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Neurology Step-up
Neurology Step-up
139
Medical
Graduate
02/16/2014

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Term
What defines a TIA?
Definition
neurologic deficit that lasts from a few minutes to no more than 24 hours
Term
What defines a "Reversible ischemic neurologic deficit"?
Definition
a TIA that lasts longer than 24 hours, but less than 2 weeks
Term
TIA implies a high risk of impending. . .
Definition
stroke in the next few months.
Term
Pts with carotid stenosis of > 75% are at risk for stroke if. . .
Definition
they experience hypOtension.
Term
What percentage of risk for stroke in individuals who experience TIA?
Definition
The risk of stroke in a pt with TIA is 10% per year.

TIAs carry a 30% 5-year risk of stroke
Term
What are the two most important risk factors for stroke?
Definition
HTN and AGE
Term
What two systems can be involved in a CVA/TIA?
Definition
Carotid system
Vertobrobasilar system
Term
What is involved in the work-up of an embolic stroke?
Definition
1. echocardiogram
2. Carotid Doppler
3. ECG, Holter monitoring
Term
Which is the most common location for a occlusion to cause CVA?
Definition
Middle cerbral artery
Term
Of the different types of stroke, which is the most common etiology?
Definition
EMBOLIC - origins include heart, internal carotid artery, Aorta, and Paradoxical (comes from a vein and goes through a PFO in the heart towards the brain)
Term
In carotid artery disease, where is the most common site of thrombus formation that causes stroke?
Definition
BIFURCATION of the COMMON CAROTID
Term
What are LACUNAR STROKES?
Definition
SMALL VESSEL THOMBOTIC disease
Term
What is the main predisposing factor for developing LACUNAR STROKES?
Definition
HTN
Term
How does HTN cause Lacunar strokes?
Definition
is causes hypertrophy of the smooth muscle of the arteries resulting in stenosis of the aterial lumen.
Term
When does a pt classically present with a THROMBOTIC STROKE?
Definition
the pt awakens from SLEEP with the neurologic deficit
Term
What is SUBCLAVIAN STEAL SYNDROME?
Definition
exercise of the left arm casuses reversal of blood flow down the ipsilateral vertebral artery to fill the subclavian artery distal to the stenosis bc it cannot supply adequete blood to the left arm
Term
What is the PATHOPHYSIOLOGY of SUBCLAVIAN STEAL SYNDROME?
Definition
caused by STENOSIS of the SUBCLAVIAN ARTERY proximal to the origin of the veterbral artery - exercise of LEFT ARM causes reversal of blood.
Term
What ultimately happens in SUBCLAVIAN STEAL SYNDROME?
Definition
Leads to DECREASED CEREBRAL BLOOD FLOW (blood is "stolen" from the basilar system)
Term
What are the NEUROLOGIC symptoms that manifest with subclavian steal syndrome?
Definition
causes symptoms of VERTEBROBASILAR ARTERIAL INSUFFICIENCY
Term
What are the physical symptoms associated with SUBCLAVIAN STEAL SYNDROME?
Definition
BP in LEFT ARM is less than that in the right arm

upper extremity CLAUDICATION
Term
What is the tx for SUBCLAVIAN STEAL SYNDROME?
Definition
SURGICAL BYPASS
Term
What is the dominant cerebral hemisphere for most people?
Definition
LEFT HEMISPHERE
Term
If the DOMINANT hemisphere is involved in a stroke, what main symptom with manifest?
Definition
APHASIA
Term
If the NON-DOMINANT hemisphere is involved in a stroke, what main symptom with manifest?
Definition
APRAXIA (inability to practice learned purposeful movements)
CONTRALATERAL BODY NEGLECT
CONFUSION
Term
With lacunar strokes, the symptoms are usually either. . .
Definition
pure MOTOR
or
pure sensory
Term
Where is the lacunar stroke if the symptoms are purely MOTOR?
Definition
INTERNAL CAPSULE
Term
Where is the lacunar stroke if the symptoms are purely SENSORY?
Definition
THALAMUS
Term
What are the four lacunar stroke syndromes?
Definition
pure Motor
pure sensory
Ataxic Hemiparesis - incoordination ispslaterally
Clumsy hand dysarthria
Term
Where is the lesion of the lacunar stroke if the person develops CLUMSY HAND SYNDROME?
Definition
Pons
Term
What is the most common location of occlusion involved in CVA?
Definition
Middle cerbral artery
Term
How long does it take for an ischemic infarct to appear on noncon CT?
Definition
48hrs
Term
How will an ischemic infarct appear on NCCT?
Definition
dark area
Term
How will a hemorrhagic stroke appear on NCCT?
Definition
white area
Term
When do you scan stroke pts with a CAROTID DUPLEX?
Definition
if they have. . .
Carotid bruit
PVD
Coronary artery disease
Term
What do you look for in a stroke pt who is less than 50 yo?
Definition
Vasculitis
hypercoagulable state
thrombophilia
Term
What do you order in a stroke pt less than 50 yo?
Definition
Protein C and S
Antiphospholipid Antibodies
Factor V Leiden
ANA, ESR, rheumatoid factor
VDRL/RPR
Lyme Serology
TEE
Term
What tests are ordered in a pt presenting with a stroke?
Definition
NCCT
ECG, CXR
CBC, platelet count
PT, PTT
Serum electrolytes
Glucose level
Bilateral Carotid Ultrasound
Echo
Term
What can you give a pt who canNOT receive ASA or plavix?
Definition
ticlopidine
Term
Is heparin or Warfarin used as a part of the treatment for stroke?
Definition
NO! they have not been proven to have efficacy in acute stroke
Term
Are BP medications given during treatment for stroke?
Definition
No, not unless there is an indication
Term
When do you give BP medications during a stroke?
Definition
if the pt has . . .

BP > 220 or diastolic >120 (MAP > 130)
acute MI
Aortic dissection
severe heart failure
hypertensive encephalopathy
Pt has high BP and is on antiplatelet therapy
Term
When does a pt get a carotid endarterectomy?
Definition
If carotid stenosis is >70% and the pt is symptomatic.
Term
What are the two major categories of hemorrhagic stroke?
Definition
Intracerebral hemorrhage
Subarachnoid hemorrhage
Term
Where does one bleed with a ICH?
Definition
bleed into the brain parenchyma
Term
Where does one bleed with a SAH?
Definition
bleed into the CSF
Term
Where is the most common location for ICH?
Definition
Basal ganglia (66%)
Term
Where is the ICH located if the pt has PINPOINT PUPILS?
Definition
PONS
Term
Where is the ICH located if the pt has PORRLY REACATIVE PUPILS?
Definition
THALAMUS
Term
Where is the ICH located if the pt has DILATED PUPILS?
Definition
PUTAMEN
Term
If a pt is found to have an ICH, where are they sent?
Definition
ICU
Term
How is the airway managed in a pt with ICH?
Definition
Intubation
Term
What is the treatment for ICH?
Definition
Supportive (there is no specific therapy)
Term
Are steroids used in ICH?
Definition
NEVER, they increase ICP
Term
What is done if a pt is found to have CERENELLAR HEMATOMA?
Definition
RAPID SURGICAL EVACUATION (could be lifesaving)
Term
Where do SACCULAR ANEURYSMS occur?
Definition
bifurcations of arteries of the circle of willis
Term
Where are the three most common sites for SAH?
Definition
1. junction of ANTERIOR COMMUNICATING a. with ANTERIOR CEREBRAL a.

2. junction of POSTERIOR COMMUNICATING a. with INTERNAL CAROTID a.

3. BIFURCATION of the MCA
Term
What inherited disorder is associated with BERRY ANEURYMS?
Definition
POLYCYSTIC KIDNEY DISEASE
Term
With SAH, an OPTHALMOLOGIC EXAM is mandatory to rule out. . .
Definition
PAPILLEDEMA
Term
if PAPILLEDEMA is present, what other procedure can you NOT perform?
Definition
LP
Term
Why can you NOT perform an LP in someone with PAPPILEDEMA?
Definition
can cause herniation (repeat the CT scan first)
Term
In Parkinson's disease, dopiminergic receptors are depleted in what two parts of the midbrain?
Definition
SUBSTANTIA NIGRA
LOCUS CERULEUS
Term
What disese process is PARKINSON'S + AUTONOMIC INSUFFICIENCY?
Definition
SHY-DRAGER
Term
What are LEWY BODIES?
Definition
HYALIN INCLUSION BODIES
Term
Does the tremor with Parkinson's get better or worse at rest?
Definition
WORSE
Term
What is PROGRESSIVE SUPRANUCLEAR PALSY?
Definition
denegerative condition of the brainstem, basal ganglia, and cerebellum, most commonly affecting middle-aged and elderly men.

Very similar to parkinson's with a few notable differences
Term
What are the differences between PSP and PARKINSON'S?
Definition
Unlike Parkinson's, PSP:
- DOES NOT CAUSE TREMOR
- DOES CAUSE OPTHALMOPLEGIA (palsy of eye movement)
Term
Do pts with tremor as a major symptom of parkinson's disease have a better or worse prognosis?
Definition
TREMOR = BETTER
Term
What causes HUNTING'S CHOREA?
Definition
it is caused by a mutation on chromosome 4 (expanded triplet repeat) - CAG leads to a loss of GABA-producing neurons in the striatum
Term
What are the general CHARACTERISTICS of HUNGTING'S CHOREA?
Definition
AUTOSOMAL DOMINANT, so lack of family history makes this diagnosis unlikely.

Onset is between 30 - 50.
15 years between onset of disease and death
Term
What are the CLINICAL FEATURES of HUNTING'S CHOREA?
Definition
CHOREA - involving face, head, neck, tongue, trunk, and extremities

ALTERED BEHAVIOR - irritability, personality changes,antisocial behavior, depression, OCD, psyhcosis

DEMENTIA - worsens with disease progression

GAIT is unsteady and irregular. Bradykinesia and rigidity prevail.

INCONTINENCE
Term
What will MRI show with HUNTING'S CHOREA?
Definition
atrophy of the HEAD OF CAUDATE NUCLEI
Term
What disease should always be kept in mind in a YOUNG PATIENT with movement disorders?
Definition
WILSON'S DISEASE
Term
What type of tremor is WORSE with INTENTIONAL ACTIVITY such as drinking from a cup, or use of utensils?
Definition
ESSENTIAL TREMOR
Term
Which type of tremor is MARKEDLY DECREASED WITH ALCOHOL?
Definition
ESSENTIAL TREMOR
Term
What is used to treat ESSENTIAL TREMOR?
Definition
propranolol
Term
What is COPROLALIA?
Definition
involuntary swearing seen in Tourette's Syndrome (although not seen in all pts with Tourette's)
Term
If you suspect a pt has Tourette's, what other diagnosis must be ruled out?
Definition
Seizures
Tardive Dyskinesias
Huntington's Disease
Term
What is BINSWANGER'S DISEASE?
Definition
a type of dementia, that has an insidious onset, due to diffuse subcortical white matter degeneration, most commonly seen in pts with LONG-STANDING HTN and ATHEROSCLEROSIS
Term
What is the pharmacologic treatment of dementia?
Definition
vitamin E
tacrine
donepezil
Term
What is tacrine?
Definition
an anticholinesterase that also acts as an anticholinergic
Term
How will the brain look in someone with Alzheimer's disease?
Definition
CEREBRAL ATROPHY secondary to neuronal loss. VENTRICLES will correspondingly be ENLARGED
Term
CT SCAN or MRI of the brain of an Alzheimer's pt will show . . .
Definition
CORTICAL ATROPHY and ENLARGEMENT of the VENTRICLES
Term
What part of the brain is affected with Binswanger's disease?
Definition
SUBcortical (white matter)

(whereas AD affects the cortical layer of the brain)
Term
If a pt is in a coma, and they have a pupillary light reflex, what does this imply?
Definition
the MIDBRAIN IS INTACT and not the cause of the coma
Term
If a pt is in a coma, and they are breathing on their own, what does this imply?
Definition
their brainstem is functioning
Term
If a pt is in a coma, and they have DOLL'S EYES, what does this imply?
Definition
they have a brainstem injury
Term
What is LOCKED IN SYNDROME?
Definition
a syndrome that mimics a coma, pts are completely paralyzed (with sparing of muscles required for RESPIRATION, BLINKING, and VERTICAL EYE MOVEMENT
Term
With LOCKED IN SYNDROME pts are _________ and capable of __________
Definition
FULLY AWARE
FEELING PAIN
Term
What causes LOCKED IN SYNDROME?
Definition
usually caused by infarction of the VENTRAL PONS
Term
What are the "plaques" in MS?
Definition
multifocal zones of demyelination
Term
Where are the classic locations of the plaques in MS?
Definition
ANGLES of the LATERAL ventricles
Term
Does MS affect the white matter or the grey matter?
Definition
WHITE matter
Term
What makes a CLINICALLY DEFINITE diagnosis of MS?
Definition
TWO episodes of symptoms
EVIDENCE of TWO white matter lesions
Term
What is considered a LABORATORY-SUPPORTED DEFINITE MS diagnosis?
Definition
TWO episodes of symptoms

evidence of at least ONE WHITE LESION on MRI

OLIGOCLONAL BANDS IN CSF
Term
What makes a PROBABLY MS diagnosis?
Definition
TWO epidoses of symptoms
AND
ONE white matter lesion
or OLIGOCLONAL BANDS in CSF
Term
What is one of the most common complaints in MS?
Definition
FATIGUE
Term
the symptom of INTERNUCLEAR OPHTHALMOPLEGIA strongly suggests what diagnosis?
Definition
MS
Term
Do steroids alter the outcome of MS?
Definition
NO - they are only used to shorten a ACUTE exacerbation
Term
Can you use oral steroids when treating an ACUTE EXACERBATION of MS?
Definition
NO - oral steroids have not shown to be efficacious. Only IV STEROIDS
Term
What diagnostic tests are used to evaluate for MS?
Definition
MRI
CSF
Evoked action potentials
Term
How are EVOKED ACTION POTENTIALS used to diagnose MS?
Definition
Evoked action potentials measures speed of nerve conduction within the brain - Newly remyeliniated nerves will conduct sensory impulses more slowly
Term
What is Myasthenic crisis?
Definition
a medical emergency in pts with Myasthenia Gravis that occurs in 15% of pts.

DIAPRAGM and INTERCOSTAL FATIGUE result in RESPIRATORY FAILURE (often requires mechanical ventilation)
Term
What is the TEST OF CHOICE diagnosing MYASTHENIA GRAVIS?
Definition
Acetylcholine receptor antibody test (most specific)
Term
Can someone have Myasthenia Gravis and be antibody negative?
Definition
YES - up to 20% of people with Myasthenia Gravis are antibody negative
Term
What imaging test must you get when diagnosing someone with Myasthenia Gravis?
Definition
CT of the neck to look for Thymoma
Term
What percentage of people with Myasthenia Gravis will have a THYMOMA?
Definition
15% (but 75% of pts will have abnormal Thymoma Tissue)
Term
What medications exacerbate symptoms of Mysthenia Gravis?
Definition
Antibiotics - aminoglycosides, tetracyclines

beta-blockers

Antiarrhythmics - quinidine, procainamide, and lidocaine
Term
What is the inheritence pattern of Duchenne's muscular dystrophy?
Definition
X-linked
Term
What is the pathophysiology in Duchenne's muscular dystrophy?
Definition
mutation on the gene that codes for DYSTROPHIN PROTEIN (dystrophin is absent causing muscle cells to die)
Term
Is there inflammation in Duchenne's Muscular Dystrophy?
Definition
NO
Term
What are the clinical signs and symptoms of Duchenne's Muscular Dystrophy?
Definition
muscle weakness is progressive, symmetric, and starts in childhood

PROXIMAL MUSCLES are primarily affected (PELVIC GIRDLE). Eventually involves the respiratory muscles.
Term
What is GOWER'S SIGN?
Definition
Pt uses hands to get up from the floor bc the weakness in the proximal lower extremity muscles makes it difficult to arise without support
Term
What will be noticed in the lower extremities in someone with Duchenne's Muscular Dystrophy?
Definition
PSEUDOHYPERTROPHY - (true muscle hypertrophy at first, followed by PSEUDOHYPERTROPHY as FAT REPLACES MUSCLE
Term
When do individuals with Duchenne's Muscular Dystrophy typically die?
Definition
third decade
Term
What is the tx for DUCHENNE'S MUSCULAR DYSTROPHY?
Definition
1. Prednisone - sig increase in strength, muscle function, and pulmonary function (may reduce risk of scoliosis)

2. Surgery to correct progressive scoliosis
Term
What is BECKER'S MUSCULAR DYSTROPHY?
Definition
less common than DUCHENNE'S
X-linked recessive
Similar to Duchenne's, but there is LATER ONSET and LESS SEVERE COURSE. (some dystrophin is present)
Term
What medications exacerbate symptoms of Mysthenia Gravis?
Definition
Antibiotics - aminoglycosides, tetracyclines

beta-blockers

Antiarrhythmics - quinidine, procainamide, and lidocaine
Term
What medications exacerbate symptoms of Mysthenia Gravis?
Definition
Antibiotics - aminoglycosides, tetracyclines

beta-blockers

Antiarrhythmics - quinidine, procainamide, and lidocaine
Term
What are the characteristics of NEUROFIBROMATOSIS TYPE I?
Definition
Cafe Au Laits Spots
neurofibromas
CNS tumors
axillary or inguinal freckling
iris hamartomas (Lisch Nodules)
bony lesions
Term
What are some other features of both types of NEUROFIBROMATOSIS?
Definition
seizures
mental retardation, learning disability
short height
macrocephalic
Term
What is the inheritance pattern of Neurofibramatosis?
Definition
autosomal DOMINANT
Term
What are the clinical features of NEUROFIBROMATOSIS TYPE II?
Definition
BILATERAL ACOUSTIC NEUROMAS - classic finding

cataracts

similar symptoms to that of type I
Term
What is RAGGED RED MUSCLE FIBERS?
Definition
MITOCHONDRIAL disorder caused by accumulation of diseased mitochondria in the subsaccrolemal space
Term
What are the physical manefestations of RAGGED MUSCLE FIBERS?
Definition
progressive myoclonic epilepsy
short stature
hearing loss
lactic acidosis
exercise intolerance
poor night vision
Term
What is the inheritence pattern of VON HIPPEL-LINDAU?
Definition
Dominant
Term
What are the important features of Von Hippel-Lindau?
Definition
Cavernous Hemangiomas of the brain stem
Renal Angiomas
Cyst in multiple organs
Term
What TUMOR is associated with Von Hippel-Lindau?
Definition
RENAL CELL CARCINOMA
Term
What ADRENAL DYSFUNCTION is associated with Von Hippel-Lindau?
Definition
PHEOCHROMOCYTOMA
Term
What is SYRINGOMYELIA?
Definition
central cavitation of the CERVICAL CORD due to abnormal collection of fluid within the SPINAL CORD PARENCHYMA
Term
What are the clinical features of SYRINGOMYELIA?
Definition
bilateral loss of PAIN AND TEMPERATURE in a CAPE LIKE DISTRIBUTION around the shoulders

(Most often asymptomatic and discovered incidentally on MRI)
Term
What is involved in LOSS OF PAIN AND TEMPERATURE?
Definition
SPINOTHALAMIC TRACT
Term
What is involed in LOSS OF VOLUNTARY MOVEMENT?
Definition
LATERAL CORTICOSPINAL TRACT
Term
What is the diagnosis when the pt presents with . . .

CONTRALATERAL loss of Pain and temp
IPSILATERAL hemiparesis
IPSILATERAL loss of position/vibration?
Definition
BROWN-SEQUARD SYNDROME
Term
What is BROWN-SEQUARD SYNDROME?
Definition
hemisection of the spinal cord
Term
What is the prognosis for BROWN-SEQUARD SYNDROME?
Definition
very good!
Term
What is TRANSVERSE MYELITIS?
Definition
rare condition that specifically affects the tracts across the HORIZONTAL aspect of the spinal cord at a given level. THORACIC SPINE is most commonly involved.
Term
What are the CLINICAL FEATURES of Transverse Myelitis?
Definition
LOWER extremity weaknes or plegia
Back Pain
Sensory Deficits below the level of lesion
Sphincter Disturbance - especially URINARY RETENTION
Term
What is the IMAGING study of choice for Transverse Myelitis?
Definition
MRI WITH CONTRAST
Term
What is TX for Transver Myelitis?
Definition
HIGH DOSE Steroid therapy
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