central nervous system Flashcards
holoprosencephaly is due to a defect in-
SHH signaling
flow of CSF
choroid plexus lateral ventricles interventricular foramen of monro 3rd ventricle cerebral aquduct 4th ventricle subarachnoid space (Magendie and Luschka)
most common cause of hydrocephalus in newborns
cerebral aqueduct stenosis
dandy-walker malformation
failure of cerebellar vermis to form, leads to massive dilation of the 4th ventricle
AC type I
low lying cerebellar tonsils- later in life with HA, dizziness
AC type II
congenital herniation of tonsils and vermis, presents in infancy with hydrocephalus
AC type I can lead to development of
syringomyelia
syringomyelia presentation
loss of pain and temp sensation along the arms bilaterally (shaw sign)
expansion of syrinx can lead to 2 complications
UE weakness (anterior motor horn) Horner syndrome (lateral horn)
early sign of ALS
weakness and atrophy of hands
ALS is differentiated from syringomyelia in that it lacks
sensory sx
familial ALS is due to
mutation in SOD-1 that leads to increased free radical damage
degeneration of ___ leads to ataxia in Friederich’s ataxia
spinocerebellar tracts
inheritance/gene/problem of Friederich’s ataxia
AR
GAA repeat frataxin gene
impaired mitochondrial iron regulation leads to increased free radicals via Fenton reaction
classic triad of Friederich’s ataxia
ataxia, cardiomyopathy, kyphoscoliosis
brown-sequard presentation
hemisection of the cord
ipsilateral: PT loss at level, UMN signs and loss of vibe/light touch below
contralateral: loss of PT starting 2-3 segments below injury
tabes dorsalis symptoms (3)
impaired vibratory sense (destruction of dorsal columns), arygll-robinson pupils (accommodate but don’t react), +rhomberg
fungal meningitis CSF
lymphocytes, low glucose
areas of the brain most susceptible to ischemia (4)
watershed areas
pyramidal neurons in layers 3/5/6 cortex
pyramidal neurons in hippocampus
Purkinje cells of the cerebellum
thromboembolic stroke- m/c source, type of infarct
m/c source- plaque in carotid artery
pale infarct
embolic stroke- m/c source, type of infarct
m/c source- atrial fibrillation
hemorrhagic infarct
lacunar stroke- pathophysiology
hyaline arteriolosclerosis of the lenticulostriate branches of MCA secondary to chronic hypertension
histologic changes after ischemia- day 0
12 hours- red neurons (eosinophilic, pyknosis, cell shrinking)
histologic changes after ischemia- day 1
24 hours- liquefactive necrosis
1-3 days- neutrophils
4-7 days- macrophages (microglia)
histologic changes after ischemia- 1 week
granulation-like tissue
histologic changes after ischemia- 1 month
reactive astrocytes yield gliosis
hemorrhagic strokes are also called
intraparenchymal hemorrhages
m/c cause of intraparenchymal hemorrhages
rupture of charcot-bouchard aneurysms
charcot-bouchard aneurysm pathophysiology
develop secondary to hyaline arteriolosclerosis, complication of long-standing HTN
presentation of intraparenchymal hemorrhage
focal neurologic signs, headache
presentation of SAH
sudden, severe HA
nuchal rigidity
m/c cause of SAH
rupture of berry aneurysm
common sites of berry aneurysm
bifurcations- ACA and anterior communicating artery (due to lack of intima media layer)
conditions associated with increased risk of berry aneurysms
ADPKD
EDS