This PPT focuses on the diagnosis and treatment of the primary headache disorders, with special emphasis on migraine, the headache most likely to bring patients to physicians and pharmacists. warning signs of the ominous headache, which, although rare, can herald a life-threatening condition. Clinical characteristics of the primary headache types, migraine, tension-type headache, and cluster headache, are described
2. Objectives
Be able to identify common types of primary headache syndromes seen in primary care:
Migraine
Cluster Headache
Muscle Tension Headache
Avoid triggers contributing to medication overuse headache
Differentiate between treatment options for migraines, both acutely and as a preventative
Be aware of emerging therapies for migraines
3. Common Secondary Headaches
Medication Overuse Headache
Giant Cell Arteritis
Low Pressure Syndromes (CSF leak)
High Pressure Syndromes (venous occlusion,
mass, edema)
Infectious Headache
Traumatic Head or Neck etiologies
Acute Stroke or Blood
Nocturnal Hypoxia
4. Prevalence
½ - ¾ of adults have suffered from a headache within the past year.
30% have had a migraine in the past year.
1.7-4% have had a headache at least 15 days or more each month.
Severe headache / migraine reported in 1out of 6 Indian over a 3 month period.
9.7% males, 20.7% females
Fifth leading cause of ER visits
Third leading cause among females age 18-44
1.3% of outpatient visits
Third highest cause nationwide of years lost to disability [YLD].
Headache. 2018 Apr;58(4):496-505. doi: 10.1111/head.13281. Epub 2018 Mar 12.
World Health Organization fact sheet, 2016
8. Medication Overuse Headache
Also known as Rebound Headache
Defined as:
Headache present on >15 days/month.
Regular overuse for >3 months of one or more drugs that can be taken for acute and/or symptomatic
treatment of headache.
Headache has developed or markedly worsened during medication overuse.
Ther Adv Drug Saf. 2014 Apr; 5(2): 87–99.
9. Medication Overuse Headache
Can be precipitated by many agents:
NSAIDs
Acetaminophen
Aspirin
Caffeine
Triptans
Opioids
Butalbital
Ergotamines
10.
11. Pathophysiology
Etiology is uncertain given multiple medication triggers
Present in patients predisposed to headache
Consideration given to chronic low serotonin, elevated CGRP and central sensitization
Ther Adv Drug Saf. 2014 Apr; 5(2): 87–99.
12. Medication Overuse Headache
Goal: withdrawal of offending agent
Baseline headache pattern can therefore be established
Achieved by one of three methods:
Abrupt withdrawal
Gradual wean
Steroid taper – data does not prove superiority
After successful wean, relapse is 20-40%
Limit future abortive use to no more than twice weekly in susceptible patients
15. Introduction
Prevalence:
Women 25% (lifetime)
Men 8% (lifetime)
Highest from 25-50 years of age
Genetics
About 70% of migraineurs have a positive family history in a first-degree relative
Unknown mode of transmission
17. Migraine
The International Classification of Headache Disorders, 3rd edition
A. At least five attacks fulfilling criteria B–D
B. Headache attacks lasting 4–72 hours (when untreated or unsuccessfully treated)
C. Headache has at least two of the following four characteristics:
1. unilateral location
2. pulsating quality
3. moderate or severe pain intensity
4. aggravation by or causing avoidance of routine physical activity (e.g. walking or climbing stairs)
D. During headache at least one of the following:
1. nausea and/or vomiting
2. photophobia and phonophobia
E. Not better accounted for by another ICHD-3 diagnosis.
Cephalalgia 2018, Vol. 38(1) 1–211
22. Time Is Critical in Preventing Migraine
From Becoming Full-blown
Within minutes of a
migraine being triggered,
the peripheral neurons
that innervate meningeal
blood vessels become
sensitized
If migraine is left untreated,
those peripheral pain
neurons activate and
sensitize central neurons,
leading to central
sensitization
Central sensitization
signifies full-blown migraine,
when central neurons are
continually firing and the
attack becomes more
difficult to treat
Harvard Research Suggests:
A Sequence of Events Leads to Central Sensitization
23. STAGES OF MIGRAINE
Adapted from Cady RK. Clin Cornerstone. 1999;1(6):21-32.
Phases of a Migraine Attack
Premonitory/
Prodrome
Aura Mild Moderate to
Severe HA Postdrome
Pre-HA Post-HAHeadache
Time
Intensity
25. Aura
15% of patients
Episode of focal
neurologic changes
Develop over 5 to 15
minutes & last up to
60 minutes
Visual, weakness,
numbness, confusion
26. Headache
Headache lasts hours to days
Migraine head pain unilateral in 56 – 68% of patients
90% of patients have coexisting nausea
Constitutional symptoms common
28. Treatment philosophy
If the pain can be stopped early, the cascade of pain responses can be
controlled
Headache needs to be caught before central sensitization occurs
Patients may receive the greatest benefit from their migraine medication
if they:
Practice early intervention
Use a fast-acting migraine medication
30. Treatment Options
Two Treatment Approaches
• Acute therapy
Work quickly to relieve migraine pain and other symptoms
Are taken only at migraine onset
• Preventative therapy
Prevent or reduce the number of migraine attacks
Are taken on a daily basis
33. Acute Treatment
Triptans
Selective 5-HT1B/1D agonists
Block actions of 5-HT such as dilation of cranial arteries/AV anastomoses, neurogenic dural plasma extravasation
Use early!
More effective in mild/moderate pain
Caution about rebound
37. Acute Treatment
Ergotamine tartrate
Available for over 50 years
Vasoconstrictors
Oral, SL, IV, PR
Caution about rebound, dependence
Contraindicated:
CVD
CAD
PVD
Severe HTN
Sepsis
CKD
Hepatic disease
Pregnancy
38. Acute Treatment
OTC agents
Cautious of rebound!
Opioids are NOT considered appropriate abortive agents except in cases
of last resort.
39. Status Migrainosus
Migraine lasting greater than 72 hours in duration
Refractory to conventional treatment
Steroid burst – oral methylprednisolone, prednisone
“Headache cocktail”:
Ketorolac 60mg IM
Diphenhydramine 50mg IM
Prochlorperazine 10mg IM
Patient must have a driver
40. Prophylactic Treatment
Indicated in patients with:
>2 migraines per month
Attacks lasting for several days per week
Severity/frequency that critically impacts patient’s daily life
Abortive therapies are contraindicated, ineffective, overused, not tolerated
Uncommon migraine type (hemiplegic, basilar, prolonged aura, migrainous infarction)
41. Prophylactic Treatment
Start low and go slow!
Adequate trial with adequate dose
Consider comorbid conditions when choosing a medication
May add a second medication
48. Tension-Type Headache:
Diagnostic Criteria
At Least 10 Episodes Fulfilling the Criteria Below
Olesen J. Cephalalgia. 1988;8(Suppl 7):1-96.
Two of the following: AND
Associated Symptoms
No nausea or vomiting
Photophobia and
phonophobia are
absent, or one but
not the other
is present
Description of Headache
Pressing/tightening quality
(nonpulsating)
Mild or moderate intensity
(may inhibit, does not prohibit
activities)
Bilateral location
No aggravation by walking up
stairs or similar routine physical
activity
Headache
lasting
30 minutes
to 7 days
Both of the following:
51. Cluster Headache: Diagnostic Criteria
At Least 5 Attacks Fulfilling the Criteria Below
Olesen J. Cephalalgia. 1988;8(Suppl 7):1-96.
Associated Symptoms
One of the Following
Description of Headache
All of the Following:
Severe
Unilateral orbital,
supraorbital, and/or
temporal location
Lasts 15 to
180 minutes
(untreated)
Conjunctival
injection
Lacrimation
Rhinorrhea
Nasal congestion
Forehead and facial sweating
Miosis
Ptosis
Eyelid edema
Frequency
of attacks:
1 every
other day
to 8 per
day
Present on the Pain Side:
AND
52. Cluster Headache
Location: strictly unilateral, often periorbital or temporal
Pain characteristics: constant, severe, burning, or boring
Frequency: 1-6(+) per day
Demographics: Males : Females 6 : 1
Duration: 15-180 minutes
Associated symptoms: autonomic symptoms – (ipsilateral to pain)
tearing, rhinorrhea, conjunctival injection, eyelid edema, ptosis,
pupillary miosis, restlessness
54. Red Flags of Secondary Headache
Arousal from sleep or precipitated by valsalva
Fever, neck stiffness with limited ROM
Significant postural component
New focal deficit or seizure
Hx of head injury
New thunderclap headache (peak intensity w/in 5 minutes)
New headache in HIV, cancer, elderly, or pregnant patient
Papilledema
Temple tenderness, jaw claudication, or fever >50 yr
55. When to image a Headache?
If hx of migraine & no red flags, imaging is NOT warranted
If no hx of migraine but diagnostic criteria met & no red flags, imaging is NOT warranted
IF atypical headache, consider imaging case by case
If red flags, Consensus opinion:
MRI brain w/o gadolinium is more sensitive
CT head w/o contrast is more sensitive for acute blood
56. Work up in Setting of Red Flags
“Every headache does not need every evaluation”
Exertional headaches CTA or MRA
New deficit not consistent with aura MRI without contrast
Focal Tenderness in elderly +/- jaw claudication ESR or CRP
Obese w/ visual complaints dilated eye exam
Thunderclap headache CT
Fever, meningismus CT and lumbar puncture
High pressure features MRV or CTV
57. What if Patient Demands Imaging?
CT imaging is very low yield in routine headache
cases
Counsel patients on risk of imaging and chance
of a distracting incidental finding
1 in 8100 risk of cancer for routine head CT in
women and 1 in 11,080 in men
Evans RW. Diagnostic testing for the evaluation of headaches. Neurol Clin.
1996;14;1-26.
58. Case Review
28 yr obese female presents with 1 month of increasing headaches that
are frontal in nature with phonophobia and light sensitivity, often worse in
the morning.
She also reports vague transient visual obscurations throughout the
day with position change.
Upon questioning, she also has some pulsatile tinnitus. Your exam
reveals an obese female with a nonfocal exam.
Your aren’t confident in your funduscopic exam but you cannot see
spontaneous visual pulsations.
59. What features suggest this is not migraine?
1. Visual obscurations with position change
40%
2. Exclusively Frontal Nature
9%
3. Absence of Spontaneous Venous Pulsations
9%
4. All of the Above
43%
60. Answer: D All of the above
Diagnosis: Idiopathic Intracranial
Hypertension
61. Idiopathic Intracranial
Hypertension: Initial Work up
Send for dilated eye exam if you cannot be
certain of papilledema
Urgent (within 48 hours) MRI/MRV of brain to
exclude mass or sinus thrombosis
Referral for LP for opening pressure and neuro
consult for definitive treatment
62. Trigeminal Autonomic Cephalgias:
Not your Mother’s Migraine
Primary headaches w/ brief episodes of SEVERE unilateral
headaches w/ ipsilateral AUTONOMIC features
Within the group of TACs, difficult to distinguish
Distinction from MIGRAINE is important because
-TAC Headaches are disabling
-Treatment Strategies are different
-Misdiagnosis can be costly
63. What are the Trigeminal
Autonomic Cephalgias?
Highest Attack Lowest Attack
Frequency_______________________________________ Freq
ency
Short lasting
Neuralgiform
Headache
Paroxysmal
Hemicrania
Cluster Hemicrania
Headaches Continua
(SUNCT/SUNA)
Shortest Duration_______________________________Longest Duratio
64. Clinical Features of TACS
Pain is knife like, boring, or stabbing
SEVERE to VERY SEVERE pain
Site is often temple or orbit
Duration of attacks are shorter than migraine on the
order of minutes (except HC)
Autonomic features are always present with attacks
Often episodic or clustering
65. Autonomic Features of TAC:
Requires > 1 ipsilateral
Conjunctival injection
Lacrimation
Nasal congestion or discharge
Miosis
Ptosis
67. Do you need to Image the TACs?
YES!!! Non-urgent MRI brain w/o contrast. Lesions
in or around the pituitary can mimic TACs
Persistent INTER-ATTACK autonomic features
require CTA brain and neck emergently
TACs can mimic carotid dissection
68. Role of Primary Care
Recognize TAC
Order appropriate imaging (MRI for all, CTA for
persistent autonomic exam findings)
Initiate abortive and bridge therapies
Consult electronically or formally with neurology
69. Case Presentation:
A 44 yo man presents with right sided, knifelike, periorbital attacks waking him
from sleep.
He reports nasal congestion and watering of the right eye with the attacks. The
attacks peak quickly, are intolerable making him restless, and seem to relent
within 20-30 minutes.
He has had 5 attacks mostly nocturnally in 2 weeks but none prior. His
neurologic and general medical exam are normal, but on medication review you
can see he has a new prescription for Tadalafil in the last month.
70. What is the likely Diagnosis?
1. Spontaneous Carotid Dissection
6%
2. Hypothalamic Mass
0%
3. Cluster Headache
66%
4. Paroxysmal Hemicrania
28%
71. Answer? Cluster Headache
Nocturnal attack predominance
Short duration (15-180 mins)
Autonomic features during attack
Male predominance 1:3
Alcohol, NTG, or PDE-5 inhibitors can triggers
72. Work-up: New Cluster Headache
Non-urgent MRI brain w/o gadolinium
EKG to screen for heart block for utilization of CCB
Consider consult electronically or formally with neurology
73. Treatment: Cluster Headache
Abortive: 1st Line: Trial of high flow 02 (10-15 L
via nonrebreather) prn onset of attack
2nd Line: Sumatriptan 4-6 mg SQ or
20 mg nasal spray up to bid
Bridge Therapy: Prednisone, 60-80 mg/day taper over 2-
4 weeks.
Preventive: Verapamil 240-480 mg/d divided in 3 doses,
short acting preferred, titrate slowly
74. Case Presentation:
A 56 yr female presents with 4 months of steady, 3/10 side-locked
headaches with superimposed attacks of severe, stabbing temple pain 3-
4x week lasting 2-4 hours without nausea,photophobia, phonophobia
During the severe attacks, she has a perception of a foreign body in her left
eye and left eyelid appears “droopy”.
She has tried rizatriptan and sumatriptan with minimal response and takes
amitriptyline 50 mg qhs with no reduction in frequency after 8 weeks.
She does not use additional analgesics.
75. What is the likely Diagnosis?
1. Giant cell Arteritis
10%
2. Chronic Migraine
6%
3. Hemicrania Continua
78%
4. Medication Overuse Headache
6%
76. Answer? Hemicrania Continua
Often misdiagnosed as migraine
Side locked steady headache with
superimposed severe unilateral attacks
Autonomic features during severe attacks which
can last hours to days
Female predominance 2:1
Uniquely responsive to indomethacin
77. Work-up: New Hemicrania Continua
Non-urgent MRI brain w/o gadolinium
Serum creatinine for planned indomethacin use
Consider formal or electronic consult with
neurology
78. Treatment: of Hemicrania Continua
Abortive: Indomethacin up to 300 mg daily
(often requires higher than FDA approved
maximum daily dose of 150 mg).
Preventives: topirimate, melatonin, occipital
nerve blocks and occipital nerve stimulators
79. Case Presentation
34 yr female with pmhx of anxiety, insomnia and migraine w/o aura
presents with a 5 day history of her typical migraine to your clinic.
She is tearful and overwhelmed after trying home strategies of rizatriptan
plus ibuprofen for 3 doses over 2 days.
She appears uncomfortable but has a nonfocal exam.
80. Diagnosis? Status Migrainosus
Description:
A debilitating migraine attack lasting for more than 72
hours.
Diagnostic criteria:
Features of Migraine without aura typical of previous
attacks except duration
Headache has both:
unremitting for >72 hours and severe intensity
Not attributed to another disorder
81. Status Migrainosus Treatment Pearls
In future, treat typical migraine attack quickly & early to
avoid central sensitization
Recurrence w/in 24 hours = effective therapy with TOO SHORT of
HALF LIFE! Change to LONG-ACTING triptan (frovatriptan) or repeat
second dose of initial medication (table 1-1)
Failed Response to Initial Appropriate therapy = Novel or
combination RESCUE Strategy needed
Consider using combination of lower risk therapies which
can be synergistic
82. In-office Rescue Therapies: Initial Steps
Step 1: Start an IV and hydrate
Step 2: Provide a dopamine receptor antagonist, IM or IV (risks of
akathisia, dystonia, and hypotension):
metoclopramide 10 mg IV
promethazine 12.5-25 mg IM or IV
prochlorperazine 10 mg IV
Step 3: Consider a repeat trial of DHE or triptan unless cardiac risks or
max dose already received
Sumatriptan 6 mg SQ or 20 mg intranasal
Dihydroergotamine 0.5-1 mg IV
83. In-office Rescue treatments: Step 4
Abortive Agent Dosing and route Risks/comments
Hypotension
Gastritis
Magnesium Sulfate 500-1000 mg IV
Ketorolac 30-60 mg IM or IV
400-1200 mg IV
Sodium Valproate Risk of acute
hyperammonemia if on TPX
Methylprednisolone 100-200 mg IV Risk of avascular necrosis, data
mixed
Dexamethasone 4-16 mg IV Risk of avascular necrosis,
evidence in HA >72 hours
84. Case presentation
32 yr F with migraine since 16 yr, frequency 2-4x/month until 1 year ago
with now nearly 25 days of headache a month,15 of which are severe.
Often awakening her in the morning with her typical migraine features
(unilateral, nausea, photophobia) and other days having more mild diffuse
headache with allodynia.
She has used abortive combination of , aspirin, caffeine for 7 years and
currently uses 4-6 pills on bad days and 2 pills on good days with
intermittent use of ibuprofen 600 mg.
She is inconsistently taking propranolol 20 mg bid.
Her neurologic and general exam including fundi are entirely normal.
85. What factors have increased the frequency of her headache?
1. Type of Abortive compound used
2%
2. Frequency of use of abortive
15%
3. Pre-existing headache type
0%
4. All of the above
84%
15
87. Diagnosis: Medication Overuse
Headache (MOH)
Headache > 15 days a month
Regular overuse of abortive treatments for > 3 months
Pattern has worsened during medication overuse
Headache improves within 2 months of removing
overuse
Preventives FAIL to reduce headaches
88. MOH Approach and Treatment
Withdraw Abortive (wean barbiturates and opioids, stop
triptans, NSAIDS, DHE, OTCs abruptly)
Treat Withdrawal Headache (steroid taper)
Amplify Preventive (use evidence base migraine
preventives)
Re-Introduce selective, infrequent (<2x/week) and
appropriate abortive
Encourage complimentary therapies and overall
reduction in triggers
89. A child with pulsatile headache and vomiting
A 6 years and 10 months child, was admitted to vomiting and nonfebrile unilateral headache.
Neurologic examination had normal results. The episodes were preceded by a sensation of
sickness, and lasted about 5–10 minutes each. Pallor, poorly defined abnormal ocular
movements, and transitory unresponsiveness were also reported by his parents.
After the episode, the child asked to sleep.
Acetaminophen and ibuprofen were prescribed to Control symptoms. BUT NOT RESPONDE
Al episode observed during clinical examination: the child reported a sudden
feeling of sickness and a severe unilateral pulsatile headache, followed by nausea.
Left eyelid myoclonus followed, and the child described a short-lasting sensation of blindness.
Then his head turned toward the right and he became unresponsive for about 20 seconds.
Soon after, he vomited and became bradycardic (sinus rhythm, 35– 40 bpm).
90. Questions for consideration:
1. What is the differential diagnosis?
2. What features of the history help make certain entities more or less likely?
3. What testing would you obtain at this point to confirm the diagnosis?
4. What is the prognosis for this patient?
5. Would you prescribe a treatment, and, if yes, which one?
91. D/D ; Intracranial mass (tumor, bleed, infection) and encephalitis
Migraine (mainly basilar migraine), gastroenteritis, vagal syncope, cyclic vomiting
syndrome, intoxication, and partial seizures (occipital or temporal lobe epilepsy).
Vascular syndromes (Klippel-Trenaunay-Weber, arteriovenous malformations of the
brain), familial dysautonomia (e.g., Riley-Day syndrome), breath-holding spells of early
infancy progressing to isolated syncope, postural orthostatic tachycardia syndrome
(POTS), and metabolic diseases.
92. The diagnosis of autonomic seizures is suggested by the episodic
recurrence of unexplained vomiting or abdominal pain, migraine, or other
autonomic symptoms, with EEG showing focal OCCIPITAL seizure activity.
93. A 33-year-old woman with severe postpartum occipital headaches
A 33-year-old woman with history of occasional “migraines” complained of severe occipital headache,
following an uncomplicated full-term vaginal delivery under epidural anesthesia.
This headache was qualitatively and quantitatively different from her usual headaches.
The diagnosis of low intracranial pressure headache related to inadvertent dural puncture was considered
and 2 epidural autologous blood patches were performed with no relief.
One week postpartum she presented to US with complaints of poor concentration, difficulty in finding
words, getting dressed, and feeding herself, and left arm numbness.
Examination showed a blood pressure of 179/119 mm Hg, poor attention span, apraxia, and decreased
sensation in the left hand. General physical examination was unrevealing.
94. Head MRI (day 0) showed fluid-attenuated inversion recovery (FLAIR)
hyperintensities and diffusion restriction with positive apparent diffusion coefficient
(ADC) map in the right parietal lobe and in the splenium of the corpus callosum.
The diagnosis of posterior reversible encephalopathy syndrome (PRES)
ON the third hospital day, she became cortically blind and mute, and had motor
perseverations and left-sided weakness.
Repeat head MRI showed marked worsening with lesions involving the cortex and
subcortical white matter of the parietal, posterior frontal, and occipital lobes,
bilaterally.
Question for consideration:
1. What is the differential diagnosis?
95. The differential diagnosis of multifocal infarcts in the distribution of many
vascular territories is wide.
Emboli from heart and aorta, disseminated intravascular coagulopathy,
thrombotic thrombocytopenic purpura, moyamoya disease, vasculitis, or
viral/bacterial/fungal infections and primary CNS angiitis.
Question for consideration:
1. What studies/tests should be performed??
RCVS