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35 Cards in this Set
- Front
- Back
What bony change is seen with osteochondroma?
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Exostosis - cartilage covered bony outgrowth at the metaphyses
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If you had a pt present with osteoid osteoma, what would you expect to see?
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A teen to young adult with small, PAINFUL tumors in their extremity(ies)
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What are the locations of osteoma, osteoid osteoma, and osteoblastoma?
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Osteoma: face/skull
Osteoid osteoma: extremities Osteoblastoma: spine |
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What malignant bone tumor appears as a tender, swollen area that is commonly mistaken as an infection?
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Ewing's sarcoma - onion skin, t(11;22)
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What malignant bone tumor is associated with Codman's triangle and/or a sunburst pattern seen on x-ray?
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Osteosarcoma - most painful bone cancer
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What type of patient would you expect to have a +Gower's sign?
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Duchenne Muscular Dystrophy pts - walk themselves upright with their hands
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What type of neural tube derived brain tumor accounts for 80% of all adult primary brain tumors?
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Fibrillary Astrocytoma
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What tumor presents a pseudopalisading, butterfly shaped tumor that involves both cerebral hemispheres?
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Glioblastoma multiforme
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What type of tumor presents with a "fried egg" appearance on histologic exam?
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Oligodendrogliomas
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What is the histologic presentation of a meningioma?
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Whirling pattern and psammoma bodies (laminated calcifications)
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What disease is associated with both meningiomas and schwannomas?
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Neurofibromatosis 2 d/t loss of NF2 gene tumor suppressor
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From what do craniopharyngiomas arise and in what population are they seen?
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Rathke's pouch remnants
Childhood or adolescence |
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What type of vision disturbance does a pituitary adenoma cause and why?
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Bitemporal heteronymous hemianopsia (loss of peripheral vision)
d/t mass effect of the tumor on the optic chiasm |
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What symptoms are associated with prolactinoma?
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Amenorrhea
Galactorrhea Loss of libido Infertility Visual disturbance (mass effect) |
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What does a corticotroph tumor secret in excess?
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ACTH resulting in excess cortisol
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With what disease is hemangioblastoma associated? Is it benign/malignant? Life threatening?
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von Hippel-Lindau disease
Life threatening d/t mass effect in brain but otherwise benign |
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What are of the brain is affected by Alzheimers?
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Nucleus basalis of Meynert - marked decrease in neurons
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What gene/allele/chromosome increases the risk of Alzheimers? What does the gene do?
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Apolipoprotein E gene
Epsilon4 allele Chromosome 19 ApoE binds amyloid beta protein plaques |
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What changes are seen histologically in a pt with Alzheimers? Hint: 3 changes
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Senile plaques (Abeta protein spheres)
Neurofibrillary tangles (tau protein) Hirano bodies (eosinophilic rods in hippocampus neurons) |
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What population is most at risk for developing Alzheimers and why?
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Down's Syndrome d/t trisomy of chromosome 21 which carries the amyloid precursor protein (APP)
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What neurotransmitter will be decreased in Alzheimers?
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ACh
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What areas of the brain are affected by Parkinsons and what will you see on biopsy?
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Substantia nigra (loss of dopamine production) which causes loss of locus ceruleus (dependent on dopamine)
+ Lewy bodies (pink eosinophilic inclusions of alpha synuclein) |
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Where are Lewy bodies first formed and what symptom do they cause?
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Olfactory bulb
Results in changes in sense of smell - one of first symptoms |
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What areas of the spinal cord are affected in ALS and are they UMN or LMN?
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Corticospinal tract - UMN
Anterior horn cells - LMN |
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What are the signs of a LMN lesion?
UMN lesion signs? |
LMN: decreased DTRs, weakness, fasciculations, and symmetrical mm atrophy
UMN: increased DTRs, spasticity and Babinskis, loss of fine motor dexterity |
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What neurons are affected in Huntington's dz?
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Cholinergic and GABA-ergic neurons - primarily the medium spiny striatal neurons responsible for modulating motor output from the basal ganglia
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What symptoms are seen in Friedreich's Ataxia?
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Ataxia
Dysarthria Decreased DTRs Babinski Sensory and proprioceptive loss Pes cavus Progressive kyphoscoliosis T1DM Cardiomyopathy (often the cause of death) Degeneration of sensory peripheral neurons and dorsal root ganglia |
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What chromosome/trinucleotide repeat/deficiency is associated with Friedreich's Ataxia?
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Chromosome 9
GAA repeat Deficiency in frataxin |
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What is Werdnig-Hoffman syndrome?
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Infantile spinal muscular atrophy d/t destruction of the anterior horn cells of the spinal cord
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What will appear on the MRI for an MS pt?
What test is confirmatory for MS after this suspicious MRI? |
+ plaques in white matter
Oligoclonal bands in CSF indicating high IgG and protein |
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What change is seen in CSF of a pt with Guillain-Barre?
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Albumino-cytologic dissociation - d/t significantly increased protein concentration with only mild increase in cell count of CSF
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What is adrenoleukodystropy?
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X linked deficiency in peroxisomal transporter enzyme resulting in inability to catabolize very long chain fatty acids
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What sphingolipidosis disorder results in a deficiency of arylsulfatase A?
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Metachromatic leukodystrophy
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What virus is associated with Progressive Multifocal Leukoencephalopath?
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JC virus causing complete destruction of all oligodendrocytes
*in the context of severe immunosuppression |
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What is the difference in Wernicke's syndrome vs Korsakoff's encephalopathy? What is the deficiency?
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Vitamin B1 (Thiamine) deficiency
Wernicke's: confabulation, focal hemorrhage and necrosis of mammillary bodies, opthalmoplegia, ataxia, REVERSIBLE Korsakoff's: M-phage accumulation at hemorrhage sites of Wernicke's in areas that eventually become open cysts resulting in psychosis and dementia, IRREVERSIBLE |