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47 Cards in this Set
- Front
- Back
Temporal arteritis
age. sex? genetic association? Type of arterial thickening? Comorbid Sxs |
Most common vasculits
Seen in elderly females Association: HLA-DR4 Nodular intimal thickening Frequently co-exists with polymyalgia rheumatica occlusion of opthalmic art can lead to blindness |
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Takayasu Arteritis
"pulseless dz" |
UNDER 50. (if over 50 = giant cell aortitis)
Seen in asian girls and women Irregular Transmural thickening of the wall of the aortic arch perivascular cuffing of the vasa vasorum Loss of pulse in upper extremities |
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Multiple Sclerosis
Age, sex? CSF finding? |
MC demylinating dz
seen in women 20-40 Oligoclonal bands seen in CSF indicated demylination Association: HLA-DR2 |
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MS plaques
Acute vs Chronic |
Acute lesions- well circumcised plaques w/ loss of myelin, bilateral distribution, frequently periventricular
Chronic lesions- no inflammation, axons showing remyelination. Remylination is defective because myelin sheaths are thinner with shorter internodes |
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Multiple, unrealted neurological deficits in sensory and motor function
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MS
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50 y/o male c/o dropping things, cramping, difficulty with fine motor tasks & Fasiculations, pulmonary infection
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ALS
Loss of Upper and Lower motor neurons in a middle aged male No sensory deficits |
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What mutation is responsible for ALS pathology?
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Zinc-copper superoxide dismutase (SOD) mutation on chromosome 21
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What tracks are affected in ALS?
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Anterior (Ventral) Horn: progressive spinal muscular dystrophy
Corticospinal tract: primary lateral sclerosis |
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MC primary brain tumor in children
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Cystic Cerebellar Astrocytoma
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MC primary brain tumor in Adults
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Gliobastoma Mutiforme
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Polyps in the colon + tumors in the brain + cafe au lait
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Turcots syndrome (AR)
A risk factor for CNS tumors Cafe Au Lait spots are also seen in neurofibromatosis |
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Risk factors for brain tumors
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Turcots syndrome, neurofibromatosis, Cigarette smoking
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Astrocytoma
Location? kids, adults |
Intradural & Intramedullary
kids: cerebellum, adults: frontal lobe |
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crosses midline: butterfly appearance on CT
pseudopalisading necrosis fastest growing and most deadly |
Gliobastoma multiforme
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MC B9 brain tumor in adults
Psammoma bodies (cacified) Slowest growing |
Meningioma
Intradural, extramedullary |
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Arises in the cuada equina in adults and in the 4th ventricle of kids
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Ependymoma
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highest incidence btw 30-50
frontal lobe tumor that calcifies Fried egg appearance |
Oligiodendroglioma
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ptosis and diplopia that worsens throughout the day in a female
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Myasthenia gravis
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pathophys of MG
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autoantibodies against AChR on the NMJ
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what gene is defective in DMD?
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dystrophin gene on the short arm of X chromosome 21
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inheritance of DMD?
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X linked Recessive
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5 year old with progressive muscular weakness of the proximal muscles of the shoulder and pelvis
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DMD
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name 4 things associated with DMD
hallmark sign? MSK sign? Respiratory Sx? CV Sx? |
gower's sign
calf pseudotrophy respiratory insuficiency d/t decreased mucocilliary clearance heart failure and arrythmias |
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Dx of DMD
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elevated Creatinine Kinase, muscle biopsy shows necrosis, fibrosis
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how does Becker's compare to DMD?
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Beckers is a milder form of DMD
Dystrophin gene is mutated but still functional Later onset with rare cardiac involvment |
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40 year old man with acute onset and relapsing tinnitus, vertigo, nauea, and vommiting. what is the pathophys?
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Meniere's syndrome
disease of the inner ear that kills hair cells |
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what bug causes polio? How?
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Poliovirus
destruction of cells in the anterior horn of the spinal cord producing LMN sxs |
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Malaise, headache, fever, nausea, abdominal pain, sore throat
Signs of LMN lesions – muscle weakness and atrophy, fasiculations, fibrillation, hyporeflexia. |
Polio
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CSF with lymphocytic pleocytosis with slight elevation of protein (With no change in CSF glucose)
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Polio
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What findings are associated with tertiary syphillis?
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Gummas on bones, Aortitis (vasa vasorum destruction), tabes dorsalis, Argyll robertson pupils
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If you see Broad based ataxia and Postivie Romberg, what do you screen with, and what do you confirm with?
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Tertiary syphilis
Screen with VDRL, Confirm with FTA-ABS |
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Peripheral ipsilateral facial paralysis with inability to close eye on involved side
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Bells Palsy
Seen as a complication in AIDS, Lyme dz, Herpes simplex, Sarcoidosis, Tumors, Diabetes |
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What type of thickening is seen in Takayasu's vs Temporal arteritis?
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Takayasu is Irregular Transmural thickening
Temporal arteritis is Nodular Intimal thickening |
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Perivascular cuffing of the vasa vasorum
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Temporal Arteritis
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HLA-DR2
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Myasthenia Gravis
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HLA-DR4
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Temporal Arteritis
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Intradural, Extramedullary Tumors (2)
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Meningioma
Schwanoma/neurofibroma |
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Intradural, Intramedulary Tumors (3)
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Ependymoma (midline)
Astrocytoma Glioblastoma (midline) |
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Supratentorial, cerebellar lobe and deep hemispheric Tumors (2)
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Gliomas (Astrocytomas/ gliobastomas)
Meningioma |
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Infratentorial Tumors, in Adults
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Cerebropontine angle tumors: Acoustic Schwanoma
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Infratentorial Tumors, In children, Midline (2)
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Ependymoma
medulloblastoma |
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Infratentorial Tumors, In children, Cerebellar
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Astrocytoma
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A rapidly progressive, fatal dementia involving prominent motor symptoms, such as rigidity, clumsiness, and myoclonic jerks
A transmissible “spongiform” encephalopathy involving abnormal proteins called prions Diagnosed with certainty only by brain biopsy (o) |
Creutzfeldt-Jakob Disease
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What are the 3 categories of Creutzfeldt-Jakob Disease? Which are we most concerned about?
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Sporadic CJD appearing without known risk factors accounts for 85% of cases.
Hereditary CJD involves positive family history or positive testing for a genetic mutation. 5-10% of U.S cases. Acquired CJD involves transmission by exposure to brain or nervous system tissue. 1% of cases. (****transmission from open wounds, retina transplant, etc) |
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Increased levels of CSF protein 14-3-3 would give you the diagnosis of?
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Creutzfeldt-Jakob Disease
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inflammatory demyelinating plaques in the central nervous system is the hallmark of what?
(o) |
Multiple Sclerosis (MS)
has to do with OLIGODENDROCYTES (not Schwann--peripheral) |
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T1 “Black Holes” and brain atrophy can be seen in what?
or hyperlucent on T2 |
Multiple Sclerosis
note: Gilenya ™ - Fingolimod is the first oral pill tx for MS |