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92 Cards in this Set
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subepi bullae with eosinophil inflamm infiltrate in surrounding dermis =
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bullous pemphigoid; IgG autoAb against epi BM--get characteristic IF band along BM
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dermal microabscesses with PMNs, eos @ tips of dermal papillae and IgA dep=
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dermatitis herpetiformis
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ocular albinism
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X linked; melanin dysfunction limited to eyes
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types of oculocutaneous albinism
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tyrosinase(-)-failure of conversion of tyrosin to DOPA (intermed. in melanin synthesis); tyrosinase(+)--mech unknown
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vitiligo
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aquired LOSS of melanocytes in discrete areas; autoimmune?
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blue nevus
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present at birth; nodular foci of dendritic highly pigmented melanocytes in DERMIS
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Spitz nevus
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benign! but often confused with malignant melanoma; in kids
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acrochordon
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skin tag
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dermatofibroma
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benign neoplasm that presents as firm nodule, characterized by intertwining bundles of collagen and fibroblasts
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dermatofibrosarcoma protuberans
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slow growing, well-diff'd malignant neoplasm that histologically resembles dermatofibroma (intertwining bundles of collagen and fibroblasts)
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acanthosis nigrans sometimes marker of?
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visceral malignancy
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variants of capillary hemangioma
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port wine stain; strawberry hemangioma--bright red raised; cherry hemangioma--small dome shaped papule
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Sturge-Weber syndrome
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port wine stain on face + ipsi glaucoma + vascular lesion of ocular choriodal tissue + extensive hemangiomatous involvement of meninges
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granuloma pyogenicum
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vascular pedunculated lesion characterized by numerous capillaries and edematous stroma; often develops following trauma
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histo characteristics of BCC
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darkly staining basaloid cells with typical palisade arrangement of nuclei of cells at periphery of tumor cell cluster
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nodular melanoma
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begins in vertical growth phase!! very poor prognosis
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disuse atrophy assoc'd with
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angular atrophy, mostly type II fibers
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death in Duchenne's muscular dystrophy most commonly due to?
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pneumonia caused by weakness of respiratory muscles
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facioscapulohumeral muscular dystrophy
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AD, slowly progressive but nondisabling with almost normal lifespan; mm of face then scapular then humerus
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myotonic dystrophy
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AD, CTG repeat in myotinin protein kinase gene (nl<30 repeat); myotonia, cataracts, testicular atrophy, male pattern baldness
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central core dz
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congenital myopathy (nl sr CK levels); loss of mitochondria et al in central portion of type I fibers--> weakness and hypotonia but babies eventually ambulatory
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nemaline myopathy
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congenital myopathy (nl sr CK levels); tangles of small rod-shaped granules mostly in type I fibers; varies clinically
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Kearns-Sayre syndrome
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a mitochondrial myopathy (ragged red fibers); ophthalmoplegia, pigmentary retinopathy, heart block, cerebellar ataxia; (only from mama!)
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Lambert-Eaton syndrome
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paraneoplastic (SCLC!); acquired Ab that reacts with presynaptic V-gated calcium channels
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von Recklinghausen dz of bone
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osteitis fibrosa cystica ("brown tumor"); due to primary or secondary hyperparathyroidism-->widespread osteoclastic lesions
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clinical manifestations of Rickets
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craniotabes (thin/soft parietal/occipital bones); late closing of fontanelles, rachitic rosary, Harrisons groove (depression along line of instertion of diaphragm into ribcage); pigeon chest
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complications of Paget dz (increased activity of o'blasts and o'clasts)
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bone pain (fx); high output cardiac failure; hearing loss; osteosarcoma
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None
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3 types of fibrous dysplasia
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monostotic--usually asympt but can have spontaneous fx; polyostotic; McCuneAlbright-polyostotic with precocious puberty, cafe au lait spots, short stature (young girls)
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Osgood-Schlatter dz
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avascular necrosis of tibial tubercle
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Legg-Calve-Perthes dz
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avascular necrosis of femoral head
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Kohler dz
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avascular necrosis of navicular bone
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3 eponynomous avascular necroses in children
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Legg-Calve-Perthes--head of femur; Korhler--navicular; Osgood-Schlatter--tibial tubercle
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None
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osteopetrosis--due to? assoc'd with?
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(marble bone dz); due to failure of o'clastic activity; assoc'd with anemia (dec'd BM); blindness deafness CN involvement (narrowing and impingment of neural foramina)
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bugs causing pyogenic osteomyelitis in a)kids b)newborns c)sickle cell d)IV drug users
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a)staph aureus; b)gp B beta strep, e.coli; c) salmonella; d) pseudomonas
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Brodie abscess
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wall of granulation tissue seen in pyogenic osteomyelitis
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Letterer-Siwe dz
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acute disseminated Langerhans cell histiocytosis; aggressive, usually fatal of babies; hepatosplenomegaly, lymphadenopathy, pancytopenia, pulm involvement , recurrent infections
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Hand-Schuller-Christian dz
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chronic progressive histiocytosis; triad: skull lesions, DI, exophthalmos (orbit involvement)
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variants of histiocytosis X
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Letterer-Siwe dz (v bad); Hand-SChuller-Christian dz (not as bad); eosinophilic granuloma (best prognosis)
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most common benign bone tumors
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osteochondroma-hamartoma, Ring of Ranvier defect; giant cell-"soap bubble" on XR, locally aggressive
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2-3xAP increase + Codman triangle + sunburst pattern =
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osteosarcoma
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osteosarcoma predisposing factors
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Paget dz, fibrous dysplasia, chondroma, osteochondroma; ionizing radiation; bone infarcts
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"small blue cell" tumor + t(11;22) =
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Ewing sarcoma (in early stages may mimic acute osteomyelitis)
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HLA type assoc'd with RA?
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HLA-DR4
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Felty syndrome
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RA, splenomegaly, neutropenia
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Still dz
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juvenile RA
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triad of Reiter syndrome
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urethritis, conjunctivitis, arthritis
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Heberden nodes vs Bouchard nodes
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(both osteophytes in OA); Heberden--DIP of fingers; Bouchard--PIP of fingers
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most common soft tissue sarcomas of late middle-old age?
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malignant fibrous histiocytoma
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fibrosarcoma
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malignant tumor of fibroblasts; spindle shaped cells in herringbone pattern
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spina bifida
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failure of posterior verterbral arches to close
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TORCH complex
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Toxoplasmosis, Other, Rubella, CMV, HSV; --> microcephaly, focal cerebral calcification
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hydrocephalus ex vacuo
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hyydrocephalus without obstruction or increase in CSF production; seen in d/o with dec'd cerebral mass (e.g. Alzheimers)
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external hydrocephalus
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increased CSF confined to subarachnoid space
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Arnold-Chiari malformation
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downward displacement of cerebellar tonsils and medullar thru foramen magnum; --> hydrocephalus; also usually thoracolumbar meningomyelocele
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None
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tuberous sclerosis syndrome
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AD, nodular proliferation of mulitnucleated atypical astrocytes; adenoma sebaceum of skin; "ash leafe spots"; angiomyolipoma of kidney; seizures and mental retardation beginning in infancy
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None
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most frequent site of CVA--thrombotic vs embolic?
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thrombotic occlusion--carotid bifurcation, MCA; embolic--MCA
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lacunar strokes clinical manifestations
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purely sensory(thalamus) or motor(internal capsule)
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Charcot Bouchard aneurysms
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minute dilations at small artery bifurcations in HTN; may rupture and cause intracranial hemorrhage
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intracranial hemorrhage most often where?
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basal ganglia/thalamus (most often due to HTN)
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lymphatics in CNS
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no lymphatics enter CNS
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hydrocephalus and pyogenic meningitis?
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meningitis can cause reactive fibroblastic arachnoiditis wiht scarring and obliteration of subarachnoid space--> hydrocephalus
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CSF findings in bacterial meningitis
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increased PMNs, increased proteins, decreased glucose
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most frequent cause of bacterial meningitis in a)infants/newbies b)children/young adults c) older adults
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a)listeria, gp B beta strep, e.coli; b) strep pneumo, n.meningitides; c)strep pneumo, GNR
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most common source of cerebral abscess
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middle ear
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periventricular calcification in newborn, think?
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toxoplasmosis (or CMV)
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how is CSF in viral meningitis different from bacterial meningitis?
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viral--inc'd lymphocytes, relatively increased protein, NORMAL GLUCOSE; bacterial--inc'd PMNs, inc'd protein, DECREASED glucose
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poliomyelitis
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degeneration and necrosis of anterior horn cells of spinal cord
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small cysts in CNS gray matter with striking absence of inflammatory response=
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prion dz
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lack of M compoment of measles virus--
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subacute sclerosing panencephalitis
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Charcot triad
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nystagmus, intention tremor, scanning speech; often a clinical manifestation of MS
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acute disseminated encephalomyelitis
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post infectious myelitis (delayed hypersensitivity?); widespread demyelination
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albumino-cytologic dissociation of CSF--
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very increased [protein], modest increase in cell count; seen in Guillain-Barre syndrome
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morphologic abnormalitis in Alzheimers
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neurofibrillary tangles; senile plaque; granulovaculor deg'n (w/i pyramidal cells of hippo); amyloid deposition; Hirano bodies (actin inclusions); generalized cerebral atrophy
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E4 allele of apoprotein E
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(csome 19) occurs with greater frequency in Alzheimers pts
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choline acetyltransferase deficiency
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seen in Alzheimers
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significance of nucleus basalis of Meynert?
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decreased neurons wthin this nucleus assoc'd with Alzheimers
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Binswanger dz
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assoc'd with HTN; multiple lacunar infarcts, progressive demyelination limited to subcortical area with characteristic sparing of cortex; subcortical leukoencephalopathy
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atrophy of striatum (caudate nucleus and putamen) seen in?
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HD (CAG repeat)
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genetics of HD
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AD, CAG repeat with huntingtin gene on csome 4
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Shy-Drager syndrome
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parkinsonism with autonomic dysfxn and orthostatic hypotension
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None
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progressive bulbar palsy
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brainstem and cranial nerve involvement leads to difficulty swallowing, speaking; ends in respiratory failiure
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most frequent cause of conjunctivitis?
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adenovirus infection
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retrolental fibroplasia
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retinopathy of prematurity; O2 toxicitiy--> blindness
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retinitis pigmentosa
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hereditary night blindness with progressive loss of central vision due to loss of rods, then cones; retinal pigmentation seen on ophthalmoscopy
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most common form of glaucoma?
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open angle (gradullay increasing IOP)
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increase in IOP on dilation of pupils=?
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closed angle glaucoma
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most common primary intracranial tumors in a) adults b) kinds
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a) glioblastoma multiforme, meningioma, acoustic neuroma; b) cerebellar astrocytoma; medullloblastoma
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"pseudopalisade" arrangment of tumor cells seen in?
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glioblastoma multiforme
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Name the tumor: slow growing, closely packed "fried egg" cells, foci of calcification
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oligodrendroglioma
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name the tumor: rosettes, blepharoplasts (rod shaped structures near nuclear representing basal bodies of cilia), may cause hydrocephalus
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ependymoma (usually in 4th ventricle)
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histology of meningioma
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whorled pattern of concentrically arranged spindle cells and laminated calcified psammoma bodies
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2 patterns of schwannoma
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Antoni A--interlacing bundles of elongated cells with palisading nuclei; Antoni B--looser, less cellular
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