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35 Cards in this Set

  • Front
  • Back
What bony change is seen with osteochondroma?
Exostosis - cartilage covered bony outgrowth at the metaphyses
If you had a pt present with osteoid osteoma, what would you expect to see?
A teen to young adult with small, PAINFUL tumors in their extremity(ies)
What are the locations of osteoma, osteoid osteoma, and osteoblastoma?
Osteoma: face/skull

Osteoid osteoma: extremities

Osteoblastoma: spine
What malignant bone tumor appears as a tender, swollen area that is commonly mistaken as an infection?
Ewing's sarcoma - onion skin, t(11;22)
What malignant bone tumor is associated with Codman's triangle and/or a sunburst pattern seen on x-ray?
Osteosarcoma - most painful bone cancer
What type of patient would you expect to have a +Gower's sign?
Duchenne Muscular Dystrophy pts - walk themselves upright with their hands
What type of neural tube derived brain tumor accounts for 80% of all adult primary brain tumors?
Fibrillary Astrocytoma
What tumor presents a pseudopalisading, butterfly shaped tumor that involves both cerebral hemispheres?
Glioblastoma multiforme
What type of tumor presents with a "fried egg" appearance on histologic exam?
Oligodendrogliomas
What is the histologic presentation of a meningioma?
Whirling pattern and psammoma bodies (laminated calcifications)
What disease is associated with both meningiomas and schwannomas?
Neurofibromatosis 2 d/t loss of NF2 gene tumor suppressor
From what do craniopharyngiomas arise and in what population are they seen?
Rathke's pouch remnants

Childhood or adolescence
What type of vision disturbance does a pituitary adenoma cause and why?
Bitemporal heteronymous hemianopsia (loss of peripheral vision)

d/t mass effect of the tumor on the optic chiasm
What symptoms are associated with prolactinoma?
Amenorrhea
Galactorrhea
Loss of libido
Infertility
Visual disturbance (mass effect)
What does a corticotroph tumor secret in excess?
ACTH resulting in excess cortisol
With what disease is hemangioblastoma associated? Is it benign/malignant? Life threatening?
von Hippel-Lindau disease

Life threatening d/t mass effect in brain but otherwise benign
What are of the brain is affected by Alzheimers?
Nucleus basalis of Meynert - marked decrease in neurons
What gene/allele/chromosome increases the risk of Alzheimers? What does the gene do?
Apolipoprotein E gene
Epsilon4 allele
Chromosome 19

ApoE binds amyloid beta protein plaques
What changes are seen histologically in a pt with Alzheimers? Hint: 3 changes
Senile plaques (Abeta protein spheres)
Neurofibrillary tangles (tau protein)
Hirano bodies (eosinophilic rods in hippocampus neurons)
What population is most at risk for developing Alzheimers and why?
Down's Syndrome d/t trisomy of chromosome 21 which carries the amyloid precursor protein (APP)
What neurotransmitter will be decreased in Alzheimers?
ACh
What areas of the brain are affected by Parkinsons and what will you see on biopsy?
Substantia nigra (loss of dopamine production) which causes loss of locus ceruleus (dependent on dopamine)

+ Lewy bodies (pink eosinophilic inclusions of alpha synuclein)
Where are Lewy bodies first formed and what symptom do they cause?
Olfactory bulb

Results in changes in sense of smell - one of first symptoms
What areas of the spinal cord are affected in ALS and are they UMN or LMN?
Corticospinal tract - UMN

Anterior horn cells - LMN
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
What neurons are affected in Huntington's dz?
Cholinergic and GABA-ergic neurons - primarily the medium spiny striatal neurons responsible for modulating motor output from the basal ganglia
What symptoms are seen in Friedreich's Ataxia?
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
What chromosome/trinucleotide repeat/deficiency is associated with Friedreich's Ataxia?
Chromosome 9
GAA repeat
Deficiency in frataxin
What is Werdnig-Hoffman syndrome?
Infantile spinal muscular atrophy d/t destruction of the anterior horn cells of the spinal cord
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
What change is seen in CSF of a pt with Guillain-Barre?
Albumino-cytologic dissociation - d/t significantly increased protein concentration with only mild increase in cell count of CSF
What is adrenoleukodystropy?
X linked deficiency in peroxisomal transporter enzyme resulting in inability to catabolize very long chain fatty acids
What sphingolipidosis disorder results in a deficiency of arylsulfatase A?
Metachromatic leukodystrophy
What virus is associated with Progressive Multifocal Leukoencephalopath?
JC virus causing complete destruction of all oligodendrocytes

*in the context of severe immunosuppression
What is the difference in Wernicke's syndrome vs Korsakoff's encephalopathy? What is the deficiency?
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