neuro 490 - 494 Flashcards
what neurocutaneous dz is associated with optic gliomas?
NF type 1
NF type 1 is also called
von Recklinghausen dz
what neurocutaneous dz (ND) is associated with shagreen patches?
TS
what ND is associated with pheochromocytoma?
VHL
what type of multiple endocrine neoplasia is associated with pheochromocytoma?
MEN2B
what ND is associated with GNAQ gene mutation?
sturge weber syndrome
which ND is associated with 2’ polycythemia?
VHL (hemangioblastoma –> inc EPO)
what ND is associated with inc incidence of subependymal astrocytomas and ungual fibroma
TS
which ND is associated with early onset glaucoma?
sturge weber
explain the pathophysio behind early onset glaucoma in sturge weber
ipsilateral leptomeningeal angioma & episcleral hemangioma –> inc IOP –> early onset glaucoma
which ND is associated with seizures, epilepsy, and intellectual disability?
sturge weber
what ND is associated with mitral regurge?
TS
what path is associated with tram track calcification (opposing gyri)?
sturge weber
what is nevus flammeus and what dz is associated with it?
a non neoplastic brithmark in CN V1/V2 distribution, Sturge Weber
what neurocutaneous disorder is non-inherited (somatic)?
Sturge weber
what neurocutaneous disorder is due to the developmental anomaly of neural crest derivatives?
Sturge weber
what is the role of neurofibromin in NF1?
a negative regulator of RAS on ch#17
what path is associated with shagreen patches?
TS
what path is associated with cafe au lait spots?
NF1
what path is associated with carvernous hemangioma in skin, mucosa, skin, organs?
vHL
2 neurocutaneous disorders associated with intellectual disability?
sturge weber, TS
what is the type of headache associated with repetitive brief headache?
cluster
describe the characteristics of tension headache
steady pain, no photophobia or phnophobia. No aura
how would you diff cluster, tension, migraine headache in terms of their location?
cluster: unilateral
tension: bilateral
migraine: unilateral
what type of headache is associated with excruciating periorbital pain with lacrimation and rhinorrhea with potential Horner syndrome?
Cluster
treatments for cluster headache?
100% O2, sumatriptan
target of sumatriptan?
5HT 1B/1D agonist –> inhibit trigeminal nerve activation, vasoactive peptide release –> induces vasoconstriction
how is trigeminal neuralgia diff from cluster headache?
TN produces repetitive shooting pain in the distribution of CN5 that lasts for less than 1 min
how would you diff cluster, tension, migraine headache in terms of duration?
cluster: 15 min - 3 hours
tension: more than 30 min (typically 4-6hr)
migraine: 4-72 hrs
explain the pathophysio of migraine
due to irritation of CN V, meninges, or blood vessels
in migraine, what 3 substances are released from blood vessels?
substance P, calcitonin gene related peptide, vasoactive peptides
4 DOC for migraine prophylaxis?
propranolol, topiramate, CCB, amitriptyline
4 DOC for tension headache?
analgesics, NSAIDs, acetaminophen, amitriptyline for chronic pain
mnemonics for migraine headache?
POUND
Pulsatile One day duration Unilateral Nausea Disabling
in terms of the location of the lesion, how can you diff peripheral vs ventral vertigo?
peripheral: inner ear etiology
central: brain stem or cerebellar lesion
how would you test peripheral vertigo?
positional testing to see if there is any delayed horizontal nystagmus
how would you test central vertigo?
positional testing will reveal immediate nystagmus in any direction
name 3 inner etiologies that can cause peripheral vertigo
- semicircular canal debris
- vestibular nerve infection
- Meniere dz
what brain tumor is associated with chick wire capillary pattern?
oligodendrogioma
what brain tumor is associated with drop metastases to spinal cord?
medulloblastoma
in uncal herniation, you get 2 unique clinical symptoms (other than CN3 symptoms), what are they? Explain.
- contralateral crus cerebri at the Kernohan notch –> ipsilateral paresis, a false localization sign
- ipsilateral PCA –> contralateral hmonymous hemianopia
what type of hernia can cause coma or death as a result of compressing brain stem?
cerebellar tonsillar herniation into the foramen magnum
what is uncus?
medial temporal lobe
what cranial nerve is affected by uncus herniation?
CN3
2 clinical symptoms from CN3 lesion?
blown pupil, down and out gaze
what type of hernia leads to duret hemorrhages?
downward transtentorial (central) herniation due to caudal displacement of the brain stem
duret hemorrhage is due to a rupture of what blood vessel?
paramedian basilar artery branches
what type of hernia compresses anterior cerebral artery?
cingulate (subfalcine) herniation under falx cerebri
pts with cingulate (subfalcine) herniation will present what types of focal neurological deficits?
contralateral paralysis of the lower limbs
contralateral loss of sensation of the lower limbs
childhood tumors occur normally in infratentorial. what is the exception?
craniopharyngioma (occurs in supratentorial)
which brain tumor is associated with small blue cell (Homer Wright rosetts)?
medulloblastoma
what other 2 paths are associated with small blue cells?
- Ewing (anaplastic small blue cells)
2. Neuroblastoma (neural crest cells –> Homer Wright rosette)
what 2 childhood brain tumors can affect the 4th ventricle?
medulloblastoma, ependymoma
what tumor is associated with rosenthal fibers?
pilocytic astrocytoma
what is rosenthal fibers?
eosinophilic, corkskrew fibers
what 2 brain tumors are GFAP positive?
glioblastoma multiforme (grade 4 astrocytoma) pilocytic astrocytoma
DOC for glaucoma emergenices?
pilocarpine (very effective at opening meshwork into canal of Schlemm)
what 2 opioids are used for diarrhea?
loperamide, diphenoxylate
what 2 drugs are used for maintenance programs for heroin addicts?
methadone, buprenorphine + naloxone