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NEUROLOGY

A patient with head trauma is purposely hyperventilated to produce respiratory alkalosis, which
causes cerebral vessel constriction. This decreases the risk of increased vessel permeability and
cerebral edema. Respiratory acidosis and hypoxemia cause vasodilation of cerebral vessels, which
increases cerebral vessel permeability, resulting in cerebral edema. Both conditions cause increased
activity of the K+ channels in smooth muscle cells produces hyperpolarization relaxes smooth
muscle cells (intracellular calcium) producing vasodilation with increased vessel permeability.
Cerebral edema:
intracellular and
extracellular types
Intracellular: serum
Na+ (SIADH);
dysfunctional Na+/
K+-ATPase pump (global
hypoxia)(water moves into cells)(CytotoxiC
Extracellular: vessel
permeability; meningitis,
metastasis(Vasogenic)(due to inflammation etc)

Pathology, such as infarction or neoplasm, that disrupts the endothelial cell tight junctions
will result in vasogenic edema
Respiratory alkalosis:
cerebral vessel
Vasoconstriction cos less Co2
Respiratory acidosis,
hypoxemia: cerebral
vessel permeability;
cerebral edema(Co2 control vasodilation in brain)
Intracranial
hypertension:
papilledema,
bradycardia, projectile
vomiting, hypertension
The bodys response to a decrease in CPP is to raise blood pressure and dilate blood vessels in the brain.
This results in increased cerebral blood volume, which increases ICP, lowering CPP further and causing a
vicious cycle.
Once the ICP approaches the level of MAP, it becomes more and more difficult to squeeze blood into the
intracranial space.
Increases in intracranial pressure (ICP) compress the brain within the rigid skull. This reduces cerebral blood
flow prompting reflex hypertension to maintain cerebral perfusionCushing reflex:
The increased blood pressure(due to decrease CPP) also stimulates the baroreceptors (pressure sensitive
receptors) in the carotids, leading to an activation of the parasympathetic nervous system, which slows
down the heart rate, causing the bradycardia.(also sending ach to heart and GI(in Gi activating acid leading
to cushing ulcer)
This result in Cushing's Triad: hypertension, bradycardia and irregular respirations
Pseudotumor cerebri:

intracranial pressure
without evidence of
tumor/obstruction (Treatment weight loss)
Pseudotumor cerebri:
MC young obese
Women
Pseudotumor cerebri:
CSF reabsorption in
arachnoid villi
Pseudotumor cerebri:
headache, blurry vision,
blindness, diplopia
Subfalcine herniation:
compresses ACA
Uncal herniation:
compression of CN III,
PCA, parasympathetic
Fibers
Tonsillar herniation:
coning of cerebellar
tonsils; cardiorespiratory
arrest
Uncal herniation: eye
deviated down and out;
mydriasis
Communicating
hydrocephalus:
production of CSF;
reabsorption of CSF
Noncommunicating
hydrocephalus:
obstruction CSF flow
out of ventricles
Hydrocephalus in
children: ventricles
dilate and increase head
circumference(cos their fontanelles havent fused yet
Hydrocephalus ex
vacuo: dilated ventricles
secondary to brain
atrophy
Normal pressure
hydrocephalus: dilated
ventricles + triad
dementia, urinary
incontinence, widebased
gait
Normal pressure
hydrocephalus:
potentially reversible
cause of dementia with

shunting
Wide-based gait and
urinary incontinence:
from stretching of sacral
motor fibers
Dementia: from
stretching of limbic
fibers
Neural tube defects:
lateral folds of neural
plate fail to fuse; AFP
Anencephaly: absence
of brain; maternal
polyhydramnios(cos no brain to initiate swallowing of amniotic fluid)
Dandy-Walker: partial/
complete absence of
cerebellar vermis; cystic
dilation of 4th ventricle;
hydrocephalus
Syringomyelia: cervical
cord enlargment;
fluid-filled cavity
Syringomyelia: pain/
temperature sensation
in hands; atrophy of
intrinsic muscles of
hand (due to fucked up spino thalamic tract
Syringomyelia: MRI
shows cervical
enlargement and cavity
NF: AD; incomplete
Penetrance
Both types: caf au lait
macules; neurofibromas
Contrecoup injuries:
opposite site of impact;
tips of frontal/temporal
lobes
Coup injuries: site of
impact
SWS: vascular
malformation on face;
ipsilateral arteriovenous
malformation in
meninges in some
patients
Rhabdomyoma heart:
highly predictive of
tuberous sclerosis
Hamartomas:
subependymal
proliferations,
angiomyolipomas in

kidneys, rhabdomyoma
Tuberous sclerosis: AD;
mental retardation
Key findings: seizures,
mental retardation,
angiofibromas, ash leaf
spots
NF2: bilateral acoustic
neuromas; juvenile
cataracts; meningiomas
NF1 tumor associations:
pheochromocytoma;
Wilms tumor; CML
(juvenile)
NF1: optic gliomas;
Lisch nodules; axillary/
inguinal freckling
NF1 tumor associations:
pheochromocytoma;
Wilms tumor; CML
(juvenile
NF2: bilateral acoustic
neuromas; juvenile
cataracts; meningiomas
NF2: bilateral acoustic
neuromas; juvenile
cataracts; meningiomas
Tuberous sclerosis: AD;
mental retardation
Key findings: seizures,
mental retardation,
angiofibromas, ash leaf
spots
Hamartomas:
subependymal
proliferations,
angiomyolipomas in
kidneys, rhabdomyoma
Rhabdomyoma heart:
highly predictive of
tuberous sclerosis
SWS: vascular
malformation on face;
ipsilateral arteriovenous
malformation in
meninges in some
patients
Contrecoup injuries:
opposite site of impact;
tips of frontal/temporal
lobes
Subdural hematoma:
most often caused by

trauma; risk with


cerebral atrophy
Risk factors: elderly,
alcoholics with brain
atrophy
Global hypoxic injury:
hypotensive episodes;
chronic CO poisoning
Hypoglycemia: similar
effect on brain as global
hypoxia
Red neurons: apoptotic
Neuron
Atherosclerotic stroke:
MC in MCA distribution;
other sites-internal
carotid artery near
bifurcation, basilar
artery
Atherosclerotic stroke:
loss of demarcation
between gray and white
mater
Atherosclerotic stroke:
repair by gliosis
(astrocytes, microglial
cells)
Vertebrobasilar artery
stroke: vertigo/ataxia,
ipsilateral sensory loss
in face, contralateral
hemiparesis/sensory loss
in trunks/limbs
Embolic stroke:
reperfusion occurs,
unlike atherosclerotic
stroke (pale)
Intracerebral
hemorrhage: basal
ganglia MC location
Berry aneurysms:
junctions lack internal
elastic lamina and
smooth muscle
Lacunar strokes: hyaline
arteriolosclerosis due to
hypertension/diabetes
CNS infections: most are
due to sepsis

Bacterial meningitis:
majority of organisms
originate in
nasopharynx
Encephalitis:
inflammation of brain;
headache; drowsiness;
coma
Cerebral abscess:
hematogenous;
contiguous spread
MS: CD4 TH1/TH17 cells
react against self myelin
antigens
MS: CD4 TH1 cells
secrete -IFN
activates
macrophages release
TNF-
MS: TH17 cells release
cytokines recruit
neutrophils/monocytes
leukocytes/TNF-
attack myelin sheath/
oligodendrocytes
demyelination
MS: antibodies
produced by
autoreactive B cell
damage myelin sheath oligodendrocytes
Demyelinating plaques:
white matter looks like
gray matter
Autonomic dysfunction:
urge incontinence;
sexual dysfunction;
bowel motility
dysfunction
SIN: scanning speech,
intention tremor,
nystagmus
Bilateral INO:
pathognomonic for MS;
demyelinated MLF
Adrenoleukodystrophy:
XR; peroxisomal enzyme
deficiency in -oxidation
of FAs
Metachromatic
leukodystrophy: LSD;
arylsulfatase A

Metachromatic leukodystrophy
a. Autosomal recessive disorder
Lysosomal storage disease (LSD)
b. Deficiency of arylsulfatase A
Results in accumulation of sulfatides
Krabbes disease: LSD;
-galactocerebrosidase
with
-galactocerebroside
in lysosomes
AD: phosphorylated
A neurotoxic
-Secretases cleave APP
to harmless fragments
Insulin degrading
enzyme: involved in
clearance of A;
decreased in AD
Activated GSK:
hyperphosphorylates tau
protein

Pivotal role of activated glycogen synthase kinase-3 (GSK) in neurotoxicity


of A
Activated GSK-3:
phosphorylates A
PIN1 enzyme:
dephosphorylates
hyperphosphorylated
tau protein; deficient in
some cases of AD

Pin 1 enzyme (prolyl isomerase) normally strips excess phosphate molecules from
NF, restoring it to its original shape; however, in some cases of AD, this enzyme is
absent or dysfunctional.
Amyloid angiopathy:
risk for cerebral
hemorrhage(cos it deposits in vessels and causes their weakening)
Parkinsonism: CO, drugs,
Wilson disease,
encephalitis, MPTP,
ischemia
HD: chorea; oculomotor
abnormalities;
parkinsonism
Friedreich ataxia:
hypertrophic
cardiomyopathy; type 1
DM

ALS: mutated SOD 1 or


misfolded SOD 1

due to mutated superoxide dismutase (SOD) 1 (neuron destruction by


superoxide free radicals) or misfolded SOD 1 leading to apoptosis of neurons (most
likely mechanism)
ALS: no sensory
changes; bowel/bladder
function intact
Werdnig-Hoffmann
disease: LMN disease in
children
Wilson disease: cystic
degeneration of basal
ganglia; serum free
copper, serum
ceruloplasmin
AIP: urine colorless
when first voided;
exposure to light
produces color (window
sill test)

porphobilinogen (PBG)
Urine is colorless when first voided.
(a) Exposure to light oxidizes PBG to porphobilin producing port-wine color.
(b) Classic window sill test
AIP: uroporphyrinogen
synthase; bellyful of
scars; peripheral
neuropathy; dementia
Rx AlP: carbohydrate
loading inhibits ALA
synthase
Childhood tumors: cystic
astrocytoma/
medulloblastoma, both
in cerebellum
Clinical: headache,
seizures, intracranial
hypertension
GBM: grade IV
astrocytoma; often
crosses corpus callosum;
hemorrhagic/cystic
Meningioma: MC
benign brain tumor in
adults
Meningioma: derived

from meningothelial cell


of arachnoid membrane;
parasagittal location
Meningioma:
association with NF-2,
history of radiation
Meningioma: female
predominance;
psammoma bodies
Oligodendroglioma:
frontal lobe
calcifications in adult
Primary CNS lymphoma:
occurs in AIDS;
EBV-mediated cancer
Metastasis MC brain
malignancy; lung MC
primary site

Charcot-Marie-Tooth (CMT) disease


CMT: MC hereditary
Neuropathy
CMT: lower legs have
inverted bottle
appearance

Causes atrophy of muscles of the lower legs


GBS causes ascending
Paralysis
GBM lab: CSF protein,
oligoclonal bands;
normal CSF glucose/cell
count
Rx GBS: IV
immunoglobulin or
plasma exchange
DM: MCC of peripheral
neuropathy; osmotic
damage to Schwann
cells
LMN Bell palsy:
ipsilateral weakness
upper/lower face

UMN Bell palsy:


contralateral weakness

lower face; sparing


upper face
Drugs: vincristine,
hydralazine, phenytoin
Vitamin deficiencies:
thiamine, pyridoxine,
vitamin B12
Acoustic neuroma:
tinnitus, sensorineural
hearing loss