OVERVIEW: What every practitioner needs to know
Are you sure your patient has a headache? What are the typical findings for this disease?
Headache or cephalgia is pain anywhere in the region of the head or neck.
Head pain – Determining the location, quality, duration, and frequency of head pain, along with associated symptoms, helps classify which type of headache the patient is having.
Location: unilateral versus bilateral versus entire head; frontal versus occipital versus temporal
Quality: pounding or throbbing, squeezing or tightness, stabbing or shooting
Duration: seconds to days
Frequency: sporadic, monthly, daily, multiple times per day; may also relate to specific times (eg, only with menstrual cycles)
Associated symptoms: See below under each headache subtype
Certain types of headaches can present without head pain
Headaches are classified by the International Headache Society’s International Classification of Headache Disorders (ICHD), which published the second edition in 2004. Headaches can be generally subdivided into primary headaches, secondary headaches, and cranial neuralgias/central and primary facial pain.
Primary headachesare those types in which there is no other identifiable etiology for the headaches. These are the most common type of headaches. They include migraine, tension, cluster and other trigeminal autonomic cephalgias (TAC). Some would also include chronic daily headache in the primary category. There are other rare primary headaches as classified by ICHD-2 that will not be discussed here.
Secondary headaches are those in which another etiology for the pain (symptom) can be found. These will not be discussed here.
Cranial neuralgiasare head and facial pain disorders that are short in duration but have severe pain in an anatomic distribution of the affected cranial nerve. These are exceedingly rare in children and are always, especially in children, a diagnosis of exclusion.
Migraine is one of the most common types of headaches in children and adults. It is high in prevalence with high disability and is one of the primary neurologic reasons for emergency department visits for children and adults.
In general, migraine can be subdivided into two broad categories:
Other migraine classification groups include
Childhood periodic syndromes that are precursors to migraine
Retinal migraine—not discussed here
Complications of migraine—for example, status migraine, migraine infarction, migraine-triggered seizure—not discussed here
Probable migraine—not discussed here
For more information on retinal migraine, complications of migraine, and probable migraine see ICHD-2.
Migraine Diagnostic Criteria—Pediatric (patients up to 18 years old):
1. Less than five attacks fulfilling features 2 through 4
a) Headache attack lasting 1 to 72 hours
b) Pulsating quality
c) Moderate to severe intensity
d) Aggravated by routine physical activities
3. At least 1 of the following accompanies the headache:
a) Nausea and/or vomiting
b) Photophobia and/or phonophobia (may be inferred from their behavior)
For patients over 18 years of age, there are somewhat different criteria:
2. Duration must be greater than 4 hours
3a. Unilateral only
4b. May not infer from behavior
Migraine without aura—The most common type of migraine. It typically has more frequent attacks and is typically more disabling than other types of migraine.
Can be associated with menstrual cycles
With frequent enough attacks, can be classified as chronic (see below)
Can be related to medication overuse
Migraine with aura—Less common than without aura but often those with aura will also have attacks without aura. The aura is generally a neurologic or sensory symptom(s) that occur(s) prior to or at onset of migraine. Patients may develop an aura without migraine. Auras gradually build over minutes and last 5 to 60 minutes.
Visual Auras—flickering/flashing lights, spots, lines, colors, temporary loss of vision
Sensory Auras—smell, sound, numbness, pins and needles sensation
Speech Auras—any speech disturbance from their baseline
Motor Weakness—if this is accompanied by another aura (see above) and migraine then the diagnosis of hemiplegic migraine is appropriate; if there is a positive family history the diagnosis is familial hemiplegic migraine, otherwise it is sporadic.
Basilar-Type Migraine (a subset of migraine with aura)—Migraine that has aura symptoms originating from the brainstem or bilateral hemispheres but without weakness. Must have at least two of the following:
Visual symptoms if both eyes in all fields
Decreased level of consciousness
Childhood periodic syndromes that are precursors to migraine—Grouping of three syndromes that when seen in children often will lead to migraine development later in life. These syndromes do not necessarily have headaches as part of their clinical picture.
Cyclical Vomiting—Recurrent episodic attacks of vomiting and significant nausea that are often stereotypical. Each attack lasts 1 hour to 5 days and during the attack emesis must occur at a rate of 4 times an hour for 1 hour and there must be at least five attacks over time. There can be no cause found for the emesis and the patient must be symptom free between attacks.
Abdominal Migraine—Idiopathic recurrent episodes of abdominal pain seen typically in school-aged children. The pain is midline/periumbilical or hard to localize, is dull in quality, moderate to severe in intensity, lasting 1 to 72 hours, and must be accompanied by at least two of the following: anorexia, nausea, vomiting, pallor. Five episodes must have occurred to make this diagnosis.
Benign Paroxysmal Vertigo of Childhood—At least five attacks of recurrent, brief, self-limiting attacks of vertigo in previously healthy children. While not part of the diagnosis criteria, the patient should have normal neurologic, audiologic, and vestibular examination between episodes and also should have a normal electroencephalogram (EEG).
The most common type of primary headache seen in 30% to 80% of people. Often referred to as muscle headache, stress headache, ordinary/common headache.
In general, tension headache can be subdivided into two broad categories:
Episodic—can be infrequent or frequent
Chronic (see chronic daily headache)
Tension Headache Diagnostic Criteria:
1. 10 attacks fulfilling features 2 through 4: episodic infrequent is 1/month, episodic frequent is 1 to 15/month for 3 months
2. Headache attack lasting 30 minutes to 7 days
3. Headache has at least two of the following four features:
a) Bilateral location
b) Squeezing or pressing/tightening quality
c) Moderate to severe intensity
d) Not aggravated by routine physical activities
4. At least 1 of the following accompanies headache:
a) No nausea and/or vomiting
b) Can have photophobia or phonophobia but not both
Chronic Daily Headache
Chronic daily headaches can be migraine or tension type headaches. It is important to exclude medication overuse (rebound) headache from overuse of analgesics.
For chronic migraine, diagnostic criteria above for migraine still apply.
More than15 migraines/month for 3 consecutive months without medication overuse being a confounding factor
If the individual migraine lasts more than 3 to 5 days, then it would constitute status migrainosis
It is possible to have both status migrainosis and chronic migraine
For chronic tension headaches, diagnostic criteria above for tension headache still apply.
On average, greater than 15 headaches/month for 3 or more months
Headache may be continuous and if lasts more then 1 day then would count as separate days towards 15
Trigeminal Autonomic Cephalalgias (TACs)
This group of primary headaches incorporates headaches that are found to be brief, repetitive episodes of intense headache associated with autonomic, usually parasympathetic signs and trigeminal activation. These are overall rare in children as well as the general population.
This group includes
SUNCT (short-lasting, unilateral, neuralgiform headache with conjunctival injection and tearing)
Cluster—Severe attacks of pain that is sharp, drilling, boring, or stabbing; localized to the orbital, periorbital, or temporal region. The pain is always unilateral and does not change sides during an attack. Each attack can last minutes to hours and can occur many times a day. May be episodic or chronic.
Must include two of the following:
ipsilateral tearing and/or conjunctival injection
ipsilateral nasal congestion and/or rhinorhea
ipsilateral eyelid edema
ipsilateral facial sweating
ipsilateral miosis or ptosis
restlessness or agitation
SUNCT- SUNCT stands for short-lasting, unilateral, neuralgiform headache with conjunctival injection and tearing. TAC is seen rarely in children. These headaches are short (seconds to a few minutes) sharp stabbing orbital or periorbital pain that is always accompanied by conjunctival injection and almost always tearing. Other autonomic symptoms may be present. They may occur several hundred times a day.
Paroxysmal hemicrania—These pains are similar cluster headaches and is relatively rare in children. Key differentiation is absolute responsiveness to indomethacin. The attacks are generally unilateral, severe, sharp stabbing pain in the temporal or superorbital region with the autonomic symptoms listed above for cluster headache. The attacks last 2 to 30 minutes and can occur many times a day. They can be episodic or chronic.
What other disease/condition shares some of these symptoms?
Headaches can be primary or secondary in nature; the key is differentiating between the two so an underlying etiology is not missed. This is accomplished by thorough history and physical examination in conjunction with utilizing the headache classification system set forth in ICHD-2.
Possible conditions/diseases that can present as headaches:
Idiopathic Intracranial Hypertension – pseudotumor cerebri
Infection—chronic/acute meningitis or any other CNS infection, sinusitis, otitis, and viral including influenza
Drug Intoxication—illicit, prescription, over-the-counter, and medication overuse
Trauma—concussion, neck strain/injury, intracranial hematoma, and postsurgical
Vascular Disorder—stroke/TIA, intracranial hemorrhage including subarachnoid, vascular malformation including aneurysm, AVM, or fistula, venous thrombosis, vasculitis, and inherited such as CADASIL or MELAS
Intracranial Hypotension—post–lumbar puncture, cerebrospinal fluid (CSF) leak
Homeostasis Changes—hypertension, hypoxia, hypothyroidism, and fasting
What caused this disease to develop at this time?
It is very difficult to say why headaches present when they do or what causes them to develop. Many are familial.
Each headache type has a general age range of development; however, there are always outliers, and typically it is children who present outside of the general age range. Headaches can develop at essentially any age from toddlerhood through adulthood. They often start in puberty.
The actual precipitating event or cause that leads to headache development is typically not known and can be different for each patient.
What laboratory studies should you request to help confirm the diagnosis? How should you interpret the results?
Routine laboratory testing in general has not been shown to be helpful in the diagnosis or evaluation of headaches in children and adolescents. The American Academy of Neurology’s practice parameter does not recommend any standard laboratory tests, including lumbar puncture, be obtained during the work-up for headache in pediatrics.
Only if history and physical exam raises concern for secondary headache should laboratory testing be considered. In this circumstance, the testing should be directed to help to evaluate or confirm the suspected primary diagnosis that has led to a secondary headache.
Would imaging studies be helpful? If so, which ones?
Neuroimaging does have its place in evaluating pediatric patients with headaches. However, with a thorough history and detailed physical exam, including extensive neurological exam with fundoscopic evaluation, most patients do not warrant imaging as part of their evaluation.
If there are abnormalities on exam, including focal neurological findings, signs of increased intracranial pressure, or changes in mental status or consciousness, neuroimaging should be obtained.
If there are concerns from the history, including recent onset of severe headache, changes in headache type, neurological dysfunction, history of seizures, concerning associated symptoms along with the headaches, or “red flags” (see below) neuroimaging should be considered.
The type of neuroimaging to be obtained is based on the urgency for the study and the differential diagnosis being considered.
Brain magnetic resonance imaging (MRI)—Longer, harder to obtain (especially if a young child requires sedation), more expensive study that is very good at giving detailed picture of intracranial compartment. It will show almost any intracranial abnormality, large or small, and it can show intracranial vasculature. The MRI provides great detail without radiation. Negatives include reduced availability, length of the study, and expense. Overall, MRI is the study of choice if there is a concern for any intracranial abnormality. Often it will be obtained after screening computed tomography (CT) if there is concern for an acute abnormality.
Head CT—Quick, easy to obtain, and relatively inexpensive study that is good at visualizing overall gross intracranial structures. It demonstrates blood, intracranial masses, hydrocephalus, and bone. Negatives include radiation, poor visualization of the posterior fossa. CT scan does not offer anatomical detail, and may miss small intracranial masses. Overall a good screening study if there is an acute concern for intracranial abnormality.
Cranial/Cervical X-ray—Quick, easy to obtain, inexpensive study that visualizes bone structure. These studies should only be done if there is concern for bony abnormality, such as fracture. Overall x-ray has very limited role in evaluation of headaches.
Confirming the diagnosis
Headaches can be primary or secondary in nature – the key is differentiating between the two so not to miss an underlying etiology. This is accomplished by thorough history and physical exam in conjunction with utilizing the headache classification system set forth by the International Headache Society in ICHD-2.
Key History Points
Time pattern of headaches (new, episodic, daily, stable or worsening)
When did headaches start, any precipitating factor
How long do they last, how often do they come
Any warning signs or triggers
Where is the pain and what does it feel like
Any associated symptoms
What makes headaches better or worse
What medication/treatment is used and how often
Family history of headaches – if so what kind
Lifestyle issues: hydration, exercise, diet, sleep, stress
Key Examination Points
Vital signs – including temperature, blood pressure, and head circumference
Careful head and neck exam
Skin—screen for neurocutaneous syndromes
Complete detailed neurologic examination, including fundoscopic examination
History or Examination "Red Flags"
Acute onset of first headache
Steady worsening of headaches
Awakening from sleep due to headaches
Associated symptoms that suggest posterior fossa (for example: vision change, vertigo, vomiting, cranial neuropathies)
Concurrent infection with findings suggestive of CNS infection
Any neurologic examination finding that may suggest focality
Any abnormality on fundoscopic examination
Vital sign irregularities suggestive of marked hypertension or increased intracranial pressure
Using these key history, examination, and “red flags” as well as incorporating the ICHD-2 diagnostic criteria will allow a likely diagnosis to be made and help determine the need for ancillary testing.
If you are able to confirm that the patient has headaches, what treatment should be initiated?
Treatment of headaches can be broken down into the acute management and the chronic management. Acute management involves abortive therapy in the home or emergency department setting. Chronic management is geared to ongoing control of headaches and preventative measures, including pharmacological and non-pharmacological treatment. (See Table I, Acute/Abortive Treatment; and Table II, Prophylactic Treatment.)
|Drug||Initial Dose||Max Dose||Side Effects||When to use||Warnings|
|Ibuprofen (eg, Advil)||10mg/kg||800mg||GI upset /bleed, renal impairment||Good 1st-line agent for acute headache||Overuse can exacerbate headache|
|Acetaminophen (eg, Tylenol)||15mg/kg||1000mg||Hepatic impairment||Fair 1st line agent for acute headache||Overuse can exacerbate headache|
|Sumatriptan (eg, Imitrex) *||Nasal – 5 or 20mg Oral – 25, 100mg||Nasal – 20mg/dose, 40mg/day Oral – 25-200mg/dose, 200mg/day||Paresthesias, flushing, chest discomfort, fatigue||Good for acute migraine – often after trial of NSAIDS fails||Do not use if there is a history of cardiac disease or arrhythmia. Caution with any triptan if patient is receiving SSRIs already|
|Ketorolac (Toradol)||0.5mg/kg IV||30mg||Same as ibuprofen||Good agent in ED setting – often used in combination with fluids and antiemetic||Use caution in renal patients|
|Metoclopramide (Reglan)||0.2mg/kg IVor PO||10mg per dose; may repeat dose||Drowsiness, urticaria, extrapyramidal symptoms (EPS)||Good agent in ED setting – often used with fluids and pain medication; can be used with Benadryl to counteract EPS||Do not use if prior EPSDo not use if altered mental status presentDo not use in asthmatics|
|Prochlorperazine (Compazine)||0.15mg/kg IV or PO||10mg per dose; may repeat dose||Drowsiness, dizziness, extrapyramidal symptoms (EPS)||Good agent in ED setting – often used with fluids and pain medication; can be used with Benadryl to counteract EPS||Do not use if altered mental status present|
|Diphenhyramine (Benadryl)||1mg/kg IV or PO||50mg||Drowsiness||Good agent in ED or home – often used in combination with pain medication, antiemetic, and fluids; counteracts EPS from raglan or compazine||Do not use if adverse reaction in past|
|Valproate Sodium (Depakote)||15mg/kg IV or PO||1000mg||GI upset, sleepiness||Good second agent in ED setting if initial combination does not work||Use caution in hepatic insufficiency|
|Fluids||Oral – no limit IV – 20cc/kg||Oral or IV – unlimited IV – 1 liter||None||Good in home or ED setting||None|
* Triptans other then imitrex have been used in children/adolescents. Almotriptan is only one FDA approved in children at this time. Those that have been shown to be effective in children include: sumitriptan, almotriptan, rizatriptan, and zolmitriptan.
|Drug||Starting Dose||Goal/Max Dose||Side Effects||Advantages to Use|
|Amitriptyline (Elavil)*||10mg oral nightly||50mg oral nightly or 25mg BID||Dangerous in overdose, tiredness, dizziness||Sleep aid, may help stabilize/improve mood (only at high dose)|
|Topiramate (Topamax)*||25mg oral nightly||Max: 100mg oral BID but typically stop at 50mg BID||Weight loss, worsening of cognition, kidney stones||Good in overweight children, good success even at low dose|
|Cyproheptadine (Periactin)||4mg oral BID||8mg oral 2-3 times a day (adolescents)||Drowsiness||Well tolerated, long standing use, safe in young children|
|Propranolol (Inderal)||1mg/kg/day oral (daily or divided BID)||4mg/kg/day oral (daily or divided BID)||Low blood pressure or heart rate; do not use in asthmatics or diabetics; tiredness||Well tolerated, possible option in adolescents|
|Divalporex (Depakote)||10mg/kg/day oral (usually divided BID)||40mg/kg/day oral (usually divided BID)||Weight gain, potential birth defects, hair loss, potential liver injury||Well tolerated, general good success; treats seizures as well**, mood stabilizer|
* These are the two most common used agents in children/adolescents
** Children with seizures will often have headaches as well, which may make valproate a more suitable option
Nonpharmacologic treatment of chronic headaches to be considered
Avoid over-use of short acting agents such as ibuprofen and other over-the-counter medications as well as narcotics
Lifestyle Changes: Diet – 3 meals a day, avoid caffeine, avoid any known food triggers, try to maintain healthy weight; Sleep – good sleep hygiene with consistent bedtime and amount of sleep (may benefit from sleep clinic visit and sleep study); Hydration – avoid dehydration; Exercise – goal to lower resting heart rate over time, minimum 30 minutes 3 times a week breaking a sweat or at least increasing heart rate; Avoid smoking, drinking, illicit drugs
Counseling/Therapy – behavior psychology that specializes in headache/pain management, stress management and helps with relaxation techniques and cognitive behavior therapy
Dietary Supplements/Vitamins: B2 (riboflavin), magnesium, coenzyme Q10, melatonin
Herbs: feverfew, butterbur
What are the adverse effects associated with each treatment option?
See Table I and Table II.
What are the possible outcomes of headaches?
Once the diagnosis of headache is made, the specific type of headache has been determined and other possible conditions have been ruled out, outcomes can be discussed.
In general, the outcome is good. While once headaches develop there remains a risk of recurrence throughout life, they can usually be controlled quite well with motivation from the patient and family. The majority of methods for prevention are the responsibility of the individual or family.
By avoiding triggers, and with the treatments (especially the non-pharmacological) listed above, most patients will overcome their headaches and prevent them. Should headaches recur, the patient will be able to work through them and overcome them over time as they learn how to self treat.
There are some patients that have primary headaches that remain a significant problem despite their best efforts at prevention and treatment, and those are the patients that require prophylactic pharmacological intervention, possibly for extended periods of time. While medications often will help, if their headaches are so severe prolonged medications are required, the patient often will continue to have headaches, albeit hopefully less frequently and less severe.
What causes this disease and how frequent is it?
The underlying cause of headaches is not well understood, but some associations are known, depending upon the type of headache.
The cause of primary headaches is debated but there does seem to be some influence of genetics that plays a role (see below).
The cause of secondary headaches depends on the etiology of the primary diagnosis and the headache itself is merely a symptom of that underlying cause.
Genetics have been thought for some time to play a role in the development of headaches. While there have been some breakthroughs (including certain gene and chromosome locations), there is still ongoing investigation trying to isolate genes that may predispose individuals to developing certain types of headaches.
There often is a first degree relative with similar symptoms for patients who present with a primary headache, especially migraines.
There also can be genetic links for some secondary headaches, including those attributed to brain tumors, certain vascular disorders, or genetic disorders such as tuberous sclerosis.
Certain headache types have higher incidence based on gender and/or race.
Headache represents one of the most common reasons for seeking neurology consultation in childhood and adolescence. Headaches are one of the primary neurological complaints presenting to emergency departments.
Overall up to 90% of the population has had at least one headache at some point, and up to 90% of those are primary headaches. It has been reported that over 50% of teenage boys and 75% of teenage girls will report having at least one headache in any given month, and 50% of children under 7 and over 80% by age 15 will have had at least one headache.
Primary headaches are the most common type, and within that subclass, tension is the most common seen in up to 80% of the population, followed by migraine. Migraine, however, is the most common type of headache leading to a medical evaluation and assessment.
Some specifics relating to pediatric headaches/migraines:
Prepuberty—boys are more affected than girls; Postpuberty—girls are more affected than boys
Migraines are seen in up to 3% of children under 7, up to 11% of children under 11, and up to 23% in teenagers
What complications might you expect from the disease or treatment of the disease?
See above under treatment.
How can headaches be prevented?
See above under treatment for pharmacological and non-pharmacological prophylactic treatment.
Unfortunately, prior to the onset of a first headache, headaches cannot to be prevented. If there is potential for headaches to develop (based on familial likelihood, for instance), adhering to the lifestyle changes listed above under treatment may help delay onset or even prevent them from occurring.
What is the evidence?
Lewis, D, Ashwal, S, Hershey, A. “Practice parameter: pharmacological treatment of migraine headaches in children and adolescents: report of the American Academy of Neurology Quality Standards Subcommittee and the Practice Committee of the Child Neurology Society”. Neurology. vol. 63. 2004 Dec 28. pp. 2215-24. (An often quoted parameter on treatment of migraines in children.)
Lewis, DW, Yonker, M, Winner, P, Sowell, M. “The treatment of pediatric migraine”. Pediatr Ann. vol. 34. 2005 Jun. pp. 448-60. (A nice review.)
Lewis, DW, Ashwal, S, Dahl, G. “Practice parameter: evaluation of children and adolescents with recurrent headaches: report of the American Academy of Neurology Quality Standards Subcommittee and the Practice Committee of the Child Neurology Society”. Neurology. vol. 59. 2002 Aug 27. pp. 490-8. (Similar to the first parameter, this focuses on the evaluation and testing required.)
Eiland, LS, Hunt, MO. “The use of triptans for pediatric migraines”. Paediatr Drugs. vol. 12. 2010 Dec 1. pp. 379-89. (A review of migraines and triptan use in children.)
Ongoing controversies regarding etiology, diagnosis, treatment
At this point there is still investigation into the actual mechanism and etiology of headaches. Until those mechanisms can be determined there will continue to be various opinions as to what leads to headaches in an individual.
The criteria above are the current standard for diagnosis. There have been multiple review articles looking at the necessary workup and from those reviews the above suggested evaluation has been recommended.
There have been very few studies of the pharmacologic treatment of childhood headaches and abortive or prophylactic medications. Most of what is used or listed above is not FDA approved for children (with the exception of almotriptan in adolescents), but in several studies have been shown to be effective and safe. Additionally, many of the treatments above have been approved for use for adult headaches, and their use has been extrapolated to use in children. Currently there are several ongoing trials of new medications primarily designed to treat headaches in children.
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- OVERVIEW: What every practitioner needs to know
- Are you sure your patient has a headache? What are the typical findings for this disease?
- What other disease/condition shares some of these symptoms?
- What caused this disease to develop at this time?
- What laboratory studies should you request to help confirm the diagnosis? How should you interpret the results?
- Would imaging studies be helpful? If so, which ones?
- Confirming the diagnosis
- If you are able to confirm that the patient has headaches, what treatment should be initiated?
- What are the adverse effects associated with each treatment option?
- What are the possible outcomes of headaches?
- What causes this disease and how frequent is it?
- What complications might you expect from the disease or treatment of the disease?
- How can headaches be prevented?
- What is the evidence?
- Ongoing controversies regarding etiology, diagnosis, treatment