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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
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
Multiple Sclerosis
Age, sex?
CSF finding?
MC demylinating dz
seen in women 20-40
Oligoclonal bands seen in CSF indicated demylination
Association: HLA-DR2
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
Multiple, unrealted neurological deficits in sensory and motor function
MS
50 y/o male c/o dropping things, cramping, difficulty with fine motor tasks & Fasiculations, pulmonary infection
ALS

Loss of Upper and Lower motor neurons in a middle aged male

No sensory deficits
What mutation is responsible for ALS pathology?
Zinc-copper superoxide dismutase (SOD) mutation on chromosome 21
What tracks are affected in ALS?
Anterior (Ventral) Horn: progressive spinal muscular dystrophy

Corticospinal tract: primary lateral sclerosis
MC primary brain tumor in children
Cystic Cerebellar Astrocytoma
MC primary brain tumor in Adults
Gliobastoma Mutiforme
Polyps in the colon + tumors in the brain + cafe au lait
Turcots syndrome (AR)
A risk factor for CNS tumors
Cafe Au Lait spots are also seen in neurofibromatosis
Risk factors for brain tumors
Turcots syndrome, neurofibromatosis, Cigarette smoking
Astrocytoma
Location? kids, adults
Intradural & Intramedullary
kids: cerebellum, adults: frontal lobe
crosses midline: butterfly appearance on CT
pseudopalisading necrosis
fastest growing and most deadly
Gliobastoma multiforme
MC B9 brain tumor in adults
Psammoma bodies (cacified)
Slowest growing
Meningioma

Intradural, extramedullary
Arises in the cuada equina in adults and in the 4th ventricle of kids
Ependymoma
highest incidence btw 30-50
frontal lobe tumor that calcifies
Fried egg appearance
Oligiodendroglioma
ptosis and diplopia that worsens throughout the day in a female
Myasthenia gravis
pathophys of MG
autoantibodies against AChR on the NMJ
what gene is defective in DMD?
dystrophin gene on the short arm of X chromosome 21
inheritance of DMD?
X linked Recessive
5 year old with progressive muscular weakness of the proximal muscles of the shoulder and pelvis
DMD
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
Dx of DMD
elevated Creatinine Kinase, muscle biopsy shows necrosis, fibrosis
how does Becker's compare to DMD?
Beckers is a milder form of DMD

Dystrophin gene is mutated but still functional

Later onset with rare cardiac involvment
40 year old man with acute onset and relapsing tinnitus, vertigo, nauea, and vommiting. what is the pathophys?
Meniere's syndrome

disease of the inner ear that kills hair cells
what bug causes polio? How?
Poliovirus
destruction of cells in the anterior horn of the spinal cord producing LMN sxs
Malaise, headache, fever, nausea, abdominal pain, sore throat
Signs of LMN lesions – muscle weakness and atrophy, fasiculations, fibrillation, hyporeflexia.
Polio
CSF with lymphocytic pleocytosis with slight elevation of protein (With no change in CSF glucose)
Polio
What findings are associated with tertiary syphillis?
Gummas on bones, Aortitis (vasa vasorum destruction), tabes dorsalis, Argyll robertson pupils
If you see Broad based ataxia and Postivie Romberg, what do you screen with, and what do you confirm with?
Tertiary syphilis
Screen with VDRL, Confirm with FTA-ABS
Peripheral ipsilateral facial paralysis with inability to close eye on involved side
Bells Palsy
Seen as a complication in AIDS, Lyme dz, Herpes simplex, Sarcoidosis, Tumors, Diabetes
What type of thickening is seen in Takayasu's vs Temporal arteritis?
Takayasu is Irregular Transmural thickening

Temporal arteritis is Nodular Intimal thickening
Perivascular cuffing of the vasa vasorum
Temporal Arteritis
HLA-DR2
Myasthenia Gravis
HLA-DR4
Temporal Arteritis
Intradural, Extramedullary Tumors (2)
Meningioma
Schwanoma/neurofibroma
Intradural, Intramedulary Tumors (3)
Ependymoma (midline)
Astrocytoma
Glioblastoma (midline)
Supratentorial, cerebellar lobe and deep hemispheric Tumors (2)
Gliomas (Astrocytomas/ gliobastomas)
Meningioma
Infratentorial Tumors, in Adults
Cerebropontine angle tumors: Acoustic Schwanoma
Infratentorial Tumors, In children, Midline (2)
Ependymoma
medulloblastoma
Infratentorial Tumors, In children, Cerebellar
Astrocytoma
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
What are the 3 categories of Creutzfeldt-Jakob Disease? Which are we most concerned about?
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)
Increased levels of CSF protein 14-3-3 would give you the diagnosis of?
Creutzfeldt-Jakob Disease
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)
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