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Flashcards in Neuro Path Deck (28):
1

Alzheimers

- 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)

2

Pick disease

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

3

Lewy body dementia

- initially dementia and visual hallucinations followed by parkinsonian features
- alpha synuclein defect

4

GBS findings and treatment

- symmetric ascending weakness/paralysis
- increased CSF protein and papilledema
- treat with plasmapheresis, IVIG

5

Charcot-Marie-Tooth disease

- 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

6

adrenoleukodystrophy

- 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

7

Cluster headache

- may induce Horners
- treat with inhaled oxygen, sumatriptan

8

tension headache treatment

- treat with NSAIDs, acetaminophen, amitriptyline for chronic pain

9

migraine prophylaxis

- propranolol, topiramate, CCBs, amitriptyline

10

peripheral vertigo

- more common, inner ear etiology (semicircular cana debris, vestibular nerve infection, meniere disease)
- positional testing --> delayed horizontal nystagmus

11

central vertigo

- 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

12

meniere diseae

- 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

13

Sturge Weber syndrome

- 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

14

tuberous sclerosis

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

15

NF1 (Von Recklinghausen disease)

- 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

16

VHL disease

- 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

glioblastoma

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

meningioma

- 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

hemagioblastoma

- 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

schwannoma

- 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

oligodendroglioma

- rare, slow growing
- most often in frontal lobe white matter --> seizures
- chicken wire capillary pattern
- oligodendrocytes = "fried egg" cells
- often calcified

22

pilocytic astrocytoma

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

23

medulloblastoma

- 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

ependymoma

- 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

craniopharyngioma

- benign childhood tumor (most common supratentorial)
- derived from remnants of Rathke's pouch (same as ant pit)
- calcification is common

26

cingulate (subfalcine) herniation

- can compress ACA

27

uncal herniation

- can compress CN III (ipsilateral down and out), PCA (contralateral homonymous hemianopsia), and contralateral crus cerebri (ipsilateral paralysis)

28

cerebellar tonsilar herniation

- coma and death due to brain stem compression