Download 185 HEADACHE Robert Kaniecki, MD Director, the Headache

HEADACHE
Robert Kaniecki, MD
Director, the Headache Center
Chief, Headache Division
Assistant Professor of Neurology
University of Pittsburgh
OVERVIEW
The lifetime prevalence of headache is more than 90%. In recent populationbased surveys of U.S. adults, nearly 25% annually report recurrent episodes of severe
headache and 4% daily or near-daily headache. Prescription or non-prescription products
are used by 9% of U.S. adults each week to treat headache, matching hypertension as the
primary reason for medication use. The majority of patients presenting to physicians will
have primary headache syndromes such as tension-type, cluster, and migraine. Less than
2% of patients in office and 4% of patients in emergency department settings will be
found to have headaches secondary to significant pathology.
Recurrent headaches provoke consultation when they are debilitating, frequent, or
associated with worrisome neurologic or systemic symptoms. Episodic tension-type
headache annually affects 38% of U.S. adults yet rarely requires medical attention given
the typical absence of disability or concerning symptoms. Cluster headache generally
leads to significant disability and assorted autonomic features, but it is uncommon in
office practice due to low population prevalence (less than 0.1%). Migraine headache is
disproportionately represented in office settings because of its high prevalence, (13% of
U.S. adults) significant disability, and common association with neurological and
gastrointestinal symptoms. Research has shown that more than 90% of initial headache
consultations in a primary care office setting will involve patients experiencing attacks
meeting International Classification of Headache Disorders (ICHD-II) criteria for
migraine, while only 3% experience solely episodic tension-type headache.
DIAGNOSTIC CLASSIFICATION AND MANAGEMENT
In the diagnostic system designed by the International Headache Society,
headaches are broadly divided into two main categories: primary headaches (headache is
the problem) and secondary headaches (headache is a symptom of a problem). (Table 1)
Since headache may arise from conditions that range from benign to catastrophic,
the initial step in headache assessment requires screening for secondary origins. Acute
headache is often the most prominent complaint from patients experiencing a host of
serious nervous system disorders, including subarachnoid hemorrhage, bacterial
meningitis, encephalitis, or stroke. Subacute headache may also be associated with an
assortment of worrisome disorders, including brain tumors, intracranial hypertension, or
temporal arteritis. Thorough history combined with general and focused neurological
examinations are mandatory. Neuroimaging procedures or serum/cerebrospinal fluid
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analyses are required when one of the red flags of secondary headache presentations is
encountered. (Table 2) The available data are insufficient to recommend either computed
tomography or magnetic resonance imaging as a more sensitive modality. The routine
use of electroencephalography in the evaluation of headache patients is no longer
warranted.
The majority of patients in primary care settings will experience episodic primary
headache disorders. Traditional diagnosis is founded on a symptom-base paradigm
initially developed by the IHS for purposes of clinical research. Significant symptom
overlap among the primary headaches and between primary and secondary headaches has
raised concerns regarding the clinical specificity of such a system.
Migraine
Epidemiology and Clinical Features
More than 90% of patients who have recurrent headache on presentation to
primary care offices or emergency departments have migraine. This extraordinarily high
figure results from the high prevalence of migraine, which affects approximately 13% of
adults in the United States, and the disabling nature of the condition. Migraine usually
begins in late childhood or early adolescence and follows various courses: the headache
may go into remission after a few years, recur in cycles of variable headache activity for
many years or decades, or evolve into a chronic and more refractory state. Migraine is
more common in preadolescent boys than girls but becomes three times more common in
adult women than men. Prevalence peaks in the fifth decade of life, drops significantly in
the sixth and seventh decade, and is exceedingly uncommon in later decades. Although a
typical migraine episode consists of a unilateral, throbbing headache accompanied by
photophobia, phonophobia, and nausea, there is extensive variability in the clinical
expressions of migraine, often leading to misdiagnoses of “tension headache” in the
presence of bilateral steady pain or “sinus headache” when the discomfort is a frontal or
facial pressure. (Table 3)
Migraine is preceded or accompanied by focal neurologic symptoms termed aura
in up to 30% of patients. The most common aura is a visual experience consisting of a
flashing light or an enlarging blind spot rimmed with a shimmering edge or jagged lines
in the peripheral vision. An aura is defined as “typical” if it involves any combination of
visual, hemisensory (usually face and hand), or language abnormalities with each
symptom developing over a minimum of 5 minutes and lasting a maximum of 60
minutes. The associated migraine usually occurs within 1 hour, but some auras do not
progress to head pain. Auras should be investigated further if they extend beyond 60
minutes, involve any focal motor weakness (“hemiplegic migraine”), or display
brainstem symptoms such as diplopia, dysphagia, ataxia, vertigo, dysarthria or
synchronous bilateral sensory dysfunction (“basilar migraine”).
Migraine is subclassified not only based on the presence or absence of aura, but
also based on average monthly frequency of headache occurrences. Most have headache
fewer than 15 days per month and are classified as “episodic” migraine. Patients with
“chronic” (previously “transformed”) migraine have headache at least 15 days per month
for more than three months, with the individual headache episodes meeting criteria for
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migraine on at least 8 of those days. Chronic migraine affects 2% of the world
population and results in substantial individual disability and enormous societal costs.
Transformation of episodic into chronic migraine has been shown to occur at a rate of 3%
per year in the general population and 14% per year in clinic-based populations. Evidence
suggests risk factors for such transformation include:
 Older age
 Major life changes or significant stressors
 Female sex
 Low education/socioeconomic status
 Head/neck trauma
 Obesity
 Presence of comorbid pain, sleep, or psychiatric disorders
 High caffeine or nicotine intake
 Overuse of acute headache medications
The final risk factor for migraine transformation, overtreatment with acute medication, is
now termed “medication overuse” headache (MOH) instead of “rebound” headache.
MOH is present in up to 80% of individuals with chronic migraine presenting to medical
attention, and thus it is critical that all patients be questioned about the use of both
prescription and non-prescription products during a headache history. Several studies
assessing the relationship between migraine transformation and acute medication use
have drawn the same conclusions: opiates (critical exposure 8 days per month) and
barbiturates (critical exposure 5 days per month) are associated with high rates of
migraine progression, while nonsteroidal anti-inflammatory agents (NSAIDs) and
triptans induce progression only in those with frequent episodic migraine (10-14 days per
month) but not overall.
Migraine Pathophysiology
Migraine is a heritable biological disorder of the central nervous system characterized by
baseline hypersensitivity to external stimuli and intermittent episodes of head pain. The
condition appears to be genetically heterogeneous, with recent research identifying
genetic alterations in migraine with and without aura, adding to prior work establishing 3
different genetic abnormalities in the rare subform of migraine known as familial
hemiplegic migraine. Functional imaging techniques have linked migraine aura with a
slowly propagating electrical wave of brain depolarization and hyperpolarization known
as cortical spreading depression (CSD). Subsequent activation of the trigeminal vascular
system plays a role in migraine headaches, but the exact mechanism and the precise
connection with CSD are unknown. Basic science and clinical data support the concept
that migraine pain arises from activation of trigeminal nerve fibers that innervate the
dura. When stimulated these neurons release a number of neurochemicals (substance P,
calcitonin gene–related peptide, or neurokinins) which provoke vasodilation of dural
vessels, local sterile inflammation, and pain. Pain signals generated on the dural surface
are then transmitted and modulated by central nervous system structures, involvement of
which may help explain migraine persistence (recurrence, status migrainosus) or
progression.
Migraine Management
Evidence from well-designed placebo-controlled trials supports the efficacy of NSAIDs,
dihydroergotamine mesylate, and triptans in the management of acute migraine. Despite
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their widespread use, there is no evidence supporting the efficacy of butalbital
compounds and little evidence supporting the efficacy of isometheptene compounds in
the treatment of acute migraine. Opioids are recommended only when migraine-specific
agents and NSAIDs are contraindicated or in rare cases of rescue when such agents have
failed. Antiemetics are helpful adjunctive therapies when used with NSAIDs or triptans.
NSAID’s may be helpful in cases of mild or moderate migraines, with the data best for
ibuprofen, naproxen, aspirin, and the combination product of aspirin-acetaminophencaffeine. Among the triptans sumatriptan, rizatriptan, zolmitriptan, naratriptan,
almotriptan, eletriptan, and frovatriptan all have tablet formulations with established
efficacy in moderate-to-severe migraine. Both sumatriptan and zolmitriptan are available
in nasal preparations. The agent with greatest speed of onset and efficacy is subcutaneous
sumatriptan. In addition to acute headache management, patients with migraine should
receive lifestyle recommendations regarding headache prevention. Regulation of sleep
and meal patterns, adequate amounts of hydration and exercise, limitation of caffeine and
other stimulants, and the intake of certain nutritional supplements (magnesium, butterbur
root) are often encouraged. If migraine attacks occur more frequently than 5-6 days per
month a course (6-12 month) of daily migraine preventive medication is often
considered. The selection of a drug should be based first on efficacy, with consideration
given to coexisting psychiatric or medical disease, patient preference, and patient
adherence. Evidence in episodic migraine prevention is strongest for propranolol (60-240
mg/d), timolol (5-30 mg/d), amitriptyline (30-150 mg/d), divalproex sodium (500-2000
mg/d), and topiramate (100-200 mg/d). Recent trials have documented the efficacy of
only 2 agents for chronic migraine, oral topiramate and injectable onabotulinumtoxinA.
Tension-type headache (Table 4) is the least distinct of the primary syndromes,
defined by the absence of associated features. The pain is mild and moderate in intensity,
generally bilateral, and nonpulsatile. It typically remains unchanged or improves with
physical activity. Stress is listed as the most common trigger. Due to its limited
disability, episodic tension-type headache rarely is the basis for consultation in primary
care or specialty settings. Acetaminophen, aspirin, and NSAID’s have all been shown to
be effective for acute management. Preventive medications are not particularly helpful,
but amitriptyline is the drug of choice.
Cluster headache (Table 5) is distinguished by its distinctive temporal pattern of
grouped headache attacks recurring over several weeks or months. The episodes are
characterized by minutes-to-hours of intense unilateral periorbital pain associated with
nasal or ocular autonomic features. Due to its low population prevalence, cluster
headache is also an infrequent consultation in primary care. Given the brevity and
severity of attacks, acute management requires approaches that are rapidly effective.
Inhaled oxygen at 10-15 liters/minute for 10-15 minutes and subcutaneous sumatriptan
are the approaches of choice. Corticosteroids may help shorten a cycle of cluster, while
verapamil is the preventive drug of choice for more lengthy cycles.
There is no scientific evidence to support the existence of an independent entity
known as “sinus” headache. Although acute sinusitis may be associated with headache,
the symptom of headache is generally one of the minor features in the presentation. In the
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absence of acute sinusitis, there is no clear evidence that sinus inflammation irritation
results in headache. Interestingly the phenomenon of “sinus headache” appears to be a
uniquely American phenomenon. A recent large study of sinus headache in the United
States population revealed that approximately 90% of individuals with the self-diagnosis
or clinician-diagnosis of “sinus headache” actually meet criteria for migraine.
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Table 1 Headache Classification (ICHD-II)
Migraine headache
Tension-type headache
Cluster Headache and other trigeminal autonomic cephalgias
Miscellaneous primary headaches
Headache attributed to head and/or neck trauma
Headache attributed to cranial or cervical vascular disorder
Headache attributed to non-vascular intracranial disorder
Headache attributed to a substance or its withdrawal
Headache attributed to infection
Headache attributed to disorder of homeostasis
Headache or facial pain attributed to disorders of extracranial structures
Headache attributed to psychiatric disorder
Cranial neuralgias and central causes of facial pain
Table 2 Red Flags for Secondary Headache Disorders (Nasty Nine)
1. First/worst headache
2. Abrupt onset headache
3. Progression or fundamental change in pattern of headache
4. New headache in those less than 5 years old, greater than 50 years old
5. New headache with cancer, immunosuppression, or pregnancy
6. Headache with syncope or seizure
7. Headache triggered by exertion/valsalva/sex
8. Neurologic symptoms greater than one hour in duration
9. Abnormal general or neurological examination
Table 3
Migraine Headache
Migraine without Aura
Migraine with Aura
A. At least five attacks fulfilling B-D
B. Attacks lasting 4-72 hours (untreated or
Unsuccessfully treated)
C. At least two of the following characteristics:
1. Unilateral location
2. Pulsating quality
3. Moderate or severe intensity (inhibits or
Prohibits daily activities)
4. Aggravation by routing activity
D. At least one of the following:
1. Nausea and/or vomiting
2. Photophobia and phonophobia
A. At least two attacks fulfilling B
B. At least three of the following characteristics:
1. One or more fully reversible aura symptom
indicating focal cerebral cortical and/or
brain stem dysfunction
2. At least one aura symptom develops
gradually over more than four minutes or
two or more symptoms occur in succession
3. No aura symptom lasts more than 60
minutes. If more than one aura symptom
is present, accepted duration is
proportionally increased
4. Attack follow aura with a free interval of
less than 60 minutes (may also begin
before or simultaneously with the aura)
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Table 4 Tension-Type Headache
Tension-Type Headache
A. Headache duration 30 minutes-7 days, either for < 15 days per month (“episodic”) or
for > 15 days per month for over 6 months (“chronic”)
B. At least two of the following pain characteristics:
1. Pressing/tightening quality
2. Mild or moderate severity (may inhibit, but does not prohibit activities)
3. Bilateral location
4. No aggravation by walking stairs or similar routine physical activity
C. Both of the following:
1. No vomiting
2. No more than one of the following:
nausea, photophobia, phonophobia
Table 5 Cluster Headache
Cluster Headache
A. At least five attacks fulfilling B-D
B. Severe unilateral orbital, supraorbital, and/or temporal pain lasting 15 to 180 minutes
Untreated
C. Attack is associated with at least one of the following signs on the side of pain:
Conjunctival injection
Lacrimation
Nasal congestion
Rhinorrhea
Forehead and facial sweating
Miosis
Ptosis
Eyelid edema
Pacing or restlessness
D. Frequency: from one every other day to eight per day
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