Hanalise V. Huff, MD, MPH, and Kenneth R. Huff, MD
A 12-year-old girl is brought in with a history of headaches. Although she has been sent home from school twice in the past 6 weeks, she has experienced headaches for at least 1 year. The last episode, 1 week prior to this office visit, was typical. The headache began as a dull feeling over both eyes, radiated up to the top of her head, and eventually became pounding. She had no preliminary visual symptoms or other warning signs prior to the head pain. The episode began during an afternoon class after she had been outside on a hot, sunny day for physical education. The headache worsened after she walked home from school. After she got home, she went to her room, drew the curtains, and lay down on her bed. She experienced some nausea and loss of appetite but no vomiting. She did not get up for dinner. She denied experiencing diplopia, vertigo, ataxia, and limb weakness, and her speech was observed to be articulate and coherent. She took 2 80-mg children’s acetaminophen tablets without significant relief but eventually fell asleep. The following morning, she felt fine.
Between headaches, her behavior has not changed, and she has continued to make above-average grades. She has not experienced any major changes in her home environment. When initially questioned, her mother denied having migraines but did admit to needing to lie down because of headaches approximately once a month. A detailed neurologic examination of the girl is completely normal.
1. How do the symptoms and neurologic examination help differentiate headaches caused by an intracranial space-occupying lesion from headaches caused by a chronic migrainous condition?
2. How do the symptoms and neurologic examination help identify the etiology of the headache?
3. What are the characteristics of headaches caused by intracranial tumors and migraines?
4. How do lifestyle and environmental history help identify headache triggers and help in management of the headache?
5. What is the appropriate treatment plan for patients such as the one discussed in the case study?
Headaches, which are quite common in adolescents and older children particularly, frequently prompt parents or caregivers to have their child evaluated by a physician. The physician must differentiate headaches that are symptomatic of a progressive intracranial process from those that may possibly be intermittently disabling but do not necessitate surgery. The cornerstone of this determination is obtaining key historical information and determining whether abnormalities exist on examination of the nervous system. Decisions about management strategies are influenced mostly by etiology and the effect of the headaches on the child’s life.
The contribution of secondary headaches (ie, those related to tumors, vascular abnormalities, or meningitis) to the overall prevalence of headache is relatively small. Chronic nonprogressive headaches in children that are not related to a self-limited condition are frequently lumped together as “migraine,” which includes migraine with and without aura. The prevalence of migraine that comes to the attention of a physician is approximately 3% to 10% for all children, although as many as 75% of all children and 85% of adolescents admit to having experienced a headache in the prior year. Although initial headache symptoms begin most often between ages 6 and 12 years, many patients come to medical attention initially during adolescence, especially girls, whose headache prevalence at that age is double that of boys. In adolescents, the prevalence of chronic migraine is 0.8% to 1.8%. Headaches are also commonly associated with trauma, including concussion, acute intercurrent infection, and other systemic illness.
Parents and caregivers must interpret the nature and severity of their child’s experience and symptoms, which may be difficult, especially in younger children. Younger children more rarely complain of head pain but more commonly complain of stomachache or present with nausea or vomiting. Later, as the child ages, the same episodic setting and behavior are associated with a headache complaint. The episodic syndromes of cyclic vomiting, benign paroxysmal vertigo, benign paroxysmal torticollis, and abdominal migraine are related presentations of childhood migraine. Adolescents, however, may report persistent, dull, aching, or pounding head pain, which can recur daily. As opposed to headaches that increase in intensity or frequency and are associated with neurologic signs, which may be suggestive of an intracranial mass lesion, migraine episodes may be relatively stable in frequency over many weeks or months, accompanied by a normal neurologic and ophthalmologic examination, and similar to that experienced by other family members (Box 129.1). A multigenerational family history of “sick headaches” is a frequent finding in the child with migraine. This history must be specifically elicited by the physician, however, because its significance is often discounted by parents. Migraine with aura, which is relatively unusual in children, is distinguished by the presence of reversible specific sensory symptoms (usually visual) that precede the headache. The headaches may be associated with a prodrome of lethargy, irritability, slowness of movement and response, concentration problems, intolerance of intense sound or light, loss of appetite, and typically at least 1 facial autonomic manifestation (eg, conjunctival injection, sweating, periorbital swelling, ptosis, miosis). Migraine has a tremendous capacity to negatively affect a child’s schooling, both in attendance and performance while present.
Pain fibers are carried in the trigeminal nerves from the scalp, skull, meninges, and vessel walls within the brain. These fibers experience traction or inflammation at their endings, thereby producing the pain that is associated with mass lesions or meningeal inflammation as well as mediating the pain from an internal (brain) pain generator or perhaps an external painfully tense scalp muscle (through a more circuitous route). The “migraine generator” likely resides in the brain stem and has input to the trigeminal nucleus, which both innervates intracranial blood vessels and receives nerve signals from them. “Cortical spreading depression of Leão,” the neuronal and glial depolarization wave that may be the electrophysiologic correlate of the migraine aura, also activates trigeminal afferents. One likely pathogenically deficient neurotransmitter at the vascular nerve endings, serotonin, allows a cascade of release of local cytokines, substance P, calcitonin gene-related peptide, and histamine in and around the vessel walls. These substances in turn cause changes in vascular permeability, local extravascular space swelling, and painful inflammation.
The usual distinction between migraine and tension headache pathophysiology is more difficult to make in the child than the
adult (and perhaps is less important from a management perspective) and may involve the types of factors that trigger headaches at different ages (Box 129.2). Several different mechanisms result in the triggering of pain nerves involving environmental or internal factors in genetically predisposed individuals. For example, bright sunlight, environmental stress, or a drop in menstrual hormones likely trigger migraines by different mechanisms within the brain. The rare condition of familial hemiplegic migraine has been related to mutations in 3 different genes thus far, illustrating a quite specific predisposition. Most pediatric patients have in their pedigree other individuals who have experienced migraine at some point in their life; however, the relative roles of genetic susceptibility and environmental trigger agents are subjects of much ongoing investigation.
Box 129.1. Diagnosis of Headaches (Migraine) in the Pediatric Patient
•Head pain of ≥2 hours’ duration, often pounding in character, mostly bilateral, over the eyes, at the vertex, or in the temporal region
•Pain frequently accompanied by nausea, anorexia, photophobia, or phonophobia, and aversion to movement
•Pain sometimes preceded or accompanied by visual symptoms, such as scotomas or other sensory or dysphasic auras that fully reverse
•Frequent presence of trigger factors
•Positive family history of headaches
Emergent causes should be considered if headaches have an abrupt onset, rapidly worsen, or are especially severe or if the child describes the headache as a “thunderclap” or “the worst of my life.” Intracranial hemorrhage from a ruptured aneurysm or an arteriovenous malformation or, rarely, as a secondary effect of extreme hypertension, is foremost on the list of such causes. If a child has a condition that may potentially result in stroke, such as coagulopathy, hemoglobinopathy, or heart disease, the stroke may become hemorrhagic and produce such an acute, severe headache resulting from sudden traction of pain fibers from space-occupying extravascular blood.
Nonhemorrhagic meningeal irritation, such as the inflammation produced by bacterial or viral meningitis, may also cause acute headache. These conditions may render the patient confused and lethargic while still conscious and often are associated with fever. Both types of meningeal irritation produce nuchal rigidity and Kernig and Brudzinski signs. Less serious viral illnesses outside the nervous system also can produce headache acutely but show other reassuring signs, such as rhinitis, and lack meningeal signs or altered mental status.
Chronic Progressive Headaches
The child with brain tumor or brain abscess or with a gradual block in cerebrospinal fluid (CSF) flow producing hydrocephalus frequently has a “crescendo” history, usually of less than 6 months’ duration, of increasing severity or frequency of headaches. The headache pain may be consistently localized, or it may awaken the patient from sleep, presumably related to relative shift in intracranial traction forces resulting from gravity and the horizontal position. Of note, many children with brain tumors do not complain of a localized headache and may present with persistent vomiting without headache as a prominent symptom. Between headaches children often experience new coordination problems, lethargy, loss of attained skills, personality change, or deterioration of schoolwork. Idiopathic intracranial hypertension (IIH), like intracranial venous sinus obstruction, presents with headaches of recent onset and associated papilledema. Hypervitaminosis A, steroid withdrawal, tetracycline treatment, and obesity have all been related etiologically to IIH, which most commonly occurs in adolescent females.
Box 129.2. Triggers of Migraine
•Glare, dazzle, bright sunlight, or fluorescent lighting
•Physical exertion, fatigue, lack of exercise, lack of adequate sleep, hunger or skipping meals
•Change in ambient temperature or humidity
•Allergic reactions involving sinuses, pungent odors
•Certain foods, alcoholic beverages, and cold foods or beverages
•Anxiety, stress, and worry
•Menstruation and oral contraceptives
•Refractive errors if they produce squinting (unusual)
Chronic Recurrent Headaches
The pain of migraine headaches, the most common chronic headache type, is throbbing in character, and if headaches are severe, patients avoid intense sensory stimuli or even routine physical activity. Episodes may last 2 hours to 3 days but often resolve with sleep. Nausea or dizziness, anorexia, and visual blurring are often associated with migraine headache. The pain may be severe enough to curtail activities, or the child may participate under duress, particularly at school. Migraine with aura is preceded by 10 to 20 minutes of a sensory phenomenon, usually dark spots or visual patterns. The aura may be confused with a transient ischemic attack; however, aura proceeds more slowly and may exhibit positive sensations, such as scintillations or paresthesias.
Sinus infection may be confused with chronically recurring or acute headaches, especially if the pain is facial as well as cranial. Symptoms of chronic nasal congestion may be present with migraine, but allergies and postnasal drip may be associated with sinus headaches. In many cases the infection is a trigger for a migraine headache and the patient has primary migraine, not pain originating from the sinusitis as may have been previously assumed. Children with asthma also have recurring headaches that possibly are triggered by sinus involvement, generalized cytokine release, or other aspects of asthmatic pathophysiology, such as smooth muscle reactivity and susceptibility to medication side effects.
Other types of headache may recur without progression. In some cases, chronic daily headaches (ie, >15 headache days per month) may be “transformed migraine” or “rebound headache” resulting from abortive medication overuse. Adolescents may have frequently recurring headaches triggered by irregular lifestyles, such as not exercising enough, eating regularly, or getting adequate sleep, if they are susceptible individuals. In many individuals, symptomatology is more consistent with tension-type headaches, which are characterized by a feeling of a tightening band around the head rather than a throbbing pain, an occurrence late in the day, and an association with anxiety, depression, or a stressful environment. These “featureless” headaches are not worsened by routine physical activity or accompanied by nausea, vomiting, photophobia, or phonophobia. Overlap exists in this group with migraine headache symptoms and response to therapy. Posttraumatic headaches may be a significant problem as part of a concussion but should recur with gradually decreasing severity over several days to months.
Cluster headaches are quite different from the other types discussed. They are shorter (ie, ½–3 hours’ duration); are associated with unilateral lancinating pain, tearing, nasal stuffiness, Horner syndrome, and nighttime pacing; and are unusual in children, almost never occurring in persons younger than 10 years.
Information should be obtained from patients, parents or guardians, and other caregivers concerning when headaches first began; the quality, intensity, and location of headaches; and other associated symptoms before and during headaches (Box 129.3). Duration, clinical course, and conditions that evoke, intensify, or alleviate the pain are also important. It is useful to have the child describe a typical episode—perhaps the most recent—including the circumstances of when and where the headache began and how it affected activities at the time. The circumstances of the headache can help determine individual triggers, and the degree to which the headache problem affects the child’s lifestyle strongly influences the management plan for chronic recurrent headaches.
Box 129.3. What to Ask
•Where is the child, what time of day is it, and what is the child doing when a typical headache begins or the last recalled headache began?
•Where is the pain located? How severe is the pain? How long does the pain last?
•Is nausea, vomiting, phonophobia, or photophobia associated with the headache?
•What does the child do after getting the headache? Does the episode require cessation of activities?
•Does the child take any medication for the pain? If so, was it successful? What else, if anything, allows the child to feel better?
•How long ago did the first headache occur? Are headaches becoming more frequent or severe?
•Does the child experience motion sickness?
•Have headaches ever awakened the child at night?
•Does the child have other chronic conditions? Does the child take medications or vitamins?
•Have there been any changes in weight, speech, vision, gait, school performance, or personality or any loss of skills or abilities between headaches? Do all the accompanying symptoms of the headache resolve after each headache episode?
•Does the child have any warning symptoms before the pain? Does unilateral visual loss, weakness or numbness, diplopia, confusion, or loss of consciousness occur with the headache?
•How often does the child miss school because of a headache?
•Are there stressful circumstances or major life changes? Has there been depression or anxiety?
•Do other members of the family have headaches? Do they ever need to lie down or take medicine for these headaches?