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Path 28 CNS


"red neurons" accompany CNS hypoxia/ischemia --> necrosis/apoptosis Acute Neuronal Injury
"degeneration" neuronal death occurring as result of progressive disease process, like ALS. Cell loss and reactive gliosis. Subacute and Chronic Neuronal Injury
Reaction w/in cell body that attends regeneration of the axon, seen in ant horn cells of spinal cord when motor axons are cut. Enlarged/round cell body, peripherally displaced nucleus and Nissl subst, enlarged nucleolus (central chromatolysis) Axonal Reaction
Most important histopath indicator of CNS injury, hypertrophy and hyperplasia Gliosis (astrogliosis)
BBB disruption and increased vasc. perm. fluid shifts from intravasc to intercell spaces of brain. Paucity of lymphatics Vasogenic edema
increase in intracell fluid secondary to neuronal, glial, endothel cell memb injury from hypoxia/ischemia or metabolic damage Cytotox edema
unilat or asymm expansion of cerebral hemisphere displaces cingulate gyrus under falx cerebri --> compress ACA branches Subfalcine (cingulate) herniation
medial aspect of temporal lobe is compressed against free margin of tentorium --> compromised CN3 and PCA. If herniation large enough, compress contralat cerebral peduncle --> ipsilat hemiparesis (Kernohan's notch). also, often have Duret hemorrhages Transtentorial (uncinate) herniation
displacement of cerebellar tonsil through foramen magnum --> brainstem compress (resp and cardio centers in medulla oblongata) Tonsillar herniation
Enlarged posterior fossa. large midline cyst representing the expanded, roofless 4th ventricle. dyspasias of brainstem nuclei Dandy-Walker malform
Small posterior fossa, misshapen midline cerebellum w/ downward extension of vermis through foramen magnum. w/ hydroceph and lumbar myelomeningocele Arnold-Chiari malform
nonprogressive neurologic motor deficit w/ spasticity, dystonia, ataxia/athetosis, paresis from insults during prenatal/perinatal periods Cerebral Palsy
Middle Meningeal a. bleeds w/ temporal skull fracture. crescent shaped epidural hematoma
Bridging vv. bleed from shaking elderly and baby heads. most common over lateral aspects of cerebral hemispheres. lens shaped subdural hematoma
brain swollen, gyri widened, sulci narrowed. watershed b/w ACA and MCA at greatest risk. sickle shaped band of necrosis after hypotensive episode. Global ischemia
most common sites of thrombotic occlusions (d/t atherosclerosis). frequent association w diabetes and HTN. -->nonhemorrhagic (pale/anemic) infarcts carotid bifucation, origin of MCA, ends of basilar a.
Most frequently affected by embolic infarction from cardiac mural thrombi --> hemorrhagic (red) infarction (petechiae) distribution of MCA (direct extension of internal carotid)
Inflamm disorder that involves many small/med parenchymal and subarachnoid vessels w/ multinucleated giant cells, and vessel wall destruction Primary angiitis of CNS
Lake-like spaces d/t HTN in lenticular nuc and thalamus. also, widening of perivasc spaces w/o tissue infarct (etat crible) Lacunar infarcts
Rupture of small caliber penetrating vessels --> small hemorrhages, resorb and leave behind a slitlike cavity surrounded by brown. d/t HTN Slit hemorrhages
diffuse cerebral dysfunc w/ headaches, confusion, vomit, convulsions, can lead to coma. Binswanger disease = vascular dimentia d/t cerebral atheroscler, vessel thrombo/embol, cerebral arteriolar sclerosis from HTN that involves large areas of white matter hypertensive encephalopathy
HTN is most common underlying cause, cerebral amyloid angiopathy is also an etiology. Chronic HTN --> Charcot-Bouchard microaneurysms in small vessels w/in basal gang. Intrapenchymal hemorrhage
d/t mutant Notch 3 Receptor. recurrent strokes and dementia. Cerebral Autosomal dominant arteriopathy w/ subcort infarcts and leukoencephalopathy (CADASIL)
Most freq cause of subarachnoid hemorrhage. most common intracranial aneurysm. Most are found at branch points in anterior circulation. Ehlers-Danlos, neurofibromatosis 1, Marfans are predisposed, also smokers and HTN. >40 females. Worst headache ever. Saccular (berry) aneurysms
most common vascular malform in brain. involves vessels in subarchnoid space extending into parenchyma. tangled network of worm-like enlarged vasc channels separated by gliotic tissue. Males 10-30, presents as seizure disorder and hemorrhage affecting MCA Arteriovenous malform
vasc malform of greatly distended, loosely organized vasc channels w/ thin, collagenized walls and devoid of nervous tissue. most often in cerebellum Cavernous malform
vasc malform of dilated thin walled vasc channels separated by relatively normal brain parenchyma (nervous tissue). most often in pons. Capillary telangiectasias
Microorganisms that cause acute pyogenic meningitis. purulent CSF under increased pressure, neutrophilia, and high protein/low glucose Neonates: E. coli, group B Strep. Elders: Strep pneumoniae, Listeria monocytogenes. Teens: Neisseria meningitidis
subarach space contains gelatin/fibrous exudate at base of brain, encasing CNs. granulomas w/ caseous necrosis and giant cells. present w/ headache, malaise, confusion, vomit. REally high protein. Tuberculosis of brain (Mycobacterium tuberculosis)
a chronic meningitis involving base of brain and has oblit endarteritis (Heubner arteritis) and plasma cell rich mass lesions (cerebral gummas) Meningovasc neurosyph
Treponema pallidum invades brain --> progressive mental deficits w mood alterations (grandeur) to severe dementia. damage to frontal lobe, loss of neurons, rod cells, gliosis, and iron deposits. Paretic neurosyph
dorsal root sensory nerve damage by Treponema pallidum --> impaired joint position sense, ataxia, loss of pain (Charcot joints), lightning pains, no DTRs Tabes dorsalis
Tropics. seizures, confusion, delirium, stupor, coma, reflex asym, occular palsies. high lymphocytes/protein, norm glucose Arbovirus encephalitis
children and young adults. alterations in mood, memory, behavior. inferior and middle temporal lobes and orbital gyri of frontal lobes. Cowdry type A viral inclusion bodies HSV-1
fetuses and immunosuppressed. in utero infx --> periventricular necrosis, microcephaly and calcification. AIDS infx --> subacute encephalitis CMV
Brain edema and vasc congestion. Negri bods. malaise, headache, fever, paresthesia around wound, painful to touch, convulsions, foaming at mouth, hydrophobia, then flaccid paralysis Rabies
chronic inflamm rxn w widely distributed microglial nodules w multinucleated giant cells HIV encephalitis
viral enceph caused by JC polyomavirus, preferentially infects oligodendrocytes, demyelination. immunosuppressed. Progressive multifocal leukoencephalopathy
Most common Prion Spongiform enceph. Rapidly progressive dementia and startle myoclonus. mutations in PRNP. iatrogenic transmission. Creutzfeldt-Jakob
autoimmune demyelinating disorder w distinct episodes of neuro deficits, separated in time and has white matter lesions separated in space. most common demyelinating disorder. Women. relapsing/remitting episodes. initiated by CD4+ TH1 and TH17 MS (unilateral visual impair is initial manifestation. high IgG in CSF)
bilateral optic neuritis and spinal cord demyelination. antibods to aquaporins Neuromyelitis Optica (Devic disease)
demyelinating disease that follows viral infx. presents w headache, lethargy, coma. Acute disseminated Encephalomyelitis (ADEM). Acute necrotizing hemorrhagic Enceph (ANHE) is worse and preceded by URI, in youngers
Most common cause of dementia in elderly. Cortical atrophy w widening of frontal/temporal/parietal sulci and ventricular enlarge (hydroceph ex vacuo). Neuritic (amyloid ABeta) plaques and neurofibrillary tangles Alzheimers
early onset of behavior changes w alterations in personality and language disturbance. atrophy of frontal and temporal lobes (thin,"knife edge" gyri). Neurons swell and have cytoplasmic round/oval filamentous inclusions Pick disease
truncal rigidity w dysequilibrium and nuchal dystonia, pseudobulbar palsy and abnorm speech, ocular disturbs, mild progressive dementia. older males. Progressive Supranuclear Palsy
extrapyramidal rigidity, jerking limbs, apraxias, language probs, cognitive decline. Tufted astrocytes, coiled body oligodendrocytes, tau-positive astrocytic plaques, tau-positive threads in gray and white matter Corticobasal Degen
diminished facial express, stooped posture, slow voluntary movement, festinating gait, rigidity, pill-rolling tremor. damage to nigrostriatal dopaminergic system (pallor of substantia nigra). Lewy bods of Alpha synuclein --> Lewy neurites of Dementia Parkinsons
Autosomal dominant polyglutamine trinucleotide repeat expansion (CAG). progressive movement disorders, dementia, degen of striatal neurons in caudate nucleus (use GABA)--> chorea. increased risk of suicide Huntington
GAA repeat-->very low frataxin-->spinocrebellar degeneration beginning in babies w gait ataxia then hand clumsiness, dysarthria; DTR, joint position, vibratory sense impaired. high incidence of pes cavus, kyphoscoliosis, arrhythm, heart fail Friedreich Ataxia
ataxic-dyskinetic syndrome beginning in early childhood w telangectasisa in the conjunctiva and skin d/t mutant ATM gene. Lymph nodes, thymus, and gonads are hypoplastic and have recurrent sinopulmonary infx and unsteadiness Ataxia-Telangectasia
loss of lower motor neurons (weakness, muscle atrophy)AND upper motor neurons (hyperreflex, spasticity). 40+ men. thinned ant roots of spinal cord. dropping things, pulm infx ALS
X-linked adult onset w distal limb amyotrophy, atrophy and fasiculations of tongue and dysphagia. have androgen insensitivity, gynecomastia, testicle atrophy, oligospermia. degenerate lower motor neurons in spinal cord/brainstem. CAG repeat in androgen R. Bulbospinal Atrophy (Kennedy synd)
def of galactocerebroside Beta-galactosidase--> galactosylsphingosine a cytotoxin that causes oligodendrocyte injury--> stiffness/weakness, difficult feeding. Aggregation of engorged (globoid) macrophages Krabbe disease
def of lysosomal arylsulfatase A-->accumulate sulfatides (cerebroside sulfate). infant form most common. metachromatic material in urine. Metachromatic Leukodystrophy
myelin loss from CNS and periph nerves w adrenal insuff. mutant ALD gene. Elevated Very Long Chain Fatty Acids (can't catabolize). Adrenoleukodystrophy
pendular eye movements, hypotonia, choreoathetosis, later spasticity, dementia and ataxia. Tigroid appearance in cerebral hemispheres d/t myelin loss. X-linked Pelizaeus-Merzbacher disease
magalocephaly, mental def, blind begin in infancy relentlessly progress to death w/in a few yrs. Accumulation of N-acetylaspartic acid. Canavan disease
megalenceph, seizures, progressive psychomotor retardation. Accumulation of Rosenthal fibers around blood vessels. Mutant GFAP Alexander disease
Most common neurologic synd caused by mitochondrial abnormalities. recurrent episodes of acute neuro dysfnx, cognitive changes, weakness and lactic acidosis. Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS)
early childhood lactic acidemia, arrest of psychomotor devel, feeding probs, seizures, extra-ocular palsies, weakness w hypotonia. symmetric regions of destroyed brain tissue w spongiform appearance and blood vessel proliferation. Leigh synd
large mitochondrial DNA deletion/rearrangement --> cerebellar ataxia, external opthalmoplegia, pigmentary retinopathy, cardiac conduction probs Kearn-Sayre synd
neurologic symps w hepatic dysfx (hepatitis and bile duct prolif). early seizures, developmental delay, hypotonia, ataxia, cortical blindness. mutant DNA pol gamma Alpers disease
Most common adult primary brain tumor. Diffuse=poorly defined, gray, infiltrate tumor, w fibrillary appearance. Anaplastic=mitotic figs, eosinophil, stout processes. Glioblastoma=variable gross appearance, serpent necrosis, vasc prolif Infiltrating Astrocytoma. Diffuse =grade2 (p53 and high PDGF-A), Anaplastic=grade3, Glioblastoma=grade4 (RB and p16/CDKNaA. Pilocytic=grade1 (in young cerebellum, benign, cystic)
tumor in temporal lobe young, history of seizures. neoplastic, bizarre astrocytes, reticulin deposits, circumscription, inflamm cell infiltrates, absence of necrosis and mitotic activity. Pleomorphic Xanthoastrocytoma (grade I/IV)
Only in kid's midline cerebellum. May occlude flow of CSF-->hydrocephalus. Radiosensitive Medulloblastoma
Most common CNS neoplasm in immunosuppressed. B-cell origin infected by EBV Primary CNS Lymphoma
primary tumors along midline in pineal and suprasellar regions. CSF levels of Alpha-fetoprotein and Beta-hCG. Germ Cell Tumor
benign tumors of adults attached to dura. associated w NF2 and loss of chromosome 22 Meningiomas
associated w NF2 and loss of chromosome 22 (merlin). Antoni A (Verocay bods) and Antoni B (microcyts and myxoid stroma). most at cerebellopontine angle, attached to CN8-->tinnitus, hearing loss (acoustic neuroma). can separate from nerve. Schwannoma (Neurofibromas can't be separated from nerve)
Autosomal dominant neurofibromas, gliomas of optic nerve, pigmented iris nodules (Lisch nods), cafe au lait spots Neurofibromatosis Type 1
Autosomal dom bilateral CN8 schwannomas and multi meningiomas. Nodular ingrowth of Schwann cells into spinal cord, meningioangiomatosis (prolif meningeal cells and blood vessels in brain), glial hamartia (micronods of glial cells in cortex. less common. Neurofibromatosis Type 2
Autosomal dom hemangioblastomas in cerebellum and retina and cysts in pancreas, liver, kidneys-->renal cell carcinoma and pheochromocytoma. Von Hippel-Lindau Disease
Created by: Jakphooey