Neuro- Pathology Flashcards
What genes are associated with early onset Alz?
APP, PS-1, PS-2
What gene is associated with late onset Alz and which gene is protective?
ApoE4- late onset
ApoE2- protective
Compare senile plaques and neurofibrillary tangles?
Senile plaque- extraceulluar b amyloid core; amyloid b from cleaving APP
Neurofibrillary tanges- intracellular hyperphosphorylated tau protein
Presents as dementia, aphasia, parkinsonian aspects, change in personality
Pick disease (frontotemporal dementia)
Describe the microscopic and gross findings of Pick disease?
Pick bodies- spherical tau protein aggregates frontotemporal atrophy (spares parietal lobe and post 2/3 of superior temporal gyrus)
Presents as rapid progressive dementia with myoclonus
CJD
What are 2 important diagnostic findings in MS?
oligoclonal IgG bands in CSF
periventricular plaques
Presents as scanning speech, intention tremor, nystagums
Charcot triad of MS (SIN)
what are the treatments of MS?
b-interferon, immunosuppression, natalizumab
What 2 infections are commonly associated with Acute inflammatory demylinating polyradiculopathy?
Campy, CMV
Describe adrenuoleukodystrophy
X-linked
impaired metabolism of VLCFA- build up in nervous system, adrenal, testes
What viral infection is at increased risk with natalizumab?
PML (JC virus)
What cell type is affected in PML?
oligodendrocytes
What gene is defective in metachromatic leukodystrophy and what builds up?
arylsulfatase A, AR
buildup of sulfatides (impaired production of myelin sheath)
Genetic disorder presents as central and peripheral demyelination with ataxia, dementia
Metachromatic leukodystrophy
What genetic defect presents with scoliosis and foot deformities?
Charcot-Marie-Tooth disease
What is the inheritance of Charcot-Marie Tooth? What genes are affected?
Aut Dom
proteins involved in structure and function of peripheral nerves or myelin sheath
What is status epilepticus?
continuous seizure for >30 min or no gain of consciousness between seizures for >30 min
What area of the brain do partial/focal seizures most commonly originate?
Medial temporal lobe
Compare partial vs complex partial seizure
simple- motor, sensory, autonomic, psychic; no loss of consciousness
complex- loss of consciousness
What EEG waves are seen in abscence seizures?
3hz
Describe myoclonic, tonic-clonic, tonic, and atonic seizures
myoclonic- quick, repetitive jerks
tonic-clonic- alternating stiffening and movement
tonic- stiffening
atonic- drop seizure (often mistaken for fainting)
How does positional testing distinguish peripheral vs central vertigo?
Peripheral- delayed horizontal nystagmus
central- immediated nystagmus in any direction
What brain tumor is found in cerebral hemispheres and can cross corpus callosum
glioblastoma
What brain tumor presents with spindle cells concentrically arranged in a whorled pattern and psammoma bodies?
Meningioma
Brain tumor associated with chicken-wire capillary pattern and fried egg cells, often calcified
oligodendroglioma
Brain tumor often found at cerebellopontine angle, S-100+
shchawnnoma
What brain tumor can produce EPO?
hemangioblastoma
Brain tumor associated with von hippel lindau and often cerebellar
hemangioblastoma
Brain tmor assocated with closely arranged, thin-walled capillaries with minimal interleaving parenchyma
hemangioblastoma
Compare the common locations of children vs adult brain tumors?
Children- mostly infratentorial, adult mostly supratentorial
Brain tumor presents with Homer-Wright rosettes, small blue cells
medulloblastoma
Brain tumor shows perivascular rosettes, rod-shaped blpharoplasts
ependymoma
Brain tumor shows rosenthal fibers
pilocytic astrocytoma
Where are pilocytic astrocytomas typicall found
posterior fossa (eg cerebellum)
Where are ependymomas typically found?
4th ventricle
What is the most common childhood supratentorial tumor
craniopharyngioma
What type of herniation can compress the ACA?
cingulate herniation under falx cerebri
What type of herniation can compress the brain stem and result in coma and death
cerebellar tonsillar herniation
What can present as CNII lesion, contralateral homonymous hemianopsia, ipsilateral paralysis?
Uncal herniation
