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USMLE Step 2 - Neurology

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Parkinson's Disease -
What is it
When does it start
Life expectancy
ACh and dopamine must be
in balance for normal,
balanced movement
dec. levels of dopamine
in substantia nigra
idiopathic hypokinetic
usu. starts ~50-60 y/o
life expectancy ~ 9 yrs.
Parkinson's Disease -
Insults that lead to
"parkinsonism"
Most cases are idiopathic

insults dec. dopamine -
postencephalitic
toxic insults -
(carbon disulfide
manganese
MPTP)
bihemispheric ischemic
traumatic
iatrogenic (neuroleptic meds)
Parkinson's Disease -
History/PE
Tremor at rest (pill rolling)
cogwheel rigidity
bradykinesia
festinating gait
stooped posture
unstable posture
masked facies
memory loss
micrographia

shy-dragger-
any autonomic dysfunction
Parkinson's Disease -
Tx
1st -
is it from a secondary cause
that can be reversed?

then -
how does pt. function daily?
tremor but functioning -
> 60 y/o - amantidine
< 60 y/o - anticholinergic

if can't function -
l-dopa-carbidopa
most effective
but most side effects
"response fluctuations"
Tx fluctuations -
COMT or selegiline


list of meds -
L-dopa/carbidopa - mainstay
bromocriptine
selegiline
entacapone (COMT inhib)
amantadine & anticholinerg -
limited efficacy

If meds fail & dis. advanced-
deep brain stimulation of
GPi and subthal nucleus,
or pallidotomy -
ablation of GPi
(posteroventral GP)
rarely done now
Huntington's -
What is it
Hyperkinetic
AD
atrophy of caudate
lose GABA
CAG triple repeats on C4p
anticipation -
prog. expansion
> 39 repeats - mutant genes
Huntington's -
History/PE
Presents ~30-50 y/o
gradual onset of -
chorea
dementia
altered behavior
depression
Huntington's -
Dx
Clinical
CT
MRI
molecular genetic testing
Huntington's -
Tx
No cure
disease cannot be halted
genetic counseling
haloperidol - psychosis
reserpine -
minimize unwanted movements
Alzheimer's -
What is it
MCC of dementia in elderly

⬢ senile plaques - B amyloid
⬢ neurofibrillary tangles -
abnorm phosphorylated tau protein
⬢ loss of ACh in cortex -
basal nucleus of Meynert
⬢ amyloid angiopathy
=> lg lobar hemorrhage
⬢ hippocampus affected early
Alzheimer's -
Risk factors
Age - most important
family history
Down's (> 35 y/o)
female gender

chromosomes 21, 14
chromosome 1 - younger age
chromosome 19 - older
homozygous for ApoE4 -
high rate of Alzheimer's
Alzheimer's -
History/PE
Amnesia usually 1st sign
then language deficits
acalculia
depression
agitation
apraxia
Alzheimer's -
Evaluation
Dx of exclusion
def. Dx only on autopsy
MRI or CT -
diffuse cortical
and subcortical atrophy

Neuropsych testing -
distinguish between
dementia and depression
Alzheimer's -
Tx
Supportive therapy
Cholinesterase inhibitors -
first-line therapy
donepezil
rivastigmine
galantamine
Vit E (a-tocopherol) -
may slow cognitive decline
Alzheimer's -
Complications
Survival is 5-10 yrs from
onset of sxs
death usually secondary to
aspiration pneumonia or
other infections
Delirium -
What is it caused by
Timing of onset
Course
Caused by acute illness,
infection or drug toxicity
Sudden onset
Reversible (usually)

impairment of consciousness -
dec. awareness of your envi
can't maintain attention
Dementia -
What is it caused by
Timing of onset
Course
Caused by anatomic changes
in the brain
Slow, gradual onset
Irreversible
Subarachnoid Hemorrhage -
What is it
Due to bleeding
from ruptured aneurysm
MCC - head trauma
(Circle of Willis)
(considered separate d/o)
spontan - ruptured aneurysm
usually congenital berry
associated with APKD,
coarctation of aorta
other cause - AV malformation
~50-60 y/o
high mortality
Subarachnoid Hemorrhage -
History/PE
Sudden-onset headache
"worst headache of my life"
loss of consciousness
fever
n/v
neck stiffness
seizure
CN3 palsy - if berry aneurysm
may be preceded by
milder sentinel headaches
weeks earlier
Subarachnoid Hemorrhage -
Dx
CT without contrast immed.
blood appears white
if CT neg -
LP immediately
ck for xanthochromia
no LP if inc. ICP -
sudden dec. in CSF pressure
can cause further bleeding
4-vessel angiography
to pinpoint location
Subarachnoid Hemorrhage -
Tx
Prevent 2nd rupture -
most likely in 1st 48 hrs
obliterate aneurysm
IV fluids
keep BP OK
nimodipine -prevent vasospasm
phenytoin
lower ICP -
raise head of bed,
hyperventilation
pain meds - no NSAIDs

surgery -
clip aneurysm
IR (stent-assisted) coiling
Subarachnoid Hemorrhage -
Complications
2nd rupture -
esp. with aneurysm
extend into brain parenchyma-
esp. with AVM
arterial vasospasm
obstructive hydrocephalus
Epidural Hematoma -
What is it
Often due to blunt trauma
usually lateral skull fracture
tear of Middle Meningeal Art.
Epidural Hematoma -
History/PE
Lucid interval - min. to hrs.
then headache
progressive obtundation
hemiparesis
"blown pupil"
Epidural Hematoma -
Dx
CT -
lens-shaped
convex hyperdensity
close observation and
serial neuro exams
before surgery
Epidural Hematoma -
Tx
Neurosurgical evacuation
Subdural Hematoma -
What is it
Usually after head trauma
rupture of bridging veins
cortex to dural sinuses
esp. elderly & alcoholics
Subdural Hematoma -
History/PE
Headache
change in mental status -
days to weeks
can present as dementia
in elderly
contralateral hemiparesis
may have remote h/o fall
Subdural Hematoma -
Dx
CT -
crescent-shaped
concave hyperdensity
Subdural Hematoma -
Tx
Neurosurgical evacuation
blood may regress spontan-
eously if chronic
corticosteroids
phenytoin
Parenchymal Hemorrhage -
Causes
HTN (usually in basal ganglia)
tumor
amyloid angiopathy - elderly
vascular malformations -
AVM
cavernous hemangiomas
Parenchymal Hemorrhage -
History/PE
lethargy
headache
focal mtr & sensory deficits
some obtundation
Parenchymal Hemorrhage -
Dx
CT without contrast
check for mass effect
or edema
Parenchymal Hemorrhage -
Tx
Raise head of bed
antiseizure prophylaxis
neurosurgical evacuation -
if mass effect
esp. in posterior fossa
Coma -
What is Rule of 4
1. Thiamine, D50, naloxone
coma cocktail

2. 2 mechanisms
bilateral hemisphere or
RAS

3. 3 general dis. processes
structural
metabolism
seizures

4. 4 key aspects to exam
Coma -
General Processes: Structural
Hematoma
Infarction
Abscess
Tumor
Abnormal imaging lesion
that occupies space
Coma -
General Processes: Metabolism
Electrolyte, endocrine or
metabolic functions
substrate deprivation -
O2, glucose
vitamin deficiency
organ failure - kidney
toxins -
meds
etoh
drugs
infections
inflammatory dis.
Normal imaging
Coma -
General Processes: Seizures
Status epilepticus
postictal
Coma -
4 aspects of exam
Pupils
eye movement
motor response
ventilation pattern -
acid-base status
Coma -
Tx
Stabilize the patient -
Airway
Breathing
Circulation
Reverse the reversible -
coma cocktail
O2
ID and tx underlying cause
Prevent further damage
Broca's Aphasia -
What is it
What part of brain affected
D/o of language production
motor
broken speech
comprehension is intact
expressive or
nonfluent aphasia
post. inferior frontal gyrus
Broca's Aphasia -
Features
Repetition is impaired
frustration - cuz aware
associated with -
arm and face hemiparesis
hemisensory loss
apraxis of oral muscles
often secondary to
left superior MCA stroke
Broca's Aphasia -
Tx
Speech therapy
tx underlying condition
wide range of outcomes
intermediate prognosis
Wernicke's Aphasia -
What is it
D/o of language comprehension
nonsensical production
Wernicke's is wordy
left post. superior temporal
receptive or fluent aphasia
Wernicke's Aphasia -
Features
Neologisms
word substitutions
unaware - no comprehension
secondary to
left inf./post MCA stroke
Wernicke's Aphasia -
Tx
Speech therapy
tx underlying condition
poorer prognosis than Broca's
Brain Neoplasms -
Mets vs. Primary
Benign usually > 65 y/o

Metas -
Lots of Bad Stuff Kills Glia
Lung Breast Skin Kidney GI
metas > primary
supratentorial
at junction of gray and
white matter

Primary (1o) -
MC primary in adults -
glioblastoma and meningioma
most 1o are supratentorial

MC primary in kids -
medulloblastoma & astrocytoma
most 1o are infratentorial
Brain Neoplasms -
Dx
CT with contrast
MRI with gadolinium
CT-guided Bx
Bx during surgical tumor
debulking
Brain Neoplasms -
General Principles of Tx
Resection (if possible)
radiation
chemo
palliative tx
corticosteroids -
reduce vasogenic edema
type of therapy depends on -
type of tumor
histology
progression
site
Brain Neoplasms:
Glioblastoma -
Characteristics and
Presentation
Grade IV astrocytoma
MC primary brain tumor
prognosis grave
< 1 year to live
can cross corpus callosum
progresses fast

headache
ICP
Brain Neoplasms:
Glioblastoma -
Tx
Surgical resection
radiation and chemo
have variable results
Brain Neoplasms:
Meningioma -
Characteristics and
Presentation
2nd MC primary
often incites osteoblastic
reaction in overlying
cranial bones
orig from dura or arachnoid
good prognosis
incidence inc. with age
Brain Neoplasms:
Meningioma -
Tx
Surgical resection
radiation for unresectable
Brain Neoplasms:
Acoustic Neuroma (Schwannoma)
Characteristics and
Presentation
Derived from schwann cells
bilat schwannoma in NF2

Ipsilateral hearing loss
tinnitus
vertigo
signs of cerebellar dysfunction
facial weakness
facial sensory loss
Brain Neoplasms:
Acoustic Neuroma (Schwannoma)
Tx
Surgical removal
Brain Neoplasms:
Astrocytoma -
Characteristics and
Presentation
Grades 1 - pilocytic,
mainly kids
grade 2 - diffuse
grade 3 - anaplastic
grade 4 - glioblastoma multif

headache
inc. ICP
can cause unilat paralysis in
CN 5-7 & CN10
Brain Neoplasms:
Astrocytoma -
Tx
Resection if possible
radiation
Brain Neoplasms:
Medulloblastoma -
Characteristics and
Presentation
Highly malignant
arises from cerebellum
and 4th ventricle
can compress 4th ventricle
may seed subarachnoid space
inc. ICP
morning headaches
Brain Neoplasms:
Medulloblastoma -
Tx
Surgical resection
coupled with
radiation and chemo
Brain Neoplasms:
Ependymoma -
Characteristics and
Presentation
Common in kids
arises from ventricles
and spinal cord
commonly found in 4th ventricle
can cause hydrocephalus
Brain Neoplasms:
Ependymoma -
Tx
Surgical resection
radiation
Neurofibromatoses -
What are they
NF1 -
von Recklinghausen
AD
chromosome 17

NF2
chromosome 22
Neurofibromatoses -
Hx/PE
NF1 -
2 or more of:
neurofibromas (2)
cafe-au-lait spots (6)
freckling -
axillary or inguinal
optic glioma
lisch nodules (2)
osseous abnorm
1st degree relative with NF1

NF2 -
bilat acoustic neuromas
or
1st deg. relative with NF2
and unilat acoustic neuroma
or
1st deg. relative with NF2
and neurofibroma,
meningioma,
glioma
or schwannoma
Neurofibromatoses -
Dx
MRI -
brain
brain stem
spine
derm exam
ophthal exam
family Hx
hearing test
Neurofibromatoses -
Tx
No cure
tx symptoms
surgical removal for
acoustic neuromas
Tuberous Sclerosis -
What is it
Seizures - start as infant
mental retardation
skin and eye lesions
small benign tumors -
brain
face
eyes
kidney
other organs
very variable clinical course
AD
chromosome 9
Tuberous Sclerosis -
Hx/PE
Infantile spasms
ashleaf lesions -
hypopigmentation
trunk and extremities
shagreen patch - lumbosacral
sebac. adenoma - nose, cheeks
mulberry tumors
phakomas
Tuberous Sclerosis -
Dx
CT -
calcified tubers
periventricular areas
can => astrocytomas (rare)
Wood's UV lamp - skin lesions
EKG
renal US
Tuberous Sclerosis -
Tx
Clonazepam or valproic acid

inc. ICP -
may indicate
a tuber obstructing
Foramen of Munro

surgery
Von Hippel-Lindau -
What is it
AD
deletion of VHL gene on ch 3
hemangioblastoma -
cerebellum
medulla

retinal angioma
RCC
pheochromocytoma
Von Hippel-Lindau -
Hx/PE
Headache
N/V
cerebellar Sxs
retinal angiomas -
usu. periphery
can => retinal detachment
Von Hippel-Lindau -
Dx
CT - head, abdo
MRI -
post. fossa emphasis
serpentine signal voids
angiography - vascularity
CBC - polycythemia
VMA levels in urine
ophthal US
Von Hippel-Lindau -
Tx
Surgical resection
or
radiation
Osler-Weber-Rendu -
What is it
AD
also called -
Hereditary Hemorrhagic
Telangiectasia (HHT)
telangiectasia and AVM
in lungs, GI, brain
recurrent epistaxis
Osler-Weber-Rendu -
Hx/PE
Recurrent epistaxis
painless bleeding in bowel
hepatic AV fistula -
(hepatomegaly
RUQ pain
pulsatile mass
palpable thrill
audible bruit
high-output CHF
liver failure)
hep. enceph - portosys shunt
neuro complications
rt-to-left shunt -
(cyanosis
clubbing
hypoxemia
2ndary polycythemia
exertional dyspnea)
Osler-Weber-Rendu -
Tx
Iron
folate
symptomatic tx of epistaxis
ASA - contraindicated
embolization,
surgical excision
or ligation of AV fistulas
Closed-Angle Glaucoma -
What is it
Med emergency
usu older pts. and Asians
iris root plugs opening of
trabecular meshwork
acutely
pupillary dilation
anterior uveitis
dislocation of lens
Closed-Angle Glaucoma -
Hx/PE
Intraocular pressure inc.
very fast
extreme periocular pain
acute red eye
blurred vision
Closed-Angle Glaucoma -
Dx
Tx
Best diagnostic test -
tonometry

Tx -
acetazolamide
pilocarpine - when P drops
laser iridotomy - curative
Open-Angle Glaucoma -
What is it
Most common form
almost always bilateral
risk factors -
> 40 y/o
Black
diabetic
myopic
family Hx
diseased trabecular meshwork
obstructs proper drainage
=> intraoc P inc. gradually
progressive vision loss
vision loss -
moves periph to central
=> blindness
Open-Angle Glaucoma -
Hx/PE
Asymp initially
suspect if pt. -
> 35 y/o
freq. lens changes
mild headaches
vision disturbances
impaired adaptation to dark
earliest defect -
periph nasal fields
cupping of optic disk
Open-Angle Glaucoma -
Dx
Tonometry
ophthalmic exam of optic n.
central field testing
eval on long-term basis
can be hard to Dx until
advanced stages
Open-Angle Glaucoma -
Tx
Prevention
> 40 y/o - exam every 3-5 yrs
inc. risk factor - annually

timolol, betaxolol
pilocarpine
acetazolamide

laser trabeculoplasty -
if meds fail
Macular Degeneration -
What is it
MCC of permanent bilat
vision loss in elderly
vision loss - central
do not lose periph
atrophic - gradual loss
exudative -
faster
damage more severe
Macular Degeneration -
Hx/PE
painless loss of
central vision

atrophic -
irreg pigmentation of
macular region

exudative -
hyperpigmentation
pimple-like elevation of
macula - from hemorrhage
Macular Degeneration -
Tx
Laser photocoagulation -
may delay loss of central
vision in exudative
Retinal Artery Occlusion -
What is it
Hx/PE
From emboli or thrombi
sudden
painless
unilat blindness
pupil accommodates but reacts
sluggishly to direct light
cherry-red spot on fovea
artery may look bloodless
retinal edema
Retinal Artery Occlusion -
Tx
Thrombolysis within
8 hrs of onset of Sx
dec. intraoc P -
drain ant. chamber
IV acetazolamide
Retinal Vein Occlusion -
What is it
Hx/PE
Sudden
painless
retinal hemorrhages
cotton wool spots
edema of fundus
elderly
MCC - HTN
can => macular dis., glaucoma
Retinal Vein Occlusion -
Tx
Laser photocoagulation
Inc. ICP -
Causes
Blood -
EDH, SDH, SAH, ICH
spontaneous or traumatic
CSF -
hydrocephalus
idiopathic intracranial HTN
brain mass -
tumor
trauma -cerebral contusions
edema -
trauma
tumors
Inc. ICP -
Signs in approx. order
of appearance
N/V and headache
altered mental status
in kids - bulging fontanelles
papilledema
CN palsies - esp. CN6
Cushing's Triad -
(HTN
brady
irreg breathing)
endstage -
cerebral herniation
Inc. ICP -
Tx -
in order
Make sure BP and resp. good
sedation
elevate head of bed
IV mannitol
hyperventilate to CO2 30-35
ventriculostomy
surgery -
removal of hematoma
decompressive craniectomy
Herniation -
What is it
Types
Dx
Endpoint of untreated masses
or inc. ICP
specific signs and Sxs
depends on type of hernia
and mass lesion

uncal herniation -
CN3 compression:
ipsilat dilated pupil
midbrain compression:
ipsilat hemiplegia

tonsillar herniation -
resp. compromise

Dx -
CT without contrast -
r/o mass lesion or hemorrhage
Guillain-Barre -
What is it
Acute
rapidly progressive
acq. demyelinating autoimmune
d/o of periph nerves
recent C. jejuni infection
viral infection
recent vaccination
Guillain-Barre -
Hx/PE
Rapidly progressive
ascending paralysis
involves trunk, diaph and CN
autonomic Sxs
areflexia
Guillain-Barre -
Dx
Diffuse demyelination on -
EMG & nerve conduction studies
albuminocytologic dissociation
CSF prot. > 55 mg/dL
Guillain-Barre -
Tx
ICU -
risk of respiratory failure
plasmapheresis or IVIG
aggressive rehab
MS -
What is it
Acq. demyelinating dis.
may have T cell-mediated
autoimmune pathogenesis
environmental and genetic
female-to-male 2:1
20-40 y/o
inc. prev. with gtr distance
from equator
risk - related to where lived
the 1st 15 yrs. of life
subtypes -
benign
relapsing/remitting
2o progressive
chronic progressive
MS -
Hx/PE
Mult. neuro complaints
sep. in time and space
can't be explained by a
single lesion
limb weakness
optic neuritis
paresthesias
diplopia
internuclear ophthalmoplegia
urinary retention
vertigo
Sxs wax & wane or progress

exacerbations -
stress
infections
heat
trauma
vigorous activity
MS -
Dx
Multiple, asymmetric periven-
tricular lesions in white mtr
corpus callosum lesions -
pathognomonic
active lesions enhance with
Gado on MRI
inc. CSF IgG
oligoclonal bands
MS -
Tx
Acute - steroids

treat Sxs -
spasticity: baclofen or zanaflex
pain: phenytoin
fatigue: amantadine or provigil
depression
avoid hot climates

prophylaxis:
immunomodulators
reduce no. of attacks, disability
Avonex - once wkly IM
Betaseron- every other day subq
Copaxone - daily subq
Stroke -
Types
Ischemic - 80%
emboli
thrombi
systemic hypoperfusion

hemorrhagic
Stroke -
Risk Factors
Age
male
race
HTN
diabetes
obesity
smoking
hypercholesterolemia
AF
carotid stenosis
coke
alcohol
IV drug user
Stoke -
Hx/PE
MCA
Aphasia
neglect
gaze preference
homonymous hemianopsia
contralateral hemiparesis -
arm/face > leg
Stoke -
Hx/PE
ACA
Amnesia
personality changes
foot drop
gait dysfunction
cognitive changes
contralateral hemiparesis -
leg > arm/face
Stoke -
Hx/PE
PCA
Homonymous hemianopia
memory deficits
dyslexia/alexia
Stoke -
Hx/PE
Basilar
Coma
CN palsies
apnea
visual Sxs
drop attacks
dysphagia
Stoke -
Hx/PE
Lacunar
Unilat pure motor or sensory
dysarthria -
clumsy hand syndrome
ataxic hemiparesis
Stoke -
Hx/PE
TIA
Transient neuro deficits
last < 24 hours
most last < 1 hour
Stoke -
Dx
CT without contrast -
ischemic vs. hemorrhagic
MRI
EKG
echocardiogram
vascular studies -
carotid US
MRA
transcranial doppler or MRA
screen for hypercoagulability
PE
Stoke -
Tx
Preventive and Long-Term
* Vigilance for signs of -
brain swelling
inc. ICP
herniation
* tPA -
ischemic stroke
if admin within 3 hrs.
of onset of Sxs
* ASA -
ischemic stroke presenting
within 48 hours
* no hypotension, hypoxemia,
hypoglycemia
* tx aspiration pneumonia,
UTI or DVT

Preventive & long-term Tx -
* ASA, clopidogrel or
dipyridamole/ASA
* carotid endarterectomy
* anticoags
* management of HTN, DM and
hypercholesterolemia
Carpal Tunnel Syndrome -
What is it
Risk factors
Median n. compression where
passes thru carpal tunnel
women 30-55

Risk factors -
repetitive use injury
pregnancy
DM
hypothyroidism
acromegaly
RA
obesity
Carpal Tunnel Syndrome -
Hx/PE
Wrist pain
numbness & tingling of 3 1/2
weak grip
dec. thumb opposition
thenar atrophy
nocturnal pain & paresthesias
Sxs may awaken pt. at night
relieved by shaking wrist
Carpal Tunnel Syndrome -
Workup
Tinel's sign -
tap
tingle
Phalen's sign -
ph-lex (flex)
paresthesias
EMG - to confirm
NCV - to confirm
eval for risk factors
Carpal Tunnel Syndrome -
Tx
Neutral wrist splint
modify activity
create ergonomic work envi
NSAIDs
corticosteroids -
direct injection
surgery if Sxs persist -
carpal tunnel release
Myasthenia Gravis -
What is it
Autoimmune
Ab that bind to postsynaptic
Ach receptors (nicotinic)
block receptors
lose receptors
young adult women
can be associated with -
thymoma
thyrotoxicosis
Myasthenia Gravis -
Hx/PE
Muscle weakness inc. in
periods of activity
dec. after rest
Sx come and go
onset can be sudden
fluctuating fatigable ptosis
diplopia (double vision)
dysphagia (swallowing)
dysarthria (speech)
Sxs worsen throughout day

myasthenic crisis -
so severe,
resp. failure and aspiration
secondary to weakness
often secondary to infection
(resp. muscles compromised)

ocular myasthenia -
common variant
if doesn't spread in 2-3 yrs,
probably won't
Myasthenia Gravis -
Dx
Edrophonium (tensilon test) -
diagnostic
abnormal single-fiber EMG
dec. response to
repetitive n. stimulation
antistriatal ab if thymoma
chest CT - eval for thymoma
Myasthenia Gravis -
Tx
* Neostigmine (treat Sx)
* pyridostigmine (treat Sx)
* prednisone
* plasmapheresis or IVIG -
temp relief (wks) if severe
* thymoma resection
Amyotrophic Lat. Sclerosis -
What is it
Progressive neurodegen. dis
unknown etiology
degeneration of motor neurons
UMN and LMN degenerate
=> resp. failure and death
Amyotrophic Lat. Sclerosis -
Hx/PE
Slowly progressive weakness
may have fasciculations init
UMN and LMN
Amyotrophic Lat. Sclerosis -
Dx
Clinical presentation
EMG and n. conduction studies

CT/MRI -
cervical spine
check for lesions

r/o systemic causes -
CBC TSH SPEP UPEP
Ca2+ PTH PFTs

urine for heavy metal -
(if h/o exposure)
Amyotrophic Lat. Sclerosis -
Tx
Supportive
pt. education
pulmonary specialist
riluzole
Thiamine (B1) Deficiency -
What are Signs/Sxs
Wernicke's
inadeq intake or absorption
excessive alcohol interferes
with GI absorption
and liver storage
acute onset -
confusion
opthalmoplegia -
nystagmus
lat. rectus palsy
conjugate gaze palsy
abnormal pupils
ataxia -
vestibular dysfunction
cerebellar dysfunction
Thiamine (B1) Deficiency -
Pts.
Alcoholics
dialysis
hyperemesis
starvation
cancer
AIDS
can be triggered by large-dose
glucose admin if deficient
Thiamine (B1) Deficiency -
Tx
Reversible almost immediately
with thiamine admin
Korsakoff's Psychosis -
What is it
Late complication of
untreated pts. with Wernicke's
can be triggered by
severe or repeated DTs
can be triggered by SAH and
thalamic injuries
immediate memory affected
confabulation
Korsakoff's Psychosis -
Tx
Prognosis good -
head injury and SAH
irreversible - others
B12 deficiency -
What are Signs/Sxs
Subacute combined degen
B12 deficiency -
Pts.
Pts with pernicious anemia
B12 deficiency -
Tx
B12 injections or
large oral doses
Folate Deficiency -
What are Signs/Sxs
Irritability
glossitis
diarrhea
depression
neural tube defects
Folate Deficiency -
Pts
Alcoholics - alcohol
interferes with absorption
Pts. with pernicious anemia
Folate Deficiency -
Tx
Reversible if corrected early
Seizures -
General Evaluation
. Epilepsy -
seizure d/o
2+ seizures not related
to reversible stressors
. was seizure epileptic -
Hx
inc. prolactin = epileptic
. VITAMINS
seizure caused by -
vascular
infection
trauma
autoimmune
metabolic
idiopathic
neoplasm
sychiatric (faking it)
. anticonvulsant therapy
indicated?
1st seizure not treated
when underlying cause unknown
Partial Seizures -
What are they
Arise from focus
limited to one side
consciousness usually not lost
can progress to generalized
simple partial & complex part
Partial Seizures -
Hx/PE
Simple partial -
consciousness not impaired
motor - Jacksonian march
sensory - parietal
autonomic - BP, HR, PVR
psychic - fear, deja vu
postictally -
Todd's paralysis
resolves in 1-2 days

Complex partial -
consciousness impaired
temporal lobe
auditory or visual hallucinat
deja vu
automatisms
postictal -
confusion
amnesia
Partial Seizures -
Workup
EEG

r/o systemic causes -
CBC
electrolytes
Ca2+
fasting glucose
LFTs
tox screen
RPR
renal panel
ESR

r/o mass -
MRI or CT with contrast
Partial Seizures -
Tx
Tx underlying cause
recurrent partial -
phenytoin
oxcarbazepine
tegretol
phenobarbital
valproic acid
kids - phenobarbital
intractable temporal lobe -
WADA testing
ant. temporal lobectomy
Generalized Seizures -
What are they
Begin diffusely throughout
both sides
consciousness always lost
postictal confusion
most common types -
tonic-clonic (grand mal)
absence (petit mal)
Tonic-Clonic Seizures -
Hx/PE
Start suddenly
with tonic extension of
back and extremities
rep. symm. clonic movements
incontinence
tongue biting
may look cyanotic
consciousness slowly regained
may complain of -
muscle ache
headache
Tonic-Clonic Seizures -
Tx
Tx underlying cause
idiopathic -
valproate - 1st line
lamotrigine - adjunctive
topiramate - adjunctive
symptomatic -
same therapy as partial sz.
Absence (Petit Mal) Seizures-
What are they
Begin in childhood
subside before adulthood
often familial
Absence (Petit Mal) Seizures-
Hx/PE
Brief, often unnoticeable
episodes of
impaired consciousness
lasts 5-10 seconds
have 100's of times/day
amnestic during and
immediately after
eye flutters or lips smack
can be precipitated by
hyperventilation
Absence (Petit Mal) Seizures-
Evaluation
EEG -
3 Hz per sec. spike
& wave discharges
Absence (Petit Mal) Seizures-
Tx
Ethosuximide - 1st line
valproic acid
zonisamide
Infantile Spasms
(West Syndrome) -
What is it
Tx
Syndrome -
. infantile spasms
(generalized seizures)
symmetric
tonic
5-10 at a time while drowsy
or upon awakening
. abnorm interictal EEG
. arrest of psychomotor dev.
at age of onset
starts at 3-12 months
usu male
MR

Tx -
ACTH
prednisone
clonazepam
valproic acid
Status Epilepticus -
What is it
Common Causes
Brain in persist. state of Sz
1 cont. seizure or recurrent
w/o regaining cons. > 30 min.
med emergency - 20% mortality
common causes -
anticonv. withdrawal/noncomp
anoxic brain injury
alcohol withdrawal
metab disturb (hypoNa+)
trauma
infection
usual cause -
subtherapeutic or
noncompliance
Status Epilepticus -
Evaluation
Airway, breathing, circulation
labs -
electrolytes
anticonvulsants
get Hx
look for etiology if
no prior Hx
(tumor, encephalitis, SAH)


(EEG, CT only when stable)
Status Epilepticus -
Tx
Diazepam or lorazepam
if continues -
phenytoin or fosphenytoin
if continues -
phenobarbital
if continues -
midazalom or propoful
Headache -
Things to think about
(Evaluation)
Headache new or old
characteristics
any associated Sxs
any neurological Sxs
SAH suspected?
Migraine Headache -
What is it
More commonly affects women
and those with family Hx
vascular & 5HT abnorm
triggers -
menses
OCP
chocolate
fasting
stress
bright lights
Migraine Headache -
Hx/PE
Throbbing > 2 hrs
usually lasts < 24 hrs
n/v
photophobia
sensitive to noise

"classic" migraine -
visual aura precedes
unilat

"common" migraine -
80%
can be b/l, periorbital
Migraine Headache -
Dx
Hx
if have focal neuro deficits-
CT or MRI
Sxs acute in onset -
r/o meningitis or SAH
Migraine Headache -
Tx
Avoid known triggers
aspirin/NSAIDs
sumatriptan

prophylaxis -
beta-blockers
TCAs
Ca2+ channel blockers
valproic acid
Cluster Headache -
What is it
Men
~ 25 y/o - age of onset
Cluster Headache -
Hx/PE
Unilat, periorbital
30 min. - 3 hrs.
attacks in clusters -
same part of head
same time of day
same time of year
can be precip by alcohol or
vasodilators
ipsilat tearing of eye
Cluster Headache -
Dx
Hx
Cluster Headache -
Tx
Acute therapy with
high-flow O2
(100% nonrebreather O2)
sumatriptan
corticosteroids

prophylaxis -
Ca2+ channel blockers
valproic acid
methylsergide

prophylaxis important -
by the time abortive meds take
effect, has already resolved

methylsergine (sansert) -
need drug holiday every several months to avoid risk of retroperitoneal fibrosis
Tension Headache -
What is it
MC type diagnosed in adults
chronic
Tension Headache -
Hx/PE
Tight, bandlike pain
occipital and neck
exacerbated by -
noise
bright lights
stress
fatigue
Tension Headache -
Dx
Dx of exclusion
Tension Headache -
Tx
Avoid exacerbating factors
NSAIDs - 1st line
prophylaxis -
Ca2+ channel blockers
alpha blockers
TCAs
Benign Paroxysmal
Positional Vertigo -
What is it
Peripheral vertigo
otoliths dislodge from
usual position
migrate over time into
semicircular canals
abnorm fluid displacement
=> vertigo
Benign Paroxysmal
Positional Vertigo -
Hx/PE
Sudden onset of severe
vertigo and nystagmus
vertigo with head movement in
direction of affected ear
happens turning head in bed
5 sec. - 1 min.
n/v
Sxs dec. with repetitive tests
usu h/o recent trauma
Benign Paroxysmal
Positional Vertigo -
Dx
Nylen-Barany Maneuver
(Dix-Hallpike)
Benign Paroxysmal
Positional Vertigo -
Tx
Usually subsides spontan.
in wks to months
Epley Repositioning Maneuver
Meniere's Disease
(Endolymphatic Hydrops) -
What is it
Peripheral vertigo
from buildup of endolymphatic
fluid in inner ear
= V and P change
risk factors -
head trauma
syphilis
Meniere's Disease
(Endolymphatic Hydrops) -
Hx/PE
Sudden attacks of vertigo
lasts up to 24 hours
attacks can last hrs to days
n/v
ear fullness
tinnitus
hearing loss
signif permanent hearing loss
can occur over period of yrs
Meniere's Disease
(Endolymphatic Hydrops) -
Dx
Audiometry -
low-freq. pure tone
hearing loss
Meniere's Disease
(Endolymphatic Hydrops) -
Tx
Low-salt diet
acetazolamide
acute -
antihistamines
antiemetics
benzos
if refractory -
surgical decompression

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