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37 Cards in this Set
- Front
- Back
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alzheimers familial: early and late onset, products and genes -what is protective |
Early Onset -APP (chromo 21) presenilin 1 on chromosme 14 -presenilin-2 (chromo 1) Late Onset -ApoE4 (chromo 19)
ApoE2 (chromo 19) is protective |
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Alzheimer's gross appearance -changes in neurotransmitters |
widespread cortical atrophy with narrowing of gyri and widening of sulci -decrease in ACh
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Alzheimers histology -what correlates to degree of dementia |
senile plaques: extracellular B amyloid core, can cause amyloid angiopathy (intracranial hemorrhage). Abeta synthesized by cleaving amyloid precursor protein -neurofilbrillary tangles: intracellular, hyperphosphorylated tau protein= insoluble cytoskeletal elements. -tangles correlate with degree of dementia |
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Pick disease -symptoms -spares -histo -gross |
frontotemporal dementia -dementia, aphasia, parkinsonian aspects, change in personality -spares parietal and posterior 2/3 of superior temporal gyrus -pick bodies: spherical tau protein aggregates -frontotemporal atrophy |
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Lewy body dementia -order -defect |
initially dementia and visual hallucinations followed by parkinsonian features -alpha-synuclein defect |
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Triad of MS (really 5) |
scanning speech, intention tremor, incontinence, internuclear opthalmoplegia, nystgamus |
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CSF findings of MS -what is diagnostic -what is gold standard -what does MRI show |
increased IgG oligoclonal bands are diagnostic -MRI is gold standage: shows periventricuar plaques: areas of oligodendrocyte loss and reactive gliosis with destruction of neurons -multiple white matter lesions separated in space and time |
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MS treatment -disease -symptoms |
B-interferon, immunosuppression, natalizumab (anti-alpha4integrin which is for leukocyte adhesion) -symptomatic treatment for neurogenic bladder (cath, muscarinic antagonists), spasticity (baclofen, GABA receptor agonist), pain (opioids |
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CSF for guillain barre where is the inflammatory infiltrate what are classic infections its assc with |
increased CSF protein with normal cell count. the increased protein can cause papilledema -endoneurium -campylobacter and CMV (molecular mimicry) |
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treatment for Guillain Barre |
respiratory support, plasmapheresis, IVIG |
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Progressive multifocal leukoenceophalopathy -what happens assc with who gets it outcome increased risk with? |
-demyelination due to destruction of oligodendrocytes -assc with JC virus 2-4% of AIDS pts (reactivation) -rapidly progressive, usually fatal -increased risk with natalizumab |
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Acute disseminated encephalomyelitis -what is it -after what |
multifocal perivenular inflammation and demyelination after infection (commonly measles or VZV) or certain vaccinations (rabies, smallpox) |
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Metachromatic leukodystrophy what is it, inheritance -what builds up -what happens, symptoms |
autosomal recessive lysosomal storage disease -most common due to arylsulfatase A def -sulfatides build up and impair production of myelin sheat -central and peripheral demyelination with ataxia and dementia |
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Charcot-Marie-Tooth disease -other name -what is it -inheritance -assc with? |
also known as hereditary motor and sensory neuropathy (HMSN) -group of progressive hereditary nerve disorders related to the defective production of proteins involved in structure and function of peripheral nerves or the myelin sheat -typically autosomal dominant -scoliosis and foot deformities (high/flat arches) |
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Krabbe disease what is it what builds up findings |
autosomal recessive def of galactocerebrosidase -galactocerebroside and psychosine build up and destroy myelin sheath -peripheral neuropathy, developmental delay, optic atrophy, globoid cells |
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adrenoleukodystrophy -inheritance -what is the problem -where does a build up occur -outcome |
x-linked genetic disorder typically affecting males -disrupts metabolism of very long chain fatty acids --> excessive buildup in nervous system, adrenal glands, and testes -progressive disease that can lead to long-term coma/death and adrenal gland crisis |
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Types of generalized seizures (5) |
absence, myoclonic, tonic clonic, tonic, atonic |
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absence: electrical amplitude, is there a postictal |
3 Hz, no postictal confusion |
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causes of seizures by age children adults elderly |
kids: genetic, infection (febrile), trauma, congenital, metabolic adults: tumors, trauma, stroke, infection elderly: stroke, tumor, trauma, metabolic, infection |
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cluster headache -more common in -symptoms -treatment |
-more common in males -excruciating periorbital pain with lacrimation and rhinorrhea, may induce horner syndrome -give inhaled oxygen, sumatriptan |
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tension headache treatment -chronic pain? |
-analgesics, NSAIDs, tylenol -amytriptyline for chronic pain |
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migraine symptoms, duration -due to
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POUND: pulsatile, one day duration, unilateral, nausea, disabling -can have aura -due to irritation of CN V, meninges, or blood vessels (substance P, CGRP (calcitonin gene related peptide, vasoactive peptides) |
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migraine treatment |
abortive therapies: triptans, NSAIDs, and prophylactic (propranolol, topiramate, CCBs, amitriptyline) |
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causes of peripheral vertigo -positional testing |
semicircular canal debris, vestibular nerve infection, Meniere disease positional testing: delayed horizontal nystagmus |
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Central vertigo causes -findings -positional testing
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brain stem or cerebellar lesion: stroke affecting vestibular nuclei or posterior fossa tumor -directional change of nystagmus, skew deviation, diplopia, dysmetria, -focal neurological findings -positional testing: immediate nystagmus in any direction, may change directions
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Sturge-Weber syndrome -inheritance -what is the problem |
congenital, NON-INHERITED (somatic), developmental anomaly of neural crest derivatives (mesoderm, ectoderm) due to activating mutation of GNAQ gene |
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Sturge Weber symptoms |
-affects small blood vessels --> port wine stain of face on V1/V2 distribution -ipsilateral leptomeningeal angioma: seizures and epilepsy -intellectual disability -episcleral hemangioma (increased IOP--> early onset glaucoma SSTURGGE: sporadic, portwine Stain, Tram track Ca2+ (opposing gyri), Unilateral, Retardation, Glaucoma, GNAQ gene, Epilepsy |
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Tuberous Sclerosis symptoms inheritance increased incidence of what? |
HAMARTOMASS: Hamartomas in CNS and skin, Angiofibromas, Mitral regurgitation, Ash-leaf spots, cardiac Rhabdomyoma, Tuberous sclerosis, autosomal dOminant, Mental retardation, renal Angiomyolipoma, Seizures, Shagreen patches -increased incidence of subependymal astrocytomas and ungual fibromas |
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Neurofibromatosis I -other name -skin findings -other findings -gene, what does the gene product do -chromosome -skin tumors derivative of? |
von Recklinghausen disease Cafe-au-lait spots, Lisch modules (pigmented iris hamartomas), neurofibromas of skin, optic gliomas, pheochromocytomas mutated NF1 tumor suppressor gene (neurofibromin, a negative regulator of Ras) on chromosome 17 -skin tumors are derived from neural crest cells |
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von Hippel-Lindau -tumors -inheritance, gene, chromosome |
cavernous hemangiomas in skin, mucosa, organs -bilateral renal cell carcinoma -hemangioblastoma (high vascularity with hyperchromatic nuclei) in retina, brain stem, cerebellum -pheochromocytomas -Autosomal dominant, mutated VHL tumor suppressor gene on chromosome 3 which results in constitutive expression of HIF (TF) and activation of angiogenic growth factors |
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meningioma -location -may have? -histo |
convexities of hemispheres and parasagittal regions -can have dural attachment (tail) -spindle cells in whorled pattern, psammona bodies |
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Hemangioblastoma -location -assc with ? when ? -can produce? -histo |
most often cerebellar -assc with vHL which found with retinal angiomas -can produce EPO--> secondary polycythemia -closely arranged, thin walled capillaries with minimal interleaving parenchyma |
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Pilocytic astrocytoma -location -can be? -marker -histo MRI |
-in kids in posterior fossa (cerebellum) -can be supratentorial GFAP+ -rosenthal fibers: eosinophilic corkscrew fibers -cystic + solid (gross). youll see white solid and like a dark empty cystic space |
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Medulloblastoma -prognosis -origin -sequela -mets histo |
highly malignant cerebellar tumor -form of primitive neuroectodermal tumor -can compress 4th ventricle --> hydrocephalus -drop mets to spinal cord -Homer Wright rosettes. Solid, small blue cells |
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ependymoma -location -histo |
most common in 4th ventricle perivascular rosettes rod shaped blepharoplasts (basal ciliary bodies) found near nucleus |
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cingulate herniation under falx cerebri -what can it compress |
can compress ACA |
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Uncal herniation -what does it compress |
uncus is medial temporal lobe -compresses ipsilateral CN III (blown pupil, down and out gaze) ipsilateral PCA (contralateral homonymous hemianopsia) contralateral crus cerebri: ipsilateral paralysis, false localization sign |
