Headache is a common complaint in children and the source of a great deal of worry for practitioners and parents. Every pediatrician’s — and especially the parent’s — worst nightmare is that the child’s headaches are being caused by a brain tumor or other intracranial process. This causes many physicians and parents to demand unnecessary neuroimaging. Although headache is most often caused by primary entities, such as migraine or tension-type, the pain may result from secondary causes, such as brain tumors, idiopathic intracranial hypertension, chronic meningitis, hydrocephalus, or acute febrile illnesses, such as influenza. To determine the cause of a child’s headache, the evaluation begins with a thorough headache history, followed by a focused physical examination, including measurement of vital signs and a complete neurological examination. The diagnosis of primary headache disorders, such as migraine and tension-type, rests principally on clinical criteria set forth by the International Headache Society (IHS).1 Clues to the presence and identification of secondary causes of headache, and thereby, the necessity of neuroimaging, are uncovered through this systematic process of history and physical. The performance of ancillary diagnostic testing rests upon information or concerns revealed during fundamental processes.
Headaches at all ages can be classified as either primary or secondary headaches. Secondary headaches are those caused by an underlying serious medical problem, such as a brain tumor, hydrocephalus, benign intracranial hypertension, or even systemic hypertension. With primary headaches, there is no underlying illness. Primary headaches include migraine, tension-type, and chronic daily headache.
When a parent brings a child to the pediatrician with “headache,” there are three questions they want answered: “What is causing the pain?” “How do we relieve the pain?” and “Is there a life-threatening cause?” (specifically, “Is there a brain tumor?”) Knowing these are the parent’s primary concerns allows us to appreciate their perspective and to set the stage for a successful therapeutic relationship. Although the most common cause of headache is a primary process, the third question is foremost in the minds of clinician, parent, and child. Therefore, the first step in the evaluation is to reassure ourselves through the process of a thorough medical evaluation, and then, we can confidently reassure the family.
To reassure ourselves, we must approach the complaint of “headache” with the standard medical toolbox of history: physical and neurological examination. Pending the results of this crucial first step, we may conduct other testing to establish the headache diagnosis. Once the headache diagnosis is determined, the most appropriate treatment regimen can be formulated from the variety of treatment options discussed later in this issue of Pediatric Annals.
The chief complaint of headache in a child or adolescent can be daunting to the busy primary care provider. Having a practical and rational approach to the evaluation of headache in children can make the experience more efficient and effective, helping the provider and the parent feel more at ease. The headache history will, in most instances, yield the necessary information to make the correct diagnosis. Gathering the right information to exclude more serious secondary causes of headache (such as tumors, infection, intoxication, or hydrocephalus) is the first crucial step.
The Headache History
A thorough history of a patient’s headache will lead to the diagnosis in an overwhelming majority of headache situations. The headache questionnaire (see Sidebar 1, page 400) provides a series of simple questions that help elicit and organize headache features to help establish a differential diagnosis.2
Sidebar 1.The Basic Headache Questions
1. How and when did your headache(s) begin?2. What is the pattern of your headache (show diagram of head):
- sudden first headache,
- episodes of headache,
- everyday headache,
- gradually worsening, or
- a mixture (more than one type of headache)?
3. How often do your headaches occur, and how long do they last?4. What makes the headache better or worse?5. Do any activities, medications, or foods tend to cause or aggravate your headaches?6. Do the headaches occur under any special circumstances or at any particular time?The Basic Medical Questions
7. Do you have any other medical problems (eg, high blood pressure, diabetes)?8. Are you taking or are you being treated with any medications (for the headache or other conditions)?The Worrisome Headache Questions; Red Flag Answers
9. Was there any head injury associated with onset of the headaches?10. Have you ever had seizures or convulsions?11. Over the past weeks or months, have there been any changes in walking, balance, vision, handedness, behavior, speech, or school performance?12. Have there been any episodes where the headache occurred in the middle of the night or first thing upon awakening? Any vomiting at night or in the morning?The Migraine Questions
13. Are there warning signs, or can you tell that a headache is coming?14. Where is the pain located (please point): □ Front□ (Back) Occiput □Eyes or behind the eyes□Neck □Side or sides□Other □Top15. What is the quality of the pain: □Pounding□Stabbing □Squeezing□Other16. How long do the headaches last?17. Are there any other symptoms that accompany your headache: nausea, vomiting, dizziness, numbness, weakness, or other?18. What do you do when you get a headache, or do you have to stop your activities when you get a headache?19. Does your scalp or face get sensitive to touch during or after a headache (allodynia)?20. Does anyone in your family suffer from headaches?Patient’s or Family’s Mindset or Level of Anxiety
21. What do you think might be causing your headache?
Associated with idiopathic intracranial hypertension
Determining how long the headaches have been occurring is quite helpful in gauging the likelihood of an organic cause. The symptoms associated with brain tumors or pseudotumor cerebri (intracranial hypertension) will typically evolve over weeks and, very uncommonly, more than 3 to 4 months. So a patient with 2 years of intermittent headache is highly unlikely to have intracranial hypertension.
Question #2 helps to determine the temporal pattern of the patient’s headache symptom complex, and it is the most important question to clarify. This question helps to identify headaches that are more likely to be associated with underlying organic pathology, which are the ones that will need neuroimaging. Typically, children will present with one of five patterns, shown graphically in Figure 1:
- Acute onset of first episode of headache, without prior history (pattern 1),
- Acute recurrent (episodic headache with symptom-free intervals) (pattern 2),
- Chronic progressive patterns of increasing headache (pattern 3),
- Non-progressive daily or near-daily headache (pattern 4),
- Mixed pattern of daily headache with superimposed more intense attacks (pattern 5).
Figure 1. Five Temporal Patterns of Headache in Children and Adolescents.
Each of these patterns suggests its own differential diagnosis, with headache patterns 1 and 3 being of most concern. For example, pattern 1, the acute onset of first episode of headache without a prior history of headache, could be caused by a viral illness with fever and therefore self-limited. However, the explosive onset of headache during straining may suggest a vascular event, such as rupture of an aneurysm producing subarachnoid hemorrhage. In the latter instance, an urgent non-contrast head CT scan, followed by spinal fluid analysis, is warranted.
Similarly, a child who presents with a gradually, steadily, worsening headache syndrome accompanied by pain that awakens her from sleep will likely warrant brain imaging (magnetic resonance imaging, or MRI). In contrast, a recurring pattern of 2-hour long headaches associated with nausea, with headache-free periods may be diagnosed by history and physical examination as migraine, without need for neuroimaging. For patterns 2, 4, and 5, a clinical history and neurological examination have the highest diagnostic yield, and the value of neuroimaging is low.
The headache questionnaire (Sidebar 1, see page 400) divides the questions into several sets: the basic headache questions, which are the presence or absence of any background medical issues that may be associated with the cause of the headache; the “red flag” questions, which prompt consideration for neuroimaging or further testing; the primary headache questions, which point toward migraine or tension-type, and the final question, which gauges the level of anxiety of the patient and his/her family, “What do you think is causing the pain?”
The basic headache questions establish the time course and temporal pattern of the headache. Headaches that began over the past 3 weeks are increasing steadily in frequency and severity warrant consideration of increased intracranial pressure and further diagnostic testing. These headaches can be compared with 2 years of episodic headache in which the attacks last 3 hours, which points toward migraine. Daily or near-daily patterns that have been present for 6 months and are accompanied by numerous school absences suggest chronic daily headache, such as chronic migraine or chronic tension-type headache, common patterns in adolescents.
The questions “How often does the headache occur?” and “How long does the headache last?” help to identify the characteristic pattern of the individual headache attack. A 4-hour attack of pain that occurs once a week would point toward migraine or tension-type headache, whereas brief, 5- to 15-minute attacks multiple times per day point toward the trigeminal autonomic cephalalgias (ie, cluster, paroxysmal hemicrania) or primary stabbing headache).
The standard pain-related question, “What makes the headache better or worse?” helps to identify exacerbating or aggravating phenomena, which can help diagnostically and therapeutically. If certain aromas, perfumes, or paint fumes trigger a pounding, nausea-inducing headache, the diagnosis is likely migraine, and avoidance is a simple intervention.
Medications are frequent and often overlooked as causes of headache (see Sidebar 2). Oral contraceptives, and non-sedating allergy medications, or acne medications, are frequent causes of headache. Medication overuse, as defined by more than 5 doses of OTC agents per week is a common, aggravating behavior with chronic daily headache. The cycle must be stopped to break the daily pattern of headaches.
Sidebar 2.Medicines That Can Cause Headache, Migraine
Medications that Cause or Unmask Migraine22
- Oral contraceptives
- Hormone therapy (prednisone, dexamethasone, levothyroxine)
- Caffeine (or caffeine withdrawal)
- Cold medicines, such as non-sedating antihistamines and decongestants
- Ergotamine therapy
- Antihypertensive agents (vasodilators)
- Overuse of fat-soluble vitamins, such as vitamin A and vitamin D
- Atypical antipsychotics
Medications Associated with ‘Overuse Headache’
- Nitric oxide donors
- Phosphodiesterase inhibitors
*- associated with idiopathic intracranial hypertension
The food question relates to potential dietary triggers, an often overstated issue in migraine. Only about one-third of migraine sufferers can identify a dietary trigger, and in that minority of patients, those foods should be avoided. For the other two-thirds, prohibiting an arbitrary list of foods (chocolates, processed meats, cheeses) is not necessary or reasonable and will not be adhered to. The major exception to dietary triggers is caffeine overuse, which is an exacerbating phenomena for many teens with migraine or chronic daily headache and warrants at least strict moderation, if not elimination.
The headache questionnaire questions also help identify the coexistence of other symptoms or signs, such as fever, recent trauma, or other medical conditions, such as sickle cell anemia, bleeding diathesis, or autoimmune disorders. The presence of fever with acute headache must raise concerns for viral or bacterial meningitis, although in most instances, acute headache with fever is caused by self-limited illness, such as viral upper respiratory tract infection or pharyngitis.
The Worrisome Headache Questions; Red Flag Answers
Any thorough headache history must include the “red flags,” which traditionally have been linked to a higher risk of intracranial pathology and must, therefore, trigger consideration of neuroimaging. This priority list includes the following:
- patients younger than 3 years;
- early morning pattern or awakening with headache or vomiting;
- worsening headache while straining;
- explosive onset;
- steadily worsening pattern of headaches,
- presence of neurocutaneous markers, café au lait spots, hypopigmented macules
These factors should raise concern for more potentially ominous problems, such as tumors, abscesses, vascular malformations or bleeds, and they require prompt consideration for ancillary diagnostic testing (computerized tomography, or CT, MRI, magnetic resonance angiography, or MRA, magnetic resonance venography, or MRV, and/or electroencephalogram, or EEG).
‘Is This a Migraine?’
‘Are There Warning Signs or Can You Tell that a Headache Is Coming?’
About one-third of migraine sufferers will have an occasional visual or somatosensory (numbness or tingling) aura, which lasts 5 to 30 minutes and is followed within a few minutes by headache. Stereotypical complex visual auras accompanied by headache and confusion or distortion of consciousness in an elementary age child should also prompt consideration for benign occipital epilepsy. So not all auras point to migraine.
‘Where Is the Pain Located?’ and ‘What Is the Quality of the Pain: Pounding, Squeezing, Stabbing, or Other?’
This question must be asked carefully so as not to “lead the witness.” Children will often choose the last of any three choices given to them, so first ask them to describe, demonstrate, gesture, or draw the pain before resorting to a list of choices.
Occipital location may be present in basilar-type migraine, but be concerned about the subacute onset of occipital or upper neck pain. That type of pain may indicate posterior fossa neoplasms, such as medulloblastoma or cerebellar astrocytoma.
The duration of the painful attack helps to distinguish between migraine and some of the more uncommon “trigeminal autonomic cephalagias,” such as cluster or paroxysmal hemicranial. Migraine attacks last 1 to 48 hours, whereas cluster attacks typically last 5 to 15 minutes.
‘Are There Any Other Symptoms that Accompany Your Headache: Nausea, Vomiting, Dizziness, Numbness, Weakness, or Other?’
This question seeks to identify the presence of autonomic symptoms and must be explored carefully. Vomiting is an associated symptom of migraine but may also be prominent features of elevated intracranial pressure with brain tumors or idiopathic intracranial hypertension. If the vomiting occurs early in the morning or awakens the child, whose headaches are gradually and steadily increasing in frequency and severity, then mass lesions must be sought.
Similarly, the complaint of dizziness requires clarification. Does the patient mean lightheadedness, unsteadiness, or vertigo? The distinction is important because each potentially suggests differing pathophysiology. Lightheadedness suggests cerebral hypoperfusion or orthostasis. Unsteadiness or vertigo suggests ataxia or balance disorders pointing toward the vestibular or cerebellar systems, in which case neuroimaging must be considered. Numbness or weakness likewise must be clarified. Many migraine sufferers will have a perioral or hand numbness (chiro-oral) as part of the “aura” or prelude phase of their attack. “Weakness” requires exploration as well. Many headache patients feel “weak all over,” but a localizable pattern of weakness, especially if it persists, may justify neuroimaging in search for intracranial pathology.
‘What Do You Do when You Get a Headache, or Do You Have to Stop Your Activities when You Get a Headache?’
This question goes to the heart of disability. Do the headaches interfere with activities of daily living? A headache that stops the child in his or her tracks and forces the child to lie down or to ask for medicines is more disabling than a casual mention of headache as the patient passes by on the way out the door to play.
Assessing the “headache burden,” or degree of disability imposed by the headache, is an essential component of the management decision-making process. Frequent school absences or delayed activities suggest a high headache burden and a more aggressive strategy for prevention.
An often unrecognized feature, distinctive of migraine, is “allodynia,” in which the patient notices that seemingly innocuous sensations are perceived as painful. The question “Does your scalp or face get sensitive to touch during or after a headache?” is exploring for the presence of allodynia. Simple acts, such as brushing the hair or applying makeup, are quite painful because of “peripheral sensitization,” a phenomenon associated with migraine.
‘Does Anyone in Your Family Suffer from Headaches?’
Ask this question in an open-ended fashion using the term “headaches,” not a specific diagnosis, such as “migraine” because often a parent may have migraine but has been mislabeled as having “sinus” or “stress” headache. Although family history is not one of the diagnostic criteria for migraine, it is, nonetheless, a useful clue to determining if the patient has migraine.
This question should also address any other family history of neurological disorders. Brain tumors have an inherited pattern in conditions, such as tuberous sclerosis, neurofibromatosis, and von Hippel-Lindau disease. Also, certain vascular malformations may have heritable patterns (eg, cavernous angioma).
‘What Do You Think Might Be Causing Your Headache?’
The patient’s and the family’s mindset or level of anxiety can be assessed with this question, which is often the most important one to ask and addresses the inner fears of the patient and their family. Most of families who present to the office for evaluation of their child’s headache are fearful of brain tumors. Recognizing this fact can be extremely useful in establishing confidence and trust. If you are comfortable that the headache history suggests a primary headache disorder and the physical and neurological examinations are normal, you can confidently tell the family that there are not signs of brain tumors or anything “bad.” Confident reassurance is one of the most potent therapeutic interventions. Conversely, if you have not reassured yourself as to the diagnosis, then further testing or referral may be needed.
Physical and Neurological Examination
After the detailed headache history, a general physical examination is performed and must include vital signs, with blood pressure and temperature, looking for signs of hypertension or infection. Also, head circumference must be measured, even in the older children, because slowly progressive increases in intracranial pressure in children can cause macrocrania. Careful palpation of the head and neck for sinus, jaw, ocular, or temperomandibular joint (TMJ) tenderness, thyromegaly, or nuchal rigidity should be performed. Identification of trigger points or areas of maximum tenderness helps to determine the nature of the pain. The skin must also be examined for signs of a neurocutaneous syndrome, particularly neurofibromatosis and tuberous sclerosis, which, as stated earlier, are highly associated with intracranial neoplasms.
Following the general physical exam, a detailed neurological examination is essential. This is supported by the fact that, of the two-thirds of children with brain tumors who present with headache, more than 98% have objective neurological findings. Because reassuring ourselves is fundamental to reassuring our patients, the neurological examination is paramount to providing that essential reassurance.
In the neurological examination, the physician is looking for signs of increased intracranial pressure, integrity of the brain stem, asymmetry of motor or sensory systems, coordination problems, and gait problems. It is important to think about the neurological exam in an anatomical manner so that all of the key regions of the brain are evaluated. For example, the mental status exam assesses the cerebral cortex; cranial nerve exam checks the brain stem function and integrity; motor and sensory systems evaluate the descending and ascending pathways; coordination looks at the cerebellar and vestibular pathways; and gait observation puts multiple systems through a dynamic challenge. The key features include altered mental status, abnormal eye movements, optic disc distortion, motor or sensory asymmetry, coordination disturbances or abnormal deep tendon reflexes.3
Comfort with the neurological examination comes with practice and a systematic approach so that all key aspects are performed and documented. The funduscopic examination takes additional skill and practice to master to assess for papilledema, which indicates increased intracranial pressure. Seeing the optic discs in young children is often quite challenging. The key to seeing the optic disc is to have the patient focus on a “far point” and to bring the ophthalmoscope in from the side to allow the physician the best chance to clearly see the fundus (see Figure 2). Even with extensive practice, the fundus cannot always be visualized in the office setting. If there is true concern for increased intracranial pressure as a possible cause of the patient’s headaches, then a dilated ophthalmoscopic exam is required.
Figure 2. Seeing the Elusive Optic Disc.
Ancillary Diagnostic Testing
Once a detailed history and neurological examination are completed, the appropriate diagnosis can be made in most childhood headaches.
The next step is to decide if further ancillary diagnostic studies, such as laboratory testing, EEG, and neuroimaging, are warranted. The role for such testing for children or adolescents with recurring patterns of headache has been reviewed extensively in a practice parameter of the American Academy of Neurology and is available online at www.aan.org.4
Routine laboratory testing, although easy to accomplish and relatively inexpensive, has not been found to be beneficial in the diagnosis or evaluation of headaches in children and adolescents. One study has analyzed 104 children who were being evaluated by a child neurologist. Laboratory studies, including complete blood count, electrolyte levels, liver function profiles and urinalysis, were performed.5 The laboratory studies were described as “uniformly unrevealing.” With the limited published literature assessing the role of laboratory testing in the evaluation of headaches in children and adolescents, the AAN’s practice parameter stated that there is inadequate documentation to support any recommendation of routine laboratory studies or performance of lumbar puncture. The practice parameters did not, however, address patients with headache associated with fever or other signs of infection. In this instance, the clinician’s best judgment must prevail.
The parameter also stated that routine EEG was not recommended as part of the headache evaluation. Eight studies have assessed the utility of EEG in children with recurrent headaches. The data showed that EEG was not necessary for distinguishing a diagnosis of primary headache disorder in children from secondary headache caused by structural disease involving the head and neck, or those due to a psychogenic etiology.6–13 This was because the EEG was either normal or demonstrated non-specific abnormalities in most patients. Even when the EEG was abnormal, it did not provide diagnostic information concerning the etiology of the headache.
In addition, EEG is unlikely to distinguish migraine from other types of headaches. Based on the collective data from the eight studies, there was not a significant difference in EEG abnormalities in children with migraines, compared with the entire headache group. Furthermore, children who had paroxysmal EEGs while undergoing evaluation for headaches were noted to have a negligible risk for future seizures. In fact, no patients (with normal EEG or with paroxysmal EEG changes) subsequently developed new onset seizures following their headache evaluation. This led to the recommendation from the AAN that EEG is not warranted in the evaluation of headaches in children or adolescents.
Of note, it is important to remember that “seizure-related” headache is a controversial diagnosis, and there is debate as to its very existence. Data from a single study of 215 children, in which “seizure headaches” were diagnosed. Fifty-eight children (27%) described a “seizure headache” as a “paroxysmal brief headache” accompanied by nausea, vomiting, or other autonomic signs followed by postictal lethargy or sleep with “typical epileptiform discharge” on EEG recording. Because there are such limited data on “seizure headaches,” and the diagnosis itself is debatable, clinical follow-up and physician judgment should be used to decide the need for future investigations for epilepsy.
The AAN practice parameter from 1994 addressed the utility of neuroimaging in the evaluation of headache in adults with normal neurologic examinations.14 The recommendations were that routine neuron-imaging was unwarranted in patients with recurrent headaches who did not have a recent change in headache pattern, history of seizures, and no focal neurological signs or symptoms.
Seven pediatric studies are available to address the role and rationale for neuroimaging in children with recurrent headache. From these studies, an AAN Practice Parameter Committee attempted to develop guidance regarding the role and indications for neuroimaging for children with recurrent headaches.15–21 CT scans, MRIs, or both, were preformed on more than 600 children, with “abnormalities” identified in 16% of the children. However, 82% of these abnormalities were considered to be incidental, a non-surgical lesion or one that did not require specific medical management. These abnormalities included Chiari malformation, arachnoid cyst without mass effect, paranasal sinus disease, occult vascular malformations, pineal cyst, plus a variety of “incidental” structural abnormalities, such as cavum septi, ventricular asymmetry, and “hyperintense” lesions. Most important is the fact that all children who had lesions noted on CT or MRI that were deemed surgically treatable (18 children, 3%) manifested abnormalities, such as papilledema, abnormal eye movements, including nystagmus, and motor or gait dysfunction, were always described on neurological examination.
From this AAN practice parameter for the role of neuroimaging in the evaluation of children with recurrent headaches, the following recommendations were proposed:
- Obtaining a neuroimaging study on a routine basis is not indicated in children with recurrent headaches and normal neurological examinations. Neuroimaging, however, should be considered in children with an abnormal neurological examination including, but not limited to, focal findings, signs of increased intracranial pressure, or significant alteration of consciousness.
- Neuroimaging should be considered in children in whom there are historical features to suggest the following:
a recent onset of severe headache,
change in the type of headache,
neurological dysfunction, or
associated symptoms of concern that accompany the headache.
- Consider neuroimaging in children who present with headaches and also have seizures.
To determine the nature of a child’s headache, the evaluation begins with a thorough medical history, followed by methodical physical examination with measurement of vital signs, with complete neurological examination. Whether or not further testing, such as neuroimaging, is needed rests upon this fundamental process.
- Olesen J. The international classification of headache disorders. Cephalalgia. 2004; 24 (suppl 1):1–160.
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- The Childhood Brain Tumor Consortium. The epidemiology of headache among children with brain tumor. Headache in children with brain tumors. J Neurooncol. 1991;10(1):31–46.
- Lewis DW, Ashwal S, Dahl G, et al. Quality Standards Subcommittee of the American Academy of NeurologyPractice Committee of the Child Neurology Society. Practice parameter: evaluation of children and adolescents with recurrent headaches: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2002;59(4):490–498.
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- Report of the Quality Standards Subcommittee of the American Academy of Neurology. Practice parameter: the utility of neuroimaging in the evaluation of headache in patients with normal neurologic examinations (summary statement). Neurology. 1994;44(7):1353–1354.
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- Medina LS, Pinter JD, Zurakowski D, Davis RG, Kuban K, Barnes PD. Children with headache: clinical predictors of surgical space-occupying lesions and the role of neuroimaging. Radiology. 1997;202(3):819–824.
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