Flashcards in Neurology Deck (308):
MELAS def and inheritance pattern
mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes-seizure with stroke like episodes with residual neurological defect, muscle weakness, increased serum lactate levels both post exercise and at rest.
name three clinically important mitochondrial diseases and inheritance.
Liber hereditary optic neuropathy-leads to bilateral vision loss
Myoclonic epilepsy with ragged-red fibers- myoclonic seizures and myopathy associated with exercise. (MERRF) skeletal muscle biopsy shows irregularly shaped muscle fibers (ragged red)
Mitochondrial encephaomyopathy with lactic acidosis and stroke-like episodes
-inheritance is maternal
listeriosis causes (2) in which pt population (2) is transmitted how? grows well where? morphology?
sepsis and meningitis-immunocompromised host
transmitted through foods (milk products, undercooked meats, and unwashed raw veggies)
-grows well in cold temps (can contaminate cold food)
-gram positive rod with V or L formations (like corynebacterium)-"tumbling motility"
lateral geniculate nucleus vs optic tract lesion
both produce contralateral homonymous hemianopia but optic tract also produces a Marcus Gunn pupil (APD).
optic tract because nasal portion contributes more input to pretectal nucleus than the temporal portion of retina. swinging flashlight test (from unaffected to affected) shows pupils appearing to dilate b/c they constrict less than when light is shown in the unaffected eye
T-tubules are found in what cell type? location? function?
in striated muscle cells. junction of A and I bands, tubular network open to extracellular space and facilitates spread of depolarization to the inside of the cell
keratin vs kinesin
keratin is found in desmosomes (aka macula adherens) which provide structural support b/t cells.
kinesin is MT associated motor protein that mediates anterograde (towards + end) transport
do microvilli have microtubules?
no! they cover the apical plasma membrane (i.e. intestinal epithelial cells) and contain actin thin filaments
depressed pt develops HTN after a wine and cheese party is on what type of drug?
monoamine oxidase inhibitor (MAO inhbiitor)-phenelzine
presynaptic selective serotonin uptake
presynaptic selective norepinephrine uptake
presynaptic non-selective monoamine uptake
enzymatic monoamine degeneration
-bupropion (NE), FA says dopamine too?
-TCAs/SNRIs (NE or Serotonin)
-MAO- non selective (phelenzine) and selective (selegiline-MAO-B, increase Dopamine in Parkinson's)
denial vs fantasy
denial-doesn't acknowledge truth
fantasy-acknowledges truth but substitutes a less disturbing version of reality
which has better side effect prolife TCAs or SSRIs
SSRIs- sexual dysfunction
TCAs urinary retention (anticholinergic effect), cardiac arrhythmias (prolongs QRS and QT interval), seizures (clomipramine, antihistamine,antimuscarinic, anesthetic properties), orthostatic HTN (anti-alpha adrenergic effect), sedation (anti-histamine effect)
two mechanisms of polyhydraminos
impaired swallowing (GI atresia (obstructions) or anencephaly) or increased fetal urination (high CO due to anemia or twin to twin transfusion syndrome- twins share placenta unequal blood flow between them)
chronically progressive pre-senile demnetia with cortical atrophy but no radiological or laboratory abnormality
Alzheimer's (AD)- diagnosis of exclusion.
therapies- donepezil (cholinesterase inhibitor), antioxidants (vitamine E-alpha-tocopherol), and memantine (NMDA receptor antagonists-prevents exitotoxcity; mediated by glutamate increasing Ca2+ levels)
avoid-benzos (b/c of anti-depressive effects) can be used to treat associated anxiety and insomnia though
name 3 categories of benzodiazepines:
-risk of fall
-risk of dependence
three classes are short acting (<10 hrs), medium (10-20hrs) and long (days)
-longer acting are more likely to have day time somnolence and increased risk of falls and short acting do not
-longer acting are less likely to cause physical dependence and short acting are
short-alprazolam, triazolam*, oxazepam (use in elderly)
medium-estazolam, lorazepam*, and temazepam
long- chloridiazepoxide*, clorazepate, diazepam*, flurazepam* (avoid in elderly)
mutations associated with Alzheimer's
early onset: APP (21), presenilin 1 and presenilin-2
late onset: apolipoprotein E4
common conditions associated with Downs
Acute Leukemias (AML/ALL)
Congenital heart Disease (endocardial cushin defects, VSd, and ASD)
Gastrointestinal Defects:duodenal atresia and Hirschsprung disease
round eosinohpilic inclusion seen in cytoplasm of pyramidal neurons and cerebellar Purkenje cells; rabies virus
how do the terminal sulcus and foramen cecum relate to the tongue innervation regions
terminal sulcus delineates the anterior 2/3 of the tongue from the posterior 1/3.
the foramen cecum is located along terminal sulcus at the midline
which prokaryotic DNA polymerases have proof reading activity? which direction?
which one can proof read in the other direction?
how is it's repair different from the other polmerases?
all three have proof reading activity and can remove mismatched nucleotides in 3' to 5' exonuclease activity
DNA polymerase I has 5' to 3' activity and can excise and replace RNA primers and damaged DNA sequences.
lesion in brain that can cause alexia without agraphia
splenium of corpus callosum
mechanism of opiod agonist. give major example of one
bind to mu receptors (GPCRs that activate second messengers resulting in increased K+ efflux and decreased Ca2+ influx) blocking postsynaptic transmission of pain. morphine
describe three types of neurona response to injury and their associated histoplasmic changes
-acute neuronal injury (aka red neuron)- shrinkage of cell body, pykinosis of nucleus, loss of Nissl substance, eosinophilic cytoplasm
-axonal reaction- (loss of axon), enlargement of cell body, eccentric nucleus, enlargement of nucleolus, and dispersion of Nissl substance
-neuronal atrophy (progressive degenerative dz) loss of neurons and functional groups of neurons, reactive gliosis
therapy for acute mania
mood stabilizing agent (lithium, valproate, or carbamezapine) plus an atypical antipsychotic (olanzapine)
treatment of essential tremors. inheritance pattern
non specific beta-blockers (i.e propanolol)
autosomal dominant (aka familial tremor)
cystathionine synthetase deficiency-leads to what urine finding? presentation? cardiovascular risk? treatment?
Marfan like symptoms-ectopia lentis, developmental delay
-50% respond to high does vit B6 (pyridoxine)-cofactor for cystathionine synthase (converts homocystinuria to cystine)
define radial traction, when is it seen (obstructive and restrictive)
fibrotic lung exerted on conducting airways, restrictive lung disease
causes increase in expiratory flow rate
ataxia-telangiectasia-inheritance, defect, pts are sensitive to, manifests (4)
-DNA-repair gene defect
-hypersensitive to ionizing radiation
-cerebellar ataxia, oculocutaneous telangiectasias, repeated sinoplumonary infections, increased risk of malignancy
name 5 disorders caused by deficiency DNA-repair enzymes:
-ataxia-telangiectasia (DNA hypersensitivity to ionizing raditation)
-xeroderma pigmentosum- DNA is hypersensistive to UV radiation, leads to premature aging, and increased risk of cancer (melanoma and SCC)
-fanconic syndrome-hypersensitivity to DNA cross-linking agents
-bloom syndrome- generalized chromosomal instability, increase susceptibility to neoplasms
-hereditary non-polyposis colorectal cancer (HNPCC)
most common chemoprophylaxis for meningococcal meningitis-given to who? when? when is vaccine used?
what is used to treat?
-rifampin, close contacts of infect pt, within 2 weeks to be effective. follow this with ceftriaxone
-not for post-exposure prophylaxis, but to develop population immunity in at-risk populations (miliarty recuits, collage freshmen, and healthcare workers)
length constant- def, reglator
measure of how far along an electrical impulse can propagate?
effected by meylination (more increases and less decreases)
summation-def, doesn't occur where?, two types and difference between them?
additive effect of multiple postsynaptic potentials on a target neurons membrane potential. occurs in all parts but not axon
temporal=sequential impulses from neuron over time
spatial=simultaneous impulses from several different neurons
time constant-def, regulation, and why?
time is takes for a change in membrane potential. regulated by myelination
-decrease membrane capacitance, and reduces time constant
listeria- causes disease in whom? treat with? not?
neonates and immunocompromised adults
name sciatica nerve roots
decreased ankle jerk reflex, pain in posterior thigh and leg
role of B6 on levodopa therapy
vit b 6 increases peripheral metabolism of levodopa which decreases its effectiveness
commonly tested side effect of cimetidine
narcolepsy vs sleep apnea vs obesity hypoventilation syndrome
all can have daytime somnolence;
-cataplexy, sleep attacks, hallucinations, and sleep paralysis
-morning HA, depression, snoring, obesity is a risk factor
-aka Pickwickian syndrome, underventilation at all hours, abnormal blood arterial gases
-both OSA and OHS can occur together and lead to complicaitions such as pul HTN, RV heart failure, erythrocytosis, polcythemia
reaction formation vs sublimation
-rxn formation (immature)=redirection of a unacceptable impulse into it's opposite (pt with libidinous thoughts enters monastery)
-sublimation (mature)=replacing an unacceptable wish with a course of cation that is similar to the wish but doesn't conflict with one's value system (aggression redirected to perform well in sports)
hormone released in
nucleus basalis of Meynert
in anterior midbrain, responsible for motor coordination of upper extremities.
Lesch-Nyhan syndrome leads to decreased uptake of? requires increased rate of which process? presentation?
-purine salvage pathway
-purine de novo synthesis
-self mutilation, mental retardation, gout, aggression, dsytonia
hormones derived from proopiomelanocortin (POMC)
-beta-endorphins (bind mu and delta receptors to cause pain)
-MSH (melanin release)
-ACTH (cortisol release)
bonds that stabilize protein form
-ionic, hydrophobic, hydrogen bonds again
drug mode of action, common symptoms and common exam findings:
-hallucinogen, violent behavior, nystagmus
-stimulant, chest pain and seizures, mydriasis
-stimulant, psychosis, tooth decay and choreiform movements
-hallucinogen, visual hallucinations, alert and orientated
-psychoactive drug, euphoira and increased appetite, conjunctival injection
-opiod analgesic, euphoria or coma, miosis and decreased respiratory rate and decreased bowel sounds
PCP, class, mech, moderate amounts cause, higher doses cause,
-antagonizing NMDA receptor
-dissociative symptoms (detachment and withdrawl)
-hallucinations and violent behavior, nystagmus
nerve, cartilage and muscle, derivates of
-1st pharyngeal arch
abnormality results in
- trigeminial nerve,
-meckel cartilage, mandibile, malleus, incus, sphenomandibular ligament
-muscles of mastication, mylohydoid, anterior belly of digastric, tensor tympani, tensor veli palantini
-Treacher Collins Syndrome=mandibular hypoplasia, facial abnormalities
nerve cartilage and muscle derivates of 2nd pharyngeal arch
abnormality results in
-CN VII facial expression (Smile)
-reichert cartilage=stapes, styloid process, lesser horn of hyoid, Stylohyoid ligament
-muscles of facial expression (Stapedius, Stylohyoid, platySma, belly of digastric)
-Congential pharyngocutanepus fistula=persistance of cleft and pouch, fistula between tonsilar area and lateral neck
nerve, cartilage and muscle derivates of 3rd pharyngeal arch
-CN IX (stylopharyngeus, "swallow stylishly"
-cartilage of greater horn of hyoid
-stylopharyngeus "think stylopharyngeus innervated by glossopharyngeal nerve"
nerve, cartilage, and muscle derivates of 4th-6th pharyngeal arches
-CN X 4th=superior laryngeal branch ; 6th=recurrent laryngeal branch
-thyroid, cricoid, artenoids, corniculate, cuneiform
-4th= most pharyngeal constrictors, cricothyroid, levator veli palantini
-6th=all intrinsic muscles of larynx except cricothyroid
arches that form posterior 1/3rd of tongue
in U.S. the majority of overdose deaths are caused by?
prescriptions drugs, particularly opioid analgesics
Do not resuscitate order DNR usually includes
-No intubation or mech ventilation
-do defibrillation or IV drugs to acutely treat a terminal rhythm
-no chest compressions
tibial vs common peroneal vs superficial peroneal
-causes foot plantar flexion and inversion, sensory on sole of foot
-causes foot dorsiflexion and eversion. injury causes "foot drop"
-common peroneal branches to form deep peroneal and superficial peroneal nerve (foot eversion and distal anterior leg and dorsal foot sensory innervation)
CN IX is responsible for salivary secretion from which gland? which is responsible for other glands?
-CN VII (sub mandibular and sublingual)
which CN innervates sensation to tonsillar lining?
do varicella IgG antibodies protect against herpes zoster (VZV reactivation)?
no, they only protect against initially primary varicella-zoster virus infection.
withdrawl symptoms and exam findings of drugs:
-stimulants (cocaine, amphetamines)
-tremors and agitation and anxiety; tachycardia
-tremors, anxiety, insomina;tachycardia
-nausea, vomiting, muscle aches;dilated pupils, yawning, lacrimation
-intense psychomotor retardation, severe depresison ("crash")
name intracellular accumulations in
-heparan sulfate and dermatan sulfate
foamy histiocytes oh histology- disease, presentation, intracellular accumulation, mortality age
Niemann-Pick disease, loss of motor skills, hepatosplenomegaly, hypotonia and a cherry red macular spot, sphingomyelin due to deficiency of sphingomyelinase enzyme. death by age 3
avoidant vs antisocial personalilty vs Schizoid
-feelings of inadequacy, timidity, and fear of rejection, desire acceptance "think Amy from Big Bang Theory!"
-disregard for and violation of rights of others, men under 18.
-do not care if they are accepted or not, detachment from social relationships and restricted range of emotions, "think Sheldon from Big Band theory!"
very long fatty acid of fatty acids with branch points are degraded where
other fatty acids are beta-oxidized where?
peroxisomes: beta oxidation (VLCF) or alpha oxidation (branched chain like phytanic acid)
mitochondria: beta-oxidation for all other types of fatty acids
brief psychotic disorder vs schizophreiform vs schiophrenia
- < 1 month, usually stress related
- 1-6 months
-mores than 6 months
schizoaffective vs bipolar disorder, manic episode with psychotic features
-symptoms of schizophrenia in presence of prominent mood symptoms and a period of at least 2 weeks of psychotic symptoms without mood symptoms
-defined by presence of 1 manic episode (bipolar I) or a hypomanic and depressive episode (bipolar II) with psychotic symptoms (delusions, hallucinations, or disorganized speech)
ABCDE's of melanoma, derived from which embryonic layer?
Border irregularites (pigment fading off edge)
Diameter > or equal to 6mm
Evolving (changes shape, size, color; new lesion)
-neural crest cells
delusional vs schizophrenia disorder (delusion type, other psychotic symptoms, daily functioning?)
non-bizzare delusions (possible but unlikely) for at least 1 month; does not interfere with daily functioning
-bizzare delusions and auditory hallucinations; impairs daily functioning
paranoid personality vs delusional disorder
-paranoia in all aspects of life
-just one fixed delusion
Marfan's vs Fragile X syndrome vs 47,XYY karyotype
all three have tall males
-acrachnodacttyly, ectopia lentis, dilation of proximal aorta (no mental retardation, facial deformatiess or macroochidism)
-mental retardation, facial deformaties (eXtra large everything-ear, testes (macroorchidism), jaw)
-severe acne, delays in motor and language development, antisocial behavior, associated with autism spectrum disorders
mechanism of transamination reactions. essential cofactor?
amino group from amino acid is transferred to alpha-keto acid and alpha-keto acid become an amino acid.
vitamin B6 pyridoxine is needed for amino acid transamination and decarboxylation rxns.
presentation of friedreich's ataxia
hypertrophic cardiomyopathy, diabetes mellitus, kyphoscoliosis, and foot deformities
describe general features of spinal cord that helps to localize a transverse spinal cord lesion
more proximal levels have increasing amounts of white matter and more ovoid sections
lower cervical and lumbosacral regions have large ventral horns
thoracic and early lumbar sections (T1-L2) contain lateral grey matter horns
vitamin A overdose
vitamin C overdoes
-intracranial hypertension, skin changes, and hepatosplenomegaly
-false negative stool guaiac results and diarrhea and abdominal bloating, calcium nephrolithiasis
name four voltage gated sodium channel toxins
Bind to Na+ channel, inhbiting Na+ influz and preventing action potential conduction
-tetrodotoxin (puffer fish)/ saxitoxin (dinoflagellater in "red tide")
Binds to Na+ channel , keeping it open and causing persistent depolarization:
-ciguatoxin (exotic fish, Moray eel)/ batrachotoxin (South American frogs)
what 6 structures are supplied by the posterior cerebral artery?
CN III and IV
-thalamus, mesial temporal lobe, splenium of corpus callosum, parahippocampal gyrus, fusiform gyrus, and occipital lobe
ascending muscle weakness and paralysis. disease and bacteria associated with it
-campylobacter (can also cause infectious diarrhea)
decreased in spinal fluid=disease
-Hypocretin-1 (orexin-A) and hypocretin-2 (orexin-B)
-homovanillic acid (doapmine metabolite)
-narcolepsy with cataplexy (bilateral muscle tone loss associated with emotion)
-impulsive destructive behaviors, agression, suicide
-psycahitric conditions and Parkinson's
name two ways in which a pt being treated for myasthenia gravis can have exacerbation of symptoms. what test is used to distinguish between the two?
-either too much anitcholinesterase drug (cholinergic crisis) or too little drug (myasthenic crisis)
-both present with muscle weakness and fatigue (overstimulation leading to increased refractory period vs understimulation). use edrophonium (short acting cholinesterase inhibitor) (Tensilon test) to distinguish
-if improvement then it's myasthenic crisis=increase dosage
-if no improvement then it's cholinergic crisis=remove drug
clinical presentation of rabies? prophylatic treatment
-restlessness, agitation, dysphagia progressing to coma 30-50 following exposure to bats
-killed virus vaccine
common presentation of
-Osler Weber Rendu syndrome (aka hereditary hemorrhagic telangietasias)
-cerebellar or retinal hemangioblastomas. congential kidney liver, pancreas cysts associated with bilateral renal cell carcinoma.
-kidney, liver and pancreatic cysts, CNS harmatomas vs angiomatous. seizures is major complication
-no cysts vs VHL. disorder of blood vessels. telangiectasia, recurrent epistaxis, skin discolorations, AVMs, GI bleeding, hematuria
common presentation of
Von Recklinghausen aka NF1 vs NF2
-peripheral nervous systems tumor: neurofibromatomas, optic nerve gliomas, Lisch nodules (pigmented nodule of iris) and cafe au lait spots
-nervous system tumor: bilateral CN VIII schwannomas and multiple meningiomas, cataracts, ependymonas
common presentation of Sturge-Weber Syndrome (brain, skin, assocaitions)
-aka encepalotrigeminal angiomatosis. cutaneous facial angiomas, leptomenigial angiomas
-skin involvement of opthalmic V1 and maxillary V2 distributions
-mental retardation, seizures, hemiplegia, and skull radiopacities
rapidly progressive dementia and myoclonic jerk disease tht can be transmitted by pts with corneal transplants, inplanatable electrodes or preparations of GH
CJD creutzfeldt-jackob disease
other cause of carpal tunnel besides repetitive wrist movments
-hypothyroidism (increased fluid)
-dialysis associated amyloidosis (increase fluid)
-endoneural inflammatory inflitration with segmental demylination
-endoneural arteriole hylinization
-endomysial inflammatory infiltration
temporal arteritis (measure levels of? confirm diagnosis with, treatment, association)
-ESR is elevated
-temporal artery biopsy
-polymyalgia rheumatica (muscle pain and morning stiffness)
PIck's vs Alzheimer's disease (macroscopic, microscopic, symptoms, genetic basis)
-atrophy of frontal and temporal lobes vs mild to moderate brain atrophy
-Pick bodies (cytoplasmic inclusions of associated protein tau) vs neurofibrillary tangles, senile plaques and amyloid angiopathy
-behavioral abnormalities, speech and language decline, urinary incotinence vs slow memory loss
-unknown most cases sporadic vs chromosome 21 APP gene, apolipoprotein E4
destruction of inferior lobe of parietal cortex in
-Gerstmann's syndrome (right left confusion, dsygraphia, dyslexia, dyscalculia)
-apraxia and contralateral neglect
how to treat acute neonatal narcotic withdrawl
-give oral opium solution "tincture of opium" and then taper off as pts symptoms improve
-do not give naloxone (opioid antagonist) as it will only make it worse
mechanism of refractory partial seizure drug
-inhibitor of GABA uptake
-blocks Na channels and enhances the effect of GABA
-inhibits GABA-transmainase and increases GABA concentration
-increases brain GABA conentration
which DNA polymerase is the only one that has both 5'-3' exonuclease activity?
-DNA I polymerase
-this is use to remove the RNA primer and remove damaged DNA
which 2 vitamin deficiencies resemble the clinical presentation of Friedrich ataxia? not vit B1 b/c?
-vitamin E (spinocerebellar tracts, dorsal column of the spinal cord, and peripheral nerves)
also vit B12 (subacute combined degeneration- DCML, coticospinal tracts, and spinocrebellar tracts)
-Wernicke's encepaholpathy (confusion, ataxia, and opthalmoplegia)
transference vs projection vs displacement
-unconscious shifting of emotion or desires associated with one person (sibling, parent, spouse) to another (physician, therapist) can be positive or negative
-attributing one's own unacceptable feeling to another person (accusing therapist of having sexual feelings when pt is the one with feelings)
-shifting feelings or conflicts from one situation or person to another seen as a safer and less distressing (medical student berates another student after being belittled by attendings on rotation)
amyloid deposits in brain
amyloid deposits in vascular walls
presentation of trigeminal neuralgia, drug used to treat this? other second line choices
-presents with brief episodes of sudden and severe "electric shock-like" to "stabbing" pain in distribution of CN V (V2 and V3)
-carbamazepine is drug of choice
-can also use baclofen and valproic acid
serotonin syndrome treatment
cyproheptadine an antihistamine
common cause of pathologic vertebral fracture? not RA b/c?
-osteoporosis. can be caused by chronic systemic use of corticosteroids
-autoimmune inflammatory disorder that predominantly affects synovial joints in hands and feet. vertebral fractures do not have synovial space so RA doesn't work here
which hormone level is decrease in Alzheimer's? what is mechanism?
-decreased acetylcholine in nucleus basalis of Meynert (in forebrain) and hippocampus
-diminished activity of choline acetyltranferase
locus cerculus-location, neurotransmitter, implicated in which 2 diseases
-caudal pontine central gray matter
-lots of NE
-panic attacks and panic disorder
mc cause of community-acquired pneumonia in HIV pt with normal CD4 count vs low CD4 count? what are ranges? why not legionella?
-normal 400-1400cells/uL: streptococcus pneumoniae like regular population
mc cause of atypical pneumoniae
define Charcot-Bouchard aneurysm vs saccular aneurysm- size, location, associations, rupture leads to what type of hemorrhage?
-small in size, located in artioles that supply basal ganglia, internal capsule, and deep white matter and associaited with HTN, rupture causes intracerebral hemorrhage
-aka berry aneurysms, ACA, Ehler's danlos and ADPKD, subarachnoid hemorrhage (SAH) "worst HA of my life"
hyper-densities within cisterns/sucli
SAH worst HA of life
primary site of entry of cryptococcus neoformans? not nasopharynx b/c?
lungs b/c it causes pneumoniae and not sinusitis
drugs to treat agitation. can cause what complication? vs serotonin sydrome how to treat?
-neuroleptic malignant syndrome (hyperthermia, extreme generalized rigidity, autonomic instability, and altered mental status)
-no rigidity but myoclonus instead
-treat NMS with dopamine agonist and/or direct muscle relaxants (dantrolene) decrease mortality rates
all asociated with anti-psychotic use.
-twisting involuntary movements
-excessive dpamin blockage- rigidity, bradykinesia, and tremor
-subjective feeling of restlessness or objective motor restlessness
-chronic, dose-related disorder. persistant lip smacking or rhythmic tongue and chewing movements
how is lagging strand constructed? how is it synthesized?
-synthesized 5-3 via formation of Okazaki fragments
mech of beta blocker in treatment of glaucoma. not altering trabecular meschwork b/c?
-decrease aqueous humor production by ciliary epithelium*
-this isn't targeted by glaucoma drugs. note that cholinergic agonists (pilocarpine and carbachol for closed angle glaucoma) contract pupllary sphincter of iris leading to miosis and this caused increased angle leading to more accessible outflow of aqueous humor. can also contract ciliary muscle causing lens to be more convex and increasing flow (open-angle gluac)
meniere disease triad vs labryrinthtis
-hearing loss (sensorineural)
-caused by increased vol and pressure of endolymph in vestibular apparatus
- inflammation of vestibular lymph that causes acute- vertigo, nausea, and vomiting. not recurrent
thioridazine is associated with
chorpromazine is associated with
defects of fragile X syndrome are due to? not chromosome breakage b/c? presents how? gene
-increased number of CGG repeats leads to hypermethylation of cytosine bases and gene inactivation.
-fragile X is named b/c the X chromosome is thin and constricted when cultured in folate and thymidine deplete medium for karyotype analysis
-mental retardation, facial dsymorphism (large jaw), and macroochidism
-FMR1 (fragile X mental retardation 1 gene)
lesions of ventromedial nucleus result in? lateral nucleus?
-ventromedial contains satiety center and regulate food intake. lesions make you obese secondary to hyperphagia, also have aggressive savage behavior
-signals hunger lesions make you lose desire to eat
anterior hypothalamic nuclei coordinates. posterior?
-cooling by stimulation of parasympathetic nervous system
-heat conversion and heat production can body is cold
how to prevent illness by clostridium tetani (tetanus). how to protect neonates? why not give them vaccine? infection is caused how?
-proper immunization with a childhood series (2 months, 4 months, 6 months, 15-18months, and 10-12 yrs) and booster every 10 yrs in adulthood
-protect neonate by immunizing mother who can pass IgG through placenta to protect baby for 1st 2 months. can't give vaccine to neonated before this time b/c their immune system isn't developed yet?
-from colonization of umbilical stump
deficiency in factors that mediate membrane attack complex (C5b-C9) can lead to what type of infections? increased levels of what immunoglobin can also increase risk of this infection? what type of rash does this infection cause?
-IgA, prevents binding to IgG and IgM that activate complement
-small vessel vasculitis causing petichial rash affecting palms and soles
conversion disorder- define and pt pop
unconsioud manifestation of neurological symptoms when pathophysiological explanations for symptoms cannot be explained
-women after significant life stress
somatization disorder-define, time period, must begin before what age, impact what aspect of life?, name number and type of symptoms needed?
-pts with numerous physical complaints over course of years with no explaination
-start prior to age 30
-impacts social and occupational functioning
-4 pain symptoms, 2 GI, 1 sexual, and 1 pseudoneurological symptoms
central vs arcuate scotomas
-scotomas is visual field defect in a discrete area of altered vision surrounded by normal areas of vision
-lesion of macula cause central scotomas
-damage to region of optic nerve head leads to results in visual defect that follow arcurate shape of nerve fiber pattern
CN damaged in hyperacusis
-increased sensitivity to sound
-CN VII due to paralysis of stapedius muscle
describe flow of CSF in ventricles
-lateral to third vent via interventricular foramina of Monro
-to fourth vent vis cerebral aqueduct of Sylvius
-exits via lateral foraminia of Luschka (2) or medial foramen of Magendie
-returns to venous circulation via arachnoid villi (granulations)
forms of hydrocephalus (5)
-non communicating-obstruction of CSF within brain or brainstem
-communication-blockage in subarachnoid space
-normal pressure- CSF is not absorbed by arachoid vili
-hydrocephalus ex vaco-increased csf vol due to brain atrophy
-pseudotumor cerebri- related to decreased CSF outflow at arachnoid villi, seen in overweight young females
T or F symptoms of psychosis are seen with adjustment disorder. time frame
-instead look for increase anxiety, depression, disturbed behavior that develops 3 month of an stressor and last no more than 6 months after stressor
nitrogen atoms in urea cycle are derived from? rate limiting step in urea cycle? molecule that activates this step
-NH3 and aspartate
-carbamoyl phosphate synthetase I
-activated by N-acetylglutamate (NAG)
how is NAG formed?
from glutamine and acetyl- CoA with NAG synthetase in hepatic mitochondria
what causes clinical symptoms in pts with SAH from rupture of berry aneurysm? drug to treat it?
-calcium channel blocker like nimodipine
what is used to treat alcohol withdrawl. mech of action
-substitute for the action on GBA receptors (where alcohol usually works)
-use long acting (chlordiazepoxide and diazepam) for this and short acting (lorazepam and oxazepam) for advanced liver disease
superficial blanching of nest of distended capillaries
name toxin that takes the route of
-wound-neuron axons-salivary glands
-wound-motor-neuron axons-spinal cord
-fibrinous exudate-systemic circulation-cortical neruons
-food systemic circulation-peripheral nerves
-tetanospasmin from C tetani
besides CN VIII which nerves can be affected by Schwannomas?
CN VII and CN V
what type to tumors arise in pineal and suprasellar regions/ 2 symptoms
-germ cell tumors
-Parinaurd syndrome (paralysis of vertical gaze)
disease specific mortality
rate of increase of disease
-number of deaths attributable to disease/total pop
-[number of new cases per year-number of deaths (or cures)]/tot pop
-number of deaths per year/tot pop
how does methymalonic acidemia develop. no carboxylation b/c?
-defects in isomerization rxn that transforms methmalonyl CoA to succinyl CoA entering the TCA cycle
-rxn that converts propionic acid to methylmalonic acid is accomplished through biotin dependent carboxylation (via propionyl CoA carboxylase)
eating disorder characterized by binge eat and either restrictive or purging compensatory behaviors.
severity of N. meningititdis is related to serum levels of? not caspsular polysaccharide b/c?
LOS (lipooligosaccharide) is responsible for many toxic effects in meningitis and meningoccemia.
-capsule assist N. m in resisting phagocytosis but it's serum levels are not indicative of morbidity and mortality
LOS vs LPS
-LOS (lipooligosaccharide) in N. meningococci lacks repeating O-antigen of LPS
drugs used to treat absence seizures, which one can also treat tonic-clonic seizures
-ethosuximide and valproate
blood/gas partition coefficient- determined what? (values of amt need to saturate blood, rise and partial pressure in blood, equilibration wiht brain, an onset of action for a high value and low value. give examples of each.
-determines onset of action of gas anesthetic
-drugs with high blood/ gas p.c are more soluble in blood (large amt is needed to saturate) and demonstrate slower rise in partial pressure and equilibration with brain and have longer onset times (halothane)
-gases with low blood/gas pc are less soluble in blood (less amt is needed to saturate), demonstrate faster rise in partial pressure and equilibration with brain and have slow onset times (N2O)
how to calculate confidence interval? how does sample size change CI value? which z score correlate with 95% CI and 99% CI
-Mean+/- (Zscore)*SEM (aka standard error of mean)
-large sample size (n) tightens confidence interval (makes it smaller)
-95% CI has z score=1.96
-99% CI has z score=2.58
define status epilepticus-
how to treat?
-recurrent or continuous seizures that last for more than 30 mins
-IV benzo (preferably lorazepam) b/c of rapid onset
-load with phenytoin to prevent recurrence of seizures. onset is about 15 mins
-if still seizing start phenobarbital
-if still seizing give general anesthesia and intubate
area postrema-location, func
-dorsal medulla near fourth ventricle
-contains chemoreceptor trigger zone (CTZ) that causes acute nausea following administration of systemic chemotherapy.
progressively weakning diaphragmatic contraction during maximal voluntary ventilation with intact phrenic nerve stimulation indicates
-neuromuscular junction pathology (myasthenia gravis)
-abnormal rapid diaphragmatic muscle fatigue (restrictive lung or chest wall disease)
-mc cause of bacterial meningitidis of all ages.
-mc cause in outbreaks among ppl living in close quarters (prisions, colleges, army)
-mc cause in neonates
arterivenous concentration definition and application to onset of inhaled gases
-difference in concentration of gas anesthetic in arterial and venous blood
-reflects overall tissue solubility of an anesthetic
-high tissue solubility gases have large AV concentration gradients and slower onsets of action
-low peripheral tissue solubility reflects low, AV concentration gradients which allow blood concentration to equilibrate quickly which increases onset of action
what factors determine anesthetic concentration
-inhaled air- partial pressure
-lungs-pulmonary ventilation rate directly related to rise of gas tension in alveoli
-blood solubility-blood/gas partition coeffiecient (higher=slower onset; lower=faster onset)
-peripheral tissue solubility-AV concentration gradient (higher=slower onset, lower=faster onset)
types of headaches (gender prdilection, family history, onset, location, character, duration, assocaited symptoms)
-migraine, F>M, family history often present*, variable onset, unilateral, pulsatile and throbbing, 4-72 hrs, auras*, photophobia, nausea
-cluster. M>F*, no family history, during sleep, behind one eye, excruciating sharp and steady, 15-19 mins, sweating facials flushing, congestion, lacrimation and pupillary changes
-tension. F>M, no family history, under stress*, band like pattern around head (bilateral), dull tight, persistent, 30min-7 days, muscle tenderness in head, neck, and shoulders
treatment of abortive therapy during an acute migraine. treatment for prophylaxis
-triptans (serotonin agonists 5-HT-1b and 5-HT 1D)
-beta blockers, antidepressants (amitrptyline and venlafaxine) and anticonvulsants (valproate an topiramate)
pt with complete contralateral sensory loss
thalamic syndrome- damage to VPL and VPM nuclei
-no motor defects
small 5-6mm cavities in basal ganglia
-lipohylainosis (DM) and microatheromas (HTN)
treatment for hyperrexia. which should be done first?
-body temp above 40C should be treated with increasing body heat loss (cooling blankets and fans) and antipyrectics (acetaminophen-blocks PGE2 synthesis)
-use cooling measures first b/c it's effective immediately
rapid plasma decay of thiopental (barbiturate like IV anesthetic) is due to? not liver metabolism b/c?
is due to redistribution of drug not metabolism of drug?
cause of Arnold-Chiari malformation. types and their age of presentation
-impaired development of posterior fossa
-type 1 benign and manifest in adulthood
-type II is severe and manifest in newborn
mc cause of death in PCP intoxicity
-trauma and violence
-MI and stroke
side effect of
-agranulocytosis and seizures
name medications that may cause seizures and their uses?
-clozapine (antipsychotics at high doses)
-isoniazid (anti-TB; give pyridoxine and this risk decreases)
what amino acid is converted to urea?
-arginase is an enzyme of the urea cycle that produces urea and ornithine from arginine
senses responsible for:
-pain and temp/ positions and proprioception
-VPL (ventral posterolateral nucleus) via spinothalamic tract and medial lemniscus
-VPM (ventral posteromedial nucleus)
-lateral geniculate body from optic nerve
-medial geniculate body via superior olivary nucleus and inferior colliculus of pons
what is methadone the drug of choice for heorin abuse?
vry potent, long acting opiate with good oral bioavailability
-helps to suppress withdrawl symptoms
all effects of organophosphates can be reversed by atropine except? why? which other drug can be given to reverse all effects?
-b/c organophosphates inhibit both muscarinic and nicotinic receptors
-pralidoxime b/c it restores cholinesterase from its bond with these substances
cholinergic receptors activate release of NO from endothelial cells in blood vessels true or false? NO is also known as?
endothelium-derived growth factor
what factor allow for E. Coli to cause meningitis in neonates?
-capsule (K-1 antigen) allows bac to survive hematogenous spread and to establish meningeal infection
presentation of cerebral hyperperfusion vs cardiac embolism
-aka ischemic-hypoxic encephalopathy. bilateral wedge shaped band of necrosis over the cerebral convexity (lateral to "interhemispheric fissure")
-mutiple infarcts in vascular territories not inbetween them
name P450 actvators. their relation to hypercoagulabilty?
chronic alcoholic Mona steals phen-phen and Never refuses greasy carbs
-chronic alcohol use, Modafinil, St. John's Warts, Phenytoin*, Phenobarbital*, Nevirapine, Rifampin*, Griseofulin*, and Carbamazepine*
-Warfain is metabolized by P-450 systems and it's effects are reduced with these drugs
heat kill bacteria vaccines (3)
bordetella pertussis, vibro cholerae, yersinia pestis
which general anesthesia can cause damage to the liver? what type of damage is seen on histo? not intrahepatic cholestatsis because?
-massive hepatic necrosis (centrilobular)
-drugs that cause this are OCPs, steroids and chlorpromazine; all cause biliary tract obstruction
occlusion of posterior inferior cerebellar art vs posterior cerebral art
-lateral medullary (Wallenburg syndrome); contralateral loss of pain and temp, ipsilateral defectis of CN V, VIII, IX, X, XI; and Horner's
-contralateral homonymous hemianopia with macular sparing
-supressing one's feeling by thinking about the problem
-surgeon who avoids his feeling of failure by thinking abt every step of surgery in meticulous detail
name the poison:
-antimuscarinic effects only
-overstimulation of ach+ CNS
-competitive antagonist of muscarinic acetylcholine receptors (atropine or scopolamine)
-botulinum toxin both nicotinic symps include (diplopia and dysphagia)
-acetylcholinesterase inhibitors (organophosphate compounds)
CNS complication of measles? missing which protein? T or F there is hemagglutinin on the capsule
subacute sclerosing encephalitis
-antibodies to M protein are missing (allow virus to persist in CNS after initial infection is cleared)
anemia + CNS symptoms
vit B12 defiency
CN damaged in vertical diplopia? not CN III b/c?
CN III innervated the superior rectus, medial rectus, inferior rectus and inferior oblique (would cause both vertical and horizontal diplopia)
alternative drug for depression treatment without sexual side effects
contrast on CT indicates? not ischemic stroke b/c
acute intraparenchymal hematoma (Charcot-Bouchard pseudoaneurysms and saccular aneurysm)
-ischemic stroke would have clear CT
charcot-bouchard pseudoaneuryms vs berry (saccular aneurysms)
-result of rupture
-symptoms of rupture
-HTN vs ADPKD, Marfan's, Ehler's Danlos syndrome
-small art that perfuse basal ganglia vs circle of willis, anterior and posterior comms, middle cerebral
-<1mm vs variable 2-25mm
-intracerebral hemorrhage in basal ganglia, internal capsule, thalamus, pons vs subarachnoid hemorrhage
-focal deficits vs altered level of consciousness and more prominent than focal symptoms
lobar parenchymal hemorrhage vs basal ganglia and thalami hemorrhage
-cerebral amyloid angiopathy vs HTN (Charcot-bouchard pseudoanerysms)
endoneural inflammatory infiltration vs endomysial inflammatory inlitration
-Guillain-Barre syndrome (demyelination)
vs polymyositis (affects muscles not nerves)
occlusion of left anterior cerebral art
right hemiplegia with lower extremeties affected more than upper. (urinary incontinence and primitive reflexes (Monro, grasp, tonic reflexes) may also be present
elimination of lithium? drugs that can affect this? presentation? and treatment?
-kidneys in PCT
-drugs that cause renal injury or increased Na+ reabsorption in PCT (NSAIDs, thiazide diuretics, ACE inhibitors)
-neuromuscular excitability, coarse tremors, fascicular twitching, ataxia, delirium
mech of H. influenzae spread vs N. meningitidis
-pharynx to lymphatics to meninges
-pharynx to blood to choroid plexus to meninges
what cofactor is needed to synthesize tyrosine, DOPA, serotonin, and NO
action of dihydrobiopterin reductase
convert BH2 to BH4 for DOPA, tyrosine, serotonin, and NO synthesis
neonatal intraventricular hemorrhage usually occurs where? complication?
germinal matrix (blood in lateral ventricles)
long-term neurodevelopmental impairment
name come complications of prematurity
respiratory distress, PDA, broncopulmonary dysplasia, intraventricular hemorrhage, retinopathy of prematurity
mech of action of ethosuximide, use
block T-type calcium channels in thalamic neurons causing hyperpolarization
which drugs acts by decreasing sodium current in cortical neurons
-blocks NMDA receptors and affecting K current
-phenytoin, carbamazepine, and valproic acid
name typical high potency antipsychotics
haloperidol, fluphenazine, pimozide
name typical low potency antipsychotics
name atypical antipsycotics
clozapine, risperidone, olanzapine, and quetiapine
"It's atypical for old closets to quietly whisper"
first generation vs second generation (atypicals) treatment of Schizo
-only treat positive symptoms
-treats both positive and negative symptoms
positive and negative symptoms of schizo
-positive=hallucinations, delusions, disorganized speech, behavior
-decreased emotional range, poverty of speech, and loss of interest in living
name three extrapyramdial side effects of antipsychotics
acute dystonic reaction-sudden-onet, sustained muscle contractions
akathisia-subjective restlessness with inability to sit still
drug induced parkinsonism-tremor, rigidity, bradykinesia, and masked facies
thiamine is cofactor for (3)
-pyruvate dehydrogenase (pyruvate to acetyle CoA)
-transketolase (pentosese to glyceraldehyde 3P)
-can measure activity of these enzymes to determine thiamine defiency
name three important dopaminergic system pathways
mesolimbic mesocortical pathway, function and associated disease
nigrostriatal pathway function and associated disease
coordination of voluntary movements
tuberoinfundibular pathway function and disease
-controls prolactin secretion
mc cause of death in TCA? treatment
-refractory hypotension and cardiac arrhythmias
-inhibits fast sodium channels
-normal saline and hypertonic sodium bicarbonate administration
skull and dura
dura matter and arachnoid
arachnoid and pia mater
-epidural (middle menigeal art)
-subdural (bridging cortical veins)
-subarachnoid (anerysm or AV malformation of anterior and post comm)
symptoms and CT scan of
-lucid interval followed by loss of consciousness (biconvex hematoma)
-gradual onset of HA and confusion (crescent shaped hematoma)
-severe HA, fever, nuchal rigidity (blood in basal cisterns)
lacunar infarct symps/ location
pure motor (hemiparesis)
pure sensory (stroke)
dysarthria-clumsy hand syndrome
-posterior limb of internatl capsule
-ventroposterolateral or ventroposteromedial thalamus
-base of pons
-base of pons or genu of internal capsule
mech and resolution of lacunar infarct
-hypertensinve changes in arterioles (sclerosis)
- small cystic lesions filled with CSF =lacunas "lake like"
intracranial hemorrhage that lower mortality rate
cerebral amyloid angiopathy
two toxicities of lithium?
hypothyroidism and nephrogenic diabetes insipidus which can build up in pts with renal insuffiency
name 2 first generation anti-histamines? what is responsible for majority of side effects? not anti histamine b/c?
dipheydramine and bdlorpheniramine haev antimuscarinic, anti-alpha adrenergic and anti-serotonergic properties that are responsible for the majority of side effects (blurry vision, pupillary dilation etc...)
-this causes drowsiness and cognitive dsyfunction
name receptor for viron:
-CD4 and CXCR4/CCR5
-nicotinic acetylcholine receptor
name common drug interactions implicated in serotonin syndrome
-SSRIs, SNRIs, MAOs, TCA
-5-HT3 receptor antagonists (ondansetron)
symptoms of serotonin syndrome
-neuromuscular excitation, autonomic stimulation, altered mental status
tolerance to what side effects of opiods does not readily occur?
constipation and miosis
-treat pts prophylactically with adequate fluid intake and daily laxatives
name two class of dopamine agonists and give 3 examples
ergot compounds: (bromocriptine and pergolide)
non ergot: pramipexole and ropinirole
name short acting hypnotic drug that is similar to benzodiazepine but has much lower risk for tolerance and dependence
name three types of GABA receptors, location, structure, and effect of stimulation?
-GABAa=brain, ion channel, Cl- influx
GABAb-brain, g-protein, K+ efflux, decreased Ca2+ influx, inhbition of adenylyl cyclase
GABA c=retina, ion channel, Cl- influx
benzodiazepine binding receptor vs bacterial toxins (pertussis and cholera)
-GABAa vs GABAb
abdominal mass in child that has opsoclonis-myoclonus (rapid eye movement back and forth). not Wilm's tumor b/c?
-neuroblastoma causes opsoclonus-myoclonus (paraneoplastic syndrome. located in adrenal medulla
-Wilms affects children but no paraneoplastic syndrome
effect of N-acetylglutamine in urea cycle, formed how?
-activator of carboamoyl phophate synthase I (rate limiting step)
-formed by actely-CoA and glutamate (N-acetylglutamate synthetase)
what area in brain is damaged during global ishemia?
which is damaged first?
-pyramidal cells of hippocampus and neocortix and purkinje cells of cerebellum
what can cause cystic degeneration of putamen vs caudate
1st sign of uncle herniation
fixed and dilated pupil on side of herniation
-ipsilateral paralysis of oculomotor muscles, con or ipsi hemiparesis, and contr homonymous hemianopsia with macular sparing
mutation of myelin protein gene leading to "neural form" of muscular atrophy
-Charcot-Marie Tooth disease
-weakness of foot dorsiflexion b/c common peroneal nerve is mc nerve affected
mech of upper motor injury in hydrocephalus
stretching of periventricular pyramidal tracts
undoing coping mechanism
-symbolically nullifying an unacceptable or guilt provoking thought, idea, or feeling by confession or atonement
treat depression and insomnia? HY side effect
hypersomnolence disorder vs narcolepsy
-sleepiness vs sleep attacks
-not feeling refreshed vs feeling refreshed
on-off phenomenon vs wearing off phenomenon in parkinson's disease
-unknown etiology vs due to destruction of striatonigral dopaminergic neurons
-can't be fixed with increasing dose vs can be fixed with increasing dose
-unpredictable vs predictable
signs of opioid overdose and channels related to them. reverse with?
-mu-respiratory and cardiac depression, reduced GI motlity
-reverse with naloxone
an old infarct (months or years) consists of a csytic cavity surround by dense wall made up of?
astrocyte processes that have proliferated (gliosis)
-urine that changes dark color with standing + neurological disturbances
-urine that changes dark color + blistering cutaneuos photosensitivity
-urine that changes dark color+ arthralgias or benign presentation
-acute intermittent porphyria
-cutaneous porphyria tarda
-alkaptonuria (aka ochronosis)
conus medullaris syndrome vs cauda equina syndrome
-L2 lesion, flaccid paralysis of bladder and rectum, impotence an saddle anesthesia (S3-S5)
-lesion from massive rupture from intervert disk compressing any of 18 roots, low back apin, saddle anesthesia, loss of anocutaneous reflex (anal wink)
damage to S1-S2
loss of Achilles (ankle-jerk) reflex
how to treat TCA vs digitoxin associate cardiotoxicities
component tested in mental status examination:
-providing name, location, and current date
-following multi step commands
-reciting months of year backwards
-recalling three unrelated works after 5 mins
-proving details of significant life events
-writing sensible sentence containing noun and verb
-drawing clock face
-short term memory
-long term memory
early Duchenne muscle dystrophy vs late Duchenne muscle dsytrophy
-hypertrophy of muscle fibers
-pseudohypertrophy. replacement of muscle fibers by fat and connective tissue
accumulation of lipids within muscle fibers vs fibrofatty muscle replacement
-carnitine palmitotyltranferase deficiency
-Duchenne muscle dsytrophy
effect of beta 2 receptors on uterus vs effect of alpha 1 receptors on bladder
-relaxation of uterus (tocolysis)
-contraction of internal urethral spincter
antiviral agent used to treat Parkinsons
amantidine (treats influenza)
what is neurologists favorite way to treat parkinsons
used combination of selegiline, anticholinergics, and amantadine until they no longer provide control of symptoms then used levodopa/carabidopa
antibodies against pili in meningococus is protective why? no against hematogenous dissemination b/c?
protects against pharyngeal colonization
-meningococcus is protected from phagocytosis in blood due to capsule.
triad of NPH
wacky, wobbly, wet (ataxia, CNS problems, and urinary incontinence)
how to treat malignant hyperthermia?
blocks ryanodine receptors and prevents release of Ca into cytoplasm of skeletal muscle fibers
how to treat crytococcal menigitis
amp B and flucytosine
toxicities of carbamezapines (3)
bone marrow suppression, hepatotoxic, SIADH
medulloblastoma-epi, age, location, histo, prognosis
-2nd mc cause of brain neoplasm in childhood
-cerebellum at vermis
-small blue cells (prmitive neuroectodermal tumors)
vit B12 deficiency vs amyotrophic lateral sclerosis
-subacute combine degeneration, ascending (dorsal column) and descending (corticospinal pathways)
-upper and lower motor neuron lesions
CNS syndrome associated with large vessel arteritis aka giant cell vasculitis?
why should pt wait 14 days before switching from MAO inhibitors to SSRIs?
b/c MAO enzyme needs time to regenerate to avoid serotonin syndrome
mech of NPH cause urge incontience
-caused by stretching of descending cortical fibers that run in distended paraventricular area (coordinates relaxation of external urethral sphincter with bladder contraction)
-caused by stretching of sacral micturition center (responsible for bladder fullness sensation and contraction when full)
-stretching of cerebral cortex (responsible for inhibiting sacral micturition center)
enzymes needed to convert branched amino acids into TCA cycle and diseases caused if missing
-alpha keto acid dehydrogenase converts branched a.a. (valin and isoleucine) to propionyl CoA (maple syrup disease)
-propionyl CoA carboxlyase with biotin cofactor converts propionyl CoA to methlymalonyl CoA (propionic acidemia)
-methylmalonyl CoA isomerase with vit B12 as cofactor converts methylmalonyl CoA to succinyl CoA
which of the branched a.a does not enter TCA as succinyl CoA
-alpha keto acid dehydrogenase converts all branched a.a. into TCA cycle intermediates
-valine and isoleucine enter as succinyl CoA
-leucine enters as acetyl CoA
presentation of NFT-1 and aka
-von Recklinghausen disease
-cutaneous and subcutaneous neurofibromas (skin colored or pink nodules with rubbery texture that button hole with gentle pressure)
-can also see pigmented spots (cafe au lait) and headaches due to gliomas
schwannomas can arise from any CN except? histology
biphasic pattern= loose (Antoni B) and dense (Antoni A) growth patterns
receptor clozapine works on vs other receptors? HY toxicity
agranulocytosis and seizures
which antipsycotic causes prolonged QT
-steven johnson syndrome
-acute intermittent pophyria
-serum-sickness like syndrome and undesriable cosmetics (hirtuism, acne, gingival hypertrophy) and lympadenopathy
how is tetanus diagnosed
clinical. based on history
name opioid narcotic designed to produce analgesic effects with little to no abuse potential. mech? effect on pt who are dependent or tolerant on morphine or other opioids
-partial agonist and weak antagonist at mu receptors
-causes withdrawal symptoms
germinomas are most common found in what gland? histo, symptoms (3)
-similar to testicular seminomas
-precocious puberty (beta-hCG production)
-obstructive hydrocephalus (aqueductal compression)
-Parinaud syndrome (paralysis of upward gaze and convergence due to compression of tectal area of midbrain)
if you see paralysis of upward gaze think?
Parinaud syndrome assocaited with tumor of pineal region. mc tumor in this area is germinomas
gardiner with atropine poisoning like symptoms
-Jimson Weed (Datura stramonium poisoning)
-aka Gardener's mydriasis
oligoclonal bands of IgG in CSF
when do you se oligodendrocyte depletion
progressive multifocal leukoencephalopathy and multiple sclerosis
two cells involves in resolution of cerebral ischemic infarct
-microglial phagocytize the fragments of neurons, mylein, and necrotic debris
-then astrocytes forms a glial scar along periphery
intracerebral hemorrhage vs subarachnoid hemorrhage. which one is associated with ADPKD?
-HA, vomiting, and sudden focal neurological defects
-worst HA of life, no focal defects, neck stiffness b/c blood irritated meningies, papiliedema or pupillary dilation may also be noted
-subarachnoid hemorrhage in circle of willis
innervation of ear
-posterior part of external canal
-external tympanic membrane
-inner surface of tympanic membrane
-which of these can cause syncope?
X by small auricular branch (can lead to vasovagal syncopal episode)
V3 by auriculotemporal branch
IX by tympanic branch
which receptors inhibit insulin release and which one stimulates insulin release?
three important things to know abt phenytoin metabolism
-dose dependent metabolism
-depends on function of p450 system
-induces 450 system
name commonly prescribed drugs that are metabolized by p-450 system
Always, always, always think when starting others
which neuronal tumors stain for GFAP vs synaptophysin
-GFAP (glial origin, astrocytomas, ependymomas, and oligodendrogliomas)
-protein found in presynatic vesicles of neurons, neuroendocrine and neuroectodermal cells
Broca's aphasia vs precentral gyrus leisions
-language deficit (broken speech)+can also lead to right sided hemiparesis and oral apraxia
-dsyarthria due to paresis of skeletal muscles of mouth no language deficits
Broca's vs Wernicke's vs Conduction
-Broca's nonfluent speech (look for right hemiparesis)
-Wernicke's fluent speech+ greatly impaired comprehension (look for right superior visual field defect)
-Conduction some fluency+some comprehension+no repetition*** (no other associated features)
treatment for toxoplasmosis vs crytococcus neoformans
-pryimethamine and sulfadiazine (look for ring enhancing lesions)
-amphotericin B (no ring enhancing lesions)
primary CNS lymphoma in HIV pts is composed of? not macs b/c
-mac are found in granulomas that can affect the skull. no ring enhancing lesions
astrocytes vs oligodenrocytes vs microglia (nuclei and processese)
-round nuclei, no halo
-round dark nuclei, with pale halo
-elongated nuclei, many processes
tissue repair in peripheral system vs CNS
-mediated by fibroblasts
-mediated by astrocytes
mytonic dsytrophy vs mitochondrial myopathies
-sustained muscle contration (myotonia)+cataracts+frontal balding+selective type 1 fiber loss
-oxidative phophorylation problem+all muscle types affected+ragged red fibers
benefit of 1st vs 2nd gen antihistamines
-2nd gen has minimal sedative and antimuscarinic effects (fexofenadine)
-1st gen (chlorpheniramine, diphernhydramine, and promethazine)
how do penicillins and cephasporins work vs vancomycin
-these bind to penicillin bind to proteins (i.e. transpeptidase)
-this binds to terminal D-ala residues of cell wall glycoproteins and prevents cross linking by transpeptidase
treatment for narcolepsy
scheduled daytime naps and psychostimulants (modafinil)
neoplasms associated with myasthenia gravis vs lambert-eaton syndrome
-MG has an increased risk for thymoma
-LE presents due to a pre-existing malignacy (small cell lung carcinoma)
how 1st gen anti-psychotics are classified? difference in side effects
-low potency (non neurologic side effects- sedation, anticholinergic side effect, orthostatic hypertension) (chlopromazine and thioridazine)
-high potency (extrapyramidal symptoms) (Haloperidol and fluphenazine)
rapid correction of hyponatriemia
-central pontine myelinolysis
-nothing if correction to normal. hypocalcemia can cause carpopedal spasms
malformation vs disruption
-primary defect in cells or tissue that form an organ (congential heart disease, holoprosencephaly, polydactyly etc..)
-secondary breakdown of previously normal tissue (rupture of amnion that produces bands that compress or amputate fetal limbs-aminotic band syndrome)
deformation vs sequence
-fetal structures occur due to extrinsic mechanical forces (congenital hip dislocation, potter's syndrome clubbed feet)
-a number of abnormalities results from a single primary defect (i.e potter's syndrome)
damage to CNIII due to DM vs damage due to nerve compression
-CN III para and symp have different blood supplies.
-DM leads to ischemia of somatic fibers only (ptosis, down and out eye that's reactive to light)
-compression causes loss of both sympathetic and parasympathetic fibers (ptosis, down and out and non-reactive to light)
which virus tend to effect temporal lobe? meningies? what can cause a mixed meningoencephalitis?
-herpes simplex virus 1
-mycobacteria and fungi
mech of phenytoin and gingival hyperplasia
-increased expression of PDGF (plt dervied grwoth factor)
-gingival mac due to PDGF stimulate proliferation of gingival cells and alveolar bone
oligoclonal band on electrophoresis of CSF think what?
IgG think MS
paraneoplatice cerebellar degeneration is associated with which tumors and antibodies
-lung anti-HU and anti-P/Q
chronic MS plaques vs acute MS plaque
both have loss of myelin
-chronic will have loss of axons
-acute will still have axons
mutation of a myelin protein gene vs a muscle structural protein gene
-Duchenne's muscular dystrophy
-Charcot-Marie tooth disease (look for foot drop due to common peroneal nerve involvement)
atypical depression vs major depression
how to best treat atypical depression or treatment resistant depression
-atypical will have mood reactivity (leaden fatigue (arms and legs feel heavy) rejection sensitivity (overly sens to slight criticism), reverse vegetative signs (increased sleep and appetite))
-major will not have change in mood in response to something positive, also they have suicidal ideations
-wing beating tremor, psychosis, and cirrhosis, Kayser-Fleischer rings in cornea (Wilson's disease aka hepatolenticular degeneration)
-post-stroke pain (contralateral)
which antidepressants have higher risk of inducing mania and susceptible pts
TCAs and venlafaxine
describe simple partial vs complex partial seizures
-one body part involved, no loss of consciousness or postictal confusion
-from temporal lobe (mood changes, illusions, hallucinations), impaired consciousness and postictal state are present*
describe tonic-clonic vs myoclonic vs absence
-tonic extension then clonic rhythmic* movements, loss of consciousness, and prolonged postictal confusion
-brief arrhythmic* jerking, clusters of few seconds, no loss of con.
-brief epidoses of staring, no partial confusion
name two partial seizures and their 1st line treatment options
simple partial and complex partial
use carbamazepine for both
name three generalized seizures and their 1st line drug treatment option
-tonic clonic (phenytoin, carbamezapine, and valproate)
-myoclonic (valproic acid)
-absence (ethosuximide, 2nd line valproate)
describe axonal reaction vs compression atrophy vs irreversible cell injury
-cell body responds (generate more protein for repair) due to severed axon, increased size* and rounded with nucleus displaced to periphery and Nissl substance become dispersed (central chromatolysis)
-cell decreases in size* and number
-cell decreases in size and becomes eosinophilic
name three substances that stimulate gastric cells? which one increases parietal cells growth also? mucosal cell growth?
-histamine, acetylcholine, and gastrin
-TGF-alpha from carcinomas, macs, or epithelial cells
look at a picture of neurofibromas/ and Lisch nodules. what are they associated with? what other findings should you look for?
-many short, sessile, pedunculated lesions that vary in size
-lisch nodules (pigmented asymptomatic hamaratomas of iris)
-look for cafe au lait spots too
mech of NMDA blockers decreasing morphine tolerance
-exact is unknown
-possible ideas are
-increased phosphorylation of opioid receptors, increased adenylyl cyclase activity, or increased NO
-activation of NMDA receptors by glutamate can enhance morphine tolerance by increasing phosphorylation of opioid receptors
-NMDA blockers can decrease morphine tolerance
name 3 mood stabilizing agents? which ones can be used to treat seizures?
-lithium, valproic acid, and carbamezapine
-val and carba
risk factors of candida vaginitis?
antibiotic use, contraceptive use, corticosteroid therapy, DM, and immunosupression
mech of class of drugs that have anxiolytic, anticonvulsant, and muscle relaxant effects as well as sedative-hypnotic effects
increase frequency of opening of GABA channels
6-MP and 6-TG require activation by? are degrade how?
both of these are cytotoxic purine analogs
-activated by HGPRT
-degraded by xanthine oxidase
name biofilm producing organisms
-staphylcococcus epidermidis (prosthetic devices)
-streptococcus mutans/sanguinis (dental plaques)
-Pseudomonas aeruginosa (cystic fibrosis pneumonia, contact lens)
-viridans group strep (endocarditis)
-nontypable H. influ (otitis media)
amaurosis fugax-def and associations
-transient monocular blindness
-associated with TIA
symptoms that worsen with heat exposure? other findings?
-heat sensitivity MS
-SIIN (scanning speech, INO, incontinence, intension tremor nystagmus )
lymph drainage of testis vs scrotum
-para aortic vs superficial inguinal lymph nodes
drainage of superficial vs deep inguinal lymph nodes
-superficial-cutaneous lymph from umbilicus including external genitalia and anus up to dentate line exceptions are testis, gland penis and cutaneous portion of posterior calf
-deep- glans penis and cutaneous portion of posterior calf
*note* superfiical nodes eventually drain into deep lymph nodes
mech of vit E toxicity
-vit E protects against fatty acid antioxidation
-without it cells like neurons an RBCs are suceptible to oxidative stress