are the histo findings?
A
schwannoma = mostly benign
arise from vestibular branch of CN VIII at the cerebellopontine angle
histo= spindle cells with pallisading nuclei arranged around Verocay bodies (eosinophilic cores ) = Antoni A pattern
15
Q
what three organisms cause meningitis in neonates (0-3 months)? what 2 organisms cause meningitis in older infants (>3 months)?
what lab/microscopy findings set them apart from each other
A
grp b strep
- G+ cocci, facultative anaerobe
- capable of complete hemolysis, catalase -
e. coli:
- G -, motile, facultative anaerobe, rod,
- ferments lactose and glucose
- can grow on blood, MacConkey (bc of the lactose), eosin Methylene blue agar plates
listeria monocytogenes
- G+ rod, motile, grows in cold
- NONspore forming, use lysteriolysin O to skip phagolysosomes
strep pneumo
- G+ cocci, facultative anaerobe
- alpha hemolytic, catalase -, capsulated
n. meningitidis - G- dipplococci, facultated anaerobe
- capsulated, endotoxin LPS
16
Q
which virus is the most common cause of fatal sporadic encephalitis
etiology?
A
HSV-1
17
Q
what is the etiology that predisposes people to migraines/aura? what does aura look like?
A
–inc cerebral excitability –>a wave of corticol spreading depression
-aura can be visual or sensory (i.e. unilat extremity parasthesias)
=abn activation of trigeminal afferents–> spreads to the meninges and intracranial vasculature –> release calcitonin-gene related peptide (CGRP) ∝ pain transmission
-CGRP also causes local vasodilation and inc neurogenic inflammation –> worsens pain
18
Q
what 3 systems send signals to the medullary vomiting center to cause N/V
what NT and receptors are used
A
GI = irritation (infection, chemo, distension)
=send 5-ht3 (serotonin) from the stomach –> vagal + spinal afferent Ns –> medullary VC,
and CN XI from the tongue directly
vestibular system= motion sickness and vertigo
H1+ M1 receptors in ear send signal to medulla via CN VIII
chemoreceptor trigger zone =
emotogenic substances activate D2 receptors in the area postrema, right next to the vomiting center
19
Q
what are the specific indications for the following bipolar drugs, and what are the AE of each
lithium
valproate
carbamezapine
lamotrigine
A
20
Q
where are wernicke’s area and broca’s area + Brodmann’s area #s
(be able to point it out or use your words)
A
wernicke = posterior section of the superior temporal gyrus, L = brodmann’s area 22
broca = pars opercularis and pars triangularis of inferior frontal gyrus, L = brodmann’s 44+45
21
Q
where are the
primary visual cortex,
the primary auditory cortex,
primary motor cortex
primary somatosensory cortx
A
primary visual cortex = posterior occipital lobe
the primary auditory cortex= superior temporal lobe
primary motor cortex = precentral gyrus
primary somatosensory cortx = postcentral gyrus
22
Q
where are the each of the following located, and what is their function
- somatosensory association cortex
- premotor cortex
- frontal eye field
- prefrontal cortex
- limbic association area
- arcuate fasciculus
A
- somatosensory association cortex
- directly posterior to post-central gyrus, parietal lobe
- higher mental and emotional processes, including memory, learning, speech, and the interpretation of sensations
- premotor cortex
- anterior to primary motor cortex, frontal lobe
- associated w Ms of the trunk
- frontal eye field
- intersection of the middle frontal gyrus and the prefrontal gyrus
- saccadic eye movements for visual field perception and awareness, and voluntary eye movements
- communicate w extraocular Ms via paramedian pontine reticular formation
- lesion = deviation of eyes to ipsilateral side
- prefrontal cortex
- front part of frontal lobe
- planning complex cognitive behavior, personality expression, decision making, moderating social behavior
- limbic association area
- medial side of frontal lobe
- helps form memories and translate that to motor responses
- also help processes emotion and guides emotional response
- arcuate fasciculus
- a fiber tract connecting caudal temporal cortex and inferior frontal lobe
- bundle of axons that connects broca’s and wernickes areas
- a fiber tract connecting caudal temporal cortex and inferior frontal lobe
23
Q
what is the difference between schizoid personality and avoidant personality
A
schizoid: content w being alone from people, voluntary withdrawal from others, limited emotional expression
avoidant: hypersensitive to rejection and criticism, socially inhibited and timid, but desires relationships with others
24
Q
what kind of neurotransmitter is glycine (activating/inhibiting)
what is glycine encephalopathy
A
inhibiting
glycine encephalopathy = congenital defect in glycine metabolism
–> myclonic seizures and rapidly progressive, eventually ends in respiratory distress
–> floppy baby syndrome
25
myasthenia gravis is due to antibodies to what receptor type specifically
nicotinic Ach R
26
what is the difference between partial (2) and generalized (5) seizures?
list and describe the types of each
partial seizures = focal, single area of brain effected (mc in medial temporal lobe)
* simple partial: pt retains awareness/consciousness : can be motor, sensory, autonomic, psychic
* complex partial: impaired consciousness, movements made without conscious thought or intentions (automatism)
general seizures = diffuse brain damage
* petit mal= absence seizures= blank stare, no postictal confusion, 3 hz spike and wave discharges
* myoclonic= quick, repetitive jerks
* grand mal = tonic-clonic= alternating stiffening and movement, postictal confusion, urinary incontienence, tongue biting
* tonic = stiffening
* atonic= drop seizures (falls to floor), commonly mistaken for fainting
27
an eye that is down and out, dilated, and ptosis --suggests what pathology
_oculomotor nerve palsy_ associated w _posterior communicating A_
28
what antipyschotics can be used for schizophrenia
olanzapine, clozapine
aripiprazole, asenapine, quetiapine,
iloperidone, paliperidone, risperidone
lurasidone, ziprasidone
29
what structures are derived from neural crest cells
PNS and surrounding structures
\***MOTEL PASSES**\*
**M**elanocyte
**O**dontoblasts
**T**racheal cartilage
**E**nterochromaffin cells
**L**eptomeninges (arachnoid, pia)
**P**NS ganglia (cranial, dorsal root, autonomic)
**A**drenal medulla
**S**chwann cells
**S**piral membrane (aorticopulmonary septum)
**E**ndocardial cusions (+ mesoderm)
**S**kull bones
30
what is the clinical presentation of toxoplasmosis
how is it transmitted
how is it treated
**immunocompromised** patient comes in w **seizures**, brain CT/MRI shows **ring enhancing lesions** in the **frontal and parietal lobe** = **brain abscesses**
+chorioretinitis, lymphadenopathy, fever, focal weakness,
transmission of *toxoplasma gondii:*
inhale from cat feces, consume cysts in undercooked meat
trx=
acute: sulfadiazine + pyrimethamine
prophylactic: antiretroviral therapy; TMP/SMX for those w CD4\<200
31
what is the trx of an acute cluster HA
**100% oxygen** + sumatriptan/zolmitriptan
\*\*triptans = 5HT1 receptor agonists, also used in acute migraines
32
what trx is given to prevent the development of seizures in the setting of pre-eclampsia with severe featurs
1. give magnesium = to prevent seizures
2. induce labor w oxytocin
33
what is somatic sx disorder
= 1 or more somatic sx that are distressing or result in significant disruption of daily life
* disproportionate and persistant thoughts about the seriousness of the sx
* high levels of anxiety about one's health or sx
* spending excessive time/energy on one's health or sx
*
34
what structure in the brain makes up the "vomiting center"
where does it receive input from
NTS= nucleus tracus solitarius
- in medulla: coordinates responses from other medullary centers
- input from GI tract (serotonin activated CN X), vestibular system (CN VIII-motion sickness), area postrema (in the 4th ventricle, responds to changes in blood and CSF, especially chemo agents)
35
people with panic disorder are at increased risk of developing what phobia
agorophobia
fear of going out and being in situations they can't get out of = avoid situations where they might be trapped or helpless in the event of another panic attack
36
Down syndrome is related to what complications in each of the following systems
* neuro
* cardio
* GI
* endo
* heme
* rheumatology
* neuro
* early onset Alz (AB amyloid, early on show high levels of amyloid precursor protein
* cardio
* septal defects
* GI
* duodenal atreisa and hirschprung
* endo
* hypothyroid, type 1 diabetes, obesity
* heme
* acute leukemia
* rheumatology
* atlantoaxial instability
37
opiates act on which receptors on which side of the synapse, causing what change in which ion movement
where are the receptors found
mu receptors, in the spine
- work PREsynpatically to reduce Ca influx --\> dec excitatory NT release
- work POSTsynpatically to open K channels --\> K efflux --\> membrane hyperpolarization
38
speed of conductance down an axon is based on what two things?
the length constant : how far down an axon an electrical impulse can propogate without requiring regeneration
-depends on the resistance to conductance
the time constant : how long it takes the membrane potential to respond to a change in membrane permeability
-depends on membrane resistance and capacitance
39
thiamine deficiency will affect what 4 enzymes in what 4 pathways
niacin deficiency will affect what
x thiamine
1. pyruvate dehydrogenase
1. pyruvate --\> acetyl CoA (citric acid cycle)
2. alpha ketoglutarate dehydrogenase (citric acid cycle)
3. branched chain alpha keto-acid dehydrogenase (AA catabolism)
4. transketolase = for PPP
x niacin =
1. x NADPH + NADP
1. x glutathione reductase :metabolism of harmful reactive oxygen species (ROS)
40
- common presentation of MG
- pathophys of the ds
- trx
-MG = weakness worse at night, better in morning
* often can present with diplopa/ptosis, difficulty chewing, etc
* auto-Ab against post-synaptic nAchR --\> endocytosis of the receptors --\> reduced amplitude of end motor plate action potential in response to stimulation --\> dec M firing
* trx w anticholinesterase, immunosuppression, and a thymectomy
* the anticholinesterase such as pyridostigmine, which is not N/M specific, would cause mAchR ovestimulation-- so use N specific drugs= glycopyrrolate, hyoscyamine, propantheline
41
where in the synapse is the mechanism of action of the botulinsm toxin?
organophosphates?
botulin toxin = blocks pre-synpatic Ach release into both nAchR and mAchR
organophosphates = AchE-inhibitors
42
which brain tumor presents with a biphasic histologic pattern with highly cellular cells mixed w myxoid regions of low cellularity
-what other histologic finding is associated with these tumors
how do these tumors present, where are they usually found
Schwannomas
highly cellular cells = Antoni A pattern
myxoid regions of low cellularity = Antoni B pattern
* also see Verocay bodies = palisading patterns w interspersed nucear free zones
- MC at cerebellopontine angle
- usually present w tinnitus, vertigo, and hearing loss
43
S-100 is a marker for what tumor origin
-which are S100 (+)
neural crest origin
=melanoma and schwannomas
44
what physical exam findings are shown below?
* what is the embryologic origin of these lesinos
what is the dx, inheritance, and what other exam findings do you expect to see
NF1 = AD ch 17
* cutaenous neurofibromas = **hallmark**
* numerous, fleshy, dome shaped/pedunculated lesions that vary size: usually multiple, disperse across the body
* = made from **schwan cells --\> from neural crest cells**
* cafe au lait spots
* patches of hyperpigmented skin (light brown spots) that are either smooth or irreguar borders
* may be associated with axial or inguinal freckles
* optic N glioma
* can cause vision loss
* Lishc nodules
* pigmented, asymptomatic hamartomas of the iris
* bone abnormalities
* congenitial psuedoarthritis, scoliiosis, sphenoid dysplasia
* other tumors
* meningiomas, astrocytomas, gliomas, pheochromocytomas
45
pt presents w hx of sudden, explosive headache --\> loss of conscioussness --\> regain consciousness, now with signs of meningeal irritation
dx?
how will their mental status fair moving forward? any complications?
SAH
* will probs have been alert when they woke up, but now will have deterioting consciousness and have somnolence
* a few days later, vasospasm may cause sudden worsening of sx again
-present w blood layering the sulci
46
MRI diff between epidural, subdural, and SAH
47
fracture of the cervical spine transverse foramine can injure what big A?
this A lets off what branch that results in a characteristic spinal cord syndrome? describe teh syndrome
vertebral A runs through the transverse foramina of the C spine
PICA will branch off of it up near the lateral medulla --\> lateral medullary syndrome aka Wallenberg syndrome
48
functions of the CN VII
lesion causes what syndrome, presents how?
CNVIi = facial N
function:
* motor output to facial Ms
* para innervation to lacrimal ,submandibular, sublingual salivary glands
* taste ant 2/3 tongue
* somatic afferent pinna and external auditory canal
x= bell's palsy
* suddent onset unilat facial M wkns
* =trouble opening eye, uneven smile, thinnning of nasolabial fold
* ipsi eyebrow sag and mouth droop
* may have dec tearing and taste on that side
\*\*preserve palate movement = CN 9+10\*\*
49
where is the locus ceruleus
- function?
- effects of loss?
locus cerulus = pigmented nucleus at the **posterior rostral pons at the lateral floor of the 4th ventricles**
= **principle site of NE synthesis**
associated w mood, arousal (**reticular activating system-RAS**), sleep-wake cycles, cognition, autonomics
- dysfunction associated w **anxiety** disorders
- lose one side = impaired consciousness, e***xtensor posturing***, pinpoint pupils
- bilat pontine hemorrahage= **coma** bc no activation of RAS
50
a pt recently started on antipsychotics who now complains of suddent onset M stiffness or spasms is experiencing what?
- likely cause?
- pathophys?
- trx?
**acute dystonic reaction** aka spasmodic torticollis
=a type of EPS sx in response to the antipsychotic
* most likel causes= **_haloperidol and fluphenazine_**
* pathophys = Dopamine - Ach imbalance
* the niagrostiatal pathway requires both Dopamine (D2) and Ach (M1) to balance each other out
* D2 antagonists (antipsychotics) will remove the inhibitory effect of dopamine --\> constant activation of M1 receptors --\> inc movement, spasticity --\> pain and stiffness
* can be in major muscle groups, in just eye movements, in the laryngeal Ms. ...
* trx with M1 receptor antagonists co-administration to re-establish balance
* i.e. benztropine
* can also trx w an antihistamine = diphenydramine
51
lesion of the anterior cerebral A--\> ?
unilat vs bilat
x supply to the medial ipsi hemisphere = x sensory motor to contra foot and leg
* i..e climbin stairs
bilat= behavioral changes (i.e. inablity to lie, x willpower) and urinary incontinence
52
compare the blood solubility and the blood:gas coefficient of drug A with nitrous oxide
what does they say about their action as inhaled anesthetics
drug A has a higher blood solubility which is why it takes longer for it to reach the plateau PARTIAL PRESSURE than it it took NO
higher blood gas solubity = higher blood gas coefficient = slower ONSET OF ACTION
(nothing to do w potency aka how much you need)
53
what are the big 5 childhood primary brain tumors
* location (w any location specific sx)
* histology
* **_Pilocytic astrocytomas_**
* usually in the posterior fossa = cerebellum, but may also be supratentorial
* GFAP + (glial cell origin) : rosenthal fibers : corkscrew fibers : cysitc and solid, well circumscribe
* **_medulloblastomas_**
* commonly involve cerebellum, can also compress the 4th ventricle --\> noncommunicating hydrocephalus :**drop metastasis** to spinal cord can be seen
* = PNET (primitive neuroectoderm) associated w Homer-Wright rosettes, small blue cells
* **_ependymoma_**
* MC found in 4th ventricle, can cause hydrocephalus= poor prognosis
* ependymal cell orogin w characteristic **perivascular pseudorosettes** : rod-shaped basal ciliary bodies
* **_craniopharyngiomas_**
* MC supratentorial tumor of children = can cause **bitemporal hemianopia**
* remnant of rathke ouch (ectoderm): calcification, choelsterol rich crystals in "motor oil"
* **_pinealoma_**
* in pineal gland--\> perinaud syndrome = vertical gaze palsy (compress tectum), obstructuve hydrocephalus (compress aquadect), precocious puberty in males --\> beta-hCG production
* germ cell tumor like
54
what are the big 5 primary brain tumors seen in adults
* location and associated sx/presentation
* histology
* glioblastoma multiforme
* found in cerebral hemisphere, can cross corpus callosum = **butterfly gllioma** :grade 4 astrocyte origin: 1 yr survival
* GFAP + : _pseudopallisading_ pleomorphic tumor cells that border central areas of necrosis, hemorrhage, and/or microvasc prolif
* oligodendroglioma
* most often in frontal lobes: slow growing, often have "chicken-wire" capillary pattern
* "fried-egg" cells: round nuclei with clear cytoplasm - often calcified
* meningiomas
* often benign, seen more in Fs : near the surface of the brain and in parasagittal region : may be external to the brain parenchuma and may have a dural attachment tail
* can be asx, or present w seizure or focal neuro sx
* origin = arachnoid cell!! : **spindle cells** arranged in **whorled pattern** with **psamomma bodies** (laminated calcifications)
* schannomas
* classically at the cerebelloponting angle (bonth CN 7+8 often affected, tho can affect any CN) : ten localized to internal acoustic meatus --\> vestibuar schwannomas
* bilat vesitublar schwannomas area ssociated w NF2
* schwann cell origin, S-100 (+)= biphasice w pallisading areas of spindle cells alternating w hypocellular myxoid areas
55
how us succinylcholine metabolized in the body
-admin must be reconsidered in pt's with what heriditary disorder
succinylcholine is hydrolyzed by pseudocholinesterases in the plasma
pt's w **pseudocholinesterase deficiency** (associated w BCHE gene: AR or AD) can have less or none --\> prolonged M paralysis as the nAcHR remains blocked (depolarizing agent) for hours longer than expected
56
organophosphates inhibit which receptors
what is the trx needd to reverse ALL sx
organophoshates will cause over stimulation of mAchR AND nAchR indirectly via inhibiting AchE
- trx w atropine (M antagonist) but also a specific nAchR antagonist to trx the M weakness
- can trx w **pralidoxime**= will regenerate AchE so that the Ach can be broken down and this will have an effect on all receptor types
57
symptomatic presentation of west nile virus infection of the CNS
* associated with who
* pathology
- type of virus, found where
- common presentation without the CNS involvement
* acute confusion, fever, assymetric LE paralysis
* not common to get in the CNS- associated w elderly, or immunocompromised
* associated w meningitis (HA, meninges pain) or encephalitis (AMS)
* **highly suggestive of WNV= acute ASSYMETRIC flaccid paralysis and parkinsonian like featuresb**
* (+) ssRNA flavivirus transmitted by mosquitos
* summer in the southern US, Latin America, or Africa
* West Nile can often present w general flu like sx (fever, myalgias) with **rash on back and chest** (*maculopapular or morbiliform*)
58
what Ns are involved in the corneal reflex, and which cranial hole does it go through
**optic N** = **optic canal**
= medial to superior orbital fissure: the optic canal carries only the optic N and the ophthalmic A
the **nasociiliary N** (a branch off V1) goes through the **superior orbital fissure**
* superior orbital fissure contains the nasociliar N, CN 3, 4, 6, and the superior ophthalmic A
motor component = facial N
59
brain abscesses caused by bacterial infection can reach the brain how
* a single abscess probably got their via direct, contiguous invasion
* otitis media --\> mastoid air cell invasion--\> into temporal lobe --\> temporal lobe abscess
* frontal lobe abscess \<-- from frontal, ethmoidal sinusitis
* multiple abscess
* hematogenous spread from heart (endocarditis) or lungs (abscess, empyema)
60
local spread of bacterial from the follow areas can lead to what consequences in the head and neck
- maxillary sinusitis
- nose and upper lips
- peritonsillar space
- maxillary sinusitis
* to the orbit causeing periorbital or orbital cellulitis and abscess (no direct connection to the brain tho)
- nose and upper lips
* drain into ophthalmic V--\> cavernous sinus thrombosis --\> HA, fever, oculomotor deficits, eye swelling
- peritonsillar space
* fever, sore throat, a muffled voice
* can lead to mediastinal spread, airway obstruction, or deep neck space infection
61
sudden onset severe electric facial pain suggests what pathology
- trx w MOA
- AE of the trx?
**trigeminal neuralgia**
= episodeic, unliateral, severe shock like pain along CNV
-first line trx = **carbamezapine** - inhib Na channels from recovering from inactivation --\> dec reactivation
* AE = BM suppression --\> anemia, agranulocytosis, and/or thrombocytopenia
* SIADH --\> hyponatremia
62
huntington ds is associated with neuronal loss where, leading to the loss of what key NT
loss of caudate nuclei in the striatum --\> loss of GABA inhbiitory signals
=atrophy of area will spread: first lose control of movements and motor impulses, but will eventually also develop dementia, bradykinesia, and total loss of voluntary movement
63
a couple days of fever and HA that suddenly leads to a tonic-clonic seizure + swelling of the temporal lobes
dx? CSF findings?
-explain the specific MRI findings seen with this disease
HSV-1 is the most common cause of sporadic encephalitits (the seizure)
acute onset fever, HA, AMS, focal neuro deficits/seizure with **temporal lobe edema**
* MRI will show TEMPORAL lobe edema bc HSV-1 gets into the brain via the **olfactory tract** and goes to the olfactory cortex in the temporal lobe
CSF
* as a virus --\> show inc lymphocytes, inc protein, n/dec glucose + **elevated erythrocytes** (bc of hemorrhagic inflammation in the temporal lobes)
64
histo brain changes seen in
* MS
* AD
* parkinson
* huntington
* MS
* plaques are characteristically with periventricular inflammatory infiltrates of T cells and Møs
* Møs contain myelin debris and you can see patchy areas of demylination
* AD
* neurfibrially tangles and neuritic plaques
* parkinson
* build up of alpha synuclein abn protein --\> inc amyloid and lewy bodies in the substantia nigra
* huntington
* atrophy of the caudate nuclei in the striatum
65
what is lamotrigrine used for
what AE must be monitored for
= an anticonvulsant
=need to watch out for steven johnson and toxic epidermal necrolysis
* discontinue the drug at the first sign of a rash
66
differentiate between the etiology, findings, and clinical presentations of
* chiari I malformation
* chiari II malformation
* Dandy-walker malformation
* chiari I malformation
* cerebellar **tonsils** are abn shaped and displaced downwards --\> compression of the brainstem
* associated with syringomyelia = spinal cord cavitation --\> cape distribution loss of pain and temp
* asx in childhood, present in adulthood
* mean age 18yo,
* HA secondary to meningeal irritation = "cough HA that is worse w cough
* ataxia
* CN dysfunction
* chiari II malformation = ARNOLD CHIARI
* herniaton of **vermis and tonsils** through foramen magnum
* present w hydrocephalus (associated w aquaductal stenosis) and almost alwasya have lumosacral meningomyelocele
* usually can detect prenatally/at birth bc of the meningomyelocele
* present w paralysis/sensoery loss @/below level of meningomyelocele
* as a child: hydrocephalus will present w inc head circumference on growth chair, anterior fontanelle distension, and split sutures
* brainstem malformations from the increased pressure --\> "beaked brainstem"
* Dandy-walker malformation
* cystic enlargment of the 4th ventricle and enlarge posterior fossa
* DW cyst in the 4th ventricle will seperate the cerebellar hemispheres --\> flat cerebellum w hypoplastic or agenesis of the vermis
* 4V cyst will lead to noncommunicated hydrocephalus and spina bifida
* children present w: **hydrocephalus + delayed development + motor dysfunction** (x crawling and walking)
67
* what are the primary neural tube bulges and when do they form
* what are the secondar neural tube bulges and when do they form
* what are the adult derivatives from these bulges
neural tube bulges= brain, brainstem
wk 3-4
* forebrain = prosencephelon
* midbrain= mesencephalon
* hindbrain= rhombencephalon
wk 5
* FB --\> telencephalon + diencephalon
* MB --\> mesencephalon
* HB --\> metencephalon + myelencephalon
adult derivatives
* telencephalon --\> cerebral hemispheres + lateral ventricles
* diencephalon --\> thalamus and hypothalamus + third ventricle
* mesencephalon --\> midbrain and cerebral aquaduct
* metencephalon --\> pons and cerebellum + upper part of 4th ventricle
* myelencephalon --\> medulla and lower part of 4th ventricle
68
what is the shared etiology of spina bifida oculta, meningocele, and meningomyelocele and how are they different
how can they be detected in utero
what are risk factors
how are they all different from anencephaly (which will present how)
all= failure to caudal neuropores to fuse in wk 4
* they all have the defect in bony formation of the vertebra
* detect w US, inc AFP (+inc AchE for confirmation)
* risk= maternal diabetes, dec folic acid (happens before mom knows shes preg), maternal obesity, maternal use of valproic acid or carbamezapine
spina bifida oculta= bony malformation but dura is intact and nothing comes out
* have tuft of hair or skin dimple at level of bony defect
meningocele
* meninges alone protrudes out of the hole in the bone, associated with spina bifida cystica
meningomyelocele
* meninges and neural tissue (i.e. cauda equina) herniate through the bony defect
* almost always associated with a chiari malformation, which will inc the P and push he out
vs ANENCEPHALY = failure of **rostral** neuropore to close --\> open calvarium which leaves the forebrain and brainstem exposed in utero so they don't develop normally
* not compatible with life- will die in utero or shortly thereafter
* "frog-like" appearance of the fetus
* associated with polyhydramnios bc the there is no swallowing center in the fetal brain to drain out the fluid made
69
freidrich ataxia
- cause
- pathophys
- sx
- how will the patient initally present
freidrich ataxia
* AR GAA trinucleotide repeat on ch 9
* x frataxin (iron binding protein) --\>
* x mitochondrial functioning
* pathophys
* x CS tract --\> spastic paralysis
* x spinocerebellar tract --\> ataxia
* dorsal columns --\> x vibratory sense, proprioception
* dorsal root ganglia --\> loss of DTRs
* sx
* staggering gait + frequent falling
* nystagmus
* dysarthria
* pes vacus, hammer toes
* DM
* hypertrophic cardiomyopathy (common COD)
* presentation
* childhood with kyphoscoliosis
70
what are the cerebellar tracts and how will lesions to these present
* cerebrocerebellum tract = fine motor movements
* *_x @ pontine nucleus --\>_*
* **intention tremor**, **dysarthria** + dysmetrira, ataxic gait, **dysdiachokineses**
* vestibulocerebllum tract = coordination
* *_x vestibular nuclei_*
* **nystagmus**, **truncal** swaying+titubations, x sobriety walk
* spinocerebellar tract = gait
* x *_red nucleus/ olive_*
* gait ataxia w staggering base, **wide base w heavy steps**, x sobriety walk
71
classic sx of pseudotumor cerebri vs NPH
LP and imaging
pseudotumor cerebri = pulsatile tinnitus, disabling HA, and papilledma resulting in a peripheral vision loss
* LP= INC opening pressure
* n ventriclular size on imaging
NPH= wet, wobbly, wacky
-incontinence, gait ataxia, dementia
* n opening pressure
* LARGE ventricles
72
brainstem anatomy
* general location of the CNs
* blood supply to the regions
MIDBRAIN
* CNs
* **medial** = 3+4
* BS
* **medial** \<--PCA --\> **lat**
PONS
* CNs=
* **medial** = 6 ------ **lateral** = 5,7,8
* BS=
* **medial** = basilar A : **lateral** = AICA
MEDULLA
* CNs=
* **medial** = 12 --- **lateral** = 9, 10, 11
* BS=
* **medial**= ASA --- **lateral** = PICA
73
presentation of whipplei ds
**pas**s the **foamy whip**ped cream in a **can**
*_PAS(_*+), f*_oamy_* macrophages in the intestinal lamina propria and mesenteric LNs
*_CAN_* = **c**ardiac sx, **a**rthralgias, **n**euro sx
+ late onset diarrhea/steatorrhea
associated w older men
74
treatment for anxiety (GAD) vs panic disorder vs OCD
GAD =
* 1st line= CBT+ SSRI/SNRI
* 2nd line= buspirone, TCA, benzo
panic disorder
* 1st line- CBT + SSRIs + venlafaxine
* in the acute setting, can use benzodiazepenes
OCD=
* 1st line= CBT + SSRI
* 2nd line= clomipramine+ venlafaxine
75
MOA of methyphenidate and amphetamines
-indications
**_methyphenidate and amphetamines_**
=increase DA and NE in the synaptic cleft (indirect agonists)
=indicated in narcolepsy, ADHD, binge-eating disorder
76
embryologic origin of the anterior pituitary gland and rathke's pouch
anterior pituitary gland : embryonic buccal(oral) cavity
rathke's pouch = diverticulum of the roof of the embryonic oral cavity
77
contents of the cavernous sinus
what sinus is it located directly above, and what structure is it directly below
internal carotid A
CN 3, 4, 6, V1+V2
right under pituitary gland, right above sphenoid sinus
78
x frontal lobe --\>
disinhibited and x concentration, orientation, judgement
may regress to primitive reflexes
79
x frontal eye fields --\>
destructive lesions (MCA stroke) --\> eyes look towards brain lesion
= away from side of the hemiplegia
(frontal - keep eyes on the road, AWAY from the car wreck)
80
x paramedian pontine reticular formation --\>
eyes look away from the brain lesion (towards the side of the hemiplegia)
(paramedian = look at the car wreck)
81
x medial longitudinal fasciculus --\>
associated w what ds
internuclear ophthalmoplegia (x impaired adduction of ipsilateral eye, nystagmus of contralateral eye with ABduction)
associated w MS
82
lesion of dominant parietal cortex --\>
vs
lesion of NONdominant parietal cortex --\>
agraphia, acalculia, finger agnosia, left-right disorientation
vs
agnosia of the contralateral side of the world (inability to interpret sensations/recognize the contra side of the world)
83
x bilateraly hippocamus --\>
anterograde amnesia
84
basal ganglia lesion --\>
associated with what diseases
tremor at rest, chorea, athetosis
-- seen with PD, huntington, wilson ds
85
x subthalamic nucleus --\>
contralateral hemiballismus = frequent, irregular flailing of the extremities
86
x bilateral mamillary bodies --\>
wernicke -korsakoff syndrome
87
x bilateral amygdala --\>
associated w what cause
kluver-bucy syndrome = disinhibited behavior (hyperphagia, hypersexuality, hyperorality)
associated w HSV-1 encephalitis
88
lesion of the dorsal midbrain --\>
3 big causes
parinaud syndrome = vertical gaze palsy, pupillary light near dissocation, lid retraction, convergence-retratction stagmus
causes: stroke, hydrocephalus, pinealoma
89
lesions of the cerebellar hemisphere vs vermis
x hemisphere = **l**ateral **l**ocation, affect **l**imbs
* intention tremor, limb ataxia, lose balance IPSI deficits = fall TOWARDS side of lesion
x vermis = vermis is central, affect central body (assocated w chronic alc)
* truncal ataxia (wide based, "drunken sailor" gait), nystagmus
90
lesions of the red nucleus (in what part of the brainstem?) lead to what
red nucleus = midbrain
* decorticate posture (flexed) if lesion is above the red nucles --\> flex UEs and extend LEs
* decerebrate posturing (Extensor) if lesions is below the red nucleus --\> extend both UEs and LEs
91
which recreational drug is associated with nystagmus
what other sx does it present with
PCP (phenylcyclidine)
* classically presents with nystagmus
* also violent behavior, hallucinations, and ataxia
* resolves after ~8ish hours
92
what is this physical exam finding
* what is the etiology
what non-neoplastic pathology is often associated with this
* classic presentation
papilledema = blurred and enlarged optic disk
* inc P --\> compress optic N --\> dec axoplasmic flow --\> bilateral optic disk edema
associated with pseudotumor cerebri
* =intracranial HTN that presents in ***_young women who have daily HA and transiet visual changes_***
* worse with valsalva bc it inc the P
93
1. a white pupillary reflex is associated with what pathology?
2. this pathology is associated with a mutation in what gene?
3. germline mutation of this gene is associated with inc risk of what?
1. retinoblastoma ∝ white pupillary reflex
2. ∝ with xRb (~40% are familial, rest are not ) --\> associated w 2 hit hypothesis (inc risk w germline mutaiton bc any one chr only needs one hit)
3. inherited x Rb is associated w increased risk of **osteosarcoma** and other sarcomas
94
what are cholesteatomas
* etiology
* common presentations
cholesteatomas = build up of squamous cell debris in the middle ear that form a **round, pearly mass behind the tympanic membrane**
* etiology = congenital or acquired
* can be primary= due to chronic negative pressure in the middle ear --\> cause retraction pockets in the TM that become cystic
* secondary to trauma, surgery, infection
* MC present with **painless otorrhea** (ear drainage) and a pearly mass
* as it grows it can erode into the auditory ossicles, can cause conductive hearing loss
* can also erode into surrounding bone and impinge on the facial N -\> vertigo or facial palsy
95
what structures are derived from neural crest vs neural tube vs surfact ectoderm
neural TUBE = CNS tube
neural crest =
* ganglia + schwann cells + adrenal medulla
* heart septum and endocardial cushions
* branchial arches (--\> bones and cartilage) + skull bones
* melanocytes
96
Huntington Ds
* etiology
* pathophys + histo
* population
* clinical presenation
HD
* etiology
* AD inheritance of **CAG** repeats on chr 4 --. **GOF HTT on ch4** = x huntingtin protein --\> inc **DNA histone deacetylation**--\> DNA silencing by inhibition transcription
* pathophys + histo
* atrophy of the caudate and putamen with ex vacuo ventriculomegaly
* inc Dopamine leves, with dec GABA and Ach levels
* \*\***CAG**= **C**audate loses **A**ch and **G**aba\*\*
* neuronal death = via NMDA-R binding and glutamate excitotoxicity
* presents around 20s-50s (can be mistaken for drug abuse at first)
* anticipation of genes
* clinicall presentation
* chorea, athetosis, aggression, depression, dementia
*
97
frontotemporal dementia vs lewy body dementia
frontotemporal dementia = Pick's Ds
* =early personality and behavior changes or primary progressive aphasia
* degen of the frontotemporal lobe
* histo= **hyperphosphorylated tau** (round Pick bodies) or **ubiquinated TDP-43 protein**
Lewy body dementia
* visual hallucinations are the hallmark = haLEWYcinations
* dementia w flucuating cognition and alertness
* REM sleep behavior disorder and parkinsonism
* \*\*only lewy body dementia if the coginitive and motor sx develop within a year of each other vs demenita secondary to parkinsoniasm\*\*
* histo= intracellular lew body ds (alpha synuclein buildup)
98
elderly patients are at increased risk of medication induced delerium with what drugs
* sleed aids (benzos, sedative hypnotics (zolpidem, and the other two Zs))
* opioids
* anticholinergic agents
99
what is an essential tremor and what is 1st line treatment
essential tremor= slowly progressive, symmetric postural/kinetic tremor that gets worse when holding the same position
- can often run in the family : often improves with ingestion of some alcohol
- TOC = propanolol
100
* where in the world is West Nile found
* how is it transmitted
* what are the hallmarks of the West Nile neuroinvasive ds
* in *_southern US,_* Latin America, and Africa
* **_birds_** are a hug reservoir --\> picked up by **mosquitos** --\> give it to humans
* WEST NILE
* fever= flu like illness with maculopapular rash, self-limied
* neuroinvasive ds = meningitis, encephalitis, and flaccid paralysis
* meningitis= HA , fever, neck stiffness
* encephalitis = confusion, tremors, seizures
* "flaccid" paralysis = assymetric limb weakness with fasciculations
101
MOA of
* inhaled anesthetics
* IV anesthetics
* local anesthetics
* inhaled anesthetics
* unknown
* ""-urane"s and N.O
* IV anesthetics
* facilitate GABA (ketamine via NMDA antagonism)
* local anesthetics
102
what drugs are used to treat Parkinsons, and what is their MOA
bromocriptin + pramipexole = dopamine agonists
103
what exits through the
* superior orbital fissure
* foramen ovale
* foramen rotundum
* foraem spinosum
* jugular foramen
104
a child presenting with extremity weakness and abn involuntary (choreoathetoid) movements, cognitive and motor delay, and growth development delay and levels of arginine suggests what pathology
arginase deficiency
= no make urea --\> build up of arginine --\> toxic
-no hyperammonia seen\*
105
MLF lesion causes what
internuclear ophthalmoplegia
x ipsilateral adduction during horizontal gaze, but convergence is maintained
106
age related visual loss is often associated with what
sx and clinical presentation
cataracts = gradual opacification of the eye
sx= gradual vision loss, excessive glare from bright lights,
clin= loss of red reflex
107
disease of which organ can lead to build up of opioids in the body --\> toxcity
the KIDNEY bc opioids are RENALLY excreted
108
what is the ETIOLOGY of diabetic peripheral neuropathy
1. ischemic n damage via the hylanization of endoneural arterioles
2. inctracellular hyperglycemia in the peripheral Ns --\> inc oxidative stress inside the Ns
109
where are serotonin releasing neurons located
in the raphe nuclei of the brainstem
(happy ralph)
110
what drug can be given prophylactically to a pt with SAH to avoid worsening sx from vasospasm in the upcoming days
nimodipine (CCB)
-inc cerebral vasodilation + dec Ca-dependent excitation of neurons--\> neuroprotective
111
what are the 3 "ragged red fiber" diseases and what is their common etiology
RRF = mitochondrial inherited ds
* MERRF
* myoclonic epilepsy w ragged red fibers
* MELAS
* mitchondrial encephalopathy (confusion, tremor, seizure) w lactic acidosis and stroke like episodes
* Leber optic neuropathy
112
which AchE inhibitors are used to treat Alzheimers and what major SE are they associated w
donepazil and rivastigmine
-can inc para tone --\> AV block and episodic bradycardia
113
the middle meningeal A branches off of what A
the MAXILLARY A
114
what are the adrenergic receptors on the
* eye
* bladder
* uterus
and what are their effects
115
describe the 3 Ns that carry sensation to the tongue
116
preventative vs abortive migraine medication
117
inhaled anaesthetics can cause what undesired effects in what 5 organs
118
what is the purpose of adding carbidopa to levidopa in the treatment of parkinsons
what will it NOT affect
help by decreasing the peripheral sx of levidopa
* N/V
* hot flashes
* postural hypotension
* tachyarrhythmias
will NOT affect central sx
* anxiety and agitation
119
which cranial Ns are the afferent and efferent in each of the following reflexes
* pupillary light reflex
* corneal reflex
* jaw jerk reflex
* vestibuloocular reflex
* carotid sinus reflex
* cough reflex
* gag reflex
120
where are each of these and what structures can be compressed the following herniations
* subfalcine herniation
* transtentorial herniation
* tonsillar herniation
* subfalcine herniation
* cingulate gyrus herniates under the falx cerebri
* potentially compress ACA
* transtentorial herniation
* when the medial temporal lobe (uncus) herniates between the crus cerebri and tentorium cerebelli
* can compress **ipsi CNIII, ipsi PCA, contra cerebral peduncle** (--\>ipsi hemiparesis), **stretch/rupture of the basilar A** (--\>hemorrhage into midbrain/pons --\> fatal)
* tonsillar herniation
* cerebellar tonsils through foramen magnum
* compress medulla
121
what is the pathway in the brain that leads to parkinsonian symptoms
what AEs can emerge with long term treatment of PD w levodopa
high frequency stimulation of what part of the brain can treat PD motor sx?
* degeneration of the **striatum and substanita nigra** --\> excessive excitation of the **globus pallidus internus** from the **subthalamic nucleus** (now unchecked) --\> excessive inhibition of the thalamus
levodopa \>5 years --\> motor fluctuations and dyskinesia
* motor fluctuations : good mobility when the drug is at higher concentrations in the body, but then increased bradykinesia and rigidity when the concentrations falls/drug wears off
* dyskinesia: excessive involuntary movements during periods of increased drug concentrations
high frequency deep brain stimulation of the **globus pallidus or subthalamic nucleus** --\>thalam-cortical disinhibition --\>better mobility
122
what is anterior cord syndrome
the lesion and the sx
123
what is the most common cause of dementia and what are the biochemical changes seen in the brain
alzheimers
* dec in Ach levels associated w decreased choline acetyltransferase
* esp in nucleus basalis of meynert = memory and cognition
* also hippocampus = formation of new memories
124
wernicke-korsakoffe syndrome results in a deficiency of what vitamin
what enzymes need this vitamin as a cofactor
vitamin B1
**pyruvate dehydrogenase** (pyruvate --\> acetyl-CoA)
**alpha ketoglutarate dehydrogenase** (alpha ketoglutarate --\> succinyl-CoA)
**transketolase** (PPP)
**branced-chain amino acid dehydrogenase** (reduces oxidative stress)
125
126
Alzheimers is associated with atrophy of the temporoparietal hemispheres and the hippocampus (hippocampal is present early in the ds)
