XI.16. A 52-year-old man presents with symmetric burning pain in both
his feet over the past 2 months. His symptoms are intermittent
but severe, occurring approximately twice weekly and lasting
several hours. When he is symptomatic, his feet are hot and turn
bright red. For relief, he immerses his feet in a bucket of ice
water. His feet feel normal between episodes. Which of the fol-
lowing has been shown to be associated with a hereditary form
of this condition?
a. A sodium channel mutation in the SCN4A gene
b. A sodium channel mutation in the SCN9A gene
c. A chloride channel mutation in the CLCN1 gene
d. A calcium channel mutation in the CACNA1A gene
e. A chloride channel mutation in the CLCN2 gene
XI.16. Answer b.
An SCN9A gene mutation of a sodium channel protein
causes an autosomal dominant inherited form of erythro-
melalgia. A mutation in the SCN4A gene causes paramyoto-
nia congenita. A mutation in the CLCN1 gene causes
myotonia congenita, and a mutation in the CLCN2 gene
causes epilepsy. Mutations in the CACNA1A gene cause
familial hemiplegic migraine and 1 form of episodic ataxia.
XI.15. Which of the following is most effective in preventing the development of postherpetic neuralgia?
a. Prevention of inciting shingles activation with the inactivated
recombinant vaccine
b. Prevention of inciting shingles activation with the live, attenu-
ated vaccine
c. Administration of antivirals within 72 hours after the eruption of
shingles
d. Administration of amitriptyline for 3 months at the onset of shingles
e. Administration of gabapentin within 1 week after the onset of
symptoms
XI.15. Answer a.
The inactivated recombinant vaccine (Shingrix) is pre-
ferred over the older live, attenuated vaccine (Zostavax)
given its greater efficacy and duration. The inactivated vac-
cine decreases the risk of shingles by more than 90%, and
if shingles does develop despite the vaccination, the risk of
postherpetic neuralgia is decreased by almost 90%.
XI.14. A 34-year-old woman who had a distal radial fracture 4 months
ago had no neurologic signs or symptoms at the time of the frac-
ture. After surgical fixation of the fracture and immobilization of
the arm in a cast for several weeks, she reported burning dysesthe-
sia and allodynia through the entire hand and distal forearm. Her
hand is swollen, red, and sweaty and has limited movement. The
skin is thin and shiny, and the nails are brittle. Which of the following is most validated for diagnosis of the suspected condition?
a. Electromyography
b. Three-phase bone scan
c. Lumbar sympathetic block response
d. History and examination
e. Thermoregulatory sweat test
XI.14. Answer d.
Complex regional pain syndrome (CRPS) is a clinical diag-
nosis. Objective measurement of temperature and periph-
eral sudomotor function can help in confirming clinical
signs that are challenging to assess definitively at the bed-
side but are not required for the diagnosis. Similarly, a bone
scan shows characteristic changes of CRPS; however, these
changes are not specific or sensitive for CRPS and should
not be used alone to definitively make or exclude the diag-
nosis of CRPS.
XI.13. If a symptomatic patient has a central neuropathic pain syndrome, which of the following clinical features should be demon-
strable in an affected limb?
a. Sensory loss to light touch
b. Impaired temperature sensation
c. Weakness
d. Hyperreflexia
e. Spasticity
XI.13. Answer b.
Central neuropathic pain refers to processes in which the
primary lesion or dysfunction (not the secondary effects)
occurs within the central nervous system. The critical fac-
tor for the development of a central neuropathic pain syn-
drome is that the neurologic lesion causes dysfunction of
spinal-thalamic-cortical pathways, which is clinically evi-
dent as impaired pain (pinprick) and temperature sensation
in the region of pain.
XI.12. A 72-year-old man presents with a 6-week history of daily head-
aches, which are moderately severe. He has scalp pain when
washing his hair and difficulty chewing meat. He had a 10-min-
ute episode of right eye visual loss yesterday. Neurologic exami-
nation findings are normal. The erythrocyte sedimentation rate
is 105 mm/h. What should be the next step in this patient’s care?
a. Ultrasonography of the carotid arteries
b. Computed tomography of the head
c. Aspirin therapy
d. Corticosteroid therapy
e. Temporal artery biopsy
XI.12. Answer d.
The patient’s symptoms and laboratory study results are
concerning for giant cell arteritis. If giant cell arteritis is
suspected, corticosteroid therapy should be initiated as
soon as possible to prevent visual loss. Temporal artery
biopsies can be performed after the initiation of therapy.
XI.11. A 60-year-old Asian woman presents with lancinating pain iso-
lated to the distribution of the right maxillary division of the
trigeminal nerve (V2). On examination she does not have weak-
ness of the jaw or sensation abnormalities of the face. Magnetic
resonance imaging of the brain does not show any abnormali-
ties. Which of the following treatments may require additional
testing before use?
a. Alcohol block
b. Glycerol block
c. Carbamazepine
d. Gabapentin
e. Microvascular decompression
XI.11. Answer c.
Patients of Asian descent who are treated with carbamaz-
epine are at higher risk for Stevens-Johnson syndrome
because that population has a high association for carrying
the human leukocyte antigen (HLA)-B*1502 allele. Testing
for the HLA-B*1502 allele before starting carbamazepine
therapy is important in the Asian population.
XI.10. A 30-year-old woman presented with recurrent thunderclap head-
ache. Computed tomography showed sulcal subarachnoid hemor-
rhage, and magnetic resonance angiography showed diffuse
narrowing of intracranial blood vessels without evidence of aneu-
rysm. Which of the following might be included in treatment?
a. Levetiracetam
b. Nimodipine
c. Gabapentin
d. Imuran
e. Calcium
XI.10. Answer b.
Patients with reversible cerebral vasoconstriction syn-
drome (RCVS) present with recurrent thunderclap head-
ache and have evidence of multifocal, segmental cerebral
vasoconstriction. In addition, patients may have cerebral
ischemia, sulcal subarachnoid hemorrhage, and seizures as
a result of the vasoconstriction. RCVS can be distinguished
from vasculitis by clinical presentation, demographic infor-
mation, and spinal fluid analysis. The mainstay of RCVS
treatment is calcium channel blockers.
XI.9. A 40-year-old woman presents with excruciating side-locked
headaches lasting 10 minutes and occurring 15 times daily. They
are associated with ipsilateral conjunctival injection, tearing,
and rhinorrhea. You suspect paroxysmal hemicrania. What treat-
ment should you try first?
a. Verapamil
b. Sumatriptan
c. Indomethacin
d. Propranolol
e. Oxygen
XI.9.
Answer c.
Paroxysmal hemicrania is an indomethacin-responsive head-
ache condition. According to the International Classification
of Headache Disorders, 3rd Edition, attacks are prevented
absolutely by therapeutic doses of indomethacin.
XI.8. A patient reports sudden-onset occipital headache provoked by
coughing and straining. Magnetic resonance imaging (MRI) is
performed to look for secondary causes. What is the most com-
mon secondary cause that might be found on MRI when a
patient presents with cough headache?
a. Chiari type I malformation
b. Third ventricle colloid cyst
c. Reversible cerebral vasoconstriction syndrome
d. Cerebral aneurysm
e. Carotid artery dissection
XI.8. Answer a.
In approximately 40% of patients with cough headache, the
headache has a secondary cause. Chiari type I malformation
is the most commonly reported secondary cause.
XI.7. A 40-year-old man presents with a history of excruciating, left-
sided periorbital headaches lasting 60 minutes. They occur once
daily at the same time every evening and are accompanied by
ipsilateral tearing and rhinorrhea. The patient prefers to pace
during the headache. What is the most likely diagnosis?
a. Migraine
b. Hemicrania continua
c. Paroxysmal hemicrania
d. Cluster headache
e. Short-lasting unilateral neuralgiform headache attacks with con-
junctival injection and tearing (SUNCT)
XI.7. Answer d.
Cluster headache, which is more common in men, is the
most likely diagnosis given the characteristics of the patient’s
headaches. His headaches are side-locked, located in the
distribution of the ophthalmic division (V1) of the trigemi-
nal nerve, and last from 15 to 180 minutes. A circadian pat-
tern and ipsilateral cranial autonomic symptoms are
described. The patient’s restlessness is more common in
cluster headache than in migraine.
XI.6. Which of the following is a basic principle to be considered when
prescribing an acute migraine medication?
a. Limit use to 5 days weekly
b. Treat as early in the attack as possible
c. If the patient has severe nausea or vomiting, oral medications
should be used
d. If therapy with 1 triptan fails, other triptans will not be effica-
cious
e. Medications of different classes (eg, a nonsteroidal anti-inflam-
matory drug and a triptan) should not be used in combination
XI.6. Answer b.
A migraine should be treated as early in the attack as possi-
ble. This early treatment should be limited to 14 days per
month with simple analgesics (eg, acetaminophen or nonste-
roidal anti-inflammatory drugs) or 9 days per month with
combination analgesics (eg, triptans or ergots). It can be help-
ful to use medications of different classes in combination to
treat migraine. Because patients may have different responses
with different triptans, several triptans should be tried before
their use is avoided. Formulations that are not given orally
are best for patients with severe nausea and vomiting.
XI.5. A 35-year-old woman presents with an 8-month history of daily
headaches. She reports that before the daily headaches began,
she had intermittent headaches that lasted about 12 hours and
were throbbing, unilateral, and associated with nausea. Eight
months ago she began to have a daily headache. She reports that
on about 12 days a month, she has headaches similar to her
previous intermittent headaches, but on the other days, the
headaches are bilateral, mild, and without associated nausea. he takes ibuprofen on 1 to 2 days per week. Which diagnosis
best fits this patient’s headaches?
a. Chronic daily headache
b. Medication-overuse headache
c. Chronic migraine
d. Chronic tension-type headache
e. New daily persistent headache
XI.5. Answer c.
The patient has headaches on at least 15 days per month,
and the headaches on at least 8 days per month meet the
criteria for migraine. This pattern has been going on for at
least 3 months, so the criteria for chronic migraine are met.
XI.4. Which of the following drugs is the first choice for prophylactic
management of tension-type headache?
a. Topiramate
b. Tizanidine
c. Divalproex sodium
d. Amitriptyline
e. Onabotulinum toxin A
XI.4. Answer d.
Amitriptyline is the only medication that has been exten-
sively studied for tension-type headache, so it is the drug of
choice. Tizanidine may be useful, but strong data are lack-
ing. Topiramate, divalproex sodium, and onabotulinum
toxin A have not been found to be beneficial in tension-type
headache.
XI.3. Which of the following is true regarding thunderclap headache?
a. Thunderclap headache reaches peak severity within 5 minutes
after onset
b. Recurrent thunderclap headache may be present in patients with
reversible cerebrovasoconstrictive syndrome
c. If a patient has negative findings on computed tomography (CT)
of the head, all serious causes of thunderclap headache can be
ruled out
d. The location of thunderclap headache is typically occipital
e. Thunderclap headache may be evaluated in the outpatient
setting
XI.3. Answer b.
By definition, a thunderclap headache has an abrupt onset
and reaches maximal intensity in less than 1 minute. This
can be the heralding symptom for serious conditions, such as
subarachnoid hemorrhage, reversible cerebrovasoconstric-
tive syndrome (RCVS), arterial dissection, venous thrombo-
sis, and other conditions. CT of the head is important for
evaluating subarachnoid hemorrhage. However, CT or mag-
netic resonance angiography and venography, lumbar punc-
ture, and magnetic resonance imaging may be necessary for
further evaluation. In patients with RCVS, recurrent thunder-
clap headaches are common.
XI.2. Which statement about headache is false?
a. Headache with Valsalva maneuver is considered a red flag for a
possible secondary cause of headache
b. Migraine affects 18% of all women
c. The diagnosis of migraine requires normal findings on magnetic
resonance imaging of the brain or computed tomography of the
head
d. Tension-type headache is the most prevalent primary headache
condition in the world
e. Pregnancy or postpartum headache is considered a red flag for a
XI.2. Answer c.
The diagnosis of migraine does not require brain imaging.
Brain imaging might be considered if the patient had any of
the red flag symptoms or signs listed in Box 97.1.
XI.1. Which of the following is considered a red flag when taking a
headache history?
a. Unilateral lacrimation
b. Headache is brought on by being upright and resolves with lying
supine
c. Headache causes the patient to avoid routine physical activity
d. Osmophobia
e. Stabbing quality of pain
XI.1. Answer b.
A headache that is brought on by being upright and resolves
with lying supine might suggest a spontaneous cerebrospi-
nal fluid leak. Unilateral lacrimation may be seen in the tri-
geminal autonomic cephalgias. Patients with many types of
headache, including migraine, may avoid physical activity
while they have head pain. Osmophobia can be associated
with migraine headaches. A stabbing quality of pain can
accompany many types of primary headache disorders. The
SNOOP4 red flags should be kept in mind (see Box 97.1).
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