Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
75 Cards in this Set
- Front
- Back
|
Osteogenesis Imperfecta
|
fragility of bone due to disorder in COLLAGEN
see thin blue sclera and increaded fractures newborn type most severe |
|
|
Osteopetrosis
|
sclerosis of vertebral endplate-looks like soccer jersey w stripes
increased density and abnomal skeletal modeling= very BRITTLE bones |
|
|
Achondroplasia
|
AD
defective cartiladge synthesis and decreased epiphysis formation short limbs normal size trunk and head |
|
|
Aseptic necrosis
|
cut in the blood supple can be secondary to trauma
Osgood Schlatter & leg Calve perthese Dz |
|
|
Osteoporosis
|
Thinned cortical bone and enlarged meduallary cavity seen on radiology
Less bone tissue but what is there is normal easy fractures; wedge fracture that gives up kyphosis white, women that are small boned NORMAL serum findings |
|
|
Types of primary osteoporosis
|
idiopathic-unknown-kids and adolescents
type 1-postmenopausal women type 2-senile osteo-men and women over 70 |
|
|
Treatment of osteoporosis
|
physical activity
calcium 1000-1500 Vit D Bisphosphonates-DOC |
|
|
Osteomalacia
|
inadequate bone mineralization
kids known as rickets when caused by Vit D deficiency low Ca & phosphate High alk phos bow legs |
|
|
osteitis fibrosa cystica
|
focal bony damage bc sever hyperparathyroidism
microfractures and secondary hemorrhages that cause ingrowth of fibrous tissue along with hemosiderin deposition (BROWN TUMOR) |
|
|
Paget's Dz
|
increased, uncontrolled bone turnover with formation outpacing destruction
frontal bossing, deafness, bone pain, compression of spinal nerve roots, chalkstick fractures of lower extremities, high output cardiac failure extremely high Alk phos ( like hepatocellular carcinoma) |
|
|
Osteochondroma
|
benign tumor of bone and cartiladge-usually end of long bone
most common bone tumor tumor covered by cartilaginous cap 10-20 increase risk for chondrosarcoma |
|
|
Enchondroma
|
tumor or cartiladge ehere cartilage does not normally exist
increase rik os chondrosarcoma |
|
|
Chondroblastoma
|
RARE
10-20 femur, tibia, humerus epiphysis |
|
|
Chondrosarcoma
|
malignant
spine and pelvic bones slower growing that osteosarcoma |
|
|
Osteoma
|
multiple PAINLESS usually on facial bone
|
|
|
Osteoid Osteoma
|
small, PAINFUL
extremities of teens and young adults |
|
|
Osteoblastoma
|
larger, milder pain-dull and achy
Spine |
|
|
Osteosarcoma
|
any bone w/ equal frequency
1st symptime often pathologic fracture PAINFUL and progressively enlarging mass Codman's Trigangle-triangular, right angled mass representing tumor growth that has broken through the cortex and lifted the periosteum. bimodal: under 20 and the old |
|
|
Ewing Sarcoma
|
10-15y/o Caucasion
arises in the medullary cavity and invades the cortex and periostieum = soft tissue mass + Homer Wright rossettes on histology PAINFUL-surrounding area in tender and swollen "Onion skin" |
|
|
Duchenne Muscular Dystrophy
|
lack of dystrophin
2-3 y/o-wheelchair by 12-die by 20 usually pneumonia :( skeletal muscle atrophies and dies, pectoral muscle weakening first +gowers sign, hypertrophied calves |
|
|
Beckers
|
less severe Duchenne's
onset 5-15 and ambulatory to 15 |
|
|
Myotonic
|
AD with CTG repeat
onset at any age born w/ long fact and temporal and masseter muscle weakening |
|
|
If a person has a headache esp when laying down what should you suspect?
|
Brain Tumor
|
|
|
Astrocytoma 2 typess
|
1-fibrillary
2-pilocytic |
|
|
Fibrillary Astrocytoma-Glioblastoma multiforme
|
Most common brain tumor in adults
extemely high grade butterfly shape crossed the corpus callosum always fatal :( Bodi |
|
|
Pilocytic Astrocytoma
|
kids and young adults
cerebellum hair like processes rarely infiltrates and grows slowly |
|
|
Medulloblastoma
|
Most common childhood brain tumor
cerebellum and in the midline rapid growth may block csf= hydrocephalus very poorly differentiaed highly malignant and untreated = death very radiosensitive |
|
|
Oligodendrogliomas
|
middle age w/ calcifications
"fried egg" appearance seizures less agressive |
|
|
Ependymoma
|
epyndymal lining of the ventricular system
CSF dissementaion is common teens-4th vent older-spinal cord "rosettes or perivascular pseudorosettes" |
|
|
Meningioma
|
benign
assoc w/ NF2 mutation encapsulated well defined mass compressing underlying brain tissue "Whirling pattern" Psammoma bodies >40 |
|
|
Schwannoma
|
Acoustic neuroma
loss of NF2 vestibular branch of CN 8 at the cerebellopontine angle assoc w/ Neruofibromatosis II |
|
|
neurofibroma
|
connective and nervous tissue
assoc w/ NF1 and Neruofibromatosis I Benign |
|
|
Craniopharyngioma
|
derived from remnants of Rathke's pouch
may encroach: hypothalamus,ventricles, optic chiasm kids or teens most common SUPRATENTORIAL tumor of kids (most of infratentorial) |
|
|
Pituitary Adenoma
|
excess production of ant pit hormoes : ACTH TSH FSH LH GH
30-60 y.o mincroadenoma <10mm macroadenoma >10mm-greater propensity for compressing the optic chiasm |
|
|
Prolactinoma
|
type of Pituitary Adenoma
most common and most macro's prolactin so amenorrhea, galactorrhea, loss of libido (1st sign), infertility & visual distubances |
|
|
Growth Hormone
|
type of Pituitary Adenoma
2nd most common GH so get acromegaly or giantism |
|
|
Corticotroph Tumor
|
type of Pit Adenoma
microadenoma excess ACTH= adrenal hypersecretion of cortisol Cushing's Syndrome |
|
|
Lymphoma
|
aggressive & poor chemo response
elderly and AIDS "AIDS defining cancer" if occurs in the brain of HIV pt |
|
|
Lipoma
|
delicately encapsulated and small
mature adipost tissue benign but not good if in brain bc space occupying |
|
|
Hemangioblastoma
|
assoc/ w capillaries of the cerebellum and retina
assoc/ w Von HIppel Lindau dz bening but bad in brain bc space occupying |
|
|
Alzheimers
|
Most common CNS degenerative & casue of dementia
dementia and memory loss last ab 10 years and death via pneumonia cortical atrophy marked decrease in neurons in-nuclues basalis of Meynert |
|
|
What allele of what chromo is assoc w/ AD?
|
ApoE on Chromosome 19
ApoE binds amyloid beta protein in plaques |
|
|
Critical molecule in AD for ALL cases? and on what chormosome
|
amyloid beta protein
Chromosome 21 |
|
|
What else do you see in AD?
|
senile plaques
neurofibrillary tangles HIrano bodies |
|
|
Pick's Dz
|
frontotemporal dementia
change in personality initially women>men progresses quick 2-3 years Kluver Bucy is a variant -hypersexual -hyperphasia-orally fixaed -males more w/ this |
|
|
Parkinson's Dz
|
Loss of substantia nigra and assoc w/ low dopamine
bradykinesia muscle rigidity resting tremor w/ pill rolling festinating gait FIRST sympton-loss of smell bc LEWY bodies first form in olfactory |
|
|
Treat parkinsons
|
anticholinergics-benztropine, biperident or trihexyphenidyl
dopamernergic agents-carbidopa-levodopa, promipexole, ropinrole, MOAI selective-selegline and rasagline |
|
|
ALS
|
male>female 50-70
UPPER and LOWER motor neurons but sensory is FINE SOD1-genetic defect in cooper zinc superoxide dismutase |
|
|
How does ALS start/present
|
Weakness in hands and person drops things a lot
then cramping of arms and legs eventually fasiculations |
|
|
LMN signs
|
symmetrical muscle weakness and atrophy
fasiculations hypOreflexia |
|
|
UMN signs
|
hypERreflexia
spasticity Babinsky's sign |
|
|
Huntington's Dz
|
AD with CAG CAG CAG CAG CAG CAG repeat
degenerative and atrophy in caudate nuc (esp), putamen & frontal cortex 30-40 symp begin |
|
|
Characteristics of Huntingtons
|
Whole body choreoathetosis
hypertonicity fecal and urinary incontenence anorexia weight loss depression eventual SEVER dementia and death :( sad dz |
|
|
Friedreich's ataxia
|
AR with GAA GAA GAA GAA repeat
Chromosome 9 symptoms begin ab 11 y/o ataxia, dysarthria,decreased DTR's, babinski, sensory and propripceptive loss, pes cavus, progressive kyphoscoliosis, type 1 DM, cardiomyopathy, arrhythmias |
|
|
Multi infarct dememtia
|
2nd most common behind AD
Long standing HTN Step wise decrease in cognitive fx from multiple small infarcts |
|
|
Binswanger Dz
|
long standing HTN
multiple lacunar infarcts and progressive demylination worseining confusion and concomitant mood disorder |
|
|
`Progressive Bulbar Palsy
|
brainstem and cranial nerve palsy = diarrhea, resp difficulties and hard time swallowing
|
|
|
Werdnig-Hoffman
|
AR
infantile spinal muscular atrophy seen birth to 4 months and death by 3 |
|
|
Multiple Sclerosis
|
autoimmune dz assoc w/ HLA DR2
20-40 y/o female>male #1 most common demyelinating dz and part of the CNS plaques in white matter-esp around venticles and optic nerve-seen on MRI + oligoclonal bands distinct lesions in time and space |
|
|
Charcot's Triad for MS
|
nystagmus
scanning speech intention tremor (opposite of Parkinsons) |
|
|
DOC for MS
|
Interferon Beta
glatiramer can be used too natalizumab-when all else has failed |
|
|
Devic Syndrome
|
Asia and South America
bilateral optic neuritis followed by day or weeks by transverse myelitis self limited by sometimes heralds start of MS |
|
|
Guillain Barre
|
ASCENDING motor weakness following the flu, immunization or camplobacter gastroenteritis
demyleination of the PNS |
|
|
Adrenoleukodystrophy
|
X linked
def in perozisomal transporter enzyme = inability to properly catabolize very long chain fatty acids |
|
|
Schilder's Dz
|
variant of Adrenoleukodystrophy
mimics MS but get 1-2 very large plaques of demylelination in the cerebral hemispheres |
|
|
Metachromatic Leukodystrophy
|
AR
spingolipidosis that becomes symptomatic early in childhood or adulthood def in arylsufatase A demyl in CNS & PNS CSF-high protein count forgetfullness,poor school or work performance, progressive personality changes, mask facies and strange postures |
|
|
Subacute Combined Degeneration
|
Vit B12 def
demyleination and eventual destruciton of spinal cord posterior coloums and later corticospinal tract so involved BOTH ascending and descending |
|
|
Progresisve Multifocal Leukoencephalopathy
|
JC virus (papovavvirus)
complete destruction of all oligodendrocytes severe immunmosuppression MRI-shows global white atrophy Like MS but different in that dementia and aphasia are prominent early findings death in 6-12 months |
|
|
Alcohol withdrawl
|
seizures and hallucinations
|
|
|
Hepatic encephalopathy
|
pathology in brain secondary to system delivery or ammonia and other bowel toxins due to insufficient removal
|
|
|
Wernicke's Syndrome
|
acute sever B1(thiamine) def- can be induced by IV glucose to an alcoholic with giving thiamine 1st
focal ophthalmoplegia, confusion and ataxia that is reversible w B1 |
|
|
Korsakoff's encephalopathy
|
NOT reversible and follow some cases of Wernicke's
|
|
|
Alchoholic cerebellar degeneration
|
vermis atrophies secondary to alcohol tox
ataxia of the trunk |
|
|
Alcoholic neuropathy
|
toxic effects of alcohol = path or PNS
1st vibration sense is lost, then painful disturbances occures |
|
|
Marchiafava Bignami Dz
|
Large quantitis of RED WINE
degeneration of corpus callosum and resulting frontal lobe dementia "classic WINO" |
