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Uncommon primary headaches
1.
2.
3. ◘ RSA is 38 year old female presented 1.5 years ago by recurrent
attacks of very brief left periorbital pain extending to the face,
each last few seconds and recur several times a day.
◘ O/E, there were severe left conjunctival injection, lacrimation,
ptosis, left eyelid edema, and miosis.
◘ At this moment, we thought about possible trigeminal neuralgia,
cluster headache, cavernous ICA aneurysm, Tolosa Hunt
syndrome, collagenic disorder or RCVS.
◘ She undergo repeated contrasted brain MRI, CT angiography
and battery for collagenic disorders; all were normal.
◘ She received carbamazepine and steroids and fortunately
partially improved but relapse occurred several months later.
4.
5. ◘ Headache is themost commondisordersubjectedto empirical treatment,unnecessary consultationand/orinvestigation.
◘ Migraine.
◘ Other common primary headaches (cluster, TTH).
◘ Secondary headaches which are easily diagnosed.
◘ Less easily diagnosed secondary headaches.
◘ Less common primary headaches:
– Trigeminal autonomic cephalalgias.
– Other rare primary headaches.
6. (I) The Primary Headaches:-
1. Migraine.
2. Tension-type headache.
3. Trigeminal autonomic cephalalgias.
4. Other primary headache disorders.
(II) The Secondary Headaches.
(III) Painful Cranial Neuropathies and Other
Facial Pain.
7. (I) The Primary Headaches:-
1. Migraine.
2. Tension-type headache.
3. Trigeminal autonomic cephalalgias.
4. Other primary headache disorders.
(II) The Secondary Headaches.
(III) Painful Cranial Neuropathies and Other
Facial Pain.
8.
9. ◘ TACs are a group of primary headache disorders
characterized by:
(1) Short-lived recurrent unilateral headache attacks.
(2) Ipsilateral cranial autonomic features.
(3) Restlessness/agitation.
(1) Cluster headache (episodic & chronic).
(2) Paroxysmal hemicrania (episodic & chronic).
(3) Short-lasting unilateral neuralgiform headache attacks:
- With conjunctival injection and tearing (SUNCT).
- With cranial autonomic symptoms (SUNA).
(4) Hemicrania continua (remitting & unremitting subtypes).
(5) Probable trigeminal autonomic cephalalgia
10.
11. ◘ CH is a unilateral headache syndrome with ipsilateral cranial
autonomic features and/or restlessness.
◘ It is the most common TACs, 3 times more common in males
with a prevalence of 1: 1000.
◘ Attacks of CH usually occur in series lasting for weeks or
months (cluster periods or bouts) separated by remission
periods usually lasting months or years.
◘ It is a highly regular in its recurrence (clocklike).
◘ A circadian pattern is seen with most patients having
headaches at defined times of the night. In episodic type, a
circannual pattern is also seen, with most patients having
headaches every year or every other year in spring and
autumn.
12. A. At least five attacks fulfilling criteria B – D.
B. Severe/very severe unilateral orbital, supraorbital and/or
temporal pain 15 - 180 minutes (untreated).
C. Either or both of the following:
1. At least one of the following symptoms or signs,
ipsilateral to the headache:
a) conjunctival injection and/or lacrimation
b) nasal congestion and/or rhinorrhea
c) Eyelid edema
d) forehead and facial sweating
e) miosis and/or ptosis
2. Sense of restlessness or agitation.
D. Frequency: 0.5 to 8 per day.
E. Not better accounted for by another ICHD 3 diagnosis.
13. ◘ At least two cluster periods lasting from 7
days to 1 year (when untreated) and
separated by pain free remission periods ≥
3 month.
◘ CH attacks occurring for one year or longer
without remission, or with remission
periods lasting < 3 months.
14. ◘ Three brain systems are particularly prominent: -
- The trigeminal-vascular system.
- The cranial autonomic system.
- The hypothalamus.
◘ These 3 systems are linked through: -
- The trigeminal autonomic reflex.
- The hypothalamic-trigeminal nucleus connections.
- The hypothalamic-autonomic connections.
◘ Voxel based morphometry study, detected an isolated
gray matter increase in the bilateral inferior-posterior
hypothalamus.
◘ Neurogenic inflammation of the walls of the cavernous
sinus has been also proposed.
15.
16. ◘ Cluster headache exhibits attacks of longer duration
with relatively low attack frequency.
◘ An individual cluster attack (15 - 180 minutes)
overlaps in duration with a paroxysmal hemicrania
attack (2 - 30 minutes).
◘ Approximately one-third of patients with CH have
interictal dull pain that mimics hemicrania
continua.
18. ◘ Typical migrainous features such as photophobia,
phonophobia, facial allodynia, and nausea are seen in up to
50% of CH cases.
◘ Many patients with CH reported to have auras and
premonitory symptoms.
◘ Likewise, > 50% of patients with migraine have cranial
autonomic symptoms.
◘ The misdiagnosis as migraine seems to occur more frequently
in women (CH is more common in men).
◘ CH is best differentiated from migraine based on shorter
duration (fewer than 4 hours), higher frequency (>1 per
day), rapid escalation (reaches peak pain within minutes),
and restlessness during the headache.
19. ◘ Despite the well defined criteria, diagnostic delay is common
while most patients are referred back and forth between
primary care physicians, dentists, ophthalmologists,
otolaryngologists, and other specialists.
(1) Brain MRI:
- The European Headache Federation recommended brain MRI for
all cases of CH including coronal imaging on the pituitary
gland and MRA on head and neck.
(2) Lab investigations:
- CBC, ESR (temporal arteritis), creatinine (possible lithium use),
pituitary hormonal assay and ECG (adjustment of verapamil
dose).
(3) Polysomnogram:
- Nearly 60% of CH patients have obstructive sleep apnea.
20. ◘ Management protocol for CH includes: -
(1) Acute management.
(2) Transitional medications.
(3) Preventive medications.
◘ An indomethacin trial is often warranted to rule
out hemicrania continua and paroxysmal
hemicrania.
◘ Therapies such as acupuncture and chiropractic
have not shown benefit.
21. (1) High-flow oxygen:
- Oxygen should be administered at 100% via a nonrebreather
mask at a rate of 12 – 15 L/min for at least 20 minutes and
trialed several times before being considered ineffective.
(2) Triptans: (in particular sumatriptan and zolmitriptan)
- Quicker routes of are preferred, with SC triptans being the most
effective, followed by nasal and then oral.
(3) Noninvasive vagus nerve stimulation (VNS):
- FDA approved for acute management of episodic CH (not
approved for chronic CH).
◘ Oxygen and noninvasive VNS are suitable for patients with
high frequency and need their use multiple times per day or
those with triptan contraindications.
22. ◘ Refer to preventive medications that could be uptitrated
quickly and used for short periods of time and are most
useful in two situations:
(1) As the lone preventive for patients with short headache
cycles.
(2) As a bridge in patients with longer headache cycles while
uptitrating other preventives.
(1) Greater occipital nerve blocks:
- With local anesthetic plus steroids.
(2) Steroids:
- Oral steroids, tapered over 3 weeks is generally recommended
(not > 3 courses /year). No consensus about the best form
or dose of steroids.
(3) Ergotamine tartrate:
- Controversial (level B in the European Federation).
23. (1) Verapamil:
- The drug of choice in a daily dose of 480 - 720 mg divided
into 3 doses (immediate-release formulation are
better).
- Cardiac conduction abnormalities are a feared
(lengthening the PR interval), so a pretreatment,
regular and after dose increase ECGs are
recommended.
(2) Second line treatment:
- Topiramate.
- Lithium.
- Melatonin.
- Valproate.
- Baclofen.
24. (1) Revise your diagnosis.
- Possible symptomatic CH or secondary headache
(polysomnogram, pituitary assay and imaging).
(2) Neurostimulation procedures:
- Sphenopalatine ganglion stimulation.
- Occipital nerve stimulation.
- Hypothalamic DBS.
◘ Indications: -
- 2-years of daily or almost daily attacks.
- Extensive medication trials.
◘ The American Headache Society supports sphenopalatine
ganglia stimulation while DBS seems ineffective.
◘ Occipital nerve stimulation still under-researched.
25.
26. ◘ Calcitonin gene–related peptide (CGRP) has an active role
throughout the 3 systems involved in cluster headache
pathogenesis (the trigeminovascular system, the
trigemino-parasympathetic reflex, and central
modulating systems).
◘ Monoclonal antibodies targeting CGRP (galcanezumab and
fremanezumab) have been tested in CH, with promising
results for the episodic form.
◘ Somatostatin receptor agonist (octreotide 100 mg, SC), a, is
potentially beneficial in acute treatment of CH.
◘ Pituitary adenylate cyclase-activating polypeptide
(PACAP) inhibitors are emerging treatment for migraine
and CH.
27.
28. ◘ Shares many features with CH but is slightly shorter in
the duration of attacks and completely responsive to
indomethacin.
◘ Less prevalent than CH with a prevalence rate of < 0.5 /
1000, slightly more common in females, with onset
between 30 – 40 years.
◘ The pain peaks in < 10 minutes, it is sharp, stabbing, or
throbbing and is located in the orbital, supraorbital,
and temporal areas.
◘ Unlike in cluster headache, restlessness is less common,
and the headaches are rarely circadian.
◘ Could be triggered by alcohol, neck movements, or
pressure over the neck or greater occipital nerves.
29. A. At least 20 attacks fulfilling criteria B–E
B. Severe unilateral orbital, supraorbital and/or temporal pain
lasting 2–30 minutes.
C. Either or both of the following:
1. at least one of the following symptoms or signs, ipsilateral
to the headache:
a) conjunctival injection and/or lacrimation.
b) nasal congestion and/or rhinorrhea.
c) eyelid edema.
d) forehead and facial sweating.
e) miosis and/or ptosis.
2. a sense of restlessness or agitation.
D. Occurring with a frequency of >5 per day.
E. Prevented absolutely by therapeutic doses of
indomethacin.
F. Not better accounted for by another ICHD-3 diagnosis.
30. ◘ At least two bouts lasting from seven days to
one year (when untreated) and separated by
pain-free remission periods of ≥3 months.
◘ Occurring without a remission period, or with
remissions lasting <3 months, for at least one
year.
◘ Indomethacin 75 - 225 mg/day divided in 3 doses
is often effective.
◘ For patients not tolerating indomethacin,
verapamil, topiramate, sphenopalatine ganglion
and greater occipital nerve blocks have been
tried with variable success rates.
31. ◘ SUNCT and SUNA, have prevalence of 0.05 – 1 / 1000,
more common in male between 40 – 70 years.
◘ The pain is maximal is usually stabbing, sharp, or
throbbing.
◘ Individual attacks are brief (1 – 600 seconds), peaks in 2 –
3 seconds and must occur once a day but may be as
frequent as 100 times / day.
◘ Attacks may occur in series with no interictal pain, or
they can occur in a sawtooth pattern with a
background pain lasting minutes that is punctuated by
stabs on top.
32. A. At least 20 attacks fulfilling criteria B–D.
B. Moderate or severe unilateral head pain, with orbital,
supraorbital, temporal and/or other trigeminal
distribution, lasting for 1–600 seconds and occurring
as single stabs, series of stabs or in a saw-tooth
pattern.
C. At least one of the following five cranial autonomic
symptoms or signs, ipsilateral to the pain:
1. conjunctival injection and/or lacrimation.
2. nasal congestion and/or rhinorrhea.
3. eyelid edema.
4. forehead and facial sweating.
5. miosis and/or ptosis.
D. Occurring with a frequency of at least one a day.
E. Not better accounted for by another ICHD-3 diagnosis.
33. -
-
◘ Both of the following, ipsilateral to the
pain:
1. conjunctival injection.
2. lacrimation (tearing).
◘ Not more than one of the following,
ipsilateral to the pain:
1. conjunctival injection.
2. lacrimation (tearing).
◘ Episodic SUNCT / SUNA:
- At least two bouts lasting from seven days to one
year (when untreated) and separated by pain-
free remission periods of ≥3 months.
◘ Chronic SUNCT / SUNA:
- Occurring without a remission period, or with
remissions lasting <3 months, for at least one
year.
34. ◘ Paroxysmal hemicrania or CH could be confused with the
sawtooth pattern of SUNCT / SUNA.
◘ Primary stabbing headache is also among the differential
diagnosis but lack the autonomic features, and the location
of pain is changeable.
◘ Trigeminal neuralgia is the most important differential
diagnosis which is brief, unilateral and could be triggered by
tactile stimuli, but lack the cranial autonomic features and
has a refractory period after an attack is triggered by tactile
stimuli which is lacking in the typical SUNCT / SUNA
attack.
◘ Symptomatic SUNCT / SUNA have been reported in pituitary
and posterior fossa tumors.
◘ MRI pituitary, MRI brain with trigeminal nerve as well as
vascular loop protocols may be considered.
35. ◘ Lamotrigine in a dose of 100 – 200 mg/day is the drug
of first choice.
◘ Proposed second-line treatments include topiramate,
gabapentin, carbamazepine, oxcarbazepine,
duloxetine or short term steroid use.
◘ In chronic disabling cases, continuous IV lidocaine
provide temporary relief, 1 – 3.5 mg/kg/h is highly
effective, however typical protocols include a 1-
week admission with continuous telemetry.
36. ◘ Hemicrania continua shares many features with the other
TACs and with migraine, characterized by continuous
unilateral pain and autonomic features which is completely
responsive to indomethacin.
◘ The true prevalence is unknown, but the disorder is highly
uncommon representing about 0.8% of patients with daily
headaches.
◘ Hemicrania continua has 2 types:
(1) Remitting subtype with headache is not daily or
continuous, but interrupted (without treatment) by
remission periods of ≥24 hours.
(2) Unremitting subtype with headache is daily and
continuous for at least one year, without remission periods
of ≥24 hours.
37. A. Unilateral headache fulfilling criteria B – D.
B. Present for >3 months, with exacerbations of moderate or
greater intensity
C. Either or both of the following:
1. at least one of the following symptoms or signs, ipsilateral
to the headache:
a) conjunctival injection and/or lacrimation
b) nasal congestion and/or rhinorrhea
c) eyelid edema
d) forehead and facial sweating
e) miosis and/or ptosis
2. a sense of restlessness or agitation, or aggravation of the
pain by movement
D. Responds absolutely to therapeutic doses of indomethacin.
E. Not better accounted for by another ICHD-3 diagnosis.
38. ◘ Indomethacin, by definition, has dramatic response. The dose is
75 mg 3 timed / day which withdrawal could be tried after
6-months.
◘ For patients who cannot tolerate indomethacin, several other
medications have been proposed including ibuprofen,
celecoxib), melatonin, gabapentin, verapamil, and
topiramate.
◘ Some patients have had positive responses to greater occipital
nerve blocks by botulinum toxin injections, and occipital
nerve stimulation.
◘ Symptomatic causes of hemicrania continua include cerebral
venous sinus thrombosis and brain metastases.
39. 3 : 1 2 : 3 3 : 2 1 : 2
1 – 8 5 – 50 1 – 100 Constant
15 – 180 2 – 30 0.01 – 10 > 3 months
90 : 10 35 : 65 10 : 90 15 : 85
> 80% Rare Rare Rare
Yes Yes No Yes
Yes Yes No No
No Yes Yes No
No No Yes No
70% No effect No effect No effect
90% 20% Rare effect No effect
Rare effect 100% No effect 100%
- Male : female
- Frequency (/day)
- Duration (min)
- Episodic: chronic
- Circadian rhythm
Alcohol
Nitroglycerin
e
Neck move.
Cutaneous
Oxygen
Sumatriptan 6
mg/SC
Indomethacin
Triggers
40.
41. ◘ Headaches associated with physical exertion:
(1) Primary cough headache.
(2) Primary exercise headache.
(3) Primary headache associated with sexual activity.
(4) Primary thunderclap headache.
◘ Headaches attributed to direct physical stimuli:
(5) Cold-stimulus headache.
(6) External-pressure headache.
◘ Epicranial headaches (head pain over the scalp)
(7) Primary stabbing headache.
(8) Nummular headache.
(9) Epicrania fugax.
◘ Miscellaneous primary headache disorders
(10) Hypnic headache.
(11) New daily persistent headache (NDPH).
42. A. At least two headache episodes fulfilling criteria
B–D
B. Brought on by and occurring only in association with
coughing, straining and/or other Valsalva maneuver.
C. Sudden onset
D. Lasting between one second and two hours
E. Not better accounted for by another ICHD-3 diagnosis.
◘ Primary cough headache is a rare condition, accounting for
< 1% of all headache patients.
◘ Headache is usually bursting (not pulsatile), reaches its peak
almost immediately, and then subsides over several
seconds to a few minutes.
◘ Common associated symptoms include vertigo, nausea and
sleep abnormality.
43. ◘ 40% of cases are symptomatic due to a tight posterior fossa
including Arnold–Chiari type-I, spontaneous intracranial
hypotension, subdural hematoma, posterior fossa tumors,
midbrain cyst, basilar impression, platybasia, carotid or
vertebrobasilar diseases, cerebral aneurysms and
reversible cerebral vasoconstriction syndrome.
◘ Cough headache in pediatric patients should be considered
symptomatic until proved otherwise.
◘ Treatment:
- Reassurance could be enough but some cases may need
indomethacin (50–200 mg/day) and/or acetazolamide.
44. A. At least two headache episodes fulfilling criteria B and C
B. Brought on by and occurring only during or after strenuous
physical exercise
C. Lasting <48 hours
D. Not better accounted for by another ICHD-3 diagnosis
◘ Primary exercise headache occurs particularly in hot
weather, high altitude or extreme exertion.
◘ Headache is usually pulsatile, but usually holocranial.
◘ Most investigators hypothesize vascular origin due to venous
or arterial distension during physical exercise (internal
jugular vein valve incompetence).
◘ Treatment: Indomethacin is usually effective (50–200
mg/day). Propranolol (1–2 mg/kg/day) could be used in
case of frequent attacks.
45. A. At least two episodes of pain in the head and/or neck
fulfilling criteria B–D
B. Brought on by and occurring only during sexual activity
C. Either or both of the following:
1. increasing intensity with increasing sexual excitement
2. abrupt explosive intensity just before or with orgasm
D. Lasting from one minute to 24 hours with severe intensity
and/or up to 72 hours with mild intensity
E. Not better accounted for by another ICHD-3 diagnosis.
◘ Primary headache associated with sexual activity may be
pre-orgasmic or orgasmic and is not associated with
disturbance of consciousness, vomiting or visual, sensory
or motor symptoms, whereas symptomatic sexual
headache may be.
46. ◘ On the first onset of headache with sexual activity, it is
mandatory to exclude SAH, intra- and extracranial
arterial dissection
◘ Multiple explosive headaches during sexual activities should
be considered as headache attributed to RCVS until
proven otherwise by angiographic studies.
◘ Treatment:
- Reassurance about the benign course of the disorder.
- Anticipatory treatment with indomethacin (50–200 mg/day),
30 minutes prior to sexual activity is usually effective.
- Daily propranolol (1–2 mg/kg/day) or verapamil may be
beneficial for longer time prevention (off label).
47. A. Severe head pain fulfilling criteria B and C
B. Abrupt onset, reaching maximum intensity in <1
minute
C. Lasting for ≥ 5 minutes
D. Not better accounted for by another ICHD-3
diagnosis.
◘ Primary thunderclap headache should be a diagnosis
of last resort, reached only when all organic causes
have been excluded.
49. -
-
A. At least two acute headache episodes fulfilling criteria
B and C.
B. Brought on by and occurring only during application
of an external cold stimulus to the head
C. Resolving within 30 minutes after removal of the cold
stimulus
D. Not better accounted for by another ICHD-3
diagnosis.
A. At least two episodes of acute frontal or temporal
headache fulfilling criteria B and C.
B. Brought on by and occurring immediately after a cold
stimulus to the palate and/or posterior pharyngeal wall
from ingestion of cold food or drink or inhalation of
cold air
C. Resolving within 10 minutes after removal of the cold
stimulus
D. Not better accounted for by another ICHD-3 diagnosis.
◘ Cold stimulus headache is similar to Raynaud’s, upon exposure to cold, vasoconstriction followed by dilatation with consequent triggering of the vessel
wallnociceptors.
◘ Treatment: Avoidanceof thetriggersand/orslowingestionofice-creamorcoldfood.
50. -
-
A. At least two episodes of headache fulfilling
criteria B–D
B. Brought on by and occurring within one hour
during sustained external compression of the
forehead or scalp
C. Maximal at the site of external compression
D. Resolving within one hour after external
compression is relieved
E. Not better accounted for by another ICHD-3
diagnosis.
A. At least two episodes of headache fulfilling criteria
B–D
B. Brought on by and occurring only during sustained
external traction on the scalp
C. Maximal at the traction site
D. Resolving within one hour after traction is relieved
E. Not better accounted for by another ICHD-3
diagnosis.
51. A. Head pain occurring spontaneously as a single stab or series of
stabs and fulfilling criteria B and C.
B. Each stab lasts for up to a few seconds.
C. Stabs recur with irregular frequency, from one to many per day.
D. No cranial autonomic symptoms.
E. Not better accounted for by another ICHD-3 diagnosis.
◘ Attack frequency is generally low, with one or a few per day. In
rare cases, stabs occur repetitively over days.
◘ Primary stabbing headache involves extra-trigeminal regions in
70% of cases. It may move from one area to another, in either
the opposite hemicranium.
◘ Primary stabbing headache is more common in migraineurs.
◘ Treatment: Reassurance, otherwise indomethacin. Gabapentin,
melatonin and celecoxib are also tried.
52. A. Continuous or intermittent head pain fulfilling criterion B
B. Felt exclusively in an area of the scalp, with all of the
following four characteristics:
1. sharply contoured
2. fixed in size and shape
3. round or elliptical
4. 1–6 cm in diameter
C. Not better accounted for by another ICHD-3 diagnosis.
◘ The painful area may be localized in any part of the scalp,
but is usually in the parietal region.
◘ Multiple nummular areas may be found in the same
patient.
◘ The affected area commonly shows variable combinations
of hypoesthesia, dysesthesia, paresthesia, allodynia
and/or tenderness.
◘ Treatment: NSAIDs, gabapentin or botulinum injection.
53. A. Recurrent headache attacks fulfilling criteria B–E
B. Developing only during sleep, and causing wakening
C. Occurring on ≥10 days/month for >3 months
D. Lasting from 15 minutes up to four hours after waking
E. No cranial autonomic symptoms or restlessness
F. Not better accounted for by another ICHD-3 diagnosis.
◘ Distinction from cluster headache, sleep apnea, nocturnal
hypertension, medication overuse and intracranial
disorders is necessary.
◘ Hypnic headache usually begins after age 50 years, but may
occur in younger people.
◘ Hypnic headache is usually bilateral and is not related to
sleep stage.
◘ Lithium, caffeine, melatonin and indomethacin have been
effective treatments in several reported cases.
54. A. Persistent headache fulfilling criteria B and C
B. Distinct and clearly remembered onset, with pain becoming
continuous and unremitting within 24 hours
C. Present for >3 months
D. Not better accounted for by another ICHD-3 diagnosis.
◘ NDPH is unique in that it is daily from onset in individuals
without a prior headache history.
◘ If criteria met chronic migraine, chronic tension headache or
hemicrania continua, they will be the default diagnosis.
◘ In case of medication overuse, NDPH cannot be made unless
the onset clearly predates the medication overuse.
◘ Secondary headaches such as post-traumatic headache and
headache high or low CSF should be ruled out.
55. A. A complaint of a sudden loud noise or sense of explosion in
the head at the wake-sleep transition or upon awakening
during the night.
B. Abrupt arousal following the event, often with a sense of
fright.
C. Not associated with significant pain
◘ Treatment:
- Exploding head syndrome is benign, and reassurance is the
cornerstone of treatment as the condition remits over
time.
- Treatment with clomipramine, topiramate, clonazepam,
amitriptyline, and nifedipine have been reported.
56.
57. ◘ RCVS may cause single or recurrent episodes of
thunderclap headache.
◘ Usually start during straining e.g. sexual activity,
coughing, laughing, etc. or substance abuse e.g.
cocaine or amphetamine.
◘ Angiography shows string and beads appearance
which may start few days after the initial
presentation with headache.
◘ CT may show focal sulcal SAH.
◘ Transient focal deficits (TIAs like) due to cerebral
ischemia may occur after some delay.
58.
59. ◘ Avoidthereadymade diagnosisor empirical treatment.
◘ Recognizecluesthat coulddiagnose anunusual headache.
◘ Be balanced between prescribing unnecessary investigation /
consultationor bypassingrequiredones.
◘ Every new-onset or side locked headache should be considered
secondary oneuntilprovenotherwise.