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Neuro Exam 3
Terms in this set (141)
FTD and ALS
C9ORF2 mutations causing polymorphic hexonucleotide repears are associated with what two disease?
A patient comes in with double vision and can't seem to move their eyes laterally... they are also having trouble talking. What is the highest diagnosis on your differential?
TRAP signs of Parkinsons?
carbon monoxide poisoning, treat with 100% O2
A man is admitted to the ER with bitemporal headache, worsened malaise, and decreased attention span. What is the most likely diagnosis? How do you treat him?
which population is particularly sensitive to sulfur dioxide, a colorless/irritating gas that causes bronchoconstriction and a product of incomplete combustion of fossil fuels?
nitrogen oxide; no specific treatment
brownish, irritating gas formed in fires or fresh silage. Concentrations > 50 ppm causes pulmonary edema, while concentrations > 100 ppm cause death. What is this gas? How do you treat it?
ozone, a bluish gas; severe exposure = pulmonary edema/fibrosis
If a person who lives in a city presents with blurred vision, upper respiratory tract irritation, chest discomfort, and airway inflammation, what is the most likely diagnosis? What do severe exposures result in?
CNS depression, liver (esp. carbon tetrachloride), kidney, and heart toxicity, and many different cancers
What does chronic exposure of halogenated aliphatic hydrocarbons result in?
benzene (added to gas in cold climates) (aromatic hydrocarbons, also toluene and xylene which don't cause bone marrow toxicity)
Which type of toxic chronic exposure results in aplastic anemia and leukemia, and acute exposure resulting in CNS depression?
CNS stimulation (tremor, seizures, respiratory depression, and death). Organopesticides can bioaccumulate and are also responsible for tumorigenic effects due to endocrine pathway disruption
What do organochlorine pesticides cause in humans?
inhibit acetylcholinesterase by phosphorylating its active site; they lack specificity so they can phosphorylate other enzymes in neural tissues "neuropathy target esterase" (can also result in progressive demyelination)
treat with pralidoxime
how do organophosphorous pesticides work? How do you treat poisoning with these agents?
inhibitors of acetylcholinesterase carbamoylate at the esteratic site (shorter duration of action than organophosphates) - don't treat with pralidozime
how do carbamate pesticides work?
botanical pesticide found in plants in north america (jicama, hoary pea) causes GI irritation and vomiting, pharyngitis, conjunctivitis, and rhinitis
What is rotenone? How is it toxic?
botanical pesticide that is very widely used; most feared results of ingestion is seizures, but can also cause membrane irritation, asthma, and skin rash
A patient comes in with trouble breathing and a rash. They say that they love to garden. You discover that they've been gardening with something called pyrethrum. what is pyrethrum? How is it toxic?
2,4-D and 2,4,5-T; a carcinogen (these aren't used anymore)
Which herbicides result in dioxin as a byproduct of their manufacture? What is dioxin
bipyridyl herbicides; single-electron reduction to a free radical species, accumulates slowly and causes alveolitis and progressive fibrosis. Also inhibits superoxide dismutase and causes
3 - 21 days after exposure.
A man comes into your clinic with hemorrhagic pulmonary edema and trouble breathing (duh). You discover that he is a gardener but he hasn't been to work in like 2 weeks. He says he usually uses something called paraquat as an herbicide. How does paraquat work? What class does it fall under? What toxicities does it cause?
Cadmium poisoning "cadmium fume fever"
industrial metal used in batteries, pigments, tires, and plating. Dude is a welder, blah blah, with malaise, chills, cough, fever, and pneumonia. Nephrotoxic if chronic exposure. What is the most likely cause of this guy's issues?
beryllium poisoning (autoimmune pulmonary fibrosis)
Dentists are most often affected by what type of poisoning?
Halothane has a slower induction effect because it has a greater solubility/blood:gas coefficient.
Nitrous oxide has the fastest rate of induction because it has the least solubility and the lowest blood:gas coefficient (nitrous oxide also has the fastest recovery time)
Which inhaled anesthetic has a faster induction effect? Halothane or nitrous oxide?
slowed onset and increased recovery time
What effect do increased cardiac output and pulmonary blood flow on the onset of anesthesia? On the recovery time?
Which inhaled general anesthetic is best for coronary artery disease and neurosurgery?
Which inhaled general anesthetic is best for children?
diffusion hypoxia, neurodegeneration, peripheral neuropathy, megaloblastic anemia
Key side effects of nitrous oxide?
occurs when nitrous oxide is administered with halogenated inhaled anesthetics; NO2 promotes their rapid uptake from alveoli and speeds onset of anesthesia
What is the second gas effect?
Which inhaled general anesthetic is best for someone who has experienced malignant hyperthermia in the past?
slower onset of action
higher blood:gas coefficient means:
allergies to eggs and soy
when is propofol contraindicated?
contraindicated in acute intermittent porphyria
Which intravenous general anesthetics are associated with a garlic or onion taste after administration? When are these drugs contraindicated?
Which intravenous general anesthetic is an NMDA receptor antagonist?
etomidate and isoflurane
Which anesthetics are best for patients with coronary disease?
alpha-2-adrenergic agonist: dexmedetomidine
What is the MOA and name of the drug that is best for general anesthesia for short term sedation of intubated/ventilated pts in the ICU?
A child presents to your pediatric clinic with a hx of rapid, irregular, and aimless involuntary movements of the arms and legs, trunk, and facial muscles. Their parent states that they had a sore throat and fever a few months ago. What is the most likely diagnosis?
generalized confusional state seen at times in patients with advanced cancer, who have no metastasis of cancer to the brain
An older man in the ICU presents with a confusional state characterized by disordered perception, frequent hallucinations, delusions, and sleep disturbance. What is this person presenting with?
Onion bulbing seen in segmental demyelination. Associated with Charcot Marie Tooth
What does this image show?
low temp, edema, slow relaxation of reflexes, low HR makes you think of?
ophthalmoplegia, ataxia, sensory loss, and
dysautonomia. Plasma exchange (presumably removing the offending antibodies) and intravenous immunoglobulin are the treatments of choice
campylobacter infection causing GBS
GM1 ganglioside is associated with...
CMV infection causing GBS
GM2 ganglioside associated with...
PAN "mononeuritis multiplex"
When would you perform a sural nerve biopsy?
TH1 Cell mediated; increased TNFalpha
granulomas, flat red skin lesion, nerves enclosed granulomatous reactions
TH2 humoral immunity; decreased TNFalpha
when do you see thickening of endoneurial arterioles due to increased deposition of basement membrane material?
Lambert Eaton Syndrome
antibodies against voltage-gated calcium channels in presynaptic elements of neuromuscular junction.
morton neuroma of the common digital nerve
The most frequent location is between the third and fourth metatarsals (third web space). Occurs in high heel wearers or those wearing constrictive shoes.
what organ overgrowth is associated with myasthenia gravis?
neurotoxin that blocks acetylcholine release
toxin blocks GABA resulting in spastic paralysis
acute flaccid myelitis (polio-like)
Magnetic resonance imaging (MRI) can be very helpful in diagnosing cases of AFM.
Most patients will have sudden onset of limb weakness and loss of muscle tone and reflexes following viral infection. What virus is this associated with?
Charcot Marie Tooth is what type of inheritance?
myelin protein zero
most common myelin in peripheral nerves?
From what type of embryonic tissue is schannoma from?
Antoni A; Antoni B
What is A? What is B?
what does this image show?
hamartomas, ash leaf spots/hypopigmented macules
angiofibromas associated with...
DR2 haplotype increases risk of...
MACROPHAGES CONTAINING LIPID RICH, PAS + DEBRIS. Perivascular cuffing of lymphocytes and monocytes
perivascular cuffing seen in...
oligoclonal IgG banding in CSF fluid
water channel aquaporin 4 autoimmune encephalitis. commonly found around 3rd and 4th ventricles
attack is primarily against astrocytes; associated with other autoimmune disorders
intractable vomiting unresponsive to antiemetics?
bilateral optic neuritis sparing the brain; necrosis of grey and white matter
NECROTIZING HEMORRHAGIC ENCEPHALOMYELITIS
headache, lethargy, coma, happens after viral infection or immunization
ACUTE NECROTIZING HEMORRHAGIC ENCEPHALOMYELITIS
almost invariably preceded by a recent, episode of upper respiratory infection
CENTRAL PONTINE MYELINOLYSIS
tongue signs and facial signs (AKA bulbar symptoms) locked-in syndrome
rapid correction of hyponatremic patients results in...
CENTRAL PONTINE MYELINOLYSIS
neurodegenerative disease characterized by progressive deterioration mainly involving the corticospinal tract, brainstem and anterior horn cells of the spinal cord.
neuropathological hallmark of what is the aggregation and accumulation of ubiquitylated proteinaceous inclusions in motor neurons?
toxic gain of function mutation leading to ALS
mutations in SOD1?
Huntingtons and ALS
excessive calcium entry causing glutamate excitotoxicity is seen in ...
riluzole -> inhibits glutamate release
treatment of choice for ALS?
hypothenar wasting in ALS
When do you see split hand?
NO, NO IT DOES NOT!!!
Does autonomic impairment occur in ALS?
pervenular cuffing by inflammatory T-cells and macrophages
MS lesions start with ...
Heat sensitivity occurs in...
MRI with galodinium contrast
best diagnostic tool for MS?
IV steroids followed by oral steroids
Treatment for MS?
random sequence of 4 amino acids that is homologous to myelin basic protein
elongation factor 2 degradation inhibitor
acute disseminated encephalomyelitis
• heart failure, blue green urine, reduction in left ventricular function, leukemia, teratogenicity?
• injection site reactions, rare acute chest syndrome, chest discomfort, palpitations
‣ only SC injections
• progressive multifocal leukoencephalopathy associated with JC virus and teratogenicity, flu-like symptoms
• binds to the a4 - integrin chain of the leukocyte adhesion molecule VLA-4, reducing movements of leukocytes to areas of inflammation
• inhibits the patient's immune response to myelin basic protein
4 amino acids that are similar to MBP to confuse the patient's immune system
• activates natural killer cells and macrophages, and upregulates antigen presentation to T lymphocytes
• inhibits topoisomerase II, disrupts DNA synthesis and repair, causing suppression of myeloid cell lines
‣ binds to the alpha 2 delta subunit of the voltage gated N type calcium Ca2+ channel
inhibits sphingosine, given once daily for MS
abdominal discomfort, nausea, vomiting, diarrhea and flushing
It was FDA-approved in September of 2012 for oral, once-daily dosing, and causes diarrhea, nausea, alopecia, and many adverse effects; it is NOT to be even touched by women of childbearing potential. Because it is found in semen, men who take it and want to father a child need to take cholestyramine or activated charcoal for 11 days after stopping the drug.
Adverse effects include elevated liver function tests and bradycardia with first-degree heart block, so the first dose is given with 6 hours of EKG monitoring
Treatment usually includes high- dose IV steroids, often with plasma exchange if not much acute improvement,
Treatment for NMO?
Acute disseminated encephalomyelitis
Diagnosis is easier when the history includes a recent viral illness or vaccination
MOA of haloperidol?
haloperidol, pimozide, fluphenazine
1st line treatment for tourettes?
2nd line treatment for tourettes? (Or first line in children?)
first line for essential tremor?
reserpine or tetrabenazine
first line for Huntington's?
tremor, rigidity, akinesia, postural instability
TRAP signs of parkinsons?
AEs of selegiline ?
Ropinirole (D2) /pramipexole (D3)
supplement with levodopa/carbidopa
1st line treatment for RLS?
loss of impulse control, uncontrollable tendency to fall asleep, peripheral edema, nausea, vomiting, hypotension
side effects of dopamine agonists?
blockage of VG Na+ channels via closure of H gate. More effective in active Na+ channels
MOA of local anesthetics?
long-acting ester local anesthetic?
local amide anesthetic for longur duration procedures/spinal anesthesia?
local amide anesthetic that causes cardiac dysrhythmias?
benzene hexachlorides, cyclodienes, toxaphenes, DDT are all from what class of pesticides?
CNS stimulation, tumorigenic, endocrine pathway disruption
organochlorine pesticides AEs?
Progressive Supranuclear Palsy
also early falls, inability to look upward, vertical saccades
absent, poor, or rapidly waning response to levodopa is a characteristic feature of PSP
Classic facial expression of perpetual surprise or astonishment is associated with what disorder?
cough, breath-holding, laryngospasm, hepatitis,
AEs of isoflurane?
patients at risk for hyperkalemia (severe wounds, severe burns), children < 8 yo, and angle-closure glaucoma or penetrating eye injuries
when is succinylcholine contraindicated?
slower rate of induction
larger blood:gas coefficients will have a _______ rate of induction
previous allergic reaction or elevated liver enzymes
when should amides be avoided?
normal pressure hydrocephalus
gait disturbance, urinary
incontinence, and progressive cognitive impairment
normal pressure hydrocephalus
main early symptoms of vascular dementia?
1. Alzheimer disease, 2. Lewy body disease, and 3. frontotemporal dementia 4. Cerebrovascular disease
principal diseases that cause dementia
Early signs of cognitive and brain dysfunction
Disorientation to time
Impaired sense of direction
Changes in personality or mood Executive impairment
beta amyloid, neuritic plaques, neurofibrillary tangles
1st line treatment according to Dr. Salinas for RLS?
anti-cholinergics and anti-depressants
How do you treat drug-induced movement disorders?
neuroleptic malignant syndrome
muscle rigidity, elevated temperature, altered mental status, hyperthermia, tachycardia, labile blood pressure, renal failure, and markedly elevated creatinine kinase levels
confusion, hyperthermia, tachycardia, coma, rigidity, ataxia, tremor
propranolol and diazepam
How do you treat serotonin syndrome?
• Dopa-responsive dystonia (DRD)
foot dystonia that interferes with walking, dominantly inherited gene for GTP cyclohydrolase-I
prevents release of inhibitory neurotransmitter GABA, affecting skeletal muscles
- GERD in infants causing failure to thrive
- paroxysmal episodes of generalized stiffening and opithotonic posturing that may be accompanied by apnea, staring, and minimal jerking of the extremities
- episodes abour 30 min after a feed, no projectile vomiting
fear and confusion associated with slow breathing, choking sound, and diminished awareness. Small growth pattern. Stereotypic hand movements, panic attacks. What does she have?
MECP2 gene is associated with what syndrome in girls with autism?
medication for sydenham's chorea?
Juvenile myoclonic epilepsy
uncontrollable, shock-like contractions of the extremities, particularly after awakening
15 yo with jerky arm movements
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