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304 Cards in this Set

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What imaging test should you always order in a patient with myasthenia gravis?
Chest CT b/c 65% of cases are assoc'd with thymic hyperplasia, and 10% of cases are assoc'd wtih thymomas

**Removal of the thymus has been shown to be beneficial in all patients regardless if they have thymic hypertrophy or thymoma!
How does the pathophysiology of Myasthenia Gravis differ from Lambert-Eaton syndrome?
MG = Ab's are made against the POST-synaptic Ach receptor at the neuromuscular jx

LE= Ab's are made against the PREsynaptic calcium channel - causing decreased release of ACh from nerve terminals
Most common symptoms seen in patients with Myasthenia Gravis
Diplopia and ptosis
Best confirmatory test to diagnose Myasthenia Gravis
Quantitative ACh receptor antibodies.

NOTE: The Tensilon (edrophonium) is no longer used in the U.S. When it is used, it can only be done if the pt has a quantifiable sign of MG such as ptosis.
True or false: Lambert-Eaton syndrome rarely involves occular muscles.
TRUE! Unlike myasthenia gravis, LE syndrome does not involve eye muscles. So, these pt will not present with diplopia and ptosis commonly seen in MG.
How can an EMG study help distinguish myasthenia gravis from Lambert-Eaton syndrome?
MG = decremental (decreased strength) response to rapid muscle stimulation

LE = incremental (Improved strength) response to rapid muscle stimulation
What is myasthenic crisis?
Occurs when a patient with MG has declining respiratory fx and needs to be placed on a ventilator.
What are some treatments for myasthenia gravis?
-Anticholinesterase inhibitors (pyridostigmine = Mestinon)

-Surgical removal of the thyroid gland

-Prednisone for occular myasthenia
Common age of onset of Duchenne Muscular Dystrophy
Age 3-5
What muscles are usually spared in children with Duchenne Muscular Dystrophy?
Occular and bulbar msucles (muscles that control chewing and swallowing)
What is the most common age of death in a boy with Duchenne MD?

What is the cause?
Around age 20 years old due to respiratory (diaphragm) insufficiency and aspiration.
What lab result is characteristically elevated in a boy with Duchenne MD?

What do muscle biopsies show?
CK levels (> 20,000)

Biopsies usually show decreased staining for dystrophin.
What is the pattern of inheritance for Duchenne MD?
X-linked recessive
Name this disease:

Clinically manifests in pts aged 3-5 years, with development of lordosis, a waddling gait, and Gowers sign. Calf pseudohypertrophy follows 1-2 years later. Most patients are wheelchair bound by age 12 years.
Duchenne MD
What is the pathophysiology behind pseudohypertrophy of the calve muscles seen in boys with DMD?
Enlarged calf muscles are caused by fatty and fibrotic infiltration of degenerated muscles.
What drug is often used in the treatment of Duchenne MD?
High dose corticosteroids

To date, corticosteroids are the only medication that has demonstrated a modest benefit in modifying the course of the disease.
What is emerin?
A trans NUCLEAR membrane protein coded for by the emerin gene located on the X chromosome.

Assoc'd with lamin A and C genes that also localize to the nuclear membrane.
Name this disease:

Early onset of joint contractures (elbows, ankles, and cervical spine); slowly progressive scapulohumeroperoneal pattern of weakness/atrophy; and cardiomyopathy that manifests as conduction abnormalities
Emery-Dreifuss Muscular Dystrophy

The major cause of mortality and morbidity in EDMD is cardiac disease: atrial conduction defects (eg, bradycardia, atrial arrhythmias, atrioventricular [AV] block, atrial paralysis)
Name this disease:

Mild to moderately elevated CK levels, muscle biopsy shows reduced or absent immunostaining for emerin
Emery-Dreifuss Muscular Dystrophy
Best method for management of the cardiac conduction defects assoc'd with Emery-Dreifuss Muscular Dystrophy.
Place a pacemaker in the patient.
Peak age for a woman with myasthenia gravis?

Peak age for a man?
Women = 20-30 years old

Men = 60-70 years old
What medications are contraindicated in patients with Myasthenia Gravis?
-Aminoglycosides
-Botox
-Quinine (tonic water)
-Interferon alpha
-Statins
-Calcium channel blockers
-Beta blockers
Name the criteria for migraine headaches:
1. At least 5 attacks that fulfill the following criteria
2. HA lasts 4-72 hrs
3. HA has at least 2 of the following qualities (unilateral, pulsating/throbbing, moderate - severe pain, aggravated by or causes avoidance of routine physical activity
4. During the HA, pt experiences at least one of the following (nausea and/or vomiting, photophobia or phonophobia)
5. The HA is not attributed to any other disorder
Name this type of migraine:
With aura
Classic migraine
Name this type of migraine:
Without aura
Common migraine
What is catamenial migraine?
Migraines in women that are related to hormonal changes of menstruation.
How is physical activity used to distinguish migraine HA from tension HA?
Tension HA - physical activity usually helps alleviate headache sx's

Migraine HA - physical activity worsens a patient's sx's, and thus physical activity is usually impaired when a migraine starts
What is the maximum times that abortive therapy for migraine HA can be used in one week?
A max of 3-4 days per week
What is the first line tx for episodic migraine
-ASA
-Acetaminophen (Tylenol)
-NSAID's

But, overuse of these agents can lead to rebound headache
How do triptans work in the treatment of migraine headaches?
They are serotonin agonists that activate vascular 5-HT1 receptors producing vasoconstriction
What is the most efficacious triptan and why is it often not used to treat migraines?
Sumatriptan, no used as frequently because it is administered via injection
What is a common side effect of overuse of Midrin and Fioicet for the tx of migraine HA's?
Rebound headache
Name some abortive therapies for tx of migraine HA's:
-Triptains (sumitriptan, rizatriptan)
-Midrin
-Fioricet
-Dihydro-ergotamine (only in emergency room/inpatient settings)
-IV neuroleptics (compazine droperidol)
-IV steroids
-IV anticonvulsants (valproate, Keppra)
-Muscle relaxants
What type of drug should also be given with ergotamine for the tx of migraines?
Give anti-emetics such as metoclopramide due to nausea as a common side effect of ergots
What are the 2 contraindications to using ergotamines for the tx of migraine?
1. Pregnancy
2. Vascular risk factors
TRUE or FALSE:
Narcotics are recommended in the abortive treatment of migraines.
FALSE!!! Narcotics should never be used to treat migraines b/c they can cause rebound HA and dependence issues.
What patients are good candidates for prophylactic migraine therapy?
Patients who have 4 or more migraines in a month.
Name some prophylactic treatments for migraines:
-Topiramate (Topamax)
-Verapamil
-Gabapentin
-Lamotrigine
-Baclofen
-Nadolol
-Nortriptyline
What test must you also order for patients with migraines that are treated with droperidol?
Droperidol is an IV neuroleptic. Patients taking this drug should have an EKG performed because it can cause prolonged QT intervals
What are some side effects of topiramate (topamax)?
Remember that topiramate is used as ppx for the treatment of migraines. This drug can cause the following:

-kidney stones
-metabolic acidosis
-decreased mentation (word finding difficulty)
What are some alternative therapies for the treatment of migraines?
-Accupuncture
-Massage
-Aromatheraphy
-Botox
-Biofeedback

These are considered to be 1st line therapy or adjuvants to traditional migraine treatment
Name this headache:

Affects males and females equally, bilateral, not aggrivated by physical activity, non-pulsatile
Tension-type headache
What symptoms are not associated with tension type headaches?
-Patients can have photophobia or phonophobia, BUT NOT BOTH!

-Patients can have anorexia, BUT NOT nausea or vomiting
Name the classification of tension type headache:

Less than 15 headaches/month

Greater than 15 headaches/month
< 15 = episodic

> 15 = chronic
Most commonly used therapy for acute tension type headaches:
-NSAID's
-Acetaminophen
-Naproxen
-Ibuprophen

-Can use muscle relaxers for refractory HA's (baclofen), cyclobenzaprine (Flexeril)
What are some prophylactic therapies for the treatment of tension type headaches?
-Tricyclic anti-depressants (amitriptyline, nortriptyline)
-Beta blockers
-Calcium channel blockers
-Topamax
_Tizanidine
Name this condition:

Unilateral, retro-orbital headache that affects males more than females
Cluster HA

-commonly begin in the morning hours, often awakening the pt
-throbbing/peircing
-assoc'd with runny nose and watery eyes, and Horner's syndrome
Abortive therapy for cluster headache
100% oxygen at 6-10 liters/min for a maximum of 15 minutes (overuse can lead to pulmonary fibrosis)

Can also use intranasal DHE (ergotamine), PO or IM sumitriptan, and prednisone.
Prophylactic treatment of cluster HA's
-Verapamil
-Valproic acid
Migraine aura WITHOUT the headache
Migraine equivalent

-More common in older patients (>65 yrs old)
Most common cause of the development of chronic daily headaches
Medication overuse

Also assoc'd with use of narcotics, excessive caffeine use, low education/socioeconomic factors, head injury, obesity
What is the best management for people with chronic daily headache?
Taper off all current medications. Then start ppx drug treatment and behavior modification
Painful, unilateral affliction of the face characterized by brief electric shock-like/lancinating pains found in the distribution of the trigeminal nerve
Trigeminal neuralgia

-Most common divisions involved are V2 and V3
What other medical condition must you rule out in a patient you suspect to have trigeminal neuralgia?
Shingles that presents in the opthalmic or other divisions of CN 5.
What are the first line treatments for trigeminal neuralgia?
-Carbamazepine (Tegretol)

-Oxcarbamazepine (Trileptal)
What imaging studies should you get in a patient you suspect to have trigeminal neuralgia?
-MRI to assess for compressive or demyelintating lesions

-MRA to assess for vascular abnormalities
Name some 2nd line treatments for trigeminal neuralgia
-Baclofen
-Gabapentin (neurontin)
-Lamotrigine (lamictal)
-Anticonvulsants (phenytoin, valproic acid) --> these are useful for the ER presentation for acute tx
Are there surgical therapies available for the treatment of trigeminal neuralgia?
YES!

-Radiofrequency thermocoagulation of CN 5 as it exits the foramen ovale --> side effect is that it leaves the face permanently numb

-Posterior fossa craniotomy for microvascular decompression
What is pseudotumor cerebri?
HA caused by increased intracranial pressure.

Most common in young, obese females
Inciting factors for the development of pseudotumor cerebri:
-weight gain
-pregnancy
-medications (TCA's, OCP's, prednisone, vit A, chemotherapy)
In a patient with pseudotumor cerebri, what might you see on fundoscopic exam, on a lumbar puncture, and on a head CT?
-Papilledema

-CSF opening pressure >250 mm H20

-Small ventricles
What is the likely etiology of pseudotumor cerebri?
-A mismatch in the production and absorption of CSF by the arachnoid villi

-Also damaged arachnoid villi due to calcification, post-hemorrhage, or damage due to chemotherapy.
Excessive CSF production (>30 ml/hr) can be caused by what?
-Hormones
-Caffeine
-Medications
The most common vasculitis that affects elderly patients
Temporal arteritis (Giant cell atertitis)
Name this condition:
An 80 year old man presents with scalp pain with tenderness over the temples and fatigue when chewing food that has resulted in a generalized headache
Temporal arteritis (Giant cell atertitis)
TRUE or FALSE: Temporal arteritis is not a neurologic emergency.
FALSE! This is an emergency because it can lead to permanent visual loss due to arteritic occlusion of the blood supply to the optic nerves.
How is the diagnosis of temporal arteritis made? How is the diagnosis is confirmed?

How should it be treated?
-Elevated estimated sedimentation rate (ESR > 100). Confirm with biopsy of the temporal artery.

Treat with PO prednisone 1mg/kg/day
Sudden onset of "worst headache ever"
Subarachnoid hemorrhage that can be caused by AVM, aneurysm, intracerebral hemorrhage or post-coital headache.
New onset HA after age 50
Temporal arteritis (Giant cell atertitis)

Tumor
HA with fever, stiff neck, and rash
Meningitis, encephalitis
Accelerating pattern of HA suggests what type of neurologic process?
Mass lesion, subdural hematoma, medication overuse
Leading cause of disability in persons less than 45 yrs old
Lower back pain (also the 3rd leading cause of disability in persons > 45 yrs old)
What percentage of workers with lower back pain return to work after the following time off from work:

-6 months
-1 year
-2 years
-6 mos = 50% return to work

-1 year = 25% return

-2 years = < 2% return
What are some risk factors for developing lower back pain?
-Sedentary lifestyle/poor fitness (most important factor)
-previous hx
-smoking
-muscle fatigue
-poor abdominal support
RED FLAGS of lower back pain
-pain in supine position w/o relief
-previous hx of cancer
-recent, significant unexplained wt loss
-fever/chills
-hx of osteoporosis
-bowel/bladder incontinence
-back pain as a child
-prominent leg weakness
-IV drug use and back pain --> epidural abscess
What structures are commonly involved in flexion-based back pain?
Targets the disk, especially in the lower lumbar area
What structures are commonly involved in extension-based back pain?
Targets the zygopophyseal joints (facet joints), lumbar spinal stenosis, pars defect (spondylolysis)
When is hydration of vertebral discs the worst?
In the morning b/c discs re-hydrate at night
What is the most common screening test to detect disc herniation?

What test is very specific for determining nerve root irritation?
Straight leg raise (moderately sensitive, but not specific)

Nerve root irritation can be detected by CROSSED straight leg raise
95% of herniated disks affect what nerve roots?
L5 - S1
What is the main neurotransmitter involved in the pathology of Alzheimer's dz? Are levels of this NT increased or decreased?
Acetylcholine levels are decreased due to a loss of the choline acetyltransferase in the cerebral cortex
When looking at the gross image of a whole brain of a patient with Alzheimer's disease, what are you likely to see?

What are you likely to see on a gross coronal section?
Widened sulci and narrowed gyri, mostly over the frontal and parietal regions.

Hydrocephalus ex vacuo, or ventricular dilation resulting from loss of cortex
What type of seizure is an absence seizure?
generalized
How many seizures must a person have in order to be diagnosed with epilepsy?
2 or more unprovoked seizures
What are the side effects of phenytoin (dilantin)?
-Ataxia
-Gingival hyperplasia
-Coarsening facial features
What are the side effects of carbamezepine (Tegretol)?
-Double vision
-hyponatremia
-Agranulocytosis
What are the side effects of valproic acid (depakote)?
-Tremor
-Weight gain
-Hair loss
-Hepatotoxicity
-Thrombocytopenia
What are the side effects of phenobarbital?
-Sedation
What are the side effects of ethosuximide (Zarontin)?
-GI symptoms
What is the drug of choice for the treatment of absence seizures?
Ethosuximide
What are the side effects of gabapentin (neurontin)?
-sedation
-ataxia
Rash and Stevens-Johnson syndrome are side effects of what anti-epileptic drug?
Lamotrigine (lamictal)
Metabolic acidosis, development of kidney stones, and word-finding difficulty are side effects of what anti-epileptic drug?
Topirimate (topamax)
What are the side effects of levetiracetam (Keppra)?
-Insomnia
-Anxiety
-Irritability
Think of this: "Keppra kept me up last night (insomnia) so I became anxious and irritable!"
What are the side effects of oxcarbazepine (Trileptal)?
-Sedation
-hyponatremia
What are the side effects of Zonisamide (Zonegran)?
-Renal stones
-Sedation
-Wt loss
What are the side effects of Tiagbine (Gabitril)
Sedation
What anti-epileptic drug targets the T-type Ca2+ channel?
Ethosuximide - used to treat absence seizures
What anti-epileptic drugs target the sodium channel?
-Phenytoin
-Carbamazepine
-Valproic acid
-Lamotrigine
-Topiramate
-Oxcarbazepine
In a patient that you suspect is having status epilepticus, what are the first 6 things you should do in the medical management?
1. Check ABC's
2. Get fingerstick glucose check
3. Start IV access
4. Send blood for laboratory analysis
5. Give 100 mg IV thiamine, FOLLOWED by 50% dextrose infusion
6. Give lorazepam 0.1 mg/kg IV
If status epilepticus persists after giving lorazepam, what drug should be given next?
Phenytoin 20 mg/kg IV (or a phosphenytoin equivalent)
If status epilepticus persists after giving lorazepam and phenytoin, what should be done next?
Intubate the patient if not already done, then give phenobarbital 20 mg/kg IV
If status epilepticus persists after giving lorazepam, phenytoin, and phenobarbital, what should be done next?
Induce coma with barbituates, midazolam or proprofol; then start continuous bedside EEG monitoring
What anti-epilepitic drug is most commonly associated with the development of neural tube defects?
Valproic acid
Treatment for essential tremor
-Propranolol 20 mg BID
-Mysoline 50 mg qhs
-Topiramate (Topamax)
-EtOH
-Deep brain stimulation
What type of tyhroidism is assoc'd with chorea?
HYPERthyroidism
Treatment of chorea
dopamine blockers, haldol, anti-psychotics
What movement disorder can develop in a person who had Rheumatic fever?
Sydenham chorea

Get an ASO titer as this will be elevated in these pts
What movement disorder is also assoc'd with lupus, cerebral palsy, and pregnancy?
Chorea
The SLOWEST of the movement disorders

The MOST BRIEF of the movement disorders
Slowest = dystonia

Brief = myoclonus
What condition(s) should you look for in an ICU patient with myoclonus
Acute uremia and acute hypoxic encephalopathy
What other neurological disease should you look for in a patient with dementia and myoclonus?
Creutzfeldt-Jacob disease - pts startle easily
Repetitive tongue and mouth movements are assoc'd with what movement disorder?
Tardive dyskinesia - often occurs after many years of dopamine-blocker drug usage.
What is the criteria for dx of Tourette's syndrome?
-multiple motor tics
-1 or more vocal tics
-onset BEFORE age 21
-duration more than one year
Treatment of Tourette's syndrome
Dopamine blockers and alpha-2 agonists (clonidine)
What 2 psychiatric conditions are also assoc'd with Tourette's syndrome?
ADHD and obsessive compulsive disorder
Most common cause of subarachnoid hemorrhage
-Trauma

In the absence of significant trauma, the MCC of SAH is the rupture of a cerebral aneurysm.
Most common location of post-traumatic subarachnoid hemorrhage is?
Over the cerebral convexity
Name this hematoma:

- Crescent shaped
- Hyperdense, but may contain hypodense foci due to serum, CSF or active bleeding
- Does NOT cross dural reflections
Subdural
In a subdural hematoma, between what meningeal layers in the brain does blood collect?
Blood collects in the space between the arachnoid matter and the dura matter.
In an epidural hematoma, between what meningeal layers in the brain does blood collect?
The blood from the ruptured vessel collects between the skull and dura mater.
What type of hematoma is assoc'd with skull fracture?
Epidural
Name this hematoma:

-Biconvex (lenticellular) shape
-Can cross suture lines because it is located above the dura mater
Epidural
Most common cause of significant morbidity in CNS trauma
Diffuse axonal injury
What is the most common location in the brain for diffuse axonal injury to occur?
# 1 = Subcortical white matter

Also,
#2 - Posterior limb internal capsule
#3 - Corpus callosum
#4 - Dorsolateral midbrain
The majority of all strokes are of this type:
Ischemic stroke - due to blockage of a cerebral artery

Ischemic stroke can further be divided into these categories:
thrombotic, embolic, lacunar, and hypoperfusion infarctions.
Name the 2 types of hemorrhagic stroke:
1. Intracerebral hemorrhage (most common)

2. Subarachnoid hemorrhage, due to rupture of a cerebral aneurysm
What is the chance that a person will have at least one seizure over the course of their lifetime?
11%
What percentage of the population has epilepsy?
2%
Name this type of seizure:

Sudden onset, is not assoc'd with aura, no warning
Generalized seizure
Side effects of this drug include:

-Hair curling and hair loss
-Liver failure
-Bone marrow toxicity (pancytopenia, thrombocytopenia)
-Essential tremor
-Neural tube defects
Valproic acid
Generalized seizure that involves an abrupt generalized jerk
Myoclonic generalized seizure
A 4 year old boy is eating breakfast, then suddenly loses all muscle tone and loses consciousness. What type of seizure did he just have?
Atonic generalized seizure
Treatment of tonic generalized seizures
-Dilantin
-Keppra
-Valproic acid
A seizure that occurred in the limbic system will likely result in what type of behavior?
Sexual arousal
A seizure that occurred in the temporal lobe will likely result in what type of symptoms?
Smelling odors such as burning, smoke, rotten eggs, funny tastes, deja vu, and out-of-body experiences
Unilateral paresthesias are a sign that a seizure has occurred in what part of the brain?
Contralateral sensory cortex
What type of partial seizure is most likely to spread and secondarily generalize?
COMPLEX partial seizures
What is the difference between simple and complex seizures?
Simple - NO loss of consciousness

Complex - Altered or loss of consciousness
Most common cause of NEW ONSET generalized tonic clonic seizure
Alcohol withdrawal - seizures usually begin 12-36 hrs after stopping alcohol
What drugs (street or prescription) are known to cause seizures as a side effect?
-Bupropion (smoking cessation)
-Demerol
-Cocaine
-Meropenam, Imepenam
TRUE or FALSE: Conventional anti-epileptic drugs often work in the treatment of alcohol withdrawal seizures
FALSE!

Treatment for these types of seizures includes Ativan (lorazepam) 2 mg IV, propofol, and barbituates such as phenobarbital
Name factors that LOWER the seizure threshold (make having a seizure more likely)
Sleep deprivation
Fever
Systemic illness or psychological stress
Alcohol withdrawal
What anti-epileptic drug is known to cause pancreatitis as a side effect and should not be given to patient who are known to have panreatitis?
IV Valproic acid (Depacon)
TRUE or FALSE:

Anticonvulsant drugs do not reduce the effectiveness of oral contraceptive medications.
FALSE!!! AED's can reduce the effectiveness of OCP's, so you must tell female patients this and document it in their chart.
TRUE or FALSE:

Alcohol withdrawal seizures can be effectively treated with the use of alcohol.
TRUE, but not a recommended treatment.
TRUE or FALSE:

Alcohol withdrawal seizures do respond to phenytoin, carbamazepine and valproic acid.
FALSE!

They respond to barbituates, alcohol, and lorazepam (Ativan).
What is West Syndrome>
aka Infantile Spasms, assoc'd with mental retardation in 85-90% of cases.

Treatment of choice is ACTH, also vigabetrin and oral steroids, valproate, benzodiazepine
What seizure disorder presents with a stare --> jerk--> fall down, that presents between 2-10 years of age and is preceded by encephalopathy in 60% of cases?
Lennox-Gastaut Syndrome
In patients with myasthenia gravis that are seronegative for ACh receptor Ab's, what other Ab are they likely to be positive for?
Muscle specific kinase (MuSK)
What mysathenia gravis antibodies are most commonly assoc'd with symptoms of weakness of neck extensors, shoulders, and respiratory muscles?
Muscle specific kinase (MuSK)
What is the criteria for intubation of a patient with Myasthenia Gravis having a MG crisis?
Any of the following:

-Decrease in forced vital capacity (FVC) below 15 ml/kg (or below 1L in an averaged sized adult)

-Severe aspiration from oropharyngeal weakness

-Labored breathing regardless of the FVC measurements
d-penicillamine, alpha interferon, chloroquine, quinine, quinidine, procainamide, aminoglycoside abx and botulinum toxin should be avoided in patients with what neuromuscular disease?
Myasthenia Gravis
What type of drugs can be used to treat the symptoms of myasthenia gravis if acetylcholinesterase inhibitors do not work?
Immunosuppression agents - high dose corticosteriods
What malignancy is most commonly assoc'd with Lambert-Eaton syndrome?
Small cell carcinoma of the lung

NOTE: Older pts with the disease tend to be men and have assoc'd malignancy, whereas younger pts tend to be female and have no assoc'd malignancy
What is Lambert's sign?
In patients with Lambert-Eaton syndrome on testing sustained maximal grip there is a gradual increase in power over the initial 2-3 seconds
Name some treatments for Lambert-Eaton syndrome
3,4-diaminopyridine, pyridostigmine, and guanidine.
What neuromuscular disease is assoc'd wtih proximal limb weakness legs > arms, hypoactive or absent muscle stretch reflexes, dry mouth, constipation, blurred vision, impaired sweating and a metallic taste in the mouth?
Lambert-Eaton syndrome
Prior to obtaining a lumbar puncture, what 2 lab results must you have?
-check platelet count: must be > or equal to 50,000

-check INR: should be < 1.5
What potential complications of lumbar puncture should a patient be told of prior to getting a LP?
- Back pain
-Leg pain
-Headache
-Bleeding
-Infection
-Death
In every patient with papilledema, altered level of consciousness, focal neurological deficit, or a new onset seizure, what should you do before getting a lumbar puncture?
Obtain head CT or MRI
In every patient with signs of symptoms of a mass lesion in the posterior fossa, what should you do before getting a lumbar puncture?
Obtain MRI of the head
Your patient needs to have a lumbar puncture. What position should they be placed in to perform the LP?
Lateral recumbent position with the knees and the neck flexed. The patient's back should be as close to the edge of the table as possible
What is the most common complication of a lumbar puncture?
Headache - can occur in up to 40% of patients.
What types of people are at greatest risk for developing post-lumbar puncture headache?
Thin young women and patients with HA's before the LP
Name this condition:

Bilateral generalized pressure or throbbing sensation that begins 48-72 hours after a lumbar puncture. Sx's worse when pt is upright and moves their head, and improve when pt lies down.
Post-lumbar puncture headache
How do you treat post-lumbar puncture headache?
-Patient should be encouraged to lie flat

-Drink caffeinated beverages Q4-6 hrs (substitue IV sodium benzoate 500 mg if pt hospitalized and is NPO)

-Epidural blood patch
What type of needle should be used to perform a lumbar puncture?
A Quincke needle
What are some disadvantages to using Quincke needles in a lumbar puncture?
Technically more difficult to use, cannot be used to measure opening CSF pressure, and may require the use of a syringe to obtain spinal fluid.
Lumbar puncture should not be performed on a patient if you cannot visualize what 2 structures on neuroimaging?
4th ventricle and the quadrigeminal cistern
A patient needs a lumbar puncture. You review her medication list and see that she take coumadin. Can she have a LP?
NO!!!!! b/c there is an increased risk of bleeding from the procedure. You must reverse anti-coagulation first.
TRUE or FALSE: Fungal meningitis and carcinomatous meningitis can cause elevated CSF glucose levels.
FALSE! They can cause LOW CSF glucose levels.
What is hypoglycorrahachia?
CSF to serum glucose ratio <0.6
What lab test should you perform on a patient immediately after getting a lumbar puncture?
Always check the blood glucose, but a finger accucheck IS NOT acceptable.
How many WBC's in the CSF should you see for every 700 RBC's?
1 WBC for every 700 RBC's.
TRUE or FALSE:

In a neonate, severe cardiorespiratory compromise is a contraindication for performing a lumbar puncture.
TRUE
What is empiric therapy for the treatment of bacterial meningitis?
-3rd or 4th gen cephalosporin (ceftriaxone 2 gm Q12 hrs, or cefotaxime 3 gm Q4 hrs, or cefepime 2 gm Q 12 hrs)

-Vancomycin 30-45 mg/kg/day during 8 hr interval

-Acyclovir 20 mg/kg Q8 hrs

-Doxycycline 100 mg Q12 hrs (during tic season)
What wave rhythm is associated with wakefulness?
Alpha waves = 8-12 cycles/sec
What sleep stages are considered deep sleep/slow wave sleep?
Stages 3 and 4

Assoc'd with delta waves (1-4 cycles/sec)
Stages 1-4 of sleep are called what?
Non-REM sleep (NREM)
What wave rhythm is seen in REM sleep?
The brain is very active during REM sleep, so we see alpha waves (8-12 cycles/sec) on EEG.
After falling asleep, how long does it take on average to enter REM sleep?
90 minutes
As we age, how does the time spent in non-REM sleep change?
Length of non-REM sleep DECREASES. So elderly pts spend less and less time in deep sleep stages 3 and 4.
TRUE or FALSE:

When we dream, we only dream in non-REM sleep.
FALSE: Dreaming can occur in both REM and non-REM sleep.
What is the best diagnostic test to determine if a pt have obstructive sleep apnea?
Perform a sleep study (polysomnography)
Complications of obstructive sleep apnea include?
HTN - 80% of cases!!!
CHF
Arrythmia
Ischemic heart disease
Stroke
TRUE or FALSE: It is normal for children to snore while sleeping.
FALSE!!!! This is never normal, and the child should be evaluated for sleep apnea.
Name this sleep disorder:

Primary disorders of initiating or maintaining sleep that leads to FRAGMENTATION of nighttime sleep and an intrusion of REM during periods of wakefullness
Dyssomnias

Example: narcolepsy
Name this sleep disorder:

A patient comes to you complaining of excessive daytime sleepiness, having sudden loss of all muscle tone (cataplexy), feeling paralyzed while sleeping, and having hallucinations while drifting off to sleep or when waking up from sleep.
Narcolepsy
A hallucination that occurs as a person is going to sleep
hypnogogic
A hallucination that occurs as a person is waking up from sleep
hypnopompic
How do you make the diagnosis of narcolepsy?
1st = get an all night sleep study

2nd = the next morning after the sleep study get a median sleep latency test (MSLT)
What is a median sleep latency test?

When should it be performed?
MSLT: the person is given five 20 minutes nap opportunities Q2 hrs; and the time it takes for the person to fall asleep is recorded.

It is used to diagnose narcolepsy. Persons with this disorder will fall asleep w/in 5 minutes and have REM sleep, whereas normal people will take 15 minutes to fall asleep and DO NOT have REM sleep during these naps
What drugs are used to treat narcolepsy to help increase alertness during the day?
Modafanil (Provigil)

Ritalin

Xyrem (sodium oxybate, > 6 gm/day) - ***the only drug FDA approved to treat cataplexy
Drug of choice to treat cataplexy assoc'd with narcolepsy
Xyrem (sodium oxybate, > 6 gm/day)
What are parasomnias?

What type of sleep (REM or NREM) is most commonly affected in these disorders?
Abnormal activities that occur during sleep, affects NREM sleep (slow wave sleep)

-Sleep walking, sleep terrors (night terrors), confusional arousals
Treatment for parasomnias
Tricyclic antidepressants and benzodiazepines
What sleep disorder is often seen in pts with Parkinson's disease that involves screeming and abnormal motor activity during REM sleep?
REM behavior disorder

Can treat with clonazepam
What metabolic problems are assoc'd with Restless Legs Syndrome?
Iron deficiency anemia
Uremia
Liver disease
Neuropathy
Treatments for Restless Legs Syndrome
Clonazepam
Tylenol with codiene
Ultram
Nerontin (gabapentin)

Sinemet (carbidopa-levodopa) - but this drug can cause sx's to progress over time, so must limit use to 3x/wk
In a patient with a new onset seizure, what 3 metabolic screens are likely to be low?
glucose, sodium, and calcium
Drug of choice for focal seizure disorder
Tegretol (carbamezepine)
What anti-epileptic drug will often drop the the WBC count to 3,000-5,000?
Tegretol (carbamezepine)
Phenytoin (dilantin)
vs. Valproate(depakote):

1. Drug of choice for mixed seizure disorder
2. Makes osteoporosis worse
3. Hepatotoxic
4. DOES NOT tend to impair memory and learning
1. Valproate
2. Phenytoin
3. Phenytoin
4. Valproate
Antiepileptic drug that causes memory impairment and produces speech difficulty
Topiramate (topamax)
Antiepileptic drug that is addicting and thus can cause withdrawal seizures
Phenobarbital

But is also good because it is very cheap and has a very long half life (3 days)
What type of tremor is assoc'd with Parkinson's disease?
Resting tremor
A tremor that is present when the limb is totally relaxed or when the patient is walking
Resting tremor
A tremor is present when the limb is being held up (like holding the phone, newspaper)
Postural tremor
A tremor that occurs when the limb is moved (eating, writing, applying makeup)
Action tremor
Name the root needed to produce this reflex and the muscle responsible:

Ankle jerk (Achilles tendon reflex)
S1, gastrocnemius
Name the root needed to produce this reflex and the muscle responsible:

Knee jerk (patellar reflex)
L2,3,4 - quadriceps
Name the root needed to produce this reflex and the muscle responsible:

Biceps
C5,6 - biceps
Name the root needed to produce this reflex and the muscle responsible:

Triceps
C7,C8 - triceps
Abdominal reflexes are a normal finding in normal people, but this reflex can be absent in pts with what type of motor neuron lesion (upper or lower?)
UMN lesion
Which hematoma is assoc'd with higher mortality and morbidity?
Subdural hematoma due to the assoc'd damage to the brain parenchyma
What should you do the in management of subarachnoid hematoma?
-Monitor for cerebral vasospasm, hydrocephalus and increased ICP
Small hemorrhagic lesions found in the corpus callosum and brainstem are called what?
Duret hemorrhages
What is the most common intracerebral lesion in pts with severe traumatic brain injury?
subdural hematoma
What is the most common location in the brain for a contusion to occur?
At the inferior frontal lobes or temporal lobes
A problem involving a single nerve root is termed what?
Radiculopathy
A problem involving multiple nerve roots at once is termed what?
Polyradiculopathy
What studies are needed to diagnose a single radiculopathy?
MRI of the spine
What studies are needed to diagnose a polyradiculopathy?
A lumbar puncture to look for infectious or inflammatory conditions
Name some causes of radiculopathies
-Herniated disk
-Reactivation of varicella zoster (shingles)
Gait seen in a person with cerebral palsy or cervical spinal cord lesion
spastic gait
Gait seen in a person with cerebellar disease
ataxic gait
Gait seen in a person with normal pressure hydrocephalus
apraxic gait (feet feel like they are glued to the floor)
Gait seen in a person with Charcot-Marie-Tooth disease and foot drop
High stepping gait
Gait seen in a person after they suffered a middle cerebral artery stroke
hemiparetic gait
Name the triad of symptoms seen in a person with normal pressure hydrocephalus (NPH)
1. Dementia (memory loss, word-finding difficulty)
2. Urinary incontinence (urgency/frequency)
3. Gait ataxia (apraxic)
Name the 4 hallmarks of Parkinson's disease
1. Bradykinesia
2. Postural instability (shuffling gait)
3. Rigidity
4. Resting tremor
Progressive, mainly symmetric, rhythmic, involuntary oscillation of the hands and forearms that is usually absent at rest, and present during posture and intentional movements
Essential tremor
First-line drug used in the treatment of essential tremor (ET):

Second-line treatment used in the tx of ET:
1st: Propranolol (beta blockers)

2nd: Topirimate (anticonvulsants), alprazolam (benzodiazepine -- but limited use due to sedative effects)
TRUE or FALSE: Lithium is associated with the development of chorea
TRUE. Other causes of chorea include lupus, pregnancy, hyperthyroidism, and cerebral palsy
What class(es) of drugs can be used to treat chorea?
Antipsychotics (haldol) and dopamine blockers
Sustained contraction of both agonist and antagonist muscles simultaneously, causing twisting and repetitive movements or abnormal postures
Dystonia - the slowest of the involuntary movement disorders
Diffuse cortical ribboning seen on DWI or MRI is most suggestive of what type of neurological disease?
Prion disease: CJD
What is the first test you should order in a pt you suspect has a prion disease such as CJD?
Brain MRI with diffusion-weighted imaging (DWI)
What involuntary movement disorder is most commonly assoc'd with prion diseases such as CJD?
Myoclonus - the most brief of the mov't disorders
What part of the brain is often involved in hemiballismus?
Subthalamic nucleus
What is a common cause of hemiballisumus?
A hypertensive hemorrhage or infarct
Name the disorder:

GAA trinucleotide repeat
Friedreich's Ataxia - autosomal recessive inheritnace
CTG trinucleotide repeat disorder
Myotonic dystrophy
Common presentation of a patient with Wilson's disease:
Patients are usually aged 10 to 40 years and present with either hepatic disease or a movement disorder neurologic disease.
What disease should you consider in a patient who is aged 10 to 40 years with hepatitis, cirrhosis, hepatic decompensation and symptoms suggestive of movement or psychiatric disorders
Wilson's disease - assoc'd with low serum cerulopsamin (95% cases), aminoaciduria, and increased urinary copper excretion
Why is penicillamine no longer used for the treatment of Wilson's disease?
b/c it can worsen neurologic sx's assoc'd with the disease in 10-50% of cases
What drug is used in the treatment of ACUTE Wilson's disease?
Increase the urinary excretion of copper using TRIENTINE
What drug is used in the treatment of CHRONIC Wilson's disease?
Decrease the intestinal absorption of copper using ZINC
Seizure that can be induced by hyperventilating
Absence seizures
Name this seizure type based on the EEG pattern:

EEG will demonstrate bilateral synchrony in the epileptiform activity
Generalized tonic-clonic seizure
How is the serum level of prolactin affected during and after a generalized tonic-clonic seizure?
Prolactin levels are often 2-3 fold greater than baseline.

This transient postictal elevation has been used clinically to distinguish these seizures from nonepileptic seizures that are not assoc'd with elevated prolactin levels.
Name risk factors for developing carpal tunnel syndrome
-Hypothyroidism
-Pregnancy (especially in the 3rd trimester)
-Repetitive motion of the wrist (extension and/or flexion)
Most common compression neuropathy of the upper extremity
Median nerve at the wrist = Carpal tunnel syndrome
What condition can result from the following:

chronic mechanical compression of the ulnar nerve at the ulnar groove
cubital tunnel syndrome
A patient presents with pain in the medial elbow, numbness/tingling in the 4th and 5th digits that increases with elbow flexion. What do they have?
Compression of the ulnar nerve at the elbow - Cubital tunnel syndrome
What is chance of a stroke patient having symptomatic intracerebral hemorrhage with the use of tPA?
6%
Anticoagulation, sympathomimetic drug abuse and vascular malformations are risk factors for what type of stroke?
Hemorrhagic stroke
What type of MRI should you order in a patient who presents with stroke?
A diffusion weighted (DWI) MRI stat!
What platelet count must a stroke patient have before he can be given tPA?
Platelets > 100,000
What blood pressure must a stroke patient have before he can be given tPA?
<185 systolic
<110 diastolic
TRUE or FALSE: You can give a stroke patient who has received tPA aspirin.
TRUE, but only if the aspirin is given 24 hrs after the administration of tPA and the pt has received a head CT that ruled out any evidence of intracranial hemorrhage.
A patient suffered a stroke 3 days ago. They are still hospitalized, and now their neurological deficits have worsened. What is the most likely cause?
Increased edema around the infarcted area of the brain
TIA is a potent risk factor for having a subsequent stroke. What percentage of pts will suffer a stroke within 90 days of having a TIA? Within 2 days?
90 days - 10.5%

2 days - 5%
A patient with carotid bruit is found to have 70% stenosis. How should he be treated?
Perform carotid endarterectomy
A patient with carotid bruit is found to have 50% stenosis. How should he be treated?
Medical management only, endarterectomy is NOT indicated.
A patient with carotid bruit is found to have 60% stenosis. How should he be treated?
In pts with 50-69% stenosis, you can CONSIDER endarterectomy.
What test should patients with myotonic dystrophy receive every year and why?
Must have a yearly EKG b/c this form of muscular dystrophy is assoc'd with cardiac rhythm disturbances.
A patient presents with frontal balding, temporal wasting, ptosis, atrophy of the SCM muscles and difficulty holding onto objects. When he was born, it was noted that he was a floppy baby with a tented mouth. What condition does he have?
Myotonic dystrophy
TRUE or FALSE: Myotonic dystrophy is associated with trinucleotide repeat expansion.
TRUE. It is also an autosomal dominant disease.
Muscle biopsy of a boy with Duchene muscular dystrophy will show what?
Absent dystrophin staining
How does Emery-Dreifuss muscular dystrophy differ from DMD?
Unlike people with DMD, most patients are able to walk into the third decade of life; AND EDMD is commonly assoc'd with severe musculotendinous contractures (especially of the neck and spine)
Common skin manisfestations of dermatomyositis
-Heliotrope rash
-Grotton's papules (red scaly lesions on the knuckles)
-butterfly rash on the face
-rash on the sun-exposed regions of the head/neck/trunk
-Periungual edema
Name this neuromuscular disorder:

Presents with symmetrical proximal muscle weakness, normal sensation , preserved reflexes, very little cranial nerve weakness if any, but also has DYSPHAGIA
Idiopathic inflammatory myopathies
TRUE or FALSE: The systemic manifestations of dermatomyositis and polymyositis include CHF, arryhtmia, heart block and interstitial lung fibrosis.
TRUE
Name this disease: The patient presents with years of slowly progressive weakness of the quadriceps muscles and distal finger flexor muscles.
Inclusion body myositis
You suspect a patient has inclusion body myositis. You perform an EMG needle exam. What will you likely see?
profuse fibrillations
Name the inflammatory myopathy:

Muscle biopsy shows vasculopathy, vasculitis, and perifasicular atrophy
dermatomyositis
Name the inflammatory myopathy:

Muscle biopsy shows muscle fiber necrosis, cellular infiltration
polymyositis
Name the inflammatory myopathy:

Muscle biopsy shows inflammation and rimmed vacuoles on H&E and oil red O staining. EMG shows filamentous inclusions
inclusion body myositis
TRUE or FALSE: Inflammatory myopathies are usually very painful.
FALSE! They are often painless!
TRUE or FALSE: Treatment of inclusion body myositis includes the use of corticosteroids and other immunotherapy just like the tx of dermato- and polymyositis.
FALSE! Unlike DM and PM, IBM is not responsive to steroids or other immunotherapy. Tx of IBM includes PT, OT, speech tx, and pulmonary evaulation.
How can you distinguish inclusion body myositis from dermato- and polymyositis?
-Progression: IBM progresses very slowly; DM and PM progress over months

-Response to corticosteroids: IBM is non-responsive, DM and PM are responsive

-CK levels: IBM shows normal or mild elevation; DM and PM show marked elevation in CK

-Muscle biopsy
TRUE or FALSE:

Polymyositis and dermatomyositis are found more commonly in white people compared with black people.
FALSE: DM and PM are more common in blacks than in whites.
TRUE or FALSE: Inclusion body myositis affects men more than women and it is more common in white rather than black patients.
TRUE
First-line treatment for dermatomyositis and polymyositis
Oral corticosteroids
A short-lived vertigo sensation that commonly occurs following rolling over in bed (or any change in position of the head); it is not assoc'd with hearing loss or tinnitus
Benign positional vertigo - sensation is usually caused by displacement of particles in the inner ear (MC = posterior canals)
Associated hearing loss, roaring tinnitus and aural fullness that is often exacerbated during an episode of vertigo; not provoked by positional changes
Meniere's syndrome
Often a single episode of persistent vertigo lasting days. The vertigo can be exacerbated by ANY positional change; often preceded by a nonspecific viral infection (upper respiratory)
Labyrinthitis
Treatment of acute labyrinthitis includes the following:
**Meclizine (anti-cholinergic)
**Diazepam
Anti-viral drugs
Prednisone
Treatment of Meniere's syndrome
Low salt diet and/or diuretics to reduce levels of endolymph
#1 cause of disequilibrium dizziness
Medications (diazepam, phenergan, amitryptaline)
1st sign of cauda equina syndrome
urinary retention
Treatment of cauda equina syndrome
1 gm solumedrol given in the ER followed by STAT MRI and surgical consultation; place urinary catheter
Best initial procedure for patients with suspected lumbrosacral radiculopathy
MRI
Pain from lumbar spinal stenosis is produced by what type of physical activity?
Walking DOWNHILL and standing. Pain is actually relieved by walking uphill or sitting due to changes in the AP diameter.
Most common level of stenosis in patients with lumbar stenosis
L4-5 --> L3-4 --> L2-3
What is albuminocytologic dissociation and what disease is it associated with?
On analysis of CSF, it is the presence of an elevated protein count but very low or absent WBC's. This is seen in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and GBS.
The cornerstone of treatment for alcohol witdrawal
Benzodiazepines
A condition experienced by individuals with epilepsy, in which a seizure is followed by a brief period of temporary paralysis which may be partial or complete but usually occurs on just one side of the body.
Todd's paralysis
Test of choice for diagnosis of a brain tumor
Head MRI with contrast
The most common malignant brain tumor of childhood that usually arises from the cerebellar vermis and causes increased ICP and obstructing hydrocephalus due to blockage of the ventricular system.
Medulloblastoma
A 6 yr boy presents with 3-4 weeks of morning headaches and intermittent vomiting w/o fever. The headaches improve throughout the day. Over the past two days, the headaches are lasting longer and the vomiting is more frequent, but after vomiting the headaches are much improved. On physical exam he is noted to have unsteady gait. What brain tumor do you suspect he has?
Medulloblastoma
The most common symptom of this tumor is unilateral hearing loss. Other sx's include unilateral tinnitus, vertigo, and other cranial nerve disturbances.
Acoustic neuroma (schwannoma)