neuro Flashcards
famililal forms of AD?
ApoE2: decreased risk
ApoE4: increased risk
APP, presenilin1, presenilin2: increased risk of early onset
what make up AD senile plaques?
extracellular beta-amyloid core
synthesized by cleavage of amyloid precursor protein - APP
what make up AD neurofibrillary tangles?
intracellular hyperphosphorylated tau
** # of tangles correlates with degree of dementia
what make up Pick bodies? what dz?
frontotemporal dementia
silver-staining spherical tau aggregates
deficiency of which B vitamins can cause dementia?
B1
B3
B12
Charcot triad of MS?
scanning speech
intention tremor (+ incontinence, INO)
nystagmus
what causes periventricular plaques of MS?
oligodendrocyte loss and reactive gliosis
findings in AIDP/Guillan Barre?
increased CSF protein with normal cell count
increased protein may cause papilledema
acute disseminated encephalomyelitis?
multifocal periventricular inflammation and demyelination after infection or vaccinations
** measles, VZV; rabies/smallpox vaccines
scoliosis and foot deformities (high/flat arches)?
Charcot Marie Tooth
AD
defective production of proteins involved in structure/fn of peripheral nn or myelin sheath
peripheral neuropathy, developmental delay, optic atrophy, globoid cells?
Krabbe disease
AR lysosomal storage dz - galactocerebrosidase deficiency
galactocerebroside and psychosine destroy myelin sheath
central and peripheral demyelination with ataxia, dementia?
metachromatic leukodystrophy
AR lysosomal storage dz - arylsulfatase A deficiency
sulfatide buildup –> impaired production and increased destruction of myelin sheath
CNS demyelination in AIDS patients?
PML
due to oligodendrocyte destruction
** increased risk with natalizumab, rituximab
adrenoleukodystrophy?
X-linked dz of VLCFA metabolism
–> buildup in nervous system, adrenals, testes
tx of cluster H/A?
100% O2
triptans
tx of tension H/A?
analgesics
NSAIDs
acetaminophen
chronic: amitryptiline
pathogenesis of pain in migraines?
CNV/meninges/blood vessel irritation
2/2 release of substance P, calcitonin gene-related peptide, vasoactive peptide
port wine stain on face, intellectual disability, glaucoma, epilepsy?
Sturge Weber
non-inherited activating mutation of GNAQ gene –> anomaly of neural crest derivatives
ipsilateral leptomeningeal angioma –> sz
episcleral hemangioma –> glaucoma
hamartomas, angiofibromas, ash-leaf spots?
tuberous sclerosis
AD
- cardiac findings: MR, rhabdomyoma
- increased incidence of subependymal astrocytomas, ungual fibromas
VHL?
hemangioblastomas
angiomatosis
B/L renal cell carcinomas
pheos
butterfly glioma, cerebral hemispheres, highly malignant?
GBM
astrocytes GFAP +
histo: pseudopalisading pleomorphic cells bordering necrosis/hemorrhage
tumor in convexities of hemispheres or parasagittal regions?
meningioma
arises from arachnoid cells - extra dural, may have tail
tx: resection/radiosurgery
** histo: spindle cells in whorls, PSAMMOMA BODIES
hemangioblastoma?
usually cerebellar
if with retinal angioma: think VHL
can produce EPO –> 2ary polycythemia
** histo: close thin-walled capillaries, minimal parenchyma
S100+ tumor at cerebellopontine angle?
Schwannoma
usually CNVIII
tx: resect or radiosurgery
** B/L vestibular schwannomas = NF2
frontal lobe tumor, chicken-wire capillaries, fried-egg cytology?
oligodendroglioma
often calcified
rare, slow-growing
child with posterior fossa tumor, well-circumscribed?
pilocytic astrocytoma GFAP + histo: eosinophilic corkscrew fibers = Rosenthal fibers gross: cystic and solid benign, good prognosis
child with cerebellar tumor, compresses 4th ventricle?
medulloblastoma
highly malignant
primitive neuroectodermal tumor
histo: Homer-Wright rosettes, small blue cells
** can send “drop metastases” to spinal cord
child with 4th ventricle tumor, +/- hydrocephalus?
ependymoma
poor prognosis
histo: perivascular rosettes, rod-shaped belpharoplasts near nucleus
bitemporal hemianopsia in adult vs child?
adult: pituitary adenoma
child: craniopharyngioma
herniation that compresses ACA?
subfalcine (cingulate under falx)
herniation that causes Duret hemorrhages?
transtentorial/downward herniation (central)
rupture of paramedian basilar artery branches
herniation that causes blown pupil?
uncal herniation
medial temporal lobe –> ipsilateral CN III and PCA
also –> contralateral Kernohan notch compression –> ipsilateral paresis
herniation that causes coma/death?
cerebellar tonsillar herniation into foramen magnum
