CNS Flashcards
drugs that inc the risk of JC virus infection?
natalizumab (targets alpha 4 integrin), rituximab
mech of PML (progressive multifocal leukoencephalopathy)?
- demyelination of CNS due to destruction of oligodendrocytes
- associated with JC virus
- rapidly progressive
another name for forebrain?
prosencephalon
another name for midbrain?
mesencephalon
another name for hindbrain?
rhombencephalon
forebrain (prosencephalon) gives rise to
- telencephalon
2. diencephalon
midbrain (mesencephalon) gives rise to
midbrain
midbrain contains what structure?
aqueduct
hindbrain (rhombencephalon) gives rise to
- metencephalon
2. myelencephalon
metencephalon gives rise to
- pons
2. cerebellum
myelencephalon gives rise to
medulla
notochord induces
overlying ectoderm to diff into neuroectoderm and form neural plate
neural plate gives rise to
- neural tube
2. neural crest cells
notochord becomes
nucleus pulposus
alar plate is
dorsal and is sensory
basal plate is
ventral and is motor
what structures belong to neuroectoderm?
- CNS neurons,
2. ependymal cells (inner lining of ventricles)
what makes CSF?
ependymal cells
2 structures from neural crest?
- PNS neurons
2. Schwann cells
structure from mesoderm?
microglia
benign childhood tumor associated with “tooth enamel like appearance?”
Craniopharyngioma
characteristics of craniopharyngioma?
- may be confused with “pituitary adenoma”
- bitemporal hemianopia
- most common childhood supratentorial tumor
- from Rathke pouch (Adenohypophysis)
- calcification common
the only supratentorial childhood tumor?
craniopharyngioma
what path is associated with hyperphosphorylated tau protein?
Alzheimer
what path is associated with silver staining spherical tau protein?
Frontotemporal dementia (Pick’s dz)
what path is associated with alpha synuclein defect?
Lewy body dementia
is ACh inc or dec in Alzheimer?
dec
what is the cause for communicating hydrocephalus?
dec CSF absorption by arachnoid granulations
what 2 structures make CSF?
- ependymal cells
2. choroid plexus
what path is associated with arachnoid scarring post-meningitis?
communicating hydrocephalus
what is normal pressure hydrocephalus?
- affects elderly
- CSF pressue elevated only episodically
- no increased subarachnoid space volume
- expansion of ventricles –> distorts fibers of corona radiata
what is the mech of clinical symptoms in normal pressure hydrocephalus?
expansion of ventricles –> distorts fibers of corona radiata leading to urinary incontinence, ataxia, cognitive dysfunction
mech of ex vacuo
appearance of inc CSF due to dec brain tissue seen in Alzhemier dz, advanced HIV, Pick
is ICP inc or dec in communicating hydrocephalus?
inc
is ICP inc or dec in ex vacuo ventriculomegaly?
normal ICP
what is Charcot-Marie-Tooth dz?
known as hereditary motor and sensory neuropathy, groups of progressive hereditary nerve disorders related to the defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath
what path is associated with scoliosis and foot deformities (high or flat arches)?
Charcot-Marie-Tooth dz (HMSN = Hereditary Motor and Sensory Neuropathy)
what enzyme is missing in Krabbe?
galactocerebrosidase
what enzyme is missing in Gaucher dz?
glucocerebrosidase
what enzyme is missing in metachromatic leukodystrophy?
arylsulfatase A def
what metabolite is accumulated in metachromatic leukodystrophy?
sulfatides
what path is associated with central and peripheral demyelination with ataxia, dementia?
metachromatic leukodystrophy
what path is associated with disrupt metabolism of very long chain fatty acids?
adrenoleukodystrophy due to def of peroxisomal acyl CoA synthase
what are the symptoms of adrenoleukodystrophy?
excessive FA buildup in nervous system, adrenal gland, testes leading to coma/death and adrenal gland crisis
what is the sudden wild flailing of 1 arm?
hemiballismus (subthalamic nucleus lesion from lacunar stroke due to HTN)
