Lewis Carroll brought his rnigrainous hallucinations to life when he wrote Alice m Wonderland. The fact that the images of Alices changes in size appear in a children's story is particularly appropriate, because we now understand that headache is common in children and adolescents as well as in adults.
References to headaches were noted 5000 years ago. Hippocrates described migraine 25 centuries ago, and Galen coined the term hemicrania.1 However, references to headaches in children are relatively recent, dating back only to the late 180Os. Bille 's monumental work in 1 962 reported on the frequency of headache in 9000 school children.- Bille 's study noted that by age 7, 1.4% of children had true migraine headaches, 2.5% had frequent nonmigrainous headaches, and 35% had infrequent headaches of other varieties. By age 15, 5.3% had migraine headaches, 15.7% had frequent nonmigrainous muscle contraction) headaches, and 54% had experienced infrequent nonmigrainous headaches. In our own consulting child neurology practice, approximately 40% of adolescents have migraine, another 45% have muscle contraction or tension headaches, and 15% have other forms of headache.
Headache alters the lifestyle of both adolescents and their parents and may result in time lost from school and work. Physical and psychological causes must be investigated in order to correctly diagnose and manage headaches in the adolescent patient.
The usual current approach to the classification of headaches is based on etiology, pathology, and symptom complex. Three major categories are noted:
1. vascular, including migraine,
2. organic, with symptoms of increased intracranial pressure (IICP), including tumor, and
3. psychological or functional, including tension or muscle contraction headaches, depression, and conversion reaction.3
Figure. Graph depicting headache types by plotting symptoms against time.
It is also useful to classify headaches according to their temporal pattern.4 The severity is plotted against time, and five sequences are noted, including acute, acute-recurrent, chronic-progressive, chronicnonprogressive, and mixed pattern (Figure).
The acute headache is a single event and is usually seen in the pediatrician's office or the emergency room. In an adolescent, the most frequent cause is an infection such as pharyngitis. If the patient is critically ill with fever or nuchal rigidity, meningitis and subarachnoid hemorrhage are considerations, and immediate investigation is required.
Acute-recurrent headaches appear and disappear over a few hours, only to recur at a later time in a similar fashion. If nausea and vomiting occur, these headaches are usually migrainous in nature.
Chronic -progress i ve headaches generally increase in severity and frequency over a period of months. The most worrisome symptoms are balance difficulty, lethargy, personality change, focal weakness, and loss of consciousness. The neurological examination may reveal ahn ormai i ties, leading the practitioner to suspect an active intracranial process such as hydrocéphale or a brain tumor.
If a patient presents with chronic nonprogressive headaches that are constant or very frequent in the absence of neurologic symptoms and with a normal neurologic examination, stress is most likely causative. This form of headache is commonly referred to as a tension or muscle contraction headache. This type of headache affects everyone at one time or another. If the headaches become frequent or constant, evaluation is indicated.
The mixed headache syndrome is a combination of the acute-recurrent and chronic -nonprogressi ve categories. It consists of episodic headaches with nausea and vomiting superimposed on daily, or almost daily, headaches. The patient is able to differentiate the two types of headache with ease, when prompted. The neurologic examination is usually normal, and a combined psychological and pharmacological approach to treatment must be used. The added dimensions of severity over time allow both a specific diagnosis to be made and a timely evaluation and treatment program to be planned.
Questions for Use in Evaluating Patients Complaining off Headaches
The medical model for the evaluation of a headache patient includes a comprehensive history, a general physical examination, and a detailed neurologic examination.4
A properly obtained history is the key to correct diagnosis and is necessary to differentiate among headache types. During the history, questions must be directed to both the parents and the adolescent. Practitioners are sometimes surprised at the amount of useful information the patients themselves can provide. Of course, the younger the child, the less specific the responses are likely to be. With adolescents, a private interview to discuss psychosocial issues is necessary. The answers to specific questions are necessary to generate sufficient data on which to base the diagnosis (Table).
When dealing with adolescents, data also must include information regarding pregnancy, labor and delivery, growth and development, behavior, academic function, family history, and the presence or absence of previous encephalopathic events. A systems review must look specifically at medical conditions that may contribute to the patient's headaches, as well as the use of medications that may precipitate or provoke headaches and those used to relieve headaches. An additional subset of questions should deal with the presence or absence of symptoms of UCP, including nausea, vomiting, lethargy, ataxia, visual disturbance, loss of consciousness, weakness, and personality change. It is helpful to note the affect of the parents and patient during the interview because information concerning emotional stresses may become apparent from these observations.
If a headache is severe without previous headache, is associated with straining or neurologic symptoms, is persistently localized, is a change from a previously established headache pattern, or awakens the patient from sleep, an otganic disorder should be suspected. The results of the physical examination of patients with migraine or muscle contraction headaches are normal. The presence of fever may indicate an infectious process. Blood pressure should he measured routinely as hypertension in adolescents, while rare, may cause headache. Cafe au lait spots should be noted since neurofibromatosis can be associated with a variety of central nervous system disorders. Every organ system may be related to the headache syndrome and should he examined.
The neurologic examination in adolescents determines the integrity of the central nervous system and is expanded to include measurement of the cranial circumference. Arrested hydrocephalus with macrocephaly may decompensate during the teenage years. If loud, asymmetric, machinery- like bruits are heard, an arteriovenous malformation should be suspected. The examination is incomplete without a fundoscopic examination.
The choice of laboratory tests depends on the differential diagnosis. Laboratory tests to "rule out" disease are seldom indicated and may actually be misleading. Foremost to consider are space-occupying lesions, seizures, and other paroxysmal disorders. In most patients, roentgenograms of the skull are not necessary. The EEG is of little value in the routine evaluation of headache, but it may be helpful if neurological symptoms or signs are present or if loss of consciousness has occurred. Nonspecific, insignificant EEG abnormalities are noted frequently in children.5 Both computed tomography (CT) and magnetic resonance imaging (MRI) are safe, accurate, and rapid methods of evaluating the inttacranial contents. Both types of scans are useful in a wide variety of conditions and in evaluating headaches that may be organic in etiology. If a patient has progressive symptoms, IICP symptoms, or focal symptoms, if the neurologic examination shows any abnormality, if neutocutaneous stigmata or macrocephaly are present, or if any laboratory test such as an EEG points to a focal lesion, an imaging procedure is mandatory to mie out a structural abnormality. The imaging study should include the paranasal sinuses. In patients who have both headaches and emotional problems, or if home or school difficulties seem to be playing a role, tests of general intellectual function may be useful, and a psychological or psychiatric consultation may be needed.
SPECIFIC HEADACHE SYNDROMES
The majority of acute headaches in children are relatively easy to diagnose and treat. The child frequently will localize the pain to a specific area such as the sinuses, eyes, or teeth, providing a clue to the etiology. Nonlocalized acute headache is even more common and is most often found to be secondary to fever, with secondary dilation of cerebral vessels. The vast majority of local and nonlocal acute headaches benefit from symptomatic treatment with acetaminophen or ibuprofen. If an acute headache is associated with fever, nuchal rigidity, or other central nervous system symptoms or signs, a rapid evaluation is mandatory and may be lifesaving.6
Acu te -recurrent headaches usually are migraine. Migraine is a specific genetic disorder of vascular instability characterized by paroxysmal attacks of vasoconstriction and vasodilation. Migraine will affect approximately 5% of children by the age of 15.
Prior to puberty, migraine is more common in boys; after puberty, it is more common in girls. The clinical featutes of patients with migraine are7:
* paroxysmal headache (100%),
* relief after sleep (94%),
* nausea, vomiting, and abdominal pain (90%),
* family history of migraine (69%),
* throbbing, pounding quality to pain (58%),
* unilateral headache (36%), and
* aura (17%).
Migraine attacks frequently are precipitated by ttigget phenomenon including anxiety, fatigue, stress, excessive sleep, lack of sleep, minor head trauma, exercise, travel, illness, menses, odors, diet, medica' tions, and hunger. Specific foods such as preserved meats, aged cheese, and chocolate may be implicated, not because of food allergies, but rather because the chemicals in these foods may be vasoactive in nature. Removal of the identified trigger factor will simplify treatment.8
Classic migraine begins with a visual aura that may include blurred vision, brightly colored lights, moving lights, or distorted images. The aura may be brief or last up to a half hour and is followed by a throbbing headache. The patient seeks a quiet, dark place, after which nausea, vomiting, and sleep usually follow. Attacks occur one to three times per month and last approximately 2 to 6 hours.
In common migraine, the symptoms are more variable. The aura may be vague or not present. The prodromal phase, if present, may consist only of personality change or malaise. Headache is less often unilateral, but nausea and vomiting remain prominent.
Complex migraine is the association of migraine with transient neurologic deficits and includes ophthalmoplegic and hémiplégie varieties. The deficits are secondary to vasoconstriction and ischemia. These syndromes have a favorable prognosis.7
Migraine also includes several less common variants, including confusional migraine, basilar artery migraine, paroxysmal vertigo, paroxysmal torticollis, and cyclic vomiting.7
The approach to the adolescent with migraine headaches should be individualized based on the age of the patient, the frequency and severity of attacks, the presence of an aura, the patient's reliability, and the patient's and family's attitude toward the use of intermittent and chronic medications.7
In all patients, a dietary history should be used to identify the 5% to 10% of patients who are able to recognize known trigger substances such as chocolate and preserved meats, and remove them from their diets. Stressful factors, if recognized, should be eliminated as well.
The pharmacologie management of migraine in children can be divided into symptom control, abortive measures, and prophylactic treatment. Suppression of the patient's symptoms may include the use of analgesics such as acetaminophen or ibuprofen and antiemetics such as trimethobenzamide (Tigan). In adolescent patients with frequent or infrequent attacks, if an aura is present, abortive medications such as Cafergot or Midrin are useful. The adolescent must be instructed to take the medication at the first sign of attack. Ergotamine compounds can be given as suppositories, sublingually, via inhalation, or orally. If using the oral form of ergotamine, the patient should take two tablets at the onset of the attack, a third tablet 20 minutes later if symptoms persist, and a fourth tablet 20 minutes later if symptoms still persist. No more than four tablets should be taken during an attack, and no more than eight tablets should be used in a single week. The medication does not contain an analgesic and, if tolerated, analgesics can be taken concomitantly. Beware of ergot habituation. Isometheptene mucate (Midrin) can be administered in a similar fashion as ergotamine and seems to cause less nausea. Two tablets are taken at the onset, a third tablet 1 hour later if symptoms persist, and a fourth tablet 1 hour later if symptoms still persist. This sequence should not be followed more than twice weekly. Antiemetics can be used concomitantly. The adolescent using these abortive medications must be adequately instructed in their use.
If either young patients or adolescents have fre- quent attacks with or without an aura, or if abortive medications have been unsuccessful in the adolescent patient, prophylactic medications can be useful. The use of either cyproheptadine (Periactin) or propra- nolol ( Inderal ) over a 4- to 6-month period is recommended, in hopes of relieving the attacks completely.
Cyproheptadine has both antihistamine and cal- cium channel-blocking properties. It is also a sero- tonin antagonist. In older children, 8 to 16 mg/day is used. Side effects include weight gain and sedation. If the patient can tolerate a large dose given at bedtime, sedative effects can be avoided. Administration of cyproheptadine should begin with 4 mg nightly and then increased as tolerated, giving one third of the dosage in the morning and two thirds at night.
Propranolol is an effective prophylactic agent for treating migraine. Its primary effects include prevent- ing arterial dilatation by blocking beta receptors. It also blocks catecholam ine- induced platelet aggrega- tion and decreases platelet adhesiveness. It is contra- indicated in patients with asthma or certain cardiac conduction defects. The dosage is in the range of 3 mg/kg/day. In most patients, we begin with 10 to 20 mg/day and increase it every 2 weeks until relief of symptoms or bradycardia occurs. Side effects in ado- lescent patients are uncommon, but may include lethargy, postural hypotension, nightmares, and de- creased athletic tolerance.
Chronic-progressive headaches worry parents and patients the most, but this is the least common type of headache.4 The majority of headaches due to intracra- nial abnormalities result from IICP. In addition to headache, symptoms may include nausea and vomit- ing, ataxia, weakness, seizures, lethargy, and personal- ity change. Among the disorders causing IICP are hydrocephalus, tumors, subdural hematomas, brain abscess, and pseudotumor cerebri. Computed to- mography or MRI scans are the most rapid and useful tests for diagnosing such problems.
Chronic-nonprogressive headaches are the most common type seen in pediatrie practice, particularly in patients over 12 years of age. These headaches are also called muscle contraction, functional, or psycho- genie headaches and have no organic etiology. Etiolo- gies include stress, adjustment reaction, depression, conversion reaction, malingering, and trauma. Six- teen percent of adolescents have experienced psycho- genie headache by the age of 16. The majority of patients with chronic -nonprogressi ve headaches have daily headaches that wax and wane. A smaller percentage may have headaches several times per week. Most patients are female and usually have had headaches for months to years. Some have prolonged absences from school, although these patients usually are good students. Patients may describe their symptoms in a vague and nonspecific manner. Blurred vision, fatigue, dizziness, and fainting are common, while nausea and vomiting are rare. There is no aura, and the pain is bilateral, often described as band-like. The mechanism by which pain is caused has been ascribed to prolonged muscle contraction and resultant muscle ischemia.
It is important for the practitioner to ask specific questions concerning headaches in other family members, alcoholism, divorce, parental absence from the home, recent death of a relative, or a close relative leaving home. If the patient has had other types of pain in the past for which no etiology has been found, an emotional etiology should be suspected. A certain amount of anxiety, tension, and stress may be related to school performance or peer pressure.
Patients have a normal general physical and neurologic examination. Laboratory testing generally is not needed, although parents frequently press for tests to be performed. Imaging studies are necessary to rule out an intracranial abnormality and chronic pansinusitis. A complete psychosocial evaluation is mandatory. Where stress or adjustment problems are suspected, a child psychology consultation may be indicated. In patients who demonstrate more severe psychopathology, referral to a child psychiatrist is indicated.
It should be emphasized that stress is a more acceptable diagnostic term than is psychological dysfunction. Many parents and patients are "turned off to further evaluation and therapy if they are labeled as having an emotional disorder.
Treatment modalities include reassurance that an organic process is not present, individual and family counseling, self- hypnosis, and biofeedback alone or combined with counseling.9 Amitriptyline (Elavil) has been a useful adjunct in patients with depression and even in cases without depression. Amitriptyline has been used in the treatment of migraine with or without associated depression and in the mixed headache syndrome. Treatment in adolescents is initiated with 25 mg at night and increased to 50 to 150 mg as tolerated and as needed. Measurement of blood levels can be helpful and used as a guide to treatment and compliance. Side effects include dizziness, dryness of the mouth, visual blurring, and urinary retention.
If significant depression is present, concomitant psychiatric treatment is indicated. Treatment based solely on medication is not in the best interest of the patient. Tranquilizers and narcotic analgesics should be avoided altogether. Unfortunately, adequate data are lacking to support one therapeutic regimen over another. No definitive data regarding the ultimate outcome of children and adolescents with functional headaches are available.
Mixed headache, the combination of daily headaches without neurologic symptoms secondary to stress and acute-recurrent headaches with nausea and vomiting that are migrainous in nature, is not uncommon. In these patients, stress is a common precipitant of both the chronic daily headaches and the episodic migrainous headaches. The history reveals no symptoms of increased intracranial pressure or progressive neurologic disease. The general physical examination and the detailed neurologic examination are normal. Imaging studies are necessary to rule out an intracranial abnormality and chronic pansinusitis. A thorough psychosocial evaluation is helpful. Treatment modalities include a combination of individual counseling, family counseling, biofeedback, and antidepressant medication. Data concerning the outcome of various types of treatment for patients with this form of headache are not available.
A thorough history, general physical examination, and neurologic evaluation coupled with the charting of the temporal pattern of the headache will allow the correct diagnosis to be made under most circumstances. Laboratory testing is tailored to the specific headache syndrome. Psychological factors are important in all forms of adolescent headache and should be evaluated in each and every case. Once the correct diagnosis has been made, a comprehensive approach to the patient's problems will usually result in improvement.
1. Friedman AP. The headache m history, literature and legend, Bull NY Acad Med. 1972;18:661-681.
2. Bilie BS. Migraine in school children. ArW Pdidioir Scand. 1962;51(suppl B6):l151.
3. Headache Classification Committee of the International Headache Society Proposed classificai inn and diagnostic criteria fur headache disorders, crania! neuralgias and facial pain. Cephalalgia . 1988;8 7):9-96.
4. Rothner AD. Diagnosis arid management of headache in children and adolescents. Neurol Clin. 1983;1:511.
5. Kinast M. Luders H, Rothnet AD, Erenherg G. Benign focal epileptitorm discharges in childhood migraine (RFEDC). Neurology. 1982,32:1309-1311.
6. Kandt RS. Levine RM. Headache and acute illness in children. J Child Neurd. 1987;2:22-27.
7. Rorhner AD. The migraine sy-ndrame in children ,inii Lidolescenrs. fsdiacr PJeuwi 1986;2:121-126.
8. Prandaliei A, Weinman S, Launay JH. et al. Total IgE, specific IgE and prick rests against foods in common migraine: a prospective study. Cephalalgia. 1983;3;231-234.
9. Fentress DW, Masek BJ, Mehegan JE, et al. Biofeedback and relaxation response to training in treatment of pediatrie migraine. Dn' Med Chid Neural. 1UM6;28:139-146.
Questions for Use in Evaluating Patients Complaining off Headaches