Neuro Tumors and Neurocutaneous Disorders

hezikry's version from 2015-09-18 18:04


Question Answer
patients at greater risk of Alzheimer'sDown syndrome
early onset familial Alzheimer'sAPP (21), presenilin-1 (14), presenilin-2 (1)
late onset familial Alzheimer'sApoE4 (19)
protective protein in familial Alzheimer'sApoE2 (19)
decreased neurotransmitter in Alzheimer'sACh
extracellular beta-amyloid core may causeintracranial hemorrhage in Alzheimer's
Pick's (Frontotemporal Dementia) symptomsdementia, aphasia, parkinsonian aspects, change in personality
pick's sparesparietal lobe and posterior 2/3 of superior temporal gyrus (frontotemporal dementia and atrophy)
pick's bodiesspherical tau protein aggregates
lewy body dementia symptomsinitially dementia and visual hallucinations followed by parksonian features
defect in lewy body dementiaalpha-synuclein defect
creutzfeld-Jakob disease symptomsrapidly progressive (weeks to months) dementia with myoclonus; "startle myoclonus"
creutzfeld-jakob pathologyspongiform cortex, beta-pleated sheets of prions resistant to proteases
widespread cortical atrophyAlzheimer's
Frontotemporal atrophyPick's disease
elderly patients w dementia at risk foraspiration pneumonia
dementia plus visual hallucinationsLBD
dementia plus progressive aphasiaFTD aka Picks
dementia plus ataxia and loss of pupillary light reflextertiary syphillis
dementia plus uninhibited social behaviorFTD
dementia plus urinary incontinence and magnetic gaitNPH
dementia plus syncopal episodesLBD
dementia plus myoclonusCJD


Question Answer
classic signs of multiple sclerosisscanning speech, intention tremor, internuclear opthalmoplegia, nystagmus
CSF findings in MSincreased protein (IgG)
diagnostic in MSoligoclonal bands
gold standard imaging technique in MSMRI
imaging finding in MRI in MSperiventricular plaques (areas of oligodendrocyte loss and reactive gliosis) with destruction of axons
treatment of MSbeta-interferon, immunosuppression, natalizumab
treatment for neurogenic bladder in MScatheterization, muscarinic antagonist like oxybutynin
treatment for spasticity in MSbaclofen, GABA receptor agonist
cell destroyed in acute inflammatory demyelinating polyradiculopathyschwann cells
pathogens associated with Guillain-Barrecampylobacter jejuni and CMV (molecular mimicry)
majority of guillain-barre patients recover withinweeks to months
pathogenesis of Guillain-Barreautoimmune condition that destroys Schwann cells. Leads to inflammation and demyelination of peripheral nerves and motor fibers. Symmetric ascending paralysis
these functions may be severely affected in guillain-barreautonomic; cardiac irregularities, hypertension, hypotension
critical in guillain-barrerespiratory support
additional treatment of guillain-barreplasmapheresis, IV immune globulins
findings in guillain-barrealbuminocytologic dissociation; increased CSF protein with normal cell count. papilledema (increasd protein)
progressive multifocal leukoencephalopathy (PML) pathologydemyelination of CNS due to destruction of oligodendrocytes
PML associationsJC virus, seen in 2-4% of AIDS patients due to latent viral reactivation
PML disease courserapidly progressive, usually fatal
acute disseminated postinfectious encephalomyelitis pathologymultifocal perivenular inflammation and demyelination after infection
acute disseminated postinfectious encephalomyelitis associated infectionsmeasles, VZV
acute disseminated postinfectious encephalomyelitis associated vaccinationsrabies, smallpox
genetics of metachromatic leukodystrophyautosomal recessive lysosomal storage disease, most commonly due to arylsulfatase A deficiency
pathology of metachromatic leukodystrophybuildup of sulfatides leads to impaired production of myelin sheath
charcot-marie-tooth diseaseprogressive hereditary nerve disorders related to defective production of proteins in peripheral nerves or myelin sheath
krabbe's disease geneticsautosomal recessive lysosomal storage disease due to deficiency of galactocerebrosidase
krabbe's disease pathologybuildup of galactocerebroside destroys myelin sheath
adrenoleukodystrophydisrupts metabolism of very long chain fatty acids. progressive disease that leads to death


Question Answer
status epilepticuscontinuous seizure for > 30 min, or recurrent seizures without regaining consciousness for > 30 min. medical emergency
most common area for partial (focal) seizures to originatemedial temporal lobe
simple partial seizureconsciousness intact; can be motor, sensory, autonomic, psychic
complex partial seizureimpaired consciousness


Question Answer
absence seizure (petit mal)3 Hz, no postictal confusion, blank stare
myoclonic seizurequick, repetitive jerks
tonic-clonic (gran mal) seizurealternating stiffening and movement
tonic seizurestiffening
atonic seizuredrop seizure; falls to floor


Question Answer
headache that can induce horner's ~syndromecluster headaches
excruciating periorbital pain with lacrimation and rhinorrheacluster
more common in malescluster
treatment for cluster headachesinhaled oxygen, sumatriptan
molecular mediators of migraine headachessubstance P, CGRP, vasoactive peptides
abortive therapy for migrainetriptans (e.g., sumatriptan)
prophylactic therapy for migrainepropranolol, topiramate
unilateral headachescluster and migraines
bilateral headachestension headache
steady paintension headache
short durationcluster headache


Question Answer
peripheral vertigo etiologyinner ear; semicircular canal debris, vestibular nerve infection, meniere's disease
positional testing in peripheral vertigodelayed horizontal nystagmus
central vertigo etiologybrain stem or cerebellar lesion; stroke affecting vestibular nuclei or posterior fossa tumor)
positional testing in central vertigoimmediate nystagmus in any direction; may change directions
meniere's characterized bytinnitus, vertigo and sensorineural hearing loss
pathogenesis of Menieresincreased volume and pressure of endolymph in vestibular apparatus

Adults Brain Tumors

Question Answer
adult tumors mnemonic mcMGM Studios (Metastasis, Glioblastoma, Meningioma, Schwannoma)
pseudopalisadingglioblastoma multifrome
crosses corpus callosumglioblastoma
from arachnoid cellsmeningioma
dural attachmentmeningioma
spindle cells in whorled patternmeningioma
psammoma bodiesmengioma
surface of brainmeningioma
dural attachmentmeningioma
VHL associationhemangioblastoma
can cause polycythemiahemangioblastoma
produces EPOhemangioblastoma
S100 posschwannoma
NF2bilateral schwanoma
frieg egg appearanceoligodendroglioma
chicken wire capillary patternoligodendroglioma
prolactinomapituitary adenoma
most frequent brain tumor in immunosupressedCNS lymphoma
GFAP +GBM, oligodenroglioma

Childhood Brain Tumors

Question Answer
cerebellar tumorsastrocytoma and medulloblastoma
childhood tumors mcAll Men Eat (Astrocytoma, Medulloblastoma, Ependymoma)
posterior fossapilocytic astrocytoma. GFAP +
rosenthal fiberseosinophilic corkscrew fibers in pilocytic astrocytoma
most frequent CNS tumor in immunosuppressedCNS lymphoma
homer-write rosettessolid small blue cells in medulloblastoma
drop metastases to spinal cordmedulloblastoma
compress 4th ventricle and cause hydrocephalusependymoma and medulloblastoma
perivascular pseudorosettesrod shaped basal ciliary bodies near nucleus in ependymoma
only childhood tumor supratentoriumcraniopharyngioma
remnant of rathke pouchcraniopharyngioma
tooth-enamel likecraniopharygioma
Precocious puberty+ParinaudPineal germinoma in the superior colliculi
Paralysis of upward gazeParinaud syndrome. Pineal germinoma in the superior colliculi
small blue cellsmedulloblastoma

Neurocutaneous Disorders

Question Answer
STURGE weberSTURGE (Sporadic, port-wine Stain, Tram track calcifications, Unilateral, Retardation, Glaucoma, GNAQ gene, Epilepsy)
Tuberous sclerosisash leaf spots, angiofibromas, seizures, mental retardation, increased incidency of subependymal astrocytomas, and cardiac rhabdomyoma
NF1 (Von Recklinghausen)cafe au lait, lisch nodules, pheo, optic gliomas. chromosome 17
VHLbilateral renal cell carcinomas, hemangioblastomas, pheos
Osler Weber Renduport wine stain on face. Disease about blood vessel malformations
bilateral renal cell carcinoma and retinal hemangioblastomasVHL
cafe au lait spots, axillary freckling, lisch nodules, scoliosis, pheos and optic tumorsNF1
subependymal tumors, cardiac rhadbomyoma, retinal hamartoma, ash leaf spots and renal angiomyolipomatuberous sclerosis
glaucoma, pheos, port wine stain, mental retardationsturge weber