First Aid - Neurology 4 (Pathology)
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mcalderone06 on May 29, 2012
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100 terms
Terms | Definitions |
|---|---|
 What is dementia? | Decrease in cognitive ability, memory or function with intact consciousness |
| Most common cause of dementia in the elderly? 2nd? | Alzheimer's disease = most common multi-infarct = 2nd common |
| Familial form of AD is associated with which genes: early vs. late? | Ealry onset: APP (21), presenilin-1 (14) and presenilin-2 (1) Late onset: ApoE4 (19) |
| APP is associated with what form of AD? which chromosome is it on? | early onset familial 21 |
| Presenilin-1 is associated with what form of AD? which chromosome is it on? | early onset familial 14 |
| presenilin-2 is associated with what form of AD? which chromosome is it on? | early onset familial 1 |
| ApoE4 is associated with what form of AD? which chromosome is it on? | late onset 19 |
| Which gene is protective from alzheimer's? which chromosome is it on? | ApoE2 chromosome 19 |
| NFTs are seen in what? | alzheimer's disease (intracellular abnormally phosphorlated tau protein = insoluble cytoskeletal elements, tangles correlate with degree of dementia) |
| Findings in Alzheimer's | Widespread cortical atrophy dec ACh Senile plaques (extracellular B-amyloid core) that may cause amyloid angiopathy and cerebral hemorraghe NFTs (intracellular abnormally phosphorlated tau protein = insoluble cytoskeletal elements, tangles correlate with degree of dementia) |
| Pick's disease affects which lobes? Symptoms? Findings? | Frontotemporal lobes (spares parietal lobe and posterior 2/3 of superior temporal gyrus) Dementia, aphasia, parkinsonian aspects, changes in personalit/behavioral disinhibition Pick bodies (intracelluar aggreated tau) + frontotemporal atrophy |
| Lewy body dementia -symptoms? -Defect in what? | parkinsonism with dementia and hallucinations a-synuclein defect |
| a-synuclein defect is associated with what? | Lewy body dementia and Parkinson's |
| Creutzfeld-Jakob disease -Sx? -Findings? | rapidly progressive dementia + myoclonus see spongiform cortex, prions (PrPc -> PrPsc sheet that is resistant to proteases)spongiform cortex |
| spongiform cortex is associated with what? | creutzfeld-Jakob |
| Causes of dementia? | AD, pick's disease, lewy body dementia, CJD, multi-infarct, syphilis, HIV, vit B12 deficiency, Wilson's disease, hypothyroidism, normal pressure hydrocephalus |
| Charcot's classic triad of MS | scanning speech intention tremor, incontinence, internuclear ophthalmoplegia nystagmus (SIN) |
| Inc protein (IgG) in CSF is associated with what? | MS |
| Findings in MS? | Inc protein (IgG) in CSF oligoclonal bands are diagnostic MRI is gold standar: periventricular plaques (oligodendrocyte loss and reactive gliosis) with preservaiotn of axons |
| Treatment of MS? | B-interferon, immuosuppression, natalizumab, symptomatic treatment for bladder (cath, muscarinic agonists), spasticity (baclofen, GABA receptor agonist), pain (opioids) |
| albuminocytologic dissociation | inc CSF protein with normal cell count, inc protein -> papilledema (seen in Guilain Barre) |
| Guillain Barre (acute inflammatory demyelinating polyradiculopathy) -Which nerves affected? -Findings? -Associated with? -Treatment? | --Inflammation and demyelination of peripheral nerves and motor fibers of ventral roots (sensory less severe than motor) -Symmetric ascending muscle weakness beginning in distal lower ext, +/- facial paralysis and autonomic malfunction (Cardiac irregularities, HTN or hypotension) -almost ALL pts survive and recover after weeks to months -Findings: inc CSF protein with normal cell count (albuminocytologic dissociation). Inc protein -> papilledema -RF: infections (autoimmune attack of peripheral myelin due to MOLECULAR MIMICRY) such as CAMPYLOBACTER or HERPESVIRUS, inoculations and stress. NO DEFINITIVE LINK TO PATHOGENS. -Treatment: respiratory support CRITICAL until recovery, plasmapheresis, IV IG |
| Progressive multifocal leukencephalopathy (PML) -Destruction of what? -Virus? -Prognosis? | Demyelination of CNS due to destruction of OLIGODENDROCYTES Associated with JC virus and seen in 2-4% of AIDS pts (Reactivaiton of latent viral infection) Rapidly progressive, usually fatal |
| Acute disseminated (postinfectious) encephalomyelitis -What is it? -Occurs after what> | Multifocal perivenular inflammation and demyelination after INFECTION (chickenpox, measles) or VACCINATION (e.g. rabies, smallpox) |
| Metachromatic leukodystrophy -Genetics? -Deficiency of what? Leads to buildup of what? -Pathogenesis? | -AR lysosomal storage disease -Most commonly due to deficiency of ARYLSULFATASE A deficiency -Buildup of sulfatides leads to impaired production of myelin sheat |
| JC virus causes what? | PML |
| arylsulfatase A deficiency causes what? | metachromatic leukodystrophy |
| Charcot-Marie-Tooth disease -AKA? -Related to defective production of what? | "hereditary motor and sensory neuropathy" Progressive hereditary nerve disorders related to DEFECTIVE PRODUCTION OF PROTEINS involved in structure and function of peripheral nerves or myelin sheat. |
| seizure affecting one area of brain? diffuse? | partial seizures - 1 area of brain generalized seizures- diffuse |
| Partial seizures -Most commonly originate where? -Preceeded by what? -Types? | 1 area of brain most commonly originates in MEDIAL TEMPORAL LOBE often preceeded by seizure AURA, can secondarily generalize 1) Simple partial (consciousness in tact) - motor, sensory autonomic, psychic 2) Complex partial (impaired consciousness) |
| Focal seizure with consciousness in tact | simple partial |
| focal seizure with loss of consciousness | complex partial |
| blank stare | absence seizure |
| types of generalized seizures | absence, myoclonic, tonic-clonic, tonic, atonic |
| absence seizure | petit mal, 3 Hz, no postictal confusion (blank stare) |
| myoclonic seizure | quick, repetitive jerks |
| Tonic-clonic seizure | grand mal, alternating stiffening and movement |
| Tonic seizure | stiffening |
| atonic seizure | "drop seizures" (falls to floor) - commonly mistaken for fainting |
| are febrile seizures considered epilepsy? | NO! |
| Most common causes of seizures in kids | genetic, infection (febrile), trauma, congenital, metabolic |
| most common causes of seizures in adults | tumors, trauma, stroke, infection |
| most common causes of seizures in elderly? | stroke, tumor, trauma, metabolic, infection |
| Does headache reflect irritation of brain parenchyma? | NO! Only dura, cranial nerves, or extracranial structures |
| substances involved in migraine | substance P, CGRP, vasoactive peptides |
| treatment of migraine | propoanolol, NSAIDs, sumatriptan for acute |
| which headaches are unilateral? bilateral? | unilateral: migraine, cluster headache bilateral: tension headache |
| Migraine vs. tension HA | tension not aggravated by light or noise, no aura |
| sx of cluster headache | PERIORBITAL PAIN with ipsilateral lacrimation, rhinorrhea, horner's MUCH MORE COMMON IN MEN |
| worst headache of life | subarachnoid hemorraghe |
| What is vertigo? what type is most common? | illusion of movement peripheral vs. central vertigo - peripheral more common |
| peripheral vertigo -Cause? -Position testing? | Innear ear etiology (semicircular canal debris, vestibular nerve infection, Meniere's disease) position testing: delayed horizontal nystagmus |
| central vertigo -Cause? -Positional testing? | Brain stem or cerebellar lesion (e.g. vestibular nuclei, posterior fossa tumor) Positional testing -> immediate nystagmus in any direction, may change directions |
| Sturge-Weber | Port-wine stains (nevus flemmeus) in V1 ophthalmic distribution Ipsilateral leptomeningeal angiomas, pheochromocytomas Can cause: glaucoma, seizures, hemiparesis and MR sporadic |
| tuberous sclerosis | hamartomas in CNS and skin adenoma sebaceum (Cutaneous angiofibromas) ash-leaf spots cardiac rhabdomyoma autosomal dominant MR renal angiomyolipoma seizures |
| NFT1 (von recklinghausen's) | Cafe au lait spots Lisch nodules (pigmented iris hamartomas) Neurofibromas in skin optic gliomas pheochromocytomas Scoliosis autosomal domiannt, 100% penetrant with variable expression Mutated NF-1 gene on chromosome 17 |
| Von HIppel Lindau disease | cavernous hemangiomas in skin, renal hemangiomas and liver cysts, mucosa, organs, hemangioblastoma in retina, brain stem, cereellum, pheochromocytoma autosomal dominant mutated tumor suppressor VHL on chromosome 3 and constitutive expression of HIf |
| Adult vs. Kid tumors relative to the tentorium? | adults - supratentorial kids - infratentorial |
| What proportion of adult brain tumors are mets? where do they present? | 50% Usually at the gray white junction |
| Which tumors tend to be supratentorial? | meningioma, GBM, oligodendroglioma, craniopharyngioma |
| which tumors tend to be infratentorial? | medulloblastoma, hemangioblastoma, ependymoma, schwannoma |
| Most common primary brain tumor? second? third? | 1) glioblastoma 2) mengioma 3) Schwannoma |
| glioblastoma multiforme -Grade? -Prognosis? -Location? -Stain with? -Histo? | adult primary brain tumor Most common primary brain tumor GRAVE prognosis, <1 year life expectancy cerebral hemispheres, can cross corpus callosum ("butterfly glioma") Stain astrocytes for GFAP Pseudopalisading pleomorphic tumor cells - border central areas of necrosis and hemorraghe |
| Meningioma -most often occurs where? -Arises from what cells? -Treatment? -Findings? | adult primary brain tumor Occurs in convexities of hemispheres and parasagital region, arises from arachnoid cells external to brain Resectable Spindle cells concentrically arranged in whorled pattern; psamomma bodies (laminated calcifications) |
| Pseudopalisading pleomorphic tumor cells | glioblastoma multiforme |
| Spindle cells concentrically arranged in whorled pattern; psamomma bodies (laminated calcifications) | meningioma |
| Tumor stains + for GFAP | astrocytoma (ex. glioblastoma multiform) |
| tumor stains + for S-100 | neural crest cell derivative - ex. Schwanomma, or melanoma |
| Schwanomma | adult primary brain tumor 3rd most common primary brain tumor originate from Schwann cells often localized to CN VIII -. acoustic schwannoma Rrsectable Usually at cerebellopontine angle S-100 + |
| tumor at cerebellopontine angle | Schwannoma |
| Oligodendroglioma -Location? -Histology? | adult primary brain tumor rare, slow growing frontal lobes chicken wire capillary pattern "fried egg" cells - round nuclei with clear cytoplasm, often calcified in oligodendroglioma |
| infiltrating tumor cells with perinuclear halos and prominent delicate vasculature - "chicken wire capillary pattern" | oligodendroglioma |
| tumor of Rathke's pouch | pituitary adenoma (adult) craniopharyngioma (kid) |
| bitemporal hemianopsia can come from what kind of tumor? | pituitary adenoma (usually adult) craniopharyngioma (usually kid) |
| pituitary adenomas are usually what type of tumors? | prolactinomas |
| Astrocytoma -high grade is called? low grade is called? | High grade: glioblastoma multiforme (grade IV) low grade: pilocytic astrocytoma |
| pilocytic astrocytoma -location? -prognosis? | children primary brain tumor well circumscribed in kids: posterior fossa MAY be supratentorial +gFAP benign with good prognosis See rosenthal fibers (eosinophiilc, corkscrew fibers, cystic + solid (gross) |
| rosenthal fibes | pilocytic astrocytoma |
| medulloblastoma -location? -prognosis? -type of tumor? -findings? -treatment? | children primary brain tumor highly malignant in cerebellum PNET tumor (primitive neuroectodermal) Can compress 4th ventricle -> hydrocephalus see Rosettes or perviascular pseudorosette pattern of cells. Solid (gross), small blue cells (histology) radiosensitivity |
| Rosettes or perivascular pseudorosette pattern of cells | medulloblastoma |
| characteristic perivascular pseudorosettes with rod shaped blepharoplasts near nucleus | ependymoma |
| ependymoma -location? -Prognosis? -Findings? | children primary brain tumor -4th ventricle -hydrocephalus -poor prgonosis -characteristic perivascular pseudorosettes, rod shaped blepharoplasts (basail ciliary bodies) found near nucleus |
| foamy cells, high vascularity | hemangioblastoma |
| hemangioblastoma -location? -associated with what syndrome? -paraneoplastic syndrome? -findings? | children primary brain tumor most often cerebellar associated with VHL syndrome when found with retinal angiomas can produce EPO -> 2ndary polycythemia foamy cells and high vascularity |
| brain tumor that can cause polycythemia | hemangioblastoma (produces EPO) |
| craniopharyngioma | children primary brain tumor -benign childhood tumor -confused with pituitary adenoma (can also cause bitemporal hemianopia) MOST COMMON CHILDHOOD SUPRATENTORIAL TUMOR Derived from rathke's pouch. Calcification is common (tooth enamel-like) |
| most common childhood supratentorial tumor? | craniopharyngioma |
| tumor that looks like tooth enamil | craniopharyngioma |
| cingulate (subfalcine) herniation under falx cerebri compresses what? | ACA |
| Types of herniation syndromes | 1) cingulate (subfalcine) under falx cerebri 2) Downward transtentorial (central) 3) Uncal herniation (compresses CN III) 4) Cerebellar tonsilar herniation into foramen magnum (compresses medulla) |
| uncal herniation compresses what cranial nerve? | CN III |
| cerebellar tonsilar herniation into foramen mangum compresses what? | medulla |
| uncus refers to what | medial temporal lobe |
| clinical signs of uncal herniation | ipsilateral dilated pupil/ptosis (stretching of CN III) contralateral homonymous hemianopia (compression of ipsi PCA) Ipsilateral paresis (compression of contralateral crus cerebri - Kernohan's notch) Duret hemorraghes - paramedian artery rupture (due to caudal displacement of brain stem) |
| duret hemorraghes refer to what | paramedian artery rupture |
| What is Kernohan's notch and what does it result from? | ipsilateral paresis due to compression of contralateral crus cerebri, due to uncal herniation |
| Ddx of ring enhancing lesion | metastasis (lung > breast > melanoma > kidney > GI) abscess toxoplasmosis primary CNS lymphoma (AIDS, EBV) |
| Ddx of uniformly enhancing lesion | metastatic lyphoma (often B cell non Hodgkin's) Meningioma metastases (usually ring enhancing) |
| heterogenously enhancing lesion | glioblastoma multiforme |
| which primary cancers most commonly metastasize to the brain? | lung > breast > melanoma > kidney > GI see in grey white junction |
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