Continuum - Questões Flashcards
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Which of the following types of cerebral edema is characterized by
derangements in cellular metabolism resulting in altered ionic gradients
and movement of water into brain tissue?
A cytotoxic
B hydrostatic
C osmotic
D vasogenic
A)x.
There are four types of cerebral
edema:
-
Cytotoxic edema is characterized by derangements in cellular
metabolism associated with cell death resulting in altered ionic gradients
and movement of water into brain tissue; - Hydrostatic edema results from displacement of CSF from the ventricular space through the ependymal lining and into the brain parenchyma in association with hydrocephalus.
-
Osmotic cerebral edema occurs when an osmotic gradient develops
between the brain tissue and serum that favors entry of water into the
brain, such as in dialysis disequilibrium syndrome. -
Vasogenic edema
results from dysfunction of the blood-brain barrier, with extravasation of
ions and macromolecules from the plasma and subsequent movement of
water into the extracellular space of the brain, where it collects preferentially in white matter. This is most commonly seen with brain
tumors.
Which of the following is a risk of both mannitol and hypertonic saline
in the management of cerebral edema and elevated intracranial pressure?
A acute kidney injury
B hyperglycemia
C hyponatremia
D hypovolemia
E liver failure
A)x.
Both agents increase
serum sodium and, with repeated use and high serum osmolality, can
cause acute kidney injury.
A comatose 19-year-old woman is brought to the emergency department
with severe multifocal traumatic brain injury and elevated intracranial
pressure. In addition to supportive care, which of the following initial
interventions is the most appropriate temporizing measure to reduce
intracranial pressure?
A bifrontal decompressive craniectomy
B hyperventilation to 25 mm Hg to 35 mm Hg PaCO2
C hypothermia to 32 °C to 34 °C (89.6 °F to 93.2 °F)
D methylprednisolone 2 g infusion over 1 hour
E pentobarbital to achieve EEG burst suppression
B)x.
Hyperventilation constricts cerebral blood vessels, offering a rapid but time-limited reduction in intracranial pressure that can be used as a bridge to more definitive treatment.
Steroids are contraindicated
in the management of traumatic brain injury based on the increased
mortality associated with the use of methylprednisolone therapy in the
CRASH (Corticosteroid Randomisation After Significant Head Injury) trial.
Both craniectomy and hypothermia to 32 °C to 34 °C (89.6 °F to 93.2 °F)
reduce intracranial pressure, but neither improves patient outcomes and they may lead to worse neurologic outcome or death. Thus, they are reserved as tier two (craniectomy) or tier three (hypothermia) options for refractory intracranial hypertension.
Pentobarbital titrated to burst suppression is a tier three intervention.
A 78-year-old man with a past medical history of hypertension presents
with sudden-onset headache. Head CT shows acute blood isolated to the prepontine cisterns. Which of the following is correct regarding the
patient’s most likely diagnosis?
A the most likely etiology is an aneurysm of the anterior
communicating artery
B the most likely etiology is an arteriovenous malformation
C the patient is at high risk for delayed cerebral ischemia
D the patient is at significantly high risk of acute hydrocephalus
E the prognosis is generally excellent
E)x.
Blood confined to the prepontine cisterns is most likely a perimesencephalic
subarachnoid hemorrhage.
In these patients, the presence of subarachnoid blood is isolated to the perimesencephalic or prepontine cisterns and no vascular malformations are found on digital subtraction.
The prognosis of these patients is
generally excellent, and acute hydrocephalus, cerebral vasospasm, and
delayed cerebral ischemia are not typical.
Which of the following therapeutic agents has Class I evidence for
decreasing the risk of poor outcome in patients with subarachnoid
hemorrhage?
A nicardipine
B nimodipine
C phenytoin
D simvastatin
E Triple H therapy
B).
Nimodipine at a dose of 60 mg every 4 hours should be started in all patients with subarachnoid
hemorrhage within 96 hours and continued for 21 days.
It does so by reducing the rate of delayed cerebral ischemia, although it
does not reduce the incidence of vasospasm.
Although nicardipine
reduced the incidence of symptomatic and angiographic vasospasm in
studies, no difference was seen in outcome.
e. Although the use of prophylactic anticonvulsants
is often given for a short course, no Class I evidence supports its use. If an anticonvulsant is used, phenytoin is not recommended.
Triple H (hypervolemic, hypertensive, and hemodilutional) therapy, is not recommended as it does not improve outcomes and increases
cardiopulmonary complications.
A 47-year-old woman presents to the emergency department with
1 week of severe headache. She states that the headache started
suddenly and was severe at the onset. She was thinking about coming to
the hospital but decided to wait it out at home. Her partner brought her
in today because the partner is concerned that the patient has been
getting more confused. A head CT in the emergency department is
normal. What is the sensitivity of a head CT in this patient for detecting
a subarachnoid hemorrhage?
A 20%
B 40%
C 60%
D 80%
E 90%
C)X.
06-12 hours: 93-100% os sensitivity.
01 week: 60%.
Prefer MRI in subacut/cronic SAH (GRE, SWI and FLAIR).
Which of the following is a benefit of an endovascular approach in
comparison to a surgical approach for treatment of ruptured aneurysms?
A allows for hematoma evacuation
B easier to access distal aneurysms
C higher odds for survival without disability at 1 year
D lower risk of aneurysm reoccurrence
E lower risk of delayed cerebral ischemia
C)X.
The ISAT (International Subarachnoid Aneurysm Trial) found
that endovascular coiling is associated with higher odds for survival
without disability at 1 year after subarachnoid hemorrhage; this risk
reduction lasts for at least 7 years.
The BRAT (Barrow Ruptured Aneurysm
Trial) study found patients who had an endovascular approach had fewer poor outcomes at 1 year.
A surgical approach has advantages that include:
- A lower risk of aneurysm occurrence;
- Easier access to distal aneurysms;
- And an ability to evacuate a hematoma if present.
Studies have not found
a difference in the risk of delayed cerebral ischemia between the two treatment approaches.
Which of the following drugs of abuse is most likely to increase the risk
of intracerebral hemorrhage?
A benzodiazepines
B cocaine
C inhalants
D marijuana
E opioids
B)X.
Are risk factor for ICH:
1. Cocaine;
2. 3,4-methylenedioxymethamphetamine (MDMA, ecstasy);
3. Amphetamines.
Sudden increases in blood pressure can induce hemorrhage from a preexisting cerebral aneurysm or arteriovenous malformation.
In addition to hypertension, vasoconstriction can occur.
Arteritis has been reported (although rare).
Which of the following vascular malformations is most commonly
associated with intracerebral hemorrhage?
A arteriovenous malformation
B capillary telangiectasia
C vein of Galen malformation
D venous angioma
A)x.
Arteriovenous malformations and cavernous malformations are the most common cerebral vascular malformations that cause intracerebral hemorrhage.
Seizures are another commom manifestation of MAVs.
Capillary telangiectasias and venous
angioma are typically found incidentally on imaging and rarely cause symptoms.
Vein of Galen malformation typically presents in infancy with heart failure.
A 68-year-old woman without significant past medical history is seen in
the office for brief recurrent neurologic symptoms, including focal
paresthesia and dysphasia. Review of systems is negative, and
examination is unremarkable. Brain MRI shows no acute findings, but
multiple cortical microbleeds and superficial siderosis are seen on
gradient recalled echo (GRE) imaging. Which of the following is the
most likely diagnosis?
A cavernous malformation
B cerebral amyloid angiopathy
C hypertension
D metastatic disease
E primary angiitis of the central nervous system
B)x.
> 55 years + multiple cortical microbleeds + superficial siderosis + amyloid spells.
. In addition to
intracerebral hemorrhage, patients with cerebral amyloid angiopathy
may experience recurrent transient neurologic events and acute or
subacute cognitive decline associated with an inflammatory
leukoencephalopathy or angiitis.
Dementia is common ( co-occurrence
of parenchymal amyloid deposition).
A 45-year-old woman with coronary artery disease and mechanical
mitral valve prosthesis is seen in the emergency department 1 hour after
sudden-onset headache and left hemiparesis. Her medications include
warfarin and low-dose aspirin. Her blood pressure is 146/82 mm Hg, and
her Glasgow Coma Scale score is 15. Head CT shows a 15 mL hemorrhage
in the right putamen. Her international normalized ratio (INR) is 2.9.
Which of the following interventions is the most appropriate next step
in management?
A hypertonic saline
B levetiracetam
C nicardipine infusion
D platelet transfusion
E prothrombin complex concentrate
E)x.
Warfarin-associated ICH has an increased risk of hematoma expansion even when
the INR is in the therapeutic range.
__Immediate reversal with prothrombin complex concentrate and vitamin
K is indicated.__
Routine platelet transfusion is not recommended for antiplatelet users because of an increased risk of poor outcome.
Prophylactic anticonvulsants are
not recommended because they do not improve outcome.
Which of the following would be most appropriate for patients who
present with a moderate or severe traumatic brain injury?
A barbiturates to induce coma
B hypothermia with goal temperature of 35 °C to 37 °C (95 °F to 98.6 °F)
C neuromuscular paralysis
D scheduled infusions of hypertonic saline every 4 to 6 hours
E 7-day course of antiepileptic medication for seizure prophylaxis
E)x.
Of all patients with moderate or severe
traumatic brain injury (TBI), 2% to 12% develop seizures, with the highest
risk immediately following the TBI. It is therefore recommended to give a
7-day course of antiepileptic medication for seizure prophylaxis in all patients with moderate or severe TBI. The other answer options are
possible interventions given to patients who have elevated intracranial pressure but should not be instituted in all patients with moderate or severe TBI.
Which of the following pharmacologic agents has been shown to accelerate the pace of functional recovery in patients who are vegetative or in a minimally conscious state shortly after traumatic brain injury?
A amantadine
B donepezil
C olanzapine
D propranolol
E sertraline
A)x.
Patients with severe traumatic brain injury who are given amantadine recover significantly faster than controls measured by the disability rating scale.
Propranolol, sertraline, and olanzapine can improve neurobehavioral symptoms
following moderate or severe traumatic brain injury, although they have not been shown to accelerate the pace of functional recovery.
Which of the following has been consistently shown to improve
outcomes in patients with moderate and severe traumatic brain injury?
A avoidance of systolic blood pressure below 100 mm Hg
B early large bifrontal temporoparietal decompressive craniectomy
C glucocorticoids
D hypothermia below 35 °C (95 °F)
E prophylactic hyperventilation with PCO2 target of less than 25 mm Hg
A)x.
Patients with moderate and severe traumatic brain injury often have failure of cerebral autoregulatory mechanisms, making cerebral blood flow completely dependent on systolic blood pressure.
Current recommendations include a systolic blood pressure goal greater than 100 mm Hg in patients 50 to 69 years of age and greater than 110 mm Hg in patients 15 to 49 and older than 70 years of age.
In the CRASH (Corticosteroid Randomisation After Significant Head Injury) trial, mortality was higher in the steroid arm.
Hypothermia reduces intracranial pressure but does not improve outcomes.
The DECRA (Early Decompressive Craniectomy in Patients With Severe Traumatic Brain Injury) trial showed that early large bifrontal temporoparietal decompressive craniectomy decreased intracranial
pressure but had no effect on mortality and increased unfavorable
outcomes.
Hyperventilation to a target of less than 25 mm Hg is not recommended as it can increase risk of ischemia.
Which of the following headache features is more suggestive of
reversible cerebral vasoconstriction syndrome than aneurysmal
subarachnoid hemorrhage?
A associated neck pain
B gradual onset
C multiple recurrences over days
D precipitated by sexual intercourse
E severe pain
C)x.
The headache feature most suggestive of RCVS is multiple recurrences of thunderclap headache over a time span of days, with the
pretest probability for RCVS in this setting approaching 100%.
A 56-year-old man is seen in the emergency department after a
generalized seizure. He reports severe headache, blurred vision, and
dizziness for the previous 3 days. His blood pressure is 236/125 mm Hg.
Examination is significant for somnolence and generalized
hyperreflexia. Urinalysis and toxicology screen are unremarkable.
Brain MRI reveals fluid-attenuated inversion recovery (FLAIR)
hyperintensities in both parietooccipital lobes, consistent with
vasogenic edema. Which of the following treatments is most
appropriate to institute at this time?
A aspirin
B glucocorticoids
C mannitol
D nicardipine
E triptan
D)x.
No treatment has been proven to
hasten resolution of cerebral edema or prevent complications of PRES,
but treatment of severe hypertension is recommended.
Antiseizure medications are also administered for recurrent seizures.
Glucocorticoids offer no benefit and may worsen concomitant reversible cerebral vasoconstriction syndrome.
triptans are contraindicated since they can precipitate PRES.
A 48-year-old woman with a history of migraine and four recurrences
of thunderclap headache over 1 week is found to have sulcal
subarachnoid hemorrhage and multifocal segmental stenoses of the
bilateral middle and posterior cerebral arteries. She is instructed to
discontinue the use of triptans and is treated with verapamil, with
resolution of headache over 2 weeks and no new symptoms. Angiography
is repeated at 1 month and shows persistence of vessel abnormalities.
Which of the following is the next best step in management?
A lumbar puncture
B reassurance
C start high-dose glucocorticoids
D switch verapamil to nicardipine
E toxicology screen
B)x.
Resolution of cerebral angiographic abnormalities typically lags behind resolution of symptoms in patients with reversible cerebral vasoconstriction syndrome and may take 2 to 3 months.
Which of the following conditions has a longer median duration until
seizure detection compared to the others, thereby making prolonged
continuous EEG monitoring particularly helpful?
A epidural hemorrhage
B intracerebral hemorrhage
C ischemic stroke
D subarachnoid hemorrhage
E subdural hemorrhage
D)x.
Thus, in patients with subarachnoid
hemorrhage, prolonged continuous EEG monitoring may be particularly
helpful.
Which of the following antiepileptic medications should be avoided in
patients with second- or third-degree heart block?
A fosphenytoin
B lacosamide
C levetiracetam
D sodium valproate
E topiramate
Lacosamide has been
associated with PR interval prolongation and should therefore be
avoided in patients with second- and third-degree heart blocks.
Which of the following EEG findings within the initial 72 hours is
associated with a poor outcome in patients with traumatic brain injury?
A absence of a discontinuous background
B absence of triphasic waves
C presence of N2 sleep transients
D presence of a posterior dominant rhythm
E presence of predominant delta activity
E)x.
Which of the following is most appropriate for a neurologist consulting
on a patient in the intensive care unit with respiratory failure due to
Guillain-Barré syndrome to alert the intensive care unit team to
anticipate?
A autonomic dysfunction
B disseminated intravascular coagulation
C hypernatremia
D hypersalivation
E worsening of neurologic condition for 8 to 12 weeks
A)x.
Autonomic dysfunction complicates the course of GBS in over 1/3 of patients, especially those with more severe disease:
1 - Quadriparesis;
2 - Bulbar and neck flexor weakness;
3 - Respiratory failure.
The most common manifestations are:
1 - Fluctuations in blood pressure and heart rate;
2 - Ileus;
3 - Fever.
SIADH may occur (with hyponatremia).
Drooling may occur in association with bulbar weakness, but hypersalivation is a feature of cholinergic crises in MG.
In a patient with Guillain-Barré syndrome, which of the following is an
early indicator of impending respiratory failure?
A breathing pattern in which the abdomen moves outward on inspiration
B cyanosis
C hypercarbia
D hypoxemia
E reduced forced vital capacity
E)x.
In 20% to 30% of patients with Guillain-Barré syndrome, progression of weakness leads to respiratory failure requiring mechanical ventilation.
Signs of worsening of respiratory status:
1 - Inability to speak without needing to repeatedly pause and breath;
2 - Paradoxical breathing pattern (the abdomen moves inward on inspirations to assist expansion of the chest);
3 - Maximum inspiratory and expiratory pressure;
4 - Reduced forced vital pressure.
Cyanosis, hypercarbia and hypoxemia are all late findings.
An 85-year-old woman with myasthenia gravis is admitted to the
intensive care unit with increasing generalized weakness and shortness
of breath associated with community-acquired pneumonia. Which of
the following medications would be most likely to trigger or worsen a
myasthenic crisis if administered to this patient?
A β2-agonist
B cephalosporin
C fluoroquinolone
D low-molecular-weight heparin
E proton pump inhibitor
C)x.
Fluoroquinolones and
aminoglycosides are common offenders.
A 58-year-old woman with gastrointestinal bleeding is noted to have
new left arm weakness on day 5 of intensive care unit management for
transfusion-associated acute respiratory distress syndrome (ARDS).
Examination is limited by sedation but is notable for reduced extension
but not flexion of the left arm away from noxious stimulation and
absent left triceps reflex. Which of the following additional
information should be sought in evaluating this patient?
A history of neuromuscular junction disease
B presence of fever
C recent history of hypotension
D serum sodium
E use of prone positioning
E)x.
Examination findings consistent with radial nerve palsy.
Use of the prone position for patients with ARDS may be associated with compression or traction on the nerves of the brachial plexus, leading to acquired peripheral nerve injury.
Which of the following is a helpful clue to distinguish acute flaccid
myelitis due to enterovirus infection from Guillain-Barré syndrome?
A absence of respiratory failure
B asymmetric pattern of weakness
C neuropathic pain
D prodromal illness
E sensory level on examination
B)x.
Infections from enterovirsu are typically asymmetric and may affect one or more limbs.
Prodromal illness is typically present in both disorders and both may include significant respiratory involvement or significant neuropathic pain.
In acute myelitis, T2 hyperintensities of the gray matter will be seen and, in GBS, nerve root enhancement of the cauda equina may be seen.
Sensory level is unusual in either disorder.
A 5-year-old girl presents with 4 days of fevers, rhinorrhea, sore
throat, and change in mental status. She has been sleeping more than
usual, and this morning her parents were not able to wake her up,
prompting them to bring her to the emergency department.
Fluid-attenuated inversion recovery (FLAIR) MRI brain shows
bilateral thalamic and putamen hyperintensities. Which of the
following viruses is most commonly associated with this disorder?
A enterovirus
B human metapneumovirus
C influenza
D measles
E respiratory syncytial virus
C)x.
Symptoms and imaging findings consistent with acute necrotizing
encephalitis.
Although rare, this disorder has been associated with the influenza virus, affecting pediatric patients more often than adult patients.
In addition to influenza virus, acute necrotizing encephalitis has also been reported in patients with COVID-19.
A 45-year-old man presents to the emergency department with
headaches and worsening mental status. He has had 10 days of mild
respiratory symptoms and decreased smell, and he was diagnosed by
his primary care physician as having COVID-19. Upon admission, the
patient has a head CT, which shows bilateral thalamic hypodensities; a
CT angiogram of the head does not show any occlusion. His D-dimer
is elevated to 900 ng/mL. Which of the following should be the next
step in management?
A aspirin
B CT venogram of the head
C IV recombinant tissue plasminogen activator (rtPA)
D lumbar puncture
E MRI brain with and without contrast
B)x.
This patient is presenting with symptoms and radiologic findings concerning for a cerebral venous thrombosis, best diagnosed with a CT venogram of the head.
MRI brain is less crucial acutely, and a lumbar puncture in this case is not warranted and may be dangerous.
Although anticoagulation is the treatment for cerebral venous thrombosis, IV recombinant tissue
plasminogen activator (rtPA) is not indicated.
On postoperative day 2 after craniotomy for posterior fossa
decompression, a patient reports headache, photophobia, and neck
stiffness. The headache is brought on by sitting up in bed and is
relieved by lying flat. Vital signs are within normal limits. When the
patient’s neck is passively flexed, she involuntarily flexes her hips. The
remainder of the examination is unremarkable. Which of the following
diagnoses is most likely?
A cerebral venous thrombosis
B CSF leak
C meningitis
D migraine headache
E subarachnoid hemorrhage
B)x.
This patient has postural headache, meningismus, and photophobia, symptoms and signs of CSF hypotension.
CSF hypotension due to a CSF leak may complicate craniotomy (especially posterior fossa decompression) or spinal surgery if dural closure is not complete.
A 77-year-old man with a history of left hemisphere transient ischemic
attack is noted to have facial asymmetry 1 day after left-sided carotid
endarterectomy. On examination, his face is symmetric at rest with
no nasolabial fold flattening. With attempted smile, the patient is
unable to move his left lower lip. Movements of the left upper lip and
the other facial muscles are intact. Which of the following neurologic
complications is most likely in this patient?
A cerebral hyperperfusion syndrome
B hypoglossal nerve palsy
C injury to the marginal mandibular branch of facial nerve
D lower motor neuron facial palsy associated with small brainstem
stroke
E upper motor neuron facial palsy associated with small cerebral
hemispheric stroke
C)X.
The marginal mandibular branch of facial nerve innervates the muscles of the lower lip, especially the lip depressors.
Injury to this branch, which may occur via compression by a retractor
during carotid surgery, results in mouth asymmetry during smiling
because of unopposed action of the mouth elevators.
. The hypoglossal nerve is susceptible to injury during carotid surgery because of its proximity to the carotid
bifurcation. Its damage results in ipsilateral tongue weakness and
difficulty speaking, chewing, and swallowing.
Cerebral hyperperfusion syndrome accompanying brain revascularization presents with ipsilateral headache, seizure, and focal neurologic symptoms.
A 50-year-old man is noted to have fluctuating encephalopathy and
subtle involuntary movements of the left hand 6 hours after
craniotomy for acute traumatic right cerebral convexity subdural
hematoma evacuation. Head CT shows no new infarct or hemorrhage.
Which of the following is the next best step in management?
A obtain EEG
B order brain MRI
C perform lumbar puncture
D start IV corticosteroids
E urgently take the patient back to surgery
A)x.
Craniotomy for subdural hematoma evacuation is associated with a high rate of postoperative seizures, which can manifest as convulsive activity or subtle involuntary movements, fluctuating encephalopathy, or aphasia.
Which of the following are the most valuable EEG features to aid
neuroprognostication in a patient with hypoxic-ischemic encephalopathy?
A epileptiform activity and generalized rhythmic delta activity
B epileptiform activity and loss of reactivity
C generalized rhythmic delta activity and greater than 50%
suppression of background activity in the absence of sedation
D greater than 50% suppression of background activity in the
absence of sedation and loss of reactivity
E loss of reactivity and generalized rhythmic delta activity
D)x.
EEF features with poor prognostic:
1 - Epileptiform activity;
2 - Greater than 50% suppression of background activity in the absence of sedation;
3 - Loss of reactivity.
Which of the following somatosensory evoked potential abnormalities
are most likely to be associated with poor prognosis in patients with
hypoxic-ischemic brain injury?
A absent bilateral N9 potentials
B absent bilateral N13 potentials
C absent bilateral N20 potentials
D absent bilateral P18 potentials
E absent bilateral P25 potentials
C)x.
Bilateral absence of cortical peaks (N20 potentials) following stimulation of the median nerves is helpful to predict poor outcome in hypoxic-ischemic brain injury, although the accuracy depends on technical factors and false-positive rates may reach 25%.
Which of the following is associated with poorer outcomes in patients
with hypoxic-ischemic brain injury?
A exposure to glycemic dysregulation
B high cerebral reserve
C low neuron-specific enolase level levels
D shivering during targeted temperature management
E shockable cardiac rhythm
A)x.
Both hypoglycemia and hyperglycemia can worsen the prognosis of patients who have had hypoxic-ischemic brain injury.
High cerebral reserve is an individual characteristic that can lead to a better prognosis.
A shockable cardiac rhythm as the cause of a cardiac arrest portends a better prognosis than a nonshockable cardiac rhythm.
Shivering during targeted temperature management is a good prognostic sign as it reflects
relative sparing of the hypothalamus.
An elevated neuron-specific
enolase, not decreased, predicts poor outcomes.
In a goals-of-care meeting with the family of a patient who is comatose
in the neurocritical care unit, the physician provides the medical facts
regarding the patient’s condition and prognosis but without opinion
or recommendation on the next steps in management. Which of the
following best describes the physician’s primary approach to shared
decision making in this case?
A collaborating
B directing
C facilitating
D informing
D)x.
The physician’s approach to
shared decision making in critical care scenarios may lie on a spectrum
from parentalism (in which the physician directs treatment decisions
with little input from the patient/surrogate) to autonomy (in which the physician merely informs but leaves the decision entirely to the
patient/surrogate).
A family meeting is being held the day after a 72-year-old woman is
admitted to the neurocritical care unit with a large left frontal
hemorrhage. MRI suggests probable cerebral amyloid angiopathy.
Prognosis is poor for recovery, but she is clinically stable. Which of the
following is the most appropriate initial statement after introductions
are made and the purpose of the meeting to provide an update is
explained?
A “After speaking with the doctors in the emergency department,
what is your understanding of what happened to your mother?”
B “How can we help you?”
C “I am sorry to have to share this news with you, but your mother
has had a 50-mL intracerebral hemorrhage that we think is due to
cerebral amyloid angiopathy, which unfortunately has no cure.”
D “I am sure your mother would not want to live in the condition she
is in now, so we want to be sure to respect her wishes in her care
going forward.”
E “I hope things don’t go in this direction, but have you discussed
end-of-life wishes?”
A)x.
A 38-year-old woman is admitted to the neurocritical care unit with
pontine, midbrain, and bilateral thalamic infarcts due to basilar artery
occlusion. She is unresponsive with no brainstem reflexes except for
presence of cough and gag reflexes, spontaneous breathing over the set
ventilatory rate, and posturing to noxious stimulation bilaterally. Her
husband indicates that they are not a spiritual or religious family.
Which of the following strategies is most likely to help the patient’s
family cope with uncertainty about the patient’s potential for recovery?
A advise that substantial improvement will not occur and she will require tracheostomy, feeding tube, and full-time nursing care
B describe a best case, worst case, and most likely case scenario for
the future
C indicate that given her young age, you are hopeful she will regain
consciousness and lead a normal life
D state that you cannot make any predictions about her potential for
recovery but that time will tell
E suggest that they speak with a chaplain or spiritual counselor for
help in processing their emotions
B)x.
Allows family to hope for the best while preparing for the worst.
Which of the following medications is most appropriate to discontinue
after a decision has been made to withhold or withdraw life-sustaining
treatment for a patient in the neurocritical care unit?
A anticonvulsant
B antipsychotic
C antithrombotic
D benzodiazepine
E opioid
C)X.
Medications and other interventions that do not contribute to relief of distressing symptoms should be discontinued.
This generally includes such
medications as antithrombotics, statins, cardiovascular drugs, and
hypoglycemic agents.
Anticonvulsants may be continued for seizure prophylaxis.
Benzodiazepines, opioids, and antipsychotic agents may be continued for relief of pain, dyspnea, anxiety, or agitation.
Which of the following conditions may mimic a presentation of
brain death?
A Guillain-Barré syndrome
B herpes encephalitis
C ischemic stroke
D subarachnoid hemorrhage
E traumatic brain injury
A)x.
Guillain-Barré syndrome can rarely present in a fulminant fashion, with a rapidly progressive course with patients developing flaccid quadriplegia and loss of brainstem function mimicking a patient who is brain dead.
Which of the following is a distinction between the World Brain Death
Project published in 2020 and the 2010 American Academy of
Neurology standards for brain death in adults?
A assessment of motor responses in face and extremities
B assessment of oculovestibular reflex when testing brainstem reflexes
C pH and PaCO2 levels as apnea testing targets
D preoxygenation before apnea testing
E requirement for the number of brain death examinations in adult
patients
C)x.
The World Brain Death Project requires that the “target for apnea testing should be pH <7.3 and PaCO2 >60 mm, unless the patient has preexisting hypercapnia, in which case the target should be ≥20 mm Hg above their baseline, if known.”
The addition of pH as a target addresses the impact of acidosis on medullary chemoreceptors.
A 72-year-old woman presents with a large thalamic hemorrhage. On
hospital day 3, the patient displays no motor activity or brainstem
reflexes, although she does have occasional spontaneous extensor
posturing. The patient has been off propofol and any other sedative
medications for 2 days. The family has noticed the patient’s movements
and, as a result, believes she still has brain function and a possibility for
neurologic recovery. Which of the following statements is correct?
A the clinician has a moral obligation to immediately withdraw
support to the patient regardless of whether family objects to a
brain death evaluation
B consent from family is required before a brain death assessment
C given the presence of spontaneous extensor posturing, significant
doubt exists about whether the patient would meet criteria for
brain death
D hospital lawyers should get involved as early as possible if a family
objects to a brain death evaluation
E taking a multidisciplinary approach with social workers, spiritual
counselors, palliative care specialists, and ethicists can be helpful
when a family objects to a brain death determination
E)x.
It is important to note that
consent from the family is not required before a brain death
assessment.
Which of the following neuromuscular disorders often has early involvement of respiratory muscles?
A acid maltase deficiency
B chronic inflammatory demyelinating polyradiculoneuropathy
(CIDP)
C Duchenne muscular dystrophy
D hyperkalemic periodic paralysis
E myophosphorylase deficiency
Neuromuscular disorders with early involvement of respiratory muscles (with consequent orthopnea and nocturnal hypoventilation):
1 - Acid Maltase Deficiency;
2 - Amyotrophic Lateral Sclerosis;
3 - Myasthenia Gravis.
Which of the following neuromuscular disorders typically causes
distally predominant weakness?
A botulism
B Kearns-Sayre syndrome
C Lambert-Eaton myasthenic syndrome
D myotonic dystrophy type 1
E spinobulbar muscular atrophy
D)x.
Spinobulbar muscular atrophy tends to present with orobulbar, proximally
predominant weakness, or both.
Botulism presents with more diffuse
weakness.
Kearns-Sayre syndrome typically presents with predominantly oculobulbar weakness.
Lambert-Eaton myasthenic
syndrome typically presents with proximally predominant weakness.
Which of the following neuromuscular conditions is associated with
gynecomastia?
A Leigh syndrome
B limb-girdle muscular dystrophy
C myotonic dystrophy type 2
D myotubular myopathy
E spinobulbar muscular atrophy
E)x.
Autoantibodies targeting which of the following voltage-gated ion
channels are most commonly identified in patients with Lambert-Eaton
myasthenic syndrome?
A CASPR2 potassium channel complex
B L-type calcium channels
C LGI1 potassium channel complex
D N-type calcium channels
E P/Q-type calcium channels
E)x.
P/Q in 85%.
Autoantibodies against L-type or N-type calcium channels may be identified in a smaller proportion of patients with LEMS, but are nonspecific, and the presence of these autoantibody types in the absence of P/Q-type VGCC should not be considered supportive of a diagnosis of LEMS.
Potassium-channel autoantibodies
may be associated with various peripheral or central nervous system
manifestations such as neuromyotonia or limbic encephalitis, but not LEMS.
Which of the following constellations of electrodiagnostic findings is
most characteristic of Lambert-Eaton myasthenic syndrome?
A decreased compound muscle action potential (CMAP) amplitude
on initial stimulation of a motor nerve, decrement on 2-Hz to 3-Hz
repetitive nerve stimulation after maximal voluntary contraction,
abnormal jitter on single-fiber EMG
B decreased CMAP amplitude on initial stimulation of a motor nerve,
increment on 2-Hz to 3-Hz repetitive nerve stimulation after
maximal voluntary contraction, abnormal jitter on single-fiber EMG
C decreased CMAP amplitude on initial stimulation of a motor nerve,
no change on 2-Hz to 3-Hz repetitive nerve stimulation after
maximal voluntary contraction, abnormal jitter on single-fiber EMG
D normal CMAP amplitude on initial stimulation of a motor nerve,
increment on 2-Hz to 3-Hz repetitive nerve stimulation after
maximal voluntary contraction, normal jitter on single-fiber EMG
E normal CMAP amplitude on initial stimulation of a motor nerve,
decrement on 2-Hz to 3-Hz repetitive nerve stimulation after
maximal voluntary contraction, normal jitter on single-fiber EMG
B)x.
Patients with Lambert-Eaton myasthenic syndrome should be
counseled to avoid which of the following medication classes?
A cholinesterase inhibitors
B neuromuscular blocking agents
C phosphodiesterase-5 inhibitors
D potassium channel antagonists
E sulfonamides
B)x
avoid medications with neuromuscular blockade properties, such as those used in general anesthesia, macrolide and quinolone antibiotics, and checkpoint inhibitors.
Amifampridine/3,4-diaminopyridine
(potassium-channel antagonist that blocks presynaptic potassium efflux
to prolong depolarization by maintaining calcium influx through
functional voltage-gated calcium channels and optimize acetylcholine
release) and pyridostigmine (cholinesterase inhibitor) are the mainstays of symptomatic therapy for LEMS.
Phosphodiesterase-5 inhibitors
may be used for erectile dysfunction in LEMS.
Which of the following clinical features best distinguishes botulism
from acute inflammatory demyelinating polyradiculopathy (AIDP)?
A flaccid muscle tone
B intact mental status
C lack of sensory involvement
D ophthalmoplegia
E reduced facial expression
C)X.
Which of the following subcategories of myasthenia gravis (MG) is
associated with more severe disease?
A anti–acetylcholine receptor
B anti–muscle-specific tyrosine kinase (MuSK)
C late-onset MG
D ocular MG
E seronegative MG
Anti-MuSK myasthenia gravis (MG) is associated with more
severe disease than other forms.
Late-onset MG tends to have an
overall better outcome than early-onset MG.
Which of the following medications carries a high risk of worsening
myasthenia gravis?
A immune checkpoint inhibitors
B iodinated contrast agents
C macrolide antibiotics
D quinine
E statins
The risk of myasthenia gravis exacerbation with immune checkpoint inhibitors is high, whereas it is moderate (macrolide antibiotics) or low (iodinated
contrast agents, quinine, statins) for the other agents listed.
Which of the following treatments for myasthenic crisis is preferred
due to efficacy and faster onset of action?
A albuterol
B efgartigimod
C IV corticosteroids
D IV immunoglobulin
E plasmapheresis
E)x.
Which of the following idiopathic inflammatory myopathies is
associated with extraocular muscle involvement, myocarditis, and
lymphopenia?
A antisynthetase syndrome
B dermatomyositis
C immune checkpoint inhibitor–related myositis
D immune-mediated necrotizing myopathy
E overlap myositis
C)X
Extraocular muscle weakness is very uncommon in treatable idiopathic inflammatory myopathies; the exception is myositis associated with the use of immune checkpoint inhibitors.
Myocarditis and lymphopenia are also features of immune checkpoint inhibitor–
related myositis.
Which of the following best describes a heliotrope rash associated with
dermatomyositis?
A erythematous rash in sun-exposed areas on the anterior chest
B erythematous scaly plaques over the metacarpophalangeal and
interphalangeal joints
C periorbital and upper eyelid violaceous discoloration
D periungual telangiectasias
E red on white patches with coexistent hypopigmented and
telangiectatic skin lesions
C)x.
A) The V-sign.
B) Gottron papules.
E) Red on white patches with coexistent hypopigmented
and telangiectatic skin lesions describes poikiloderma
Routine screening for cancer is recommended in adults with which of
the following idiopathic inflammatory myopathies?
A antisynthetase syndrome
B COVID-19 myositis
C dermatomyositis
D overlap myositis
E signal recognition particle–antibody immune-mediated
necrotizing myopathy
C)x.
Dermatomyositis and anti-HMGCoa-Redutase: incidence > 40%.
Overlap myositis, anti-SRP and antisynthetase syndrome: low risk. Screening is not recommended.
Which of the following is a first-line treatment for most idiopathic
inflammatory myopathies?
A antitumor necrosis factor agents
B calcineurin inhibitors
C corticosteroids
D IV immunoglobulin
E rituximab
C)x.
Firs-line: corticosteroides.
Second-line: azathioprine,
methotrexate, or mycophenolate - required in most patients for the
steroid-sparing effects.
Third-line: IV immunoglobulin.
Fourth-line: rituximab, calcineurin inhibitors, and cyclophosphamide.
Anti–tumor necrosis factor agents are avoided because they may exacerbate myositis.
Early in the course of inclusion body myositis, which of the following
hand muscles tends to be disproportionately weak?
A abductor pollicis
B flexor digitorum profundus
C flexor digitorum superficialis
D interosseous
E opponens pollicis
B)x.
Adding which of the following inclusion body myositis features to the
presence of finger flexor and quadriceps weakness produces a
relatively high sensitivity for the diagnosis of inclusion body myositis?
A elevated serum creatine kinase
B EMG with early recruitment pattern in affected muscles
C histopathology with endomysial inflammation or rimmed vacuoles
D MRI with fatty infiltration of the anterior compartment of the thigh
E serum anti-NT5C1A antibody
C)x.
The presence of finger flexor and
quadriceps weakness together with either endomysial inflammation or
rimmed vacuoles on histopathology (the European Neuromuscular
Centre 2011 criteria) resulted in an 84% sensitivity for diagnosis of
inclusion body myositis.
On examination of a patient with inclusion body myositis, notable
asymmetric atrophy is commonly seen in which of the following muscles?
A biceps brachii
B forearm extensors
C gastrocnemius
D gluteals
E quadriceps
E)x.
A 5-year-old boy is diagnosed with Duchenne muscular dystrophy with
genetic confirmation that his mother is a carrier. Which of the
following interventions would be important for his asymptomatic
mother?
A pulmonary function testing
B referral to cardiology
C serum creatine kinase level
D spine x-ray
E treatment with oral corticosteroids
B)x.
Should be referred to cardiology given the risk of cardiac involvement even in the absence of skeletal muscle symptoms.
Which of the following signs or symptoms are most commonly seen
early in patients with Duchenne muscular dystrophy?
A muscle fasciculations
B persistent toe-walking
C speech delay
D swallowing dysfunction
E upper extremity fine motor delay
B)x.
Early findings in Duchenne muscular dystrophy include:
1 - Persistent toe-walking;
2 - gross motor delay;
3 - and delayed ambulation or abnormal gait.
Speech delay is less commonly seen early in the course.
As compared to the side effects seen with corticosteroid use, which of
the following adverse effects is more common with deflazacort?
A behavioral symptoms
B cataract formation
C osteoporosis
D peptic ulcers
E weight gain
B)x.
As compared to side effects seen with corticosteroids, deflazacort is associated with a higher risk of cataract formation.
A 22-year-old man reports weakness that began around age 10 years,
and he became nonambulatory at age 18. In addition to weakness, he
has increased tone at his ankles, enlarged calves, 1+ and symmetric deep
tendon reflexes, and mild scoliosis. His serum creatine kinase is
elevated 10 times above normal and an echocardiogram reveals
significant cardiomyopathy. What is the most likely diagnosis?
A Becker muscular dystrophy
B Duchenne muscular dystrophy
C Emery-Dreifuss muscular dystrophy
D Fukutin-related proteinopathy
E intermediate muscular dystrophy
A)x.
Ambulation would be lost before age 13 in Duchenne muscular dystrophy and between 12 and 16 in intermediate muscular dystrophy.
While fukutin and Emery-Dreifuss muscular dystrophies can resemble the dystrophinopathies and be associated with cardiomyopathy, they would not be expected to produce such a high creatine kinase nor enlarged calves.
Which of the following initial symptoms is most likely in a patient with
limb-girdle muscular dystrophy?
A arm fatigue when reaching up
B back pain
C difficulty grasping objects in the hand
D inability to stand on the toes
E weakened cough
A)x.
Initial symptoms at the shoulder girdle include arm fatigue when reaching up and difficulty lifting objects overhead.
Hip girdle symptoms include difficulty getting off the floor and climbing
stairs.
Distal and respiratory weakness typically do not occur until later.
Musculoskeletal pain may develop related to compensatory posture
changes, unstable joints, immobility, or contractures, but would not be a
typical presenting complaint.
In evaluating slowly progressive proximal weakness, current clinical
practice guidelines recommend beginning with which of the following
diagnostic tests?
A electromyography
B muscle biopsy with immunohistochemistry
C muscle ultrasound
D nerve conduction study
E next-generation sequencing panel
E)x.
A patient with slowly progressive proximal weakness has a family
history suggestive of an autosomal recessive limb-girdle muscular
dystrophy. Genetic testing of the patient reveals a variant of uncertain
significance. Which of the following increases the likelihood that the
variant is pathogenic?
A computer models show no impact on genetic functioning
B single heterozygous variant detected
C two heterozygous variants present in one parent
D variant is common in the general population
E Western blot demonstrates absence of involved protein
E)x.
Variants of uncertain significance may be identified in three-fourths of individuals undergoing a next-generation sequencing panel for slowly progressive proximal weakness.
Variants of uncertain significance are more likely to be pathogenic if:
1 - They are uncommon in the general population;
2 - Computational analysis suggests
a deleterious or indeterminate effect;
3 - The number of variants matches
the inheritance pattern;
4 - Mutations are on different parental alleles (for autosomal recessive disease);
5 - Western blot or muscle biopsy
demonstrates absence of the involved protein.
What is one of the key molecular pathomechanisms for myotonic
dystrophy?
A change in expression ratio of contractile proteins
B increased permeability of muscle cell membrane
C loss of function of proteins involved in the structural integrity of
muscle fibers
D loss of muscle-specific tyrosine kinase (MuSK) activity
E sequestration of RNA-binding proteins
E)x.
DMPK and CNBP RNA transcripts with expanded repeats sequester
RNA-binding proteins, which are involved in regulating alternative
splicing of pre-mRNAs.
Which of the following clinical findings commonly develops in later
stages of facioscapulohumeral muscular dystrophy?
A cardiomyopathy
B diplopia
C foot drop
D scapular winging
E signe de cils
C)x.
Most patients with facioscapulohumeral muscular dystrophy eventually develop lower extremity weakness, often first manifesting clinically by foot drop. Proximal lower extremity weakness occurs as well, and approximately 20% of patients eventually become wheelchair dependent.
Scapular winging and signe de cils (failure to bury the eyelashes upon squeezing the eyes shut) are common early signs of the disease.
Cardiomyopathy is not associated with facioscapulohumeral muscular dystrophy, and diplopia is not a typical feature at any disease stage.
The Fazekas scale is a measure of the severity of which of the following
abnormal findings?
A hippocampal atrophy
B lateral ventricle enlargement
C meningeal enhancement
D overall brain atrophy
E white matter hyperintensity
E)x.
