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Step 1 Flash Cards > Neuro Path > Flashcards

Neuro Path Flashcards

1
Q

Alzheimers

A
  • most common cause of dementia in the elderly
  • early onset mutations - APP, presenilin-1 and presenilin-2
  • late onset associated with Apo E4
  • Apo E2 is protective
  • decreased Ach in the hippocampus and nucleus basalis
  • amyloid beta plaques and neurofibrillary tangles of hyperphosphorylated tau (tangles correlate with degree of dementia)
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2
Q

Pick disease

A
  • frontotemporal dementia
  • spares parietal lobe and pst 2/3 of superior temporal gyrus
  • pick bodies: spherical tau protein aggregates
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3
Q

Lewy body dementia

A
  • initially dementia and visual hallucinations followed by parkinsonian features
  • alpha synuclein defect
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4
Q

GBS findings and treatment

A
  • symmetric ascending weakness/paralysis
  • increased CSF protein and papilledema
  • treat with plasmapheresis, IVIG
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5
Q

Charcot-Marie-Tooth disease

A
  • aka hereditary motor and sensory neuropathy
  • AD inherited disorder of production of proteins in peripheral nerves or the myeline sheath
  • associated with scoliosis and foot deformities
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6
Q

adrenoleukodystrophy

A
  • X linked genetic disorder
  • disrupts metabolism of very long chain fatty acids
  • excessive buildup in the nervous system, adrenal gland and testes
  • progressive disease that can lead to long-term coma/death and adrenal gland crisis
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7
Q

Cluster headache

A
  • may induce Horners

- treat with inhaled oxygen, sumatriptan

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8
Q

tension headache treatment

A
  • treat with NSAIDs, acetaminophen, amitriptyline for chronic pain
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9
Q

migraine prophylaxis

A
  • propranolol, topiramate, CCBs, amitriptyline
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10
Q

peripheral vertigo

A
  • more common, inner ear etiology (semicircular cana debris, vestibular nerve infection, meniere disease)
  • positional testing –> delayed horizontal nystagmus
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11
Q

central vertigo

A
  • brain stem or cerebellar lesion
  • directional change of nystagmus, skew deviation, diplopia, dysmetria
  • positional testing –> immedate nystagmus in any direction, may change directions
  • focal neuro findings
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12
Q

meniere diseae

A
  • tinnitus +/- fullness
  • vertigo - subjective sensation of movement
  • sensorineural hearing loss
  • due to defective resorption of endolymph –> increased volume of inner ear
  • differentiate from schwannoma by episodic nature
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13
Q

Sturge Weber syndrome

A
  • congenital (non-inherited) developmental anomaly of neural crest derivatives due to activating mutation of GNAQ gene
  • port wine stain, ipsilateral leptomeningeal angioma, seizures, intellectual disability, episcleral hemangioma, early-onset glaucoma
  • STURGE: sporadic, tram track calcifications, unilateral, retardation, glaucoma, GNAQ gene, epilepsy
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14
Q

tuberous sclerosis

A

HAMARTOMASS - Harmartomas of the CNS and skin, Angiofibromas, Mitral regurg, Ash-leaf spots, cardiac Rhabdomyoma, (TS), autosomal dOminant, Mental retardation, renal Angiomyolipoma (mass of fat, smooth muscle and blood vessels), Seizures, Shagreen patches
- increased incidence of subependymal astrocytomas and ungual fibromas

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15
Q

NF1 (Von Recklinghausen disease)

A
  • cafe au lait spots, lisch nodules (pigemented iris hamartomas), neurofibromas on skin, optic gliomas, pheos
  • mutated NF1 on chr 17 (tumor suppressor gene), single gene AD inheritance
  • skin tumors are derived from neural crest cells
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16
Q

VHL disease

A
  • cavernous hemangiomas in skin, mucosa and organs
  • bilateral RCCs, hemangioblastoma in retina, brain stem, cerebellum and pheos
  • AD mutated VHL tumor suppressor gene on Chr 3
  • mutation results in constitutive expression of HIF (transcription factor) and activation of angiogenic growth factors
17
Q

glioblastoma

A

common, highly malignant primary brain tumor with ~1 year median survival

  • found in cerebral hemispheres, can cross the CC, stains + for GFAP
  • “pseudopalisading” pleomorphic tumor cells border central areas of hemorrhage and necrosis
18
Q

meningioma

A
  • common benign primary brain tumor (has estrogen receptors - more common in women)
  • arises from arachnoid cells (extra-axial)
  • may present with seizures of focal neuro signs
  • spindle cells concentrically in a whorled pattern, psammoma bodies
19
Q

hemagioblastoma

A
  • most often cerebellar
  • associated with VHL when found with retinal angiomas
  • can produce EPO –> secondary polycythemia
  • closely arranged, thin walled capillaries with minimal interleaving parenchyma
20
Q

schwannoma

A
  • ususally found at the CPA, schwann cell origin, S-100 +
  • resectable or treated with sterotactic radiosurgery
  • can also involve V and VII
  • bilateral acoustic schwannomas found in NF2
21
Q

oligodendroglioma

A
  • rare, slow growing
  • most often in frontal lobe white matter –> seizures
  • chicken wire capillary pattern
  • oligodendrocytes = “fried egg” cells
  • often calcified
22
Q

pilocytic astrocytoma

A
  • childhood tumor of the cerebellum, often well-circumscribed
  • GFAP +, benign, good prognosis
  • rosenthal fibers - eosinophilic, corkscrew fibers
  • cystic + solid components
23
Q

medulloblastoma

A
  • highly malignant cerebellar tumor
  • primitive neuroectodermal tumor (granular cells)
  • can compress 4th ventricle –> hydrocephalus
  • can have “drop mets” to the SC
  • Homer-Wright rosettes: small blue cell around neuritic processes
  • presents with headache and cerebellar dysfunction
24
Q

ependymoma

A
  • ependymal cell tumors most commonly found in 4th ventricle
  • can cause hydrocephalus, poor prognosis
  • characteristic perivascular pseudo-rosettes (gland-like structures)
  • rod-shaped blepharoplasts (basal ciliary bodies) found near nucleus
25
Q

craniopharyngioma

A
  • benign childhood tumor (most common supratentorial)
  • derived from remnants of Rathke’s pouch (same as ant pit)
  • calcification is common
26
Q

cingulate (subfalcine) herniation

A
  • can compress ACA
27
Q

uncal herniation

A
  • can compress CN III (ipsilateral down and out), PCA (contralateral homonymous hemianopsia), and contralateral crus cerebri (ipsilateral paralysis)
28
Q

cerebellar tonsilar herniation

A
  • coma and death due to brain stem compression

Step 1 Flash Cards (36 decks)

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