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Step 1 12.6.12

Neuro IV

QuestionAnswer
what does the inner part of CN III fiber carry? What are the sx of its injury? output to ocular muscles. sx: ptosis, down and out gaze, nonreactive pupils
What types of diseases injure the inner part of CNIII, why? vascular disease(diabetes glucose-->sorbitol) due to decr diffusion to the interior
What does the outer part of CNIII fiber carry? What are the sx of its injury? PSNS output, use pupillary light reflex to asess. "blown pupil"
What is the mech of retinal detatchement? What might cause it? separation of neurosensory layer of retina from pigment epithelium=degeneration of photoreceptors=vision loss. often secondary to trauma or DM
What is the mech of age related macular degeneration (ARMD)? degeneration of macula causing loss of central vision. due to fat deposits so visual loss is gradual.
What is rapid ARMD? rapid loss of vision due to neovascularization
What happens in a complete lesion of the optic nerve on the right side prior to the optic chiasm? right anopia
What happens in a lesion of the optic nerve at the crossing over of the optic chiasm? bitemproal heminopia ( nasal side of retina crosses over)
What happens in a lesion of the optic tract on the right side just after the optic chiasm? left homonymous hemianopia (left half of both eye visions lost)
What happens in a lesion of the optic nerve in Meyers loop on the right side (temproal lobe)? What artery could be invovled? left upper quadrantic anopia (right temporal lesion MCA).
What happens in a lesion of the dorsal optic radiation (parietal lobe) on the right side? What artery might be invovled? left lower quadrantic anopia (both left lowers knocked out). MCA
What happens in a lesion of the optic nerve in the visual cortex (occipital lobe). What artery might be invovled? left hemianopia, with macular sparing. macular sparing has bilateral projection to occiput. PCA
What happens in a lesion of only the macular part of the optic nerve? central scotoma (macular degeneration) loss of central vision
What is Meyer's loop? inferior retina; loops around inferior horn of lateral ventricle
What is the dorsal optic radiation? superior retina; takes shortest path via internal capsule
What is internuclear opthalmoplegia (MLF syndrome)? What do you see on PE? lesion in medial longitudinal fasciculus= medial rectus palsy on attempted lateral gaze. nystagmus in abducting eye. convergence is normal
What is a common associated disease with internuclear opthalmoplegia (MLF syndrome)? multiple sclerosis
What is the normal function and mech of the medial longitudinal fasciculus in an attempt to gaze left? when looking left, the left nucleus of CN VI fires, contracting the left lateral rectus and stimulates contralateral of CNII via right MLF to contract right medial rectus
What is dementia? decr in cognitive ability, memory or function with intact consciousness
What genes are associated with early onset Alzheimers disease? APP(@!), presenili,-1 (14), presenilin-2(1)
What genes are associtated with late onset Alzheimers? What gene is protective? late: ApoE4(19), protective:ApoE2(19)
Who tends to gets Alzheimer's? Old people and Down syndrome
What are 4 general histologic and gross findings in alzheimer's disease? decr ACH, senile plaques, neurofibrillary tangles, widespread cortical atrophy
What are senile plaques? extracellular beta amyloid core can cause amyloid angiopathy-->intracranial hemorrhage. The A beta amyloid is synthsized by cleaving an amyloid precursor
What are neurfibrillary tangles? intracellular abnormmaly phosphorylated tau protein=insoluble cytoskeletal elements; tangels correlate with degree of dementia
What are the Sx of Pick's disease? What parts of the brain are spared? dementia, aphasia, parkinsonian aspects, change in personality. Spares parietal lobe and posterior 2/3 of superior temoparal gyrus
What histologica and gross findings are seen in Pick's disease? pick bodies ( intracellular aggregated tau), frontotemporal atrophy
What are the sx of Lewy body dementia? Parkinsonism with dementia and hallucinations
What are the histologic and gross findings of Lewy body dementia? alpha synuclein defect
What is the time course and presentation of Creutzfeld-Jakob disease? rapidly progressive (weeks to months) demential with myoclonus
What are the histiologic or gross findings in Creutzfeld-Jakob disease? spongiform cortex, Prions ( PfPc-->PrPSC sheet which is resistant to proteases
what are some common causes of dementia besides the named diseases? multi infarct, syphilis, HIV, vit B12 deficiency, Wilson's disease
What is the pathophys of multiple sclerosis? autoimmune inflammation and demyelination of CNS (brain and spinal cord)
How might a multiple sclerosis patient present and what is the time course? optic neuritis (sudden loss of vision), MLF syndrome (internucler opthalmoplagia), hemiparesis, hemisensory sx, or bladder/bowel incontinence. relapsing or remitting course
Who is most likely to get MS? women in 20s and 30s,v more common in whites
What are the major findings in MS? incr protein (IgG) in CSF, oligoclonal bands, MRI is gold standard. see periventricular plaques (oligodendrocyte loss) with axon preservation
What is Charcot's classic triad of MS? Mnemonic? SIN: scanning speech, intention tremor, Incontinence, internuclear opthalmoplegia, mystagmus
How is MS tx? Beta interferon, immunosurpression, natalizumab. symptomatic treatment for bladder, spasticity, pain
What is Guillain-Barre syndrome( acute inflammatory demylinating polyradiculopathy)? What does it cause? inflammation an demyelination of periphepheral nerves and motor fibers of ventral roots. causes symmetric ascending muscle weakness beggining in distal lower extremities. also facial paralysis
What can happen in Guillain-Barre syndrome besides muscle weakness? sever effect on autonomic function sometimes ( cardiac irregularities, hyper or hypotension)
What are the typical findings in Guillain-Barre syndrome? incr CSF protein with normal cell count (albuminocytoloic dissociation), incr protein leading to papilledema
What is Guillain-Barre syndrome associated with? infection leading to Auto immune attack on peripheral myelin (campylobacter jejuni or herpes), inoculations, stress
What is the tx for Guillain-Barre syndrome? respiratory support is critical until recovery. also plasma phoresis, IV Ig
What is progressive multifocal leukoencephalopathy? What is it associted with and what is its outcome? demyelination of CNS due to destruction of oligodendrocytes. associated with JC virus and seen in 2-4% of AIDS pt (reactivation of latent viral infection). rapidly progressive and usually fatal
What is acute disseminated (post infectious) encephalomyelitis? multifocal perivenular inflammation and demyelination after infection (chickenpox, measels) or vaccinations (rabies, smallpox)
What is metachromatic leukodystophy? autosomal recessive lysosomal storage disease most commonly due to arylsulfatase A deficiency. get build up of sulfatides leading to imparied production of the myelin sheath
What is Charcot-Marie-Tooth disease? a.k.a. HMSN. group of progressive hereditary nerve disorders related to defective production of proteins invovled in the structure and function of peripheral nerves or the myelin sheath
What are the general characterisitcs of a partial seizure? 1 area of brain, most commonly originates in the medial temporal lobe, often preceded by aura
What is a simple partial vs complex partial seizure? simple: motor, sensory, autonomic, psychic affected but consciousness is intact. A complex partial seizure has impaired consciousness
What is an absence seizure? petit mal, 3 Hz, postictal confusion-blank stare
What is a myoclonic seizure? quick repetitive jerks
What is a tonic-clonic seizure? grand mal, alternating stiffening and movement
What is a tonic seizure? stiffening
What is an atonic seizure? drop seizures(falls to floor) commonly mistaken for fainting
What is epilepsy? disorder of recurrent seizures (febrile dont counmt)
What are the most common causes of seizures in children? genetic, infection, trauma, congenital, metabolic
What are the most common causes of seizures in adults? tumors, trauma, stroke, infection
What are the most common causes of seizures in the elderly? stroke, tumor, trauma, metabolic, infection
What is a headache? pain due to irritation of structures like dura, CN, extracranial structures, not the brain parenchyma
What is a migraine? unilateral 4-72hrs of pulsing pain with nausea, photophobia, phonopohobia. and or aura including visual, sensory, speech disturbances
What causes migraines? irritation of CN V and release of substance P, CGRP, vasoactive peptides
What is the tx for migraines? propanolol, NSAIDS, sumatriptan for acute migraines
What is a tension HA? bilateral > 30 mins of steady pain. no aggrivated by light or noise, no aura.
What is a cluster HA? unilateral, repetitive brief Ha characterized by periorbital pain associated with lacrimation, rhinorrhea, Horner's syndrome. much more common in males
What is the tx for cluster HA? oxygen or sumatriptan
What might be the cause of "the worst HA of my life"? subarachnoid hemorrhage
What is vertigo in general? illusion of movement, not confusion with light headedness or dizziness
What is peripheral verigo? What is the etiology? more common, inner ear etiology ( canal debris, infection, mensiere disease)
What postional testing is used to dx peripheral vertigo? delayed horizontal nystagmus
What is central vertigo? brainstemm or cerebellar lesion (vestibular nuclei, posterior fossa tumor)
What postional testing is seen in central vertigo? immediate nystagmus in any direction. may change directions
What is Sturge-Weber syndrome? congenital disorder with port-wine stains (nevus flammeus), typically in V1 distrobution. get ipsilaterl leptomeningeal angiomas, pheochromocytomas
What can Sturge-Weber syndrome cause? glaucoma, seizures, hemiparesis, mental retardation.
What is a Mneomonic for the presentation of tuberous sclerosis? HAMARTOMAS: hamartomas in CNS and skin, Adenoma sebaceum, mitral regurgitation, Ash-leaf spots, cardiac Rhabdomyoa, Tubuerous sclerosis, autOsomal D, Mental retardation, renal Angiomyolipoma, seizures
What is seen in neurofibromatosis type I ( von Recklinhausen's disease)? cafe au lait spots. Lisch nodules (pigmented iris hematomas, neurofibromas on skin, optic gliomas, pheochromocytomas
What is the inheritance and gene associated with neurofibromatosis type I ( von Recklinhausen's disease)? autoD, variable expression with mutated NF-1 gene on chr 17
What is seen in von-Hippel-Lindau disease? cavernous hemangiomas on skin, mucosa, organs; bilateral renal cell carcinoma, hemangioblastoma in retina, brain stem, cerebellum. pheochromocytomas.
What is the gene and inheritance of von Hippel-Lindau syndrome? autoD with mutated tumor surpressor VHL on Chr 3
where are the majority of adult primary brain tumors located? supratentorial
Where are the majority of childhood primary brain tumors located? infratentorial
Where do metastases to the brain normall look like? Where do they go? well circumscribed, present at the gray-white junction
What is the prognosis, location and what do you stain for in glioblastoma multiforme (grade IV astrocytoma)? most common primary brain tumor. prognosis is <1 year. found in cerebral hemispheres, can cross corpus callosum. stain astrocytes for GFAP
Pseudopallisading pleomorphic tumor cells bordering central areas of necrosis and hemmorrhage describes? glioblastoma multiforme (grave IV astrocytoma)
What is the location, prognosis and origin of meningioma? most often occurs at convexities of hemispheres and parasaggital region. arises from arachnloid cell external to brain, resectable.
Spindle cells concentrically arranged in a whorled pattern with psammoma bodies (laminated calcifications) describes what tumor? meningioma
What is a the origin of, prognosis and where are scwannomas found? scwann cell origin often localized to CNVIII-->acoustic schwannona. resectable and usually found at the cerebellopontine angle
What is a useful tumor marker for Schwannomas? S-100 positive
What type of tumor is associated with neurofibromatosis type 2? bilateral scwannoma
What is the orign and histology of the oligodendroglioma? relatively rare, slow growing. most often in frontal lobes. chicken wire capillary pattern. oligodendrocytes= fired egg cells, round nuclei with clear cytoplasm. often found calcifed
Where doe a pituitary adenoma occur and what are some common presenting sx? most common prolactinoma. bitemporal hemianopia anmd hyper-or hypo pituitarianism are sequelae
What is the prognosis, appearance and location of pilocytic (low grade) astrocytoma? well circumscribed in kids, found in posterior fossa but can be supratentorial. good prognosis
What is a marker for pilocytic astrocytoma? GFA positive
What are rosenthal fibers? What tumor are they associated with? eosinophilic, corkscrefibers associated with pilocytic astrocytoma
What is the prognosis and origin of medulloblastoma? What can it compress? highly malignant cerebellar tumor. form of primitive neuroectodermal tumor. can compress 4th ventricl causing hydrocephalus
What is seen in histology of medulloblastoma? rosettes or perivascular pseudorosette pattern of cells. small blue cells
What is an ependymoma? ependymal cell tumor cmost commonly found in 4th ventricle. can cause hydrocephalus with poor prognosis
What is seen in histology and patholgoy of ependymom? characteristic perivascular pseudorossettes. Rod shaped blepharoplasts (cbasal ciliary bodies), found near nucleus
What is a hemangioblastoma? What is it associated with? What can it cause? most often cerebellar, associated with von Hippel Lindau when found with retinal angiomas. Can produce EPO causing a secondary polycythemia
What is seen in histology of hemangioblastoma? foamy cells and high vascularity
What is a craniopharyngioma? benign childhood tumor confused with pituitary adenoma *can also cause bitemporal hemianopia). most common childhood supratentorial tumor
Where does craniopharyngioma come from? What is often seen in histology? derived from remnants of Rathke's puch. calcification is a common finding
What is a cingulate (subfalcine) herniation? What structure can be compressed? herniation under flax cerebri. can compress ACA
What is a cerebellar tonsillar herniation? herniation into the formen magnum
What might cause ipsilateral dilated pupil/ptosis in an uncal herniation? stretching of CNIII (innervates levator palpebrae)
What might cause contralateral homonymous hemianopia in an uncal herniation? compression of ipsilateral PCA
What might cause ipsilateral paresis in an uncal herniation? compression of the contralateral crus cerebri (Kernohan's notch)
What might cuase duret hemorrages or paramedian artery rupture in an uncal herniation? caudal displacement of the brainstem
What is the DDX for a ring enhancing lesion in the brain? metastases (lung>breast>melanoma>kidney>GI), absesses, toxoplasmosis, primary CNS lymphoma(AIDS, EBV)
What is the DDX for a unifromaly enhancing lesion in brain imaging? metastatic lymphoma (often B Cell NHL), menangioma, metastases
What is the DDX for a heterogenously enhancing lesion on brain imaging? glioblastoma multiforme
What is the mech of epinephrine for glaucoma? alpha agonist which decr aqueous humor synthesis due to vasoconstriction
What is the SE of epinephrine for glaucoma? mydriasis, stinging, DONT USE in closed angle
What is the mech of brimonidine in glaucoma? decr aqueous humor synth
What are the eye SE for brimonidine? none
What is the action of timolol, betaxolol, cartelol in glaucoma? SE? decr aqueous humor secretion, no pupillary SE
What is the mech of acetazolamide for glaucoma? SE? decr aqueous humor secretion due to decr HCO3 (inhbits carbonic anhydrase). no pupillary SE
What is the class of pilocarpine and carbachol for glaucoma? direct cholinomimetics
What is the class of physostigmine, echothiophate for glaucoma? indirect cholinomimetics
What is the mech of pilocarpine, carbachol, physostigmine, echothiphate for glaucoma? incr outflow of aqueous humoe, contract ciliary muscle and open trabecular meshwork; use pilocarpine in memergencies since its very effective at openign trabecular network
What are the SE of pilocarpine, carbachol, physostigmine, echothiphate for glaucoma? miosis, cyclospasm
What is the mech and SE of latanoprost for glaucoma? incr outflow of aqueous humor. Can turn iris brown
What is the class of morphine , fetanyl, codeine, heroin, methadone, merperidine, dextromethorphan, diphenoxylate? opioid analgesics
what is the mech of morphine , fetanyl, codeine, heroin, methadone, merperidine, dextromethorphan, diphenoxylate? all are opioid receptor agonisis which open K+ channels, close Ca++ and inhibit the release of ACH, NE< 5-HT, glutamate, substance P
What receptor does morphine work on? mu
What receptor does enkephalin work on? delta
What opioid receptor does dynorphin work on? kappa
What are the clinical uses of morphine , fetanyl, codeine, heroin, methadone, merperidine, dextromethorphan, diphenoxylate? pain, acute pulmonary edema
What opioid could be used to surpress cough? dextromethorphan
What opioid could be used for diarrhea? loperimide or diphenoylate
What opioid could be used for maintenance programs for addicts? methadone
What are the major SE of morphine , fetanyl, codeine, heroin, methadone, merperidine, dextromethorphan, diphenoxylate? addiction, respiratory depression, constipation, miosis (pinpoint pupils), additive CNS depression with other drugs
What 2 SE does tolerance not develop to for opioids? How is an OD tx? no tolerance for miosis or constipation. toxicity is treated with naloxone or naltrexone (opioid receptor antagonists)
What is the mech of butorphanol? partial agonist at opioid mu receptors, agonist at kappa receptors
What is the clinical use of butorphanol? pain, causes less respiratory depression than full agonists
What are the major SE of butorphanol? causes withdrawl if on full opioid agonist
What is the mech of tramadol? very weak opioid agonist, also inhibits serotonin and NE reuptake
What is the clinical use of tramadol? chronic pain
What is the major SE of butorphanol? similar to opioids. decreases seizure threshold
Created by: tjs2123