UWorld Neuro Flashcards
What does Nissl stain and where?
Stains the RER in neurons. Shows up in Dendrites and cell body. NOT in axon
How does Kinesin move?
ATP hydrolysis
Man treated for depression has severe confusion, hallucination, hypotension, mouth is dry, face is flushed. QRS prolongation and frequent preventricular beats. What is this and what do you do?
TCA can have quinidine like affects on cardiac TCA tox - Cardio, confusion, coma, biCARB. Effect is due to inhibition of Na channels.
MS is autoimmune. What are the gross and micro histological findings
scattered periventricular plaque like lesions - within plaques you see loss of myelin sheath and depletion of oligodendrocytes. May also see lipid laden M! containing products of myelin breakdownOligoclonal bands of IgG may be detected in CSF.Optic Neuritis - central scotoma, painful eye movementsInternuclear opthalmoplegia - demyelination of MLFCerebellar dysfunction (ataia, nystagmus)Sensory/motor sx - bowel/bladder dysfunction
Where do you see oligodendrocytes depletion? (2)
MS and Progressive Multifocal Leukoencephalopathy
Signs of parasagittal mengiomas?
Leg weakness and splasticity
Beta endorphins are derived from? What else is drived from this?
POMC (pro-opio-melano-cortin) Minus the dashes. POMC makes opiods, malanin, ACTH
Noxious stimuli results in what biochemical/cellular release?
opioid system. Narcotics bind to mu receptor and modulate pain.
Other name for Insulin Like Growth Factor?
Somatomedin C
Fragile X - where do you see the cytologic problem?
gap near the tip of the LONG ARM of X.
Fragile X gene mutation? Also Fragile X presentation?
FMR1 (familial mental retardation1). Large jaw, ears, testicles. Becomes evident within 1 year of life.
Short stature broad chest ammenorrhea - female. Name and cytology
Turners - 45 XO
Tall, gynecomastia, infertile male. Name and cytology
Klinefelters 47 XXY.
Short broad hands. Transverse palmar crease
Down syndrome
Sudden onset weakness and numbness. HTN.
HTN usu from intraparencyhmal hemorrhage. This is usu from charcot bouchard pseudoaneurysm (basal ganglia and thalamus small arterioles)
Charcot Bouchard Pseudoaneurysm presentation vs Saccular Aneurism rupture?
Charcot Bouchard - sudden onset of focal deficitsSaccular Aneurism rupture - sudden onset of headache (subarachnoid hemorrhage!) and altered level of consciousness are more prominent than focal neuro deficit.
Presentation of hemorrhagic stroke vs ischemic stroke?
Hemorrhagic is acute onset w/ focal neuro deficits whereas ischemic develops over hours and is boarder in scope
What is the most common cause of intraparenchymal (Brain) hemorrhage? What is the underlying pathology?
HTN. Leads to hyaline arteriolosclerosis (often in basal ganglia) Walls become weak and dilated.
3 year immigrant kid - MR, seizures, autopsy - abnormal pallor of catecholaminergic brain suggests? What is def here?
PKU. DEF of phenylalanine hydroxylase. Light pigment is due to inhibitory effect of excessive phenylalanine on melanin synth. Can classically have musty body odor.
Locus ceruleus - site for synth of what?
NE
Where do you see Dihydropteridine reductase and dopamine hydroxylase?
Phenylalanine converted to Tyrosine by phenylalanine hydroxylase and BH4. BH4 becomes BH2, and is regenerated to BH4 by Dihydropteridine reductase. Tyrosine then converted to Dopa, then Dopamine, then Dopamine hydroylase turns Dopamine into NE.
Alkaptouria. Inheritance? What is the problem? What is the s/sx.
AR in tyrosine degredation from def in homogentisic acid oxidase. Results in high levels of homogentisic acid -> connective tissue hyperpigmentation, and degenerative joint disease
What is the biochemical cause of albinism? Albinism inheritance?
AR. Dopa cannot be converted to Melanin in melanocytes due to lack of Tyrosinase enzyme
Describe embryological creation of neural tube
Dorsal surface of embryo -> neural plate. This deepens in center fo form neural groove (which is bound on both sides by neural folds. These folds fuse to form neural tube.
What is smoking during oregnancy assoc w/?
Fetal hypoxai - intrauterine growth retardiation, low birth weight and premature delivery. NOT assoc w/ neural tube defects
Insomnia headache periodic agitation and dysphagia, 30-50 days ago after cave exploration. How does this progress? WHat is the treatment composed of?
Rabes . progresses as flaccd paralysis and coma following the acute neurological phase. Death after 2 weeks of being comatose. Killed vaccine. Killed by beta propiolactone.
Synaptophysin - where is it found?
Presynaptic vesciles of neurons, neuroectodermla and neuroendocrine cells. Indicates neuronal origin (usu used for tumor analysis).
What cells stain for GFAP?
Gliomas - Astrocytes, oligodendrocytes, ependymal cells.
DSM criteria for generalized anxiety disorder?
Excssive uncrontrollable worry about multiple issues - can be accompaneid by irratability, distrurbed sleep, poor concentration, muscle tension, fatigability, restlessness. Need 3 or more NONWORRY symptoms for diagnosis and must be present for at elast 6 months. This differes from MDD in that you are less likely to have anhedonia and depressed mood. Tx for GAD is antidepressants, benzodiazepines, Buspirone
Delirium vs dementia
Delirium - acute onset - global memory impairment, fluctuations in consciousness - reversible.Dementia - gradual conset - can be antregrade/retrograde - no changes in conscousness . Usu not reversible.
How long odes brief psychotic disorder last?
Few hours to months
Vascular dementia (other name for this ) and presentation?
Mutli-infarct dementia - occurs over time - FOCAL! neurological signs
60 y.o. Behavioral abnormalitie, speech language decline, socially inappropriate behavior. Urinary incontience
Picks disease - cytoplasmic inclusions of microtubule assoc Tau.
Broke off engagement. How has random severe weakness in leg. Think?
Conversion disorder. Symptoms are not produced intentionally. Often in young women after emotional stress. 75% have spontaneous recovery.
Conversion disroder vs somatization
Conversion - occurs in young women after life stres - unconscous manifestation. Somatization requires 4 pain symptoms, 2 GI symptoms, 1 sexual symptom, 1 pseudoneurologic symptoms - relatively rare. Numerous physical complaints over years.
Damage to radial head presents as?
Wrist etensor weakness - Radial head subluxation “nursemaids elbow” - can be caused whe adult attempts to pull child upwards by arm. Can lead to weakeness of extensors. “Wrist drop” usu results from radal nerve injury. Deep branhc of radial nerve does not carry any sensory innervation
2 mechanisms that lead to diabetic neuropathy/
NEG of proteins -> hyalinization and narrowing of walls of arteries -> diabetic miroangiopathy of endoneural arterioles -> ischemic damageIntracellular hyperglycemia -> glucose -> sorbital by aldose reductase. Dmg to Axon and schwann cells.
3 Types of botulism? Where does botulism bind?
Food borne, wound, infant. Food borne type - botulinum neurotoxin - prevents the release of ACh rom nerve terminals in NMJ. CONDUCTION IS FINE. Just synapse problem.
16 Y.o w/ progressive gait instability and dysarthria.
Friedreich ataxia - . Can be mimicked by Vit E DEF.
Tabes dorsalis presentation?
Loss of position and vibration sense and severe lancinating pains. Peripheral reflexes may be absent. Argyll Robertson pupil due to damage to midbrain TECTUM. Wide based gain and + Romberg sign.
How is alanine converted to glucose?
Amino group transfered to a-KG. Alanine becomes pyruvate when a-KG receives the NH3 and is transformed into glutamate. Glutamine then literated back to AKG by glutamate dehydrogenase, and ammonia enters urea cycle to form urea. In essense, alanine is major AA responsible for transferring nitrogen to lvier for disposal.
How d calculate COnfidence interval? (95% confident of…)
Mean + 1.96SD/square root of nn = sample size.
Most common meningitis in adults?
Strep pneumo
Narcolepsy vs Cataplexy
Narcolepsy - excessive daytime sleepiness and REM skeep reated phenomena (including cataplexy, sleep paralysis, hypnagogic/hyponopompic hallucinations)Cataplexy -sudden loss of muscle tone w/o loss of consciousness - often provoked by strong emotions or surprises. Seen in 70% of narcoleptics.
Person who has recurrent Neisseria infection
Def in C5-C9 - results in recurrent neisseria infections. - Inability to form membrane attack complex.
Pure T cell Dysfunction?Ineffective intracellular Killing?Insufficient IgA prodcution?Inability to form MAC?
Pure T cell - DiGeorge/thymic hypoplasiaDEF Intracellular Killing - Chronic granulomatous disease - inability to synth NADPH oxidase. suffer from recurrent catalase postiive (staph etc)Various things. But on the flip side, TOO much IgA production binds bacteria and prevents from attaching to mucosal surfaces but BLOCKS IgM and IgG. IgA does not trigger complement cascade. IgM and IgG are more bacteriocidal.Inability to form MAC complex - C5-9 def. Neisseria infection
Dysarthria vs apraxia
Dysarthria - motor related. Apraxia - conscious speech planning and more
Which major arteries can affect the 3rd Cranial nerve?
Aneurysms of either the Poster Cerebral or Superior Cerebellar artery.
Ptosis w/ ipsilateral, dilated pupil, eye down and out. Aslo may have unilateral heardache, eye pain, diploplia
3rd nerve. Nonpupil sparing third nerve palsy. Can be from aneurysm of PCerebral or Superior Cerebellar
Anisocoria definition
Different pupil sizes
Japanese Food - Pufferfish poison - Name and activity
Tetrodotoxin - neurotoxin that binds VOltage gated sodium channels in nerve and cardiac tissue -> prevents sodium depolarization
4 types of exotic toxins -
Tetrodotoxin (puffer fish) - inhibits Na depol by binding to channels. Saxitoxin (dinoflagellates in red tide) - inhibits na depol by binding to channels. Ciguatoxin (exotic fish, Moray eel) - binds Na channel, keeps it persistently depolarizedBatrachotoxin (South American frogs) - bins to Na channel, keeps it open and causes persistent depolarizationSO Tetrodotoxin and Saxitoxin prevent depolCiquatoxin, Batrachotoxin block by causing persistent depol
Double stranded break repair failure - disease and constellation
Ataxia Telangiectasia - 4 year old kid - frequent respiratory infections (difficulty w/ class switching), ocular telangiectasias and CEREBELLAR ATROPHY. Rate of CA is greater due to inefficient DNA repair. This is from RADIATION induced mutations, Not single stranded problems.
Fanoni Anemia mechanisms
Hypersensitivity of DNA to cross-linking agents
Bloom syndrome mechanism
Chromosomal instability, INC susceptibility to neoplasms
White-yellow chrioretinal lesions in both eyes, INC head circumference/enlarged ventricles, scattered inracranial calcification. What is the problem and how did it happen?
In utero infection by Toxo.
What is the elassic triad of in utero toxo
hydrocephalus, inracranial calcifications, chorioretinitis
/Astrocytosis/Gliosis/Glial scar pathogenesis. How does this appear histologically
All the samet hing. Proliferation of astroytes in area of neuron degerneration. Scar compensates for volume loss.Histo - large nuclei w/ glial fibrils, GFAP, intense cytoplasmic EOSINOPHILIA
Intense cytoplasmic eosinophilia in the contex of neuronal shrinkage
Glial hyperplasia, reactive astrocytosis, gliosis, glial scar
Brief Psychotic Disorder vs Schizophreniform vs Schizophrenia
Brief Psychotic Disorder - less than 1 monthSchizophreniform - 1-6 monthsSchizophrenia - more than 6 monthsActive pychosis w/ progressive functional capacity decline. Can include hallucinations, delusions, negative and positive symptoms
Complication of anti-psychotic therapy?
Akathisia - movement disorder w/ restlessness and inability to stand still. Difficult to diagnose, as some physicians may wrongly thinnk the pt has WORSENING psychotic behavior and INC the dose, which is exactly the problem.
Strep pneumo leading cuase of
community acquired pneumonia, otitis media, meningitis in adults. Gram stain as lancet shaped gram + cocci in pairs
Friedreich ataxia presentation - 4 major s/sx. Cytogenetic problem? Inheritance
Usus presents in kids 5-15 y.o as gait ataxia. Wide based gait may be charateristic. Progresses to all 4 limbs due to CEREBELLAR DYSFUNCTION - also degen of dorsal columns lead to position and vibration sensation loss. Possible iron mitochondria problem w/ demyelinationKyphoscoliosis, hypertrophic Cardiomyopathy, diabetes mellitus, foot deformities. AR Chr 9 trinucleotide repeats
Location of vomiting center
Chemoreceptor trigger zone - area postrema of dorsal medulla near fourth ventricle
What does V3 do? Cranial nerve Where does it exit.
Sensation AS WELL AS invervates muscle of mastication (masseter, medial/alatera pterygoids, temporalis) Exist through Foramen Ovale.
Foramen rotundum, spinosum, lacernum, jugular foramen
Rotundum - V2Ovale - V3Spinosum - Middle meningeal artery//vein and recurrent meningeal branch of V3Lacerum - ascending pharyngeal artery and artery/nerve of pteryoid canal. Otherwise occluded by cartilage.Jugular foramen - CN 9, 10, 11, posterior meningeal artery. Inferior petrosal and sigmoid sinuses.
Elderly person w/ scalp tenderness or headache, blurred vision and claudication of jaw? Diagnosis S/Sx?
Temporal arteritis. VERY high ESR - often in the setting of polymyalgia rheumatica. Radic steroid treatment to prevent blindness
Cluster headache resentation?
Sudden unilateral periorbital pain - ispilateral rhinorrhea, watering of eye.
3 risk factors for Obstructive Sleep Apnea
Obesity, tonsillar hypertrophy, hypothyroidism.
Obstructive Sleep Apnea vs Obesity hypoventilation Syndrome (Pickwickian)
Difference is that Obstructive Sleep Apnea has normal blood gas - wake u when you have the osbtruction.Obesity hypoventilation syndrome leads to chronically elevated PaCO2 and reduced PO2. Also may eventually get polycythemia, pulmonary HTN, righ ventricular failure. Often have concomitant obstructive sleep apnea
What is first step of urea cycle?
CO2 + NH4 + 2ATP. PLUS N-acetylglutamate and Carbamoyl phosphate synthetase 1 -> carbamoyl phosphate.
Explain Lesch Nyhan pathway. What builds up?
Knock out of HGPRT. This is a failure in purine salvage and hypoxanthine and guanine degraded to URIC acid at higher rates. THUS you need to INC purine de novo synthesis. Because HGRPT and PRPP are required to push the cycle through (Guanine -> GMP and Hypoxanthine -> IMP), if HGRPT is not working, PRPP INC as well. This is good beause it’s needed for de novo purine synth.
WIlsons disease on histological section of brain
Cystic degenreation of putamen - damage to other basal ganglia structures.
Leg numbness, lifts leg higher up, foot slaps to ground. Nerve damage?
Common peroneal - MOST commonly injurged nerve in leg. Can be due to blunt trauma to lateral leg. Splits into deep peroneal and superficial peroneal
Kinesins and dynein - travel on intermediate filaments or microtubules
Microtubules
Decreate muscular stresnght in bilatereal lower extremities w/ absent deep tendon reflexes
Diarrhea 2 weeks ago w/ campylobacter - - guillain barre
Common pica?
pica can include eating ice due to anemia
Anorexia def?
Wegith loss of 25% of body weight or weight that is 15% below normal weight for age and height
Huntington disease underlying mechanism?
Hypermethylation of histones - prevents transcriptions - disrupts synthesis of neurotrophic proteins
Nicotinic cholinergic channel receptors vs Muscarinin cholinergic channel receptors - mechanism
Nicotinic -ligand gated ion channelsl that open after binding Ach - influx of Na/Ca, Efflux of K. Muscarin - M1, M3 - use IP3 pathway. activate kinase c.
Latent period vs Lead Time bias
Latent period - pathogenesis/exposure may occur years before clinical manifestations.Lead time Bias - Time between initial detection and specific outcome or measured endpoint. This is usually seen when tehre are 2 diff test for same disease but one test can detect disease at earlier stage. Comparing survival times may erroneous
Early facial, pubichair growth - enlarged genitalia, IMPAIRED upward gaze in 4 year old
Brain germinoma - most common spot is pineal region. Germinomas are similar to testicular seminomas - precocious puberty, obstructive hydrocephalus. This is NOT GnRH related
IMpaired upward gaze suggests?
Parinaud syndrome - Paralysis of upward aze and convergence - suggest compression of tectal area of mdibrain.
Alzherims has diffus brain atrophy - where is atrophy pronounced tho?
Temporoparietal lobes and HIPPOCAMPUS. Hippocampal atrophy evident in early stages and can be detected by brain MRI.
weakness an INC tone in arm. Initial resitance w/ passive extensino followed by sudden release when maxium extenisno is released. What is this and what is the problem?
Clasp knife spasticity - intial jerking resistance to passive extension followed by sudden release of resistance. Usu seen in UMN damage- corticospianl tracts, medulla, pons, midbrain, Internal capsul, precentrla gyrus. Usu just one of these. Can see other upper motor signs.This is all due to spasticity and lack of UMN inhibitory cotrol of stretch reflex arc.
Where is the first page w/ ischemic damage during global cerebral ischemia? AKA after MI etc.
Hippocampus (pyramidal cells), and Purkinje cells of cerebellum.
What is related to temporal arteritis?
Polymyalgia rheumatic (more than 50% of pt) - stiffness/pain in neck, torso, shoulder, pelvic girdle and morning stiffness. May also see fatigue, fever, weight loss.
Thiopental?
short acting barbituate general anesthetic. analogue of thiobarbital - AKA affects GABA receptor - inhibitory. Highly lipid soluble. Equilibrtes in brian tissue w/in 1 min and cuases L.o.C. Rapidly restributes to skeletal muscles and adipose. Highest CONC restributed to skeletal muscle/adipose.
Sertraline most common side effect
Sertraline (SSRI) - sexual dysfunction is relatively common side effect
Liquefactive necrosis in brain. When do you see hemorrhagic infarctions in CNS?
From reperfusion in injury.
Hperpyrexia (over 40C)- Emergent treatment
body cooling (cold blanket), and antipyretics). Do body cooling first( immediate) vs antipyretics)
INC GABA receptor chloride channel opening and are anxiolytic, anticonvuslant, and muscle relaxant w/ sedative-hyponotic effects
Benzodiazepine
Brain hitology - gray matter changes w/ unfiform vacuoles between neuron cell bodies and perikaryon of neurons. NO INFLAM present.
SPongiform encephalopathy - eventually translates to cysts. Prions are not pro-inflam.
JC polyoma virus in ICH - hist of brain?
Patches of demyelination due to preferential infection of oligodendrocytes
Mechanism drug induced parkinsonism (usu antipsychotics, neuroleptics). Treament?
Mech - D2 receptor blockade - Tx - Benztropine/amantadine (centrla acting antimuscarinc). Levodopa cant be used as it precipitates psychosis and bromocriptine can exacerbate underlying psychosis. You give benztropine because you need to maintain anti-dopamine effects while mellowing out their parkinsonism
What is the mechanism of opioid tolerance?How would you go about blocking morphine tolerance?
Not totally understood, but involves phosphorylation of opioid receptor by protein kinase. Chronic tolerance may INC adenylyl cyclase activity or NO levels.W/ morphine, GLUTAMATE thought to play a major role. Glutamate activates NMDA receptors, which can INC phosphorylation of opioid receptors and -> tolerance. If you use an NMDA receptor ANTAGONIST (Ketamine), you can prevent glutamate and its downhill morphile tolerance.
Ketamine mech?
NMDA receptor Antagonist
3 groups within Ectoderm and their products
Surface ectoderm - ANT. PIT. lens, cornea, inner ear, epidermis, salivary, sweat, mammary glandsNeural Tube - Brain and spinal cord, POST PIT. pineal gland, retinaNeural Crest - Autonomic, sensory, celiac ganglia. Schwanna cells, pia, arachnoid. Skull bones, melanocytes, adrenal medulla. Aorticopulmonary stepum and endocardial cushions
Molecular mechanism of Narcolepsy (w/ cataplexy)
Hypocretin 1 (orexinA) and Hypocretin 2 (orexin B) - are neuropeptides produced by Lateral hypothalamus that promote wakefulness and inhibit REM sleep. Most pt w/ narcolepsy w/ cataplexy have VERY LOW levels of these 2 in their CSF.
Elevated 14-3-3 in CSF suggests?
CJD
Homovanillic - what is it? Levels linked to what?
Homovanillic acid is a primary dopamine metabolite. Linked to many psychiatric conditions - substance use disorders, psychosis, mood disorders.LOW CSF levels are seen in Parkinson Disease
DEC Melatonin in CSF suggests?
Progression of Alzheimer
Common problems w/ deletions in genes?
Di Goerge, Cri du Chat, CF
Describe red neuron?
Shrinkage of cell body, pyknosis, Loss of Nissl, EOSINOPHILIC CYTOPLASM - ACUTE IRREVERSIBLE DAMAGE. Can be from ischemia, hypoxia, toxicity, or hypoglycemia. Aswtrocytes will proliferate and cause glial scar later.See 12-24 hours after injurious eventRed neurons are NOT seen w/ normal aging, although atrophy is.
What neurotransmitters are used in inhibitory interneurons (of reflex loops etc)
GABA and Glycine (BOTH are inhibitory)
Mech of C. tetani toxin
Binds to receptors at PRESYNAPTIC MEMBRANE . Migrates reetrograde to axonally to cell body. Inhibits GABA and Glycine in interneurons.
Two spherical lesions in both cerebral hemispheres in HIV pt
Toxo. Dont necessarily have to say ring enchancing
2 most common brain focal lesions in HIV pt
Toxo and CNS lymphoma
EBV in HIV pt CSF w/ ring lesion. Concern for? What type of inflam cells would you see?
CNS lymphomna. See B lymphocytes w/ CNS lymphjoma. EBV strongly related to AIDS related primary CNS lymphoma
Timolol? Mech? Role in Glaucoma?
Nonselective B blocker. DEC acqueous humor production by CAPILLARY EPITHELIUM
2 pharm drugs to DEC aqueous humor production?2 pharm that INC OUTFLOW of aqueous humor
DEC production - Nonselective B blockers (Timolol), also Acetazolamide (Carbonic Anhydrase inhibitor) INC outflow - Prostaglandin F2a (latanoprost, unoprostone, travoprost), and Cholinomimetics (Pilocarpine, carbachol - both promote miosis by contracting sphincter of iris)
5 y.o w/ bilateral lens subluxation(dislocated lens, ectopia lentis). Lots of thrombosis, stroke, renal infarcts. Disease? How could it have been ameliorated?
Cystathionine Synthetase? B6 supplMimics Marfans in many ways + many have devo delay. Higher riks of thromboembolism. 50% respiond well to B6.
Maternal inheritance?
Means FROM MITOCHONDRIA. Can see Heteroplasmy - multiple types of mitochondria (normal and mutated). MANY mitochondria in ovum
Name 3 mito disease and presentation
Mitochondrial Encephalopathy w/ Lactic Acidosis and Stroke Like Episodes (MELAS) - can also have seizuresLeber Hereditary Optic Neuropathy - bilateral vision lossMyoclonic Epilepsy w/ Ragged Red Fibers - Myoclonic seizures and myopathy w/ exercise. Ragged figers on biopsy.
NF1 - neurofibromas - what are cell origins from?
Neural crest - Neurofibroms are tumors of Schwann cells - which are from neural crest.
If pt is unresponsive, has no written documents, and is in need of resuscitation. What happens?
Decision of Closest kin (spouse, adult children, parents, adult siblings)
Wernicke encephalopathy - Triad? what can make it worse?
Triad - confusion, ataxia, opthalmoplegiaDmg to mamillary bodies - chronic THiamine def - ALWAYS GIVE thiamine before glucose. GLucose infusion can precipitate ENCEPHALOPATHY
Prophylaxis for Neisseria meningitidis?
Rifampin
In PKU, what enzyme becomes essential?
Tyrosine
Neonatal intraventricular hemorrhage occurs where? How does it present?
Fragile germinal matrix - INC frequency w/ DEC age and birthweight. Common complication of prematurity (esp born before 32 weeks)Germinal matrix - highly cellular and vascularized layer of subventricular zone from which neurons and glial cells migrate out during brian devo. Contiains numerous thin walled vessels that lack glial fibers -> INC risk of hemorrhage. Between 24-32 weeks, germinal matrix becoems less prominent and cellularity/vascularity DEC - reduced risk of IVH.Presents as prominent scalp veins, tense fontanels, blood in lateral ventricles via ultrasound
HSV1 encephalitis - presentation and histology
Fever, headache confusion, confusion, coma/death within days - TEMPORAL LOBE - can be bilateral, hemorrhagic necrosis + edema
Amyoid in brain
Basically alzheimers. You dont get amyloid w/ picks, vascular dementia (although Alzheimers can lead to amyloid in vasculature)
First sign of alcohol withdrawal? Followed by?
Tremulousness (5-10 hours), Followed by Tonic clonic seizrues (rare) (w/in 48 hours), DT (48-72 hours),
“On off phenomenon” of parkinsons - etiology?
Unpredictable, dose INDEPENDENT - Random loss of efficacy of L-dopa leading to hypokinesia. L-dopa taken few times a day to maintain constant and try to minimize Off effects. (On is better, feeling of being normal)Drug holidays NOT used in management of Parkinson.
Where is circaidian rhythm regulated (dont say pineal gland)
Suprachiasmatic nuceli of hypothalamus.
Pseudotumor cerebri
Idiopathic intracranial HTN - elevated ICP w/ normal CSF content and normal Neuroimaging - usu happens in young obese women - manifets as headache and papilledema. Possible sequelae of optic nerve atrophy and blidness
What delineates anterior 2/3 from psoterior 1/3 tongue?
Terminal Sulcus. Foramen cecum is lcoated on midline of terminal sulcus.
Tongue sensory, control
Sensation - 5, 9Special (Tase) 7,9Movement - 12, 12
Pharm - Urinary retention.
Think anticholinergic
Small focal infarct involving basal ganglia, pons, IC, deep white matterIF CT shows no pathology?
Lacunar infarcts - usus due to HYPERTENSIVE ATERIOLOSCERLOSIS - no pathologyWould be Charcot Bouchard aneurysm (also due to HTN) if hemorrhagic stroke seen on CT.
What is a precursor to Serotonin
Tryptophan
Tx for Serotonin syndrome? how does it work?
Cyproheptadine - Antihistamine w/ anti-serotonic effects
What vitamins should not be taken for pt w/ Parkinson
Parkinsons take levodopa/carbidopa. BE WARY OF OVER THE COUNTER MULTIVITAMINS - most of which contain B6. B6 INC metabolism of levodopa, which DEC its effectiveness.
What stomach drug can cause gynecomastia?
Cimetidine - H2 receptor antagonist. Inhibits P450, INC warfarin, phenytoin, propanolol, metoprolol, quinidine, theophylline
Atypical antipsychotics are less likely to cause EPS than regular antipsychotics. BUT which is most likely and least likely atypical to cause EPS
Most likely to cause EPS - RisperidoneLeast Likely to cause EPS - clozapine (agranulocytosis, seizures, weight gain instead)
Following MI, bilateral wedge shaped bands of necrosis over cerebral convexity. Follow interhemispheric fissure, just lateral to it. This describes?
Hypoxic encephalopathy due to ACA MCA watershed areas leading to necrosis. There are also watershed areas beteween the MCA and PCA
Wernickes - what branch occlusion can cause this?
MCA
Injury to ulnar nerve causes?
Claw hand. Anestheisa over pinky and a bit more
Dmg to median nerve usu from? Causes?
Carpal tunnel. Causes loss of finger flexion, loss of thumb movement, loss of pronation. Flattening of thenar eminence due to denervation atrophy -> “ape hand” deformity
Dmg to radial nerve causes?
Wrist drop - dmg to extensor compartment of forearm. Loss of sensation to posterior arm etc.
Dmg to musculocutaneous nerve?
Loss of sensation over lateral forearm and apralysis of forearm flexors at elbow.
65 y.o w/ small yellow retinal lesions clustered around macula. What do you see in visual field exam?
Central scotomas. Macular lesions impair central vision -> central scotoma (defect surrounded by relatively unimpaired field of vision). Can be from MS, diabetic retinopathy, retinitis pigmentosa, MD. ARMD - loss of central vision due to deposition of fatty tissue (drusen) behind retina) - dry;;; or neovascularization of retina wed MD.
Which tract is where - Meyers vs Optic Radiation
Meyers is lateral. optic radiation is higher
MS ocular deficits/diplopia due to which : Impaired NM transmission, Loss of Axons, Nerve compression, Muscle fiber inflam, Reduced Saltatory conduction
Saltatory conduction as it is a demyelination (often of the MLF)See demyelination, lipid laden M!, Reactive astrocytosis, infiltration by lymphocytes and mononuclear cells. Loss of Axons, Neurons, Oligodendrocytes CAN over over CHRONIC MS plaques. Not responsible for acute itnernuclear opthalmoplegia though.
Painful abdomen, dark urine and neuro symptoms after drinking/drugs. What is wrong, How to treat?
This is Acute Intermittent Porphyria. DEC in PD (porphobilinogen deaminase) leads to accumulation of Upstream content (ALA and Porphobilinogen), which are TOXIC. Tx/ w Glucose and Heme, which INHIBID the first step (ALAS) and thus DEC ALA and Porphobilinogen content.
Where is meningococci often found? Where do Pili bind?
Found in oro/nasopharynx of asymptomatic carriers. Pili create epithelial attachment to nasopharynx.
Chickenpox, zoster pathway
Primary VZV as a kid. High circulating IgG protective against reinfection w/ VZV. These IgG ab do not confer immunity against zoster, because they are hidden in dorsal root ganglia.VZV vaccine recommended for all kids 12-18 monnths. Vaccine induced immunity dissipiates over time though.
Gene mutations of Alzheiemrs
Early onset - APP (21), Presenilin 1 (14) and Presenilin 2 (1)Late onset (ApoE4)
Carbamazepine - mech? Tox concerns?
Blocks Na voltage gated channels. Stabilizes inactive state. Bone marrow suppression Also hepatotoxic. Can cause SIADH.
Most immediate physical response THC? Other s/sx.
Rapid heart rate and COnjunctival injection.Mild euphoria, laughing, slowed reflexes, dizziness, impaired coordination, short term memory loss.
Nystagmus and ataxia - think which drug agbuse?
PCP
When woudl you have high levels of arginine and asterixis in 3 year old? What is defect? What is treatment?
Arginase defect. Cant convert Arginine to Orinithine and Urea.Tx - low protein diet low in arginine.
When do you get orotic acid?
2 main UMP synthase def - Orotic aciduria onlyImpaired Ornithine transcarbamoylase. So Carbamyol phsphate is created, but cant be incorporated into Citrulline (1st step) . Get Orotic aciduria + Hyperammoniumemia
What is gamma-aminobutyric acid?
GABA! Created from decarboxylation of glutamate by glutamate decarboxylase
What is Valproic acid mech? What is a complication of Valproic acid in infant?
Na channel inactivation. INC GABA by ihibiting GABA transaminase.Valproic acid can DEC folate absorption in gut. Can lead to spina bifida/meningocele in infants
Ebstein anomly? What is it, when do you get it?
Atrialized right ventricle due to downward displacement of tricuspid valve. Seen in fants whose mother took lithium during preg
Which sensory pathway does NOT have a relay nuclei in thalamus?
Smell. Think of oflactory bulbs ar diding solo.
VPL gets impulses from?
Spinothalamic, Medial lemniscus (Pain and Temp, Proprioception, fine touch)
VPM gets impulses from?
Trigeminal and Gustatory pathways
Lateral and Medial Geniculate bodies are relay nuclei for?
Lateral - Light (visual)Medial - Music (audio)
What is the defect and what accumulates in Niemann Pick? When do you see presentation?
Very young ~ 2 year old. Sphingomyelin accmulates in phagovytes -> foamy histiocytes - due to sphingomyelinase DEF -> accumulates of ceramide phospholipid sphingomyelin
A week after brain infarct - what is absorbing lipids/stains for lipids?
Microglia - phagocytize fragmenets of neurons, myelin, necrotic debris. Eventually get glial scar
Where do you see neuronal loss in w/ huntington? What neurotransmitter is lost?Onset?
Loss of neurons in caudate and putamen. GABA is lost from trinucleotdie repeats on HD om Chr 4. Onset may be insidious personality changes followed by physical symptoms. Aggressivenes,s flat affect, depression, psychosis.
Rupture of Charcot Bouchard aneurysm causes?
Intracerebral hemorrhage - NOT SUBARACHNOID (WORSE HEADACHE OF MY LIFE). SAH caused by Berry aneurysm of Circle of Willis. assoc w/ ADPKD, Ehlers Danlos, marfans
Which neural damage causes fasiculations?
Lower. Lower -> fasciculations, atrophy, hypotonus,
What is Resting Tremor caused by in parkinson?
Loss of neurons in substania nigra.
Ataxia Telangiectasia - Mech? Inheritance? Other defects other than Ataxia and Telangiectasia?
Impaired double stranded repair mech. AR. Immune def primarily manifests as IgA def -> infection of upper/lower airways. Can get Also RISK of hematologic malignancies.
What is stimulated in motion sickness? How do we treatr this?
Muscarininc M1 and Histmainic H1 are stimulated. 1st generation H1 anthistamic drugs have both antimuscarinic, antiadrenerigic, and antiserotonergic. Such as Meclizine and Dimenhydrinate.Of course Scopolamine is antimuscarinic only. Still effective.
First line tx for trigeminal neuralgia? Mech?
Carbamazepine. Inhibits Na channel firing
RECENT Lung CA (or breast, ovary, uterus, lymphoma) with neurologic symptoms (esp cerebellar). Think?
Paraneoplastic. Tumor cell produced substances trigger autoimmune phenomenon - cross react w/ body. Common Abanti-YO (ovary breast)Anti-P/Q (lung)Anti-Hu (lung)Cause degen of cerebellum - present w/ limb and truncal ataxia, loss of coordination, nystagmus, dysarthria.
Why would you see INC levels of AChEsterase in amniotic fluid?
Neural tube defect. Also see INC AFP. This is because the failure of fusion causes LEAKAGE of these substances.
Cell body rounding, peripheral displacement of nuclei, dispersion of Nissl substance to periphery of cells. What is this, what is it caused by. is it Reversible/Irreversible?
This sounds like reversible, as irreversible has loss of Nissl, deep Eosinophilia of cytoplasm, pyknosis of nuclei. This is just swelling.This is called AXONAL REACTION - xon damaged during trauma - “WALLERIAN DEGEN” - segment of axon that has los connectino to cell body. This reflects INC protein synth to facilitate axon repair. Hence enlarged rounded cell w/ peripheral nuclei and dispersed granular Nissl substance.
Where is Ornithine transcarbomylase? DEF in this enzyme lead to what presentation? Labs too. Inheritance
XR - most common urea cycle disorder INC Orotic acid in blood/uriea, Hyperammonemia, NO megaloblastic anemia (vs orotic aciduria)Ornithine Transcarbamaylase adds CP to Ornithine to make Citrulline. If you cant add CP, then you get Hyperammonium (urea cannot be excreted, and excessive CP is redirected in pyrimidine synth to OROTIC ACID.
Best way to prevent Tetanus in newborn?
First vaccine at 2 months, then booster every 10 years. Immunized mother will pass IgG through to fetus. until fetus gets shot at 2 months of age (DtTaP. If mother has NOT gotten vaccine, then do neonatal vaccine
Can you get tetanus protection from breast feeding?
No. Breast milk is mainly IgA. Tetanus needs IgG.
Treatment for Wernicke Korsakoff (thiamine) gets rid of all s/sx but?Where is dmg in Korsakoff localized to?
Memory Loss. Localized to dorsomedial thalamic nuclei + mamillary bodies -> memory loss and confabulation.
What vitamin needed for transamination?
B6. Also B6 needed for decarboxylation, neurotransmitters (serotonin, epinephrine, NE, dopamine, GABA), Synthesis of heme, cystathionine, niacin, histamine.
Differentiate between Postpartum Blues, Postpartum Depression and Postpartum psychosis. What % for each?
Postpartum blues - 50-85%, 2-3 days, usu resolves in 10. Watchful waiting. 20% go on to devo postpartum depression w/in 1 year.Postpartum Depression - 10-15%, 2- 12 months duration. Give antidepressants, psychotherapyPostpartum psychosis /1-/2% - days to 4-6 weeks postpartum. Give antipsychotics, antidepressants, possible inpt hospitalization
Most common cause of death in PCP intox?
Violence/trauma.
Most common cause of death in cocaine?
MI and stroke
What are the 3 dopaminergic pathways/systems? What are their functions? Disease assoc?
Mesolimbic-mesocortical - behavior - SchizophreniaNigrostratal - voluntary movement - ParkinsonismTuberoinfundibular - Prolactin secretion - Hyperprlactinemia
High Orotic aciduria, neuro signs, 10% percentile height/weight, megaloblastic anemia. What is this how to treat?
Impaired De NOVO pyrimidine synthOrotic Posphorybosyl transferase - Cant add PRPP to Orotic acid to make UMP. Helps if you add Uridine to diet.
Pt using drugs w/ yawning, lacrimation, and dilated pupils. Think what?
Heroine withdrawal.
What is atypical depression characterized by? What drugs can help this?
Atypical depression -> MOOD REACTIVITY, rejection sensitivity (to slight criticism), INC sleep and appetite. MAO often used for atypical depressants.
Small cell vs Squamous cell Lung CA. Which has which paraneoplastic?
Small cell - ACTH, ADH, Lambert EatonSquamous - PTHrBOTH are central.Squamous - keratin pearls +intercellular brdigesSmall cell - neoplastic neuroendocrine (Kulchitsky cells -> small dark blue cells)
What is associated w/ Meningococcal morbidity mortality?
LOS (lipooligosaccharide) - responsible for many toxic effects of meningitis/meningococcemia - similar to LPS of GN. Coorelates w/ morbidity and mortality.
Drugs that can trigger Serotonin syndrome?
Antidepressants, Analgesics (Tramadol), Anti-emetics (5-HT3 receptor antagonists -Ondansetron), Antibiotics (LINEZOLID), Neuropsychiatrics (Triptans)
S/Sx of Serotonin Syndrome?
Neuromuscular excitation (hyperreflexia, clonus, myoclonus, ridgiit)Autonomic stim (hyperthermia, tachycardia, diaphoresis, tremor)Altered Mental status (agitation, confusion)
Characteristics of spinal tap of viral meningitis?
Normal glucose, Elevated protein, Lymphocytic predominace (90% lymphocytes)
What is the frataxin gene responsible for?
Normal mitochondrial function - leads to spinocerebellar tract degen (ascending and derscending), as well as DCML and more.
Common 450 Inducers?
Carbamazepine (Na)Phenytoin (Na)Barbituates (phenobarbital etc)Rifampin - antibioticGriseofulvin - antibiotic
Common 450 Inhibitors?
CiprofloxacinMacrolidesAzole AntifungalsIsoniazidRitonavirCImetidine (H2 antagonist)
Buproprion side effect and use?
Used for tobacco, depression, hyposexual Side effect - seizures -esp in bulimia, anorexia.Side effect - possible weight normal or LOSS. NOT gain.
Location of Brocas vs Wernicke?
Brochas is more frontal.
Opioid mechanism
Mu receptor agonist - G protein linked receptors that invovles K conductance (efflux out ) of cells. HYPERPOLARIZES CELLS - termiantes pain transmission
Intracranial calcified mass in 24 year old? Cystic spaces filled w/ thick fluid rich in cholesterol + little solid
Craniopharyngioma
What antidepressant has the least sexual side effects?
Bupropione
What bacteria can multiple in temp as low as 4 degrees C? Narrow zones of beta hemolysis.
Listeria. Why it can multiply in refrigerated meats, milk, soft cheeses and vegetables. Requires T cells/cell mediated immunity to destroy
High fever confusion and muscl erigidity after tx for agitation?
Haloperidol/other neuroleptics/typical antipsychotics - D2 blockers -> Neurolpetic Malignant Syndrome
How do diff Serotonin Syndrome From Neurleptic Malignant Syndrome?
NMS has NO myoclonus, but has RIGIDITY.
How to treat Neuleptic Malignant Syndrome?
Dopamine agonists (bromocriptine) and muscle relaxants (dantrolene) to treat the rigidity
Tetrahydrobiopterin (BH4) - cofactor for syth of what?
Tyrosine, DOpa, Serotonin, Nitric Oxide.
How is ACh synthesized?
Acetyl-Coa + Choline by choline acetyltransferase
Maple Syrup Urine Disease (MSUD) - where are branched chained aminoacids usually incorporated/degraded. What is the problem here? Why does the treatment work?
MSUD - aminoacids cant be incorporated into the TCA and broken down. Via RANCHED CHAIN A-KETOACID DEHYDROGENASE (similar to a-ketoglutarate dehydrogenase) Leucine enters as Acetyl CoaIsoleucine and Valine enter as Propinoly CoA -> Methylmalonyl CoA -> Succinyl CoA.These reaction require Thiamine (TPP, Lipoate, CoA, FAD, NAD. This is why supplementing w/ thiamine can help
Presentation of diabetic mononeuropathy of CN3? What is the pathology behind this?
CN3 only. SOMATIC and parasympathetic fiberse of CN3 have different blood supplies.CN3 neuropathy affects ONLY somatic - down and out, ptosis - but parasympathetic are fine - ISCHEMIC.If it were COMPRESSION you would have CN3 somatic and parasympathetic out.
What is Fluphenazine? What is a common side effect? Why?
Fluphenazie is a high potency antipsychotic (haloperidol, Trifluoperazine, Fluphenazine)Remember, these hae extrapyramidal side effects - (acute dystonia, akathisia, parkinsonism)
What benzo should be used in pt w/ mission critical positions? What can be used at night time and have no effect in day?
Triazolam. Benzo w/ short half life. Also Triazolam, oxazepam, midazolam
Most likely long temr side effects of hydrocephalus in infant?
UMN damage signs - muscle hypertonicity, hyperrelfexia. Stretching of periventricular pyramidal tract,s visual distrubances, learning disabilities.
Total sensory loss to contralateral side of body No motor deficits.
Think thalamic syndrome/stroke. TOtal contralateral - body and face. No motor deficits but proprioception is often affected and can lead to ambulation probles and falls. When this heals it can result in chronic pain to the total contralateral side.
What does damage to the internal capsule lead to? (stroke)
Motor deficits
Damage to caudate nucleus (stroke) leads to?
Transient hemiparesis as well as frontal lobe signs (inattentivness, abulia, forgetfulness), also maybe agitation, psychosis, choreoathetosis and other dyskinesias
Major cause of Lacunar infarcts? Go deeper into pathology than just 1 word. Also what is the gross appearance?
HTN. Microatheromas and lipohyalinosis. Lacunar infarct can lead to foam cells, tiny cavitations
Double vision when going down stairs only. What is this called and due to?
Vertical diplopia - trochlear nerve palsy
What is mechanism of treatment for cluster headaches
Sumatriptan (triptan) - mech - serotonin AGONIST. Migraines are caused by activation of trigeminal afferents that innervate meninges. This is assoc w/ vasoactive neuropeptides, such as SUbstance P and calcitonin gene related peptide (CGRP) - which result in neurogenic INFLAMMATION AND VASODILATION. So treatment would be to shut down blood flow/vasoconstrict. This is what triptans do! They inhiit release of vasoactive peptides.
How dose PTSD differ from Acute Stress DIsorder?
PTSD - symptoms longer than 4 weeks.Acute STress DIsorder - 2 days to 4 weeks
Accumulation of lipids within muscle fibers - think?
Lipid Myopathies - Carnitine palmitoyltransferase deficiency for example.Diffrent than fibrofatty muscle REPLACEMENT - duchenes
In terms of brain involvement - compare Tuberous Sclerosis to VHL
TS - see cortical and subependymal HAMARTOMASVHL - see hemangioblastomas.
How does Osler Weber Rendu present?
Hereditary hemorrhagic telangiectsia - AD - ruptures can cause epitstaxis, GI bleed, hematuria - cysts are NOT seen in this one compared to the other ones.
Posterior fossa mass + primitive cells and many mitotic figure
Medulloblastoma - SHEETS OF SMALL BLUE CELLS - poorly differentiated and bad prognosis. (medulloblastoma and ependymom are malignant)
General anestheisa - what is rare but severe side effect?
Hallothane side effect - massive hepatic necoriss - direct innjury by halothane metablites and formation of Ab against liver protiens - Light microscopy shows massive centrilobal hepatic necoriss - elevated AST, ATL, bilirubin levels
Accumulation of lipids within muscle fibers - think?
Lipid Myopathies - Carnitine palmitoyltransferase deficiency for example.Diffrent than fibrofatty muscle REPLACEMENT - duchenes
In terms of brain involvement - compare Tuberous Sclerosis to VHL
TS - see cortical and subependymal HAMARTOMASVHL - see hemangioblastomas.
How does Osler Weber Rendu present?
Hereditary hemorrhagic telangiectsia - AD - ruptures can cause epitstaxis, GI bleed, hematuria - cysts are NOT seen in this one compared to the other ones.
Posterior fossa mass + primitive cells and many mitotic figure. SHEETS OF SMALL BLUE CELLS -
Medulloblastoma - highly mitotic hence the blue (nucleic) cells - poorly differentiated and bad prognosis.
General anestheisa - what is rare but severe side effect?
Hallothane side effect - massive hepatic necoriss - direct innjury by halothane metablites and formation of Ab against liver protiens - Light microscopy shows massive centrilobal hepatic necoriss - elevated AST
What drug treatment (3) is associated w/ hepatic granulomatosis?
Methydopa, hydralazine, quinidine
Common side effect of volatile anesthetics?
ALL INC cerebral blood flow - undesirable as it INC ICPAlso, Myocardial depression, hypotension, respiratory depression and DEC renal function
Does binge eating without purging qualify for bulimia nervosa?
Yes, if they have OTHER COMPENSATORY behaviors, such as working out frequently
Saddle anesthesia, low back pain that radiates to one or both legs, and loss of anocutanoeus reflex - reflective of?
Cauda equina syndrome - dmg to S2-S4
Gardener’s mydriasis? What from? presentation?
Jimson Weed - Strong anticholinergic properties - similar to Atropine poisoning.
High Temp, Pulse high, BP low, Dry mucosa, Pupils dilated and nonreactive. What is this? How to reverse?
Anitmuscarinic poisoning (atropine etc)fix w/ physostigmine
Inability to oxidase VERY long chain fatty acids and phytanic acid (branched chain fatty acid) + hypotonia and seizures - what is this?
Peroxisomal disease - Thiss fatty acids cannot undergo mitochondrial beta oxidation - Example - Zellweger Syndrome - symptoms include hypotonia ad seizures, as well as Hepatomegaly, MR
Length constant?Time constant?
How far of a distance an axon can propogate. Demyelination DEC length constant. How long it takes for a membrane potential to achieve 63% of its new value. So lower (quicker) time constants mean faster axonal conduction speed.
Neurofibromatosis T1 - inheritance? Location? Common findings?
AD - Chr 17 - cafe au lait spots, mutliple neurofribromas, Lisch nodules
Vit C tox?
false negative stool guiaic and assoc w/ diarrhea and ab bloating. Assoc w/ Calcium oxlate nephrolithiasis (controversial)
Vit A tox leads to?
Intracranial HTN, skin changes, HSM
Opioid tolerance does not affect what 2 things as readily as the others?
Tolerance does not develop to constipation and miosis.Can develop to euphoria, sedation, nausea vomiting, respiratory depression, cough suppression, urinary retention
1st arch syndrome?
1st arch - CN 5 abnormalities of mandible, maxilla, malleus, incus, zygoma, vomer, palate and temporal bone2nd Arch - neural crest dervatives - styloid, lesser horn of hyoid, stapes
How does support and empathy differ in interview? What is facilitation?
Empathy - shows understandingSupport - shows concern w/o understandingFacilitation - encourages pt to talk more - “Then what happened?”
What do Schwann cells look like on microscopy?
Spindle cells - elongated cells w/ regular oval nuclei and areas of dense and loose growth patterns.
Psamomma body brain tumor
Meningiomas
What are two ways that Levodopa can be degraded in periphery? name drugs.
Levodopa -> Dopamine via dopa decarboxylase (inhibitors - carbidopa)Levodopa -> 3-OMD via COMT.COMT T inhibitors - Tolcapone (assoc w/ hepatotox) and Etancapone.
Anesthetic w/ LARGE AV concentration gradient. What 2 things does this mean?
Large gradient means moving form artery to venous you had a HUGe DEC. AKA HIGH solubility in peripheral tissue. If peripheral tissue is absorbing a lot then blood saturation takes longer, and brain saturation is delayed and ONSET OF ACTION IS SLOWERFor inhaled(air ->lungs -> blood->brain)
Cellular receptors for…CMVEBVRabiesRhinovirus
CMV - cellular integrinsEVC - CR2 (CD21)Rabies - Nicotininc Acetylcholine receptorRhinovirus - ICAM1 (CD54)
Weakness, atrophy, Cataracts, Frontal balding and gonadal atrophy. What is this. Mech. Inheritance pattern.
Myotonic dystrophy - AD - INC Trinucleotide repeats on myotonia protein kinase gene - sustained muscle contractions (myotonia) + weakness, atrophy, cataracts, frontal balding, gonadaal trophyCTG trinucleotdiepeat in DMPK (defected? myotonia protein kinase)
Fragile x syndrome. Inheritance? Mech?
X linked duh - mech = Defect affecting METHYLATION and expression of FMR1
Hemibalismas - most common etiology?D mg to where?
Dmg to CONTRALATEREAL SUBTHALAMIC NUCLEI - most commonly from lacunar stroke.
Where does Wilsons Disease often affect (in the brain)
Lentiform nuclei (globus pallidus and putamen)
Weight gain, dry skin, hair loss, constipation, bradycarida. Assoc w/ what psych med?
Hypothyroidism - assoc w/ Lithium. Screen for TSH in lithium therapy. Lithium exceretly amost exclusively by kidneys - ensure renal function and monitor blood levels.
What adrenergic dialtes the eye?
A1 (phenylephrine - a-adrenergic agonist) - often used to dilate pupils in opthalmoscopic exam. BETA 1 AND 2 ARE NOT IN THE EYE.
Errors in ubiquitination can be assoc w which 2 neuro disease?
Parkinson, Alzheimers
DeformationMalformationSequencedifferences?
Deformation - external forces - pressure to uterusMalformation - intrinstic devo abnormalitySequence - single primary defect leads to number of abnormalities (Potters)
Thipental - what is potency and mech of short acting?
High potency, high lipid solkubility, rapid entry into brain (used for induction, and short surgical), Effect terminated by rapid redistribution to tissue (skeletal), and fat.
Infant w/ non-rhythmic conjugate eye movements in various directions. + myoclonus. + abdominal mass. What is this?
Neuroblastoma paraneoplastic syndrome. “Opsoclonus-myoclonus” syndrome.
How do Neuroblastoma ab findings differ from Wilms tumor ab findings?
Neuroblastoma - firm irregular mass that can cross the midlineWilms tumor - smooth unilateral.
Botchy/ red ragged fibers red muscle fibers on what kind of stain? = mito myopathies. Inheritance?
Gomori. From mom only.
Delusional disorder vs Schizophrenia?
Delusional - usually have intact occupation and social functioning. “Non-bizarre” delusions for at least a month - such as being followed, cheated, or poisoned. This are unlikely but possible. Vis Bizarre, which is alien watching etc. Schizophrenia is often bizarre in nature and pt are SIGNIFICANTLY impaired.
What is affected in normal pressure hydrocephalus and what is spared?
Spared - cortical efferents and afferents (corona radiata)Spared - cerebellar, basal ganglia, pontine reticular.Wet wacky wobby from cortical. epriventricular white matter. Bladder control from descending cortical fiberes.
Methadone - half life potency bioavailability?
Long acticing opiate (long half life), very potent, and very good oral bioavailability. Long half life allows for prolonge deffects w/o withdrawal symptoms
Where does tRNA bind AA and Codon?
Codon binds to the central outpulge.AA binds to 3’ end
Where is ACh made?
Basal nucleus of Meynert.
What does splitting mean in terms of defense mechanisms?
Seeing the wolrd as black and white.
What do old infarcts look like in brain?
Cystic cavitation surroudned by dense wall (astrocyte processes)
Fainting during external auditory meatus examination?
Stimulation of the vagus - small auricular branch of the vagus nerve - posterior part of dthe external auditory canal.
Symmetrical enlargement of ventriculi?
Characteristic of communicating hydrocephalus
Menieres disease vs schwanomma?
Menieres - INC endolymph in inner ear from defect of resorption! -> TInnitus, Vertigo, Hearing lossvs Schwanommas which can have similar presentation. But Menieres is episodic w/ exacerbations and remissions, vs acoustic neuroma which is constant and progressive.
Mucosal atrophy, partial septum destruction think?
Cocaine
GABA (A, b, c)
GABA - a, c - ion channelsGABA b - G proteinBenzodiazepines, barbiturates, alcohol - GABAa. - allosteric binding - INC Cl frequence
Neisseria H fluHow it gets into brain for meningitis?
Neisseria. Pharynx -> blood -> choroid plexus -> meningesH flu. Pharynx -> lymph -> meninges
Common side effects of L dopa .How these change w/ carbidopa
Nausea vomiting (seeni n 80% of levodopa) - dopamine, postural hypotension and hot flashes (from peripheral conversion to dopamine) Agitation and anxiety is likely to persist after carbidopa because they are caused by central effects. These may even be exacerbated as tehy are INC w/ carbidopa.
LIthium excretion?
Almost exclusively kidney. Careful of volume depleetion from GI losses, decompensated CHF
Carbamazepine - DOC for?
COmplex partial seziures. Effective in tonic clonic too. Trigeminal Neuralgia
What virus requires hemagluttin in in its capsid?
Measles
How to treat essential tremor? DOC. Inheritance?
nonspecific B blocker - propanolol. Can be AD.
pupillary dilation, rhinorhea, sneezing, nasal stuffines, diarhea, nausea, vominting in child w/ mom on the streets. Tx?
Opium solution is given via diluted tincture of opium. DOC. Taper off.
Dopamine receptor stimulators. 2 types.
Ergot compounds - bromocriptine and pergolideNonergot compouds - Pramipexole, ropinirole
Drug of choice for myoclonic seizures?
Vaproic acid. Suppresses GABA and NMDA receptors? as well as Na and K channels.
what can provide sleeping aid w/ less dependence and tolerance than benzos?
Zolpidem, Zeleplon, EsZopiclone.Zolpidem (ambien) has 15 min onset time.
Which DNA exonuclease has 5 to 3 activity? 3 to 5 exonuclease activity?
5-3 - DNA pol 1 + 5-3 polymerase. 3-5 - DNA pol 3 + 5-3 polyermase.
When would erythrocyte transketolase activity be looked at?
In order to diagnose thiamine DEC.Transketolase in Hexose monophosphate pathway - utses B1 as a coenzyme. Levels of RBC transketolase are DEC in thiamine def. INC enzyme levels after thiamin sup.
Mamillary body location?>How is thiamine related to glucose?
Rostral to midbrainThiamine needed for pyruvate dehydrogenase, which is involved in glucose metabolism. (pyruvate -> acetyl coa)So if you give glucose before thiamine, all the thiamine will be used up, exacerbating the problems.
What is the papez circuit?
neural pathway of limbic system that invovles corticol control of emotions and memory
CN3 palsy leads to what apperance of the eye?
Down and out. Also ptosis. Pupillary constriction and accomodation can aslo be affected as CN3 carries parasympathetic friberes to ciliary muscle and iris sphincter.
Causes and signs of transtentorial (uncal herniation)?
Causes - hemorrhage of tumor.First sign of uncal herniation is a fixed and dialted pupil (ipsilateral) - also paralysis of oculomotor muscles. Contralateral homonymous hemianopsia w/ macular sparing.
Acting out vs Passive aggression?Describe Isolation
Acting out - expressing unconscious wishes through action (temper tantrum etc)Passive aggressive - expression of hostile feels in nonconfrontational matter. Isolation - separating idea from emotions. Soldier describes war in a frank, nonemotional way.
Most common complicaton of subarachnoid hemorrhage. Prevention?Most dangerous?
Subarachnoid (often berry aneuryms/AV malformation) - severe vasospasm 4-12 days after insult. Nimodipine - selective Ca channel blocker is preventative. Dangerous - rebleed (30%)
What are side effects of phenytoin?
Nystagmus, gingival hyperplasia, hirsutism, teratogenesis, SLE like syndrome ,. induction of cytochorme p45-, Generalized Lymphadenopathy! Steven Johnson. Osteopenia. Blocks folate -> megaloblastic anemia.
What are 3 mood stabilizers? Which of the 2 are anticonvulsantas?
Lithium, valproic acid, carbamazepine.Anticonvulsants - valproic acid and carbamazepine.
Meyers loop vs Optic radiation? Locations?
Optic - medial. Meyers - lateral.
Virulence factors for E Coli
Fimbriare - Cystitis, pyeloK capsule - Pneumonia, neonate meningitisLPS - septic shockK1 inhibits complement, phagocytosis, and other host responses. Immunogenic and anticapsular ab are protective.
First line for Narcolepsy? Overall tx?
Pt who is refreshed w/ sleep - may have hypnogogic/hypnopompic hallucinations, . May have recent MVA. Firist liek - Modafinil - non-amphetamine stimulant. Well tolerated drug abuse is rare. Schedule daytime naps and psychostimulants (modafinil) and nighttime Sodium Oxybate (GHB)
Why must you wait 2 weeks before swtiching from MAO to some other antidepressant?
Serotonin syndrome.Have to wait for MAO to regeneraete. Phenylzine (MAO) irreversibly bind and inhibit MAO.
What are two partial agonists to Mu receptor?
Naloxone, pentazocine
Alzheimer specific therapies? 3
Alzheimers - DEC AChTx - cholineesterase inhibitors (Donepezil)Antioxidants (vit E)NMDA receptor antagonists (Memantine)
W/ organophosphate poisoning + atropine antidote, what is not corrected?What else should you administer?
ACh affect on nonmuscarinc (AKA nicotininc). Muscle paralysis. Use Pralidoxime - reverse both muscarining and nicotinic effects - restores Cholinesterase from bonds with both other substrates.
Cause of death in TCA overdose?
Caridac arrhythmias and refractory hypotension.INhibiton of Fast Na channels in myocytes/his purkinje.
