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Management of headaches in children and adolescents
Vol. 8 No. 2 February 1993
Jounzal of Fain and SymptomManagement
81
A. David Rothner, MD Cleveland Clinic Foundation, Ckvelund, Ohio
AbsWct Deciding whether a child’s headache is functional or organic is difficult for even the expeknced physician. This revkw examines the diagnosis and management ofheadache syndromes in children and aakkents. A systematic hi.stq and examination together with selected laboratory data will enable the practitioner to establish thz correct diagnosis. J Pain Symptom Manage 1993;8:81-86. Key W0Id.s Children, adolescents, headache
Headache is a common problem in children and adolescents. According to a 1962 study of 9000 schoolchildren in Uppsala, Sweden, as many as 75% experience headache by age 15 yr.’ Although the vast majority of headaches in children are not associated with organic disease, parents often fear that their child has a brain tumor and seek the physician’s aid.* A. thorough and systematic history and physical examination, as well as selected laboratory tests, can usually guide the practitioner to the correct diagnosis.
The classification of headache is based on the presumed location of the abnormality, its origin, its pathophysiology, or the symptom complex with which the patient presents. The International Headache Society has provided one widely used classification.3 I have found it useful to classify headaches according to their Address reprint requests to: A. David Rothner, MD. Section of Child Neurology, Cleveland Clinic Foundation, 9500 Euclid kenue, Cleveland, OH 44195, USA. 0 U.S. Cancer Pain Relief Committee, MB Published by Elsevier, New York, New York
temporal pattern. 4 By plotting the severity of the headache over time, one of four sequences result: acute, acute and recurrent, chronic and progressive, and chronic and nonprogressive (Figure 1). The mixed-headache syndrome combines features of acute-recurrent headache and chronic-nonprogressive headache.
in When evaluating headache, a properly ob tained history is the most important factor in determining the correct diagnosis.The clinician should s*uspectthe etiology of the headache at the conclusion of the history. Questions should be directed to both the child and the parents. Even a young child can provide surprismgly useful information. The younger the child, however, the less specific and reliable the information. In adolescents, a private interview may prove useful as well. Answers to the questions listed in Table 1 will provide a data base from which to formulate a diagnosis. Additional questions concerning specific neurologic symptoms, such as ataxia, lethargy, seizures, vomiting, visual disturbances, weakness, and personality change, should aIs0 be 08853924/93/$6.OfJ
Vol. 8 No. 2 February 1993
Rothner
82
ACUTE PECURRENT
CHRONIC OGRESSWE
CHRONIC NPROGRESSIVE
Fig. 1. Headache types.
obtained, The standard pediatric history regarding pregnancy, labor and delivery, growth and development, academic function, behavior, previous encephalopathic events, and the systems review may provide information pertinent to the headache evaluation. Problems such as hypertension, chronic sinus disease, recurrent abdominal pain, previous emotional disorders, trauma, and the use of medications for other conditions may add to a better understanding of the headache. During the interview, it is important to pay attention to the &iect of the child and the parents, as well as the interaction between them. These observations may provide useful clues concerning subjects that are not directly discussed, such as depression, anxiety, familial conflict, and hostility. The general physical examination must include the measurement of blood pressure, as this may be the only indication of hypertension. Each organ system must be examined carefully. Height and weight must be plotted on a growth chart. If either are signi%icantly above or below normal, a hypothalamic or pituitary disorder could exist. A complete skin examination may reveal evidence of petechiae, striae, cafe au lait spots, hypopigmented lesions, or adenoma sebaceum. A thorough neurologic examination begins with the measurement of cranial circumference and comparison with normal values.5 If the head is significantly enlarged and this is not a familial trait, hydrocephalus or an occult tumor is possible. An underlying vascular malformation may be present if auscultation of
the cranium reveals an asymmetrical machinerylike bruit. The presence of tenderness of the scalp may indicate occult trauma, as seen in the battered-child syndrome. The funduscopic examination, which may be difficult in a small child, is best left until the end of the examination to avoid losing the cooperation of the child. Findings such as optic atrophy, hemorrhages, or papilledema may be clues to underlying systemic or central nervous system disorders. The presence of a sixth-nerve palsy may indicate increased intracranial pressure. Any combination of lower cranial nerve abnormalities, cerebellar dysfunction, or long-tract signs may be indicative of a brainstem or posterior fossa lesion. In the normal patient, strength, muscle bulk, muscle tone, and reflexes should be symmetric. If asymmetry is found at any level of the neuraxis, a pathologic process is likely. The pattern of the abnormality will allow localization within the nervous system. Throughout the history and physical examination, the patient’s mental status, language, affect, and level of consciousness should be monitored, as abnormalities may indicate the presence of diverse problems, such as encephalitis or a thought disorder. The clinician’s choice of laboratory tests depends on the differential diagnosis. Laboratory tests may not be indicated in otherwise uncomplicated migraine patients. I rarely find skull roentgenogram useful in localizing abnormalities in children with headache. When neurologic disease is present, most films show either no abnormality or nonspecific changes.
Vol. 8 No. PPkbmaly I993
Headaches in @hikeren and Aa!&scents
Tabk I
eadachedata base 1. Do you have more tiIdn one type of headache? 2. How did the headache begin? Trauma? Infection? 3. How long has it been present? 4. Are the symptoms worsening or staying the same? 5. Howoften do they occur? 6. Howlong do they last? 7. Do they occur at any special time or under specific circumstances? 8. Are they preceded by warning signs? 9. Where does it hurt? 10. What is the qualitv of the pain? Pounding? Sharp? 11. Do you have associated symptoms during the headache? Abdominal pain? Nausea, vomiting? 12. Do you stop what you are doing duringthe
headache? 13. Do you have any other medical problems? 14. Are you taking any medications regularly? 15. Are there any activities that make the headache
worse? 16. Does any particular medication make the headache better? 17. Does anyone else in your family have headaches? 18. What do you think is causing your headaches?
Likewise, the electroencephalogram is of limited value in the routine evaluation of children with chronic headaches. Nonspecific abnormalities are frequently found, not only in children with central nervous system disorders, but in many normal children as well. In acute disorders, persistent focal slowing or generalized slowing of a significant degree must be evaluated with more specific diagnostic tests. Epileptiform abnormalities occur to a slightly greater degree in migraine patients, but their clinical signiIIcance and relevance to therapy rarely are important6 Both computed tomography (CT) and magnetic resonance imaging (MRI) are safe and accurate methods of evaluating inuacrauial contents. They are useful in the assessment of many conditions, including malformations, infections, injuries, neoplasms, and degenerative and vascular disorders. They have essentially replaced skull roentgenogram, angiograms, pneumoencephalogmms, and isotope scans as the most valuable tests in evaluating patients with headache who are suspected of harboring an intracranial disorder. Cranial imaging is mandatory to eliminate the possibility of a structural abnormality in any patient who has a focal symptom, a progressive disorder, symptoms of increased inuacranial xpressure, or a neurologic examination that reveals
83
abnormality. Lhuing CT scanning, intravenous injection of contrast material increases the likelihood of visualizingvascular abnormalities or neoplasms. Magnetic resonance imaging has proven even more valuable when looking for abnormalities in the vicinityof the sella turcica, temporal lobes, posterior fossa, and cervicomedullaryjunction. This study may show white matter abnormalities, vascular problems, and neoplasms not visible on a CT scan. Lumbar puncture ic effective in determining the presence of an infectious process or increased intracranial pressure, as seen in pseudotumor cerebri. If an intracranial spaceoccupying lesion is suspected, however, lumbar puncture is associated with a riik of hemiation and is contraindicated without prior neuroimaging. Psychologic tests are useful in patients with “functional” headaches, as well as in those with migraine headaches thought to be precipitated by stressful circumstances.
A&4? An isolated, acute headache may represent a difficult diagnostic problem. Many children with systemic illnesses associated with fever have a generalized nonspecific headache. In the absence of nuchal rigidity or other abnormalities, symptomatic treatment of the headache with over-the-counter medications is indicated. If the patient is seriously ill, however, consideration must be given to the diagnoses of meningitis and subarachnoid hemorrhage. Other problems encountered in the emergency room include trauma, migraine, hypertension, and tension headaches. An unusual form of acute headache related to exertion bears mention here. These headaches occur acutely during such activities as weight lifting, running, playing football, and sexual intercourse. They are generalized, severe, and throbbing, and may last from a few minutes to several hours. At the time of initial presentation, and when accompanied by transient neurologic deficits, laboratory evaluation, including imaging, is indicated. Once a serious neurologic problem has been ruled out, exertional headache may respond to pretreatment with indomethacin. Localized acute headaches may be due to
84
sinusitis, oti&,, dental disorders, or head trauma. Headaches are a prominent symptom in up to 13% of patients with acute or chronic sinusitis. patients with sinusitis usually have frontal headache, rhinorrhea, chronic allergies, and cough. A liauted CT scan of the sinuses confirms the diagnosis.’ Astigmatism,refractory errors, eye strain, and squint are rarely responsible for headaches in children. However, an ophthalmologic examination is required if the headache is localized to the frontal area and is precipitated by watching television, reading, or doing schoolwork, and is relieved by stopping the ac tivlty. Dental disorders usually are diagnosed by localized pain in the jaw or teeth. Temporomandibular joint dysfunction is uncommon in children, but when present, willproduce unilateral pain in the ear, jaw, or mouth rather than the head. Headache associated with trauma frequently is related to the specific area of trauma and resolves within a few days. If the headache is protracted over months and the neurologic evaluation is negative, reassurance and simple analgesics usually improve the situation. If symptoms change, however, a reevaluation is indicated.
Acute-RecurrentHeadache Migraine is a specific genetic disorder characterized by episodic, periodic paroxysmal attacks of vasoconstriction and vasodilation. Five percent of children will have experienced migraine by the age of 15 yr. Prior to adolescence, migraine is more common in boys than girls, but occurs more frequently in girls after puberty. The clinical features of children with migraine include paroxysmal headache in 100%; relief after sleep in 94%; nausea, vomiting, and abdominal pain in 90%; a positive family history of migraine in 69%; a throbbing, pounding headache in 58%; unilateral headache in 36%; and an aura in 17%.s Phonophobia and photophobia are also quite common. Episodes may be triggered by anxiety, minor head trauma, exercise, menses, and travel. In a few patients, dietary factors such as chocolate, cola drinks, preserved meats (nitrates), and monosodium glutamate may play a role.8 Migraine can be classified into four groups: classic, common, complex, and variant forms. In children, common migraine is encountered most frequently.This headache may begin
Rdhner
&l. 8 No. 2 February I993
with premonitory pallor, malaise, fatigue, and irritability, which are followed by a bifrontal headache, nausea, and vomiting. The child will generally avoid light and noise and will try to sleep. The usual attack resolvesin 2-8 hr. Classic migraine usually begins with a visual aura, which is followed by a throbbing headache contralaterally. The remaining symptoms are similar to those of common migraine. Any child who presents with neurologic symptoms-generalized, focal, or progressive-should be suspected of having an organic process, such as a tumor or arteriovenous malformation. The diagnosis of complex migraine should be reserved for those patients whose diagnostic workup proves negative. Hemiplegic migraine is defined by hemiparesis that precedes, accompanies, or follows the headache. Neuroimaglng is indicated in all cases. If the attacks are frequent, prophylactic medications, such as cyproheptadine or propmnolol, are indicated. Calcium-channel blockers or anticonvulsants should be tried when the usual agents are unsuccessful. Ophthalmoplegic migraine involves eye pain and an incomplete or complete third-nerve palsy. Pupillary dilation, outward deviation of the eye, and ptosis are most common. Neuroimaging is needed to exclude a possible vascular abnormality or other intracranial process. The use of steroids is indicated during the acute processp and prophylactic medications are indicated when attacks are recurrent and frequent. Migraine variants are more common in the pediatric population than in adults. They include confusional migraine, basilar artery migraine, paroxysmal vertigo, and the periodic syndrome, also known as cyclic vomiting. Confusional migraines are episodes of altered consciousness, agitation, and receptive or expressive aphasia along with headache. To establish the diagnosis, other causes of acute encephalopathy, including drug abuse and encephalitis, must be ruled out. Basilar artery migraine is defined by symptoms referable to the brainstem and cerebellum. Symptoms include occipital headache, nausea, vomiting, vertigo, tinnitus, and facial weakness. Episodes usually clear completely. Paroxysmal vertigo occurs in young children between the ages of 2 and 6 yr. Episodes are brief and sudden and cause vertigo with difficulty maintaining pasture. Headache, nausea, and vomiting usually are not seen. Finally, episodes of cyclic vomit-
Vol. 8 No. 2 February 1993
Headaches in CMdm. and AdoIments
ing with abdominal pain, which are paroxysmal and result in acidosis and dehydration, may represent a migraine variant. Bnce structural disorders of the gastrointestinal tract and metabolic diseases have been excluded, migraine prophylaxis may be initiated. When treating pediatric patients with migraine, the age of the patient, his or her reliability, and the frequency and severity of attacks should be considered. The family’s attitude toward the chronic use of medication also should be noted. In younger patients with infrequent attacks, reassurance regarding the absence of underlying neurologic disease is important, and dietary and other triggers should be removed. Treatment is symptomatic and includes sedatives, analgesics, and antiemetics. If a young child has frequent and recurrent attacks, daily cyproheptadine or propranolo1 can be utiliied. In the older adolescent with infrequent auacks, especially if accompanied by an aura, the use of, for example, Midrin or ergot preparation (e.g., Cafergot) can be considered. The older adolescent with frequent attacks may benefit from cyproheptadine, propmnolol, or, at times, amitriptyline.8
Chronic-Progressive Headache A pathologic process within the cranial vault that increases irmacranial pressure will cause headaches that are progressive in severity and frequency_ The most common causes are brain tumors, pseudotumor cerebri, hydrocephalus, brain abscess, and subdural hematoma. The rapidity with which the process progresses determines the speed of evaluation. In addition to headache, symptoms of increased intracranial pressure include nausea, vomiting, weakness, ataxia, lethargy, personality change, visual disturbances, and seizures. The physical examination may be normal or may show papilledema and a sixth-nerve palsy, both of which are nonlocalizing signs of increased intracranial pressure. The type of pathologic process and its location may result in varying combinations of cortical, pyramidal, exttapyramidal, cerebellar, and cranial nerve abnormalities. If a structural abnormality of the central nervous system is suspected, an MRI or enhanced CT scan are the diagnostic tools of choice. At least ‘78% of children with brain tumors have headache as a presenting symptom. The headache is difhrse in 50% of the patients and awakens them or is present on awakening in
85
approximately 65% of the patients. Headaches caused by brain tumor may be caused by the mass ikelf or by hydrocephalus secondary to obstruction of cerebrospinal fluid flow. The location of the headache has limited localizing value. Exertion, change in position, coughing, and defecation may exacerbate the pain. The quality of the pain is variable and not diagnostic. The headache may be more severe in the morning and may be associated with and relieved by vomiting. Treatment depends on -the type and location of the neoplasm. Pseudotumor cerebri is a syndrome consisting of increased intracranial pressure without evidence of infection, mass lesion, or hydrocephalus. The usual signs are papihedema and sixth-nerve palsy. Visual field testing may show an enlarged blind spot. Neuroimaging is normal and should precede a lumbar puncture, which usually demonstrates normal chemistries and elevated pressure. Obesity, menstrual irregularity, chronic otitis, and withdrawal from corticosteroid therapy are among the many associated causes of this condition. Treatment involvesrepeated lumbar punctures to remove enough cerebrospinal fluid to normalize pressure. If necessary, diuretics and corticosteroids may be prescribed. Surgical decompression of the optic nerve sheath is indicated if vision is compromised. Subdural hematoma usuallyfollows a known instance of head trauma. It should be noted, however, that spontaneous rupture of a blood vessel or hemorrhage secondary to a blood dyscrasia are also causative. Symptoms and signs of increased intracranial pressure usually are present. Neuroimaging confirms the diagnosis and therapy involves drainage of the subdural collection by repeated subdural taps, external drainage, or shunting procedures. Brain abscesses are rare in children and adolescents, but may be found in association with cyanotic congenital heart disease, chronic sinusitis or otitis, or in an immunosuppressed child. The abscess may be single or multiple. Fever and the usual symptoms of increased intracranial pressure are present. Seizures and/ or focal neurologic deficits may be present, depending on the location of the abscess. Neuroimaging is diagnostic, and treatment involves antibiotics and surgical drainage. Hy&ocephdus can result from obstruction Ofthe normal flow of cerebrospinal fluid anywhere from its point of origin to its site of absorption.
86
There is an increased quantity of cerebrospinal fluid under increased pressure. In young children, macrocephaly, superficial cranial venous dilation, and the “sunset sign” may accompany symptoms of increased intracranial pressure. Neuroimaging reveals dilated ventricles. A shunting procedure is the treatment of choice.
Chronic-Nonjrrogressive Headache If a headache has persisted on a daily or almost da.ily basis for several months in the absence of neurologic symptoms and abnormalities on neurologic exam, the diagnosis usually is nonorganic headache.g Included in this category are patients with muscle contzzction headaches, headaches that are depressive equivalents, and conversion headaches. Chronic-nonprogressive headaches are much more common in adolescents than in children under 10 yr. In fact, stress-related headaches are the most common headache we in adolescents. In most instances, there is no aura, and the pain is bilateral and frontal, or may extend around the head in a circumferential pattern. Associated nausea is rare, although dizziness and fatigue are frequent. The pain may be daily or several times per week; often it is constant. The syndrome is more frequent in young women. Secondary gain in the form of frequent and prolonged absence from school is common, despite excellent academic abilities (the “valedictory” syndrome). In the assessment of the headache, it is important to ask specific questions about headaches in other family members, alcoholism or divorce in the home, absence of a sibling or parent from the home, and recent death of a relative. In cases of functional headache, the results of the general and neurologic examinations are normal. A neuroimaging study that includes the sinuses is helpful, as pansinusitiscan cause chronic headache.’ The diagnosis of headache secondary to stress should be discussed freely and openly with the patient and his or her parents. Treatment modalities include family counseling, individual counseling, or biofeedback alone or in conjunction with pharmacologic agents. We have found amitriptyline to be especially useful.
Mixed Headache The combination of daily headaches without neurologic symptoms, which are secondary to stress, and acute-recurrent headaches with
W. 8 No. 2 February=3
Rotlaw
nausea and vomiting, which are migrainous in nature, is not uncommon. The patient has no difficulty differentiating between the milder daily headaches and the intermittent, less frequent, severe headaches. In these patients, stress is a common contributor to both the chronic daily headaches and the episodic migrainous headaches. The general physical examination and neurologic examination are normal. Laboratory testing generally is unrevealing. A thorough psychosocial evaluation may support the diagnosis. Treatment modalities include counseling, biofeedback, and antidepressant medication.
c0?&si0?2 Even the experienced clinician may have difficulty determining whether a child’s headache is organic or functional. Highly specific questioning during the patient interviewusually will tell the practitioner all he or she needs to know about the headache syndrome. This information, coupled with a careful physical and neurologic exam, will guide the clinician in selecting laboratory tests and specific treatment.
w
erences
1. Bille BS. Migraine in school children. Acta
Paediatr Stand 1962;51 (suppl 136):1-51.
2. Koch C, Melchoir JD. Headache in childhood. Dan Med Bull 1969;16:109-114. 3. Olesen J_ Classification and diagnostic criteria of headache disorders, cranial neuralgias, and facial pain. Cephalagia 1988;8:1-96. 4. Rothner AD. Headaches in children: a review. Headache 1978;18:169. 5. Paine RS, Oppe TE. Neurologic examination of children (Clinics in developmental medicine, vols 20 and 21). Lavenham, UK: Spastics International Medical, 1966. 6. Kinast M, buders H, Rothner AD, et al. Benign focal epileptiform discharges in childhood migraine (BFEDC). Neurology 1982;32:1309-1311. 7. Faleck H, Rothner AD, Erenberg G, Cruse RP. Headache and subacute sinusitis in children and adolescents. Headache 1988;28:96-98. 8. Rothner AD. The migraine syndrome in children and adolescents. Pediatr Clin North Am 1986;2:121126. 9. Ling W, Oftedal G, Weinberg W. Depressive illness in childhood presenting as a severe headache. Am J Dis Child 1970:120:122-124.
Jounzal of Fain and SymptomManagement
81
A. David Rothner, MD Cleveland Clinic Foundation, Ckvelund, Ohio
AbsWct Deciding whether a child’s headache is functional or organic is difficult for even the expeknced physician. This revkw examines the diagnosis and management ofheadache syndromes in children and aakkents. A systematic hi.stq and examination together with selected laboratory data will enable the practitioner to establish thz correct diagnosis. J Pain Symptom Manage 1993;8:81-86. Key W0Id.s Children, adolescents, headache
Headache is a common problem in children and adolescents. According to a 1962 study of 9000 schoolchildren in Uppsala, Sweden, as many as 75% experience headache by age 15 yr.’ Although the vast majority of headaches in children are not associated with organic disease, parents often fear that their child has a brain tumor and seek the physician’s aid.* A. thorough and systematic history and physical examination, as well as selected laboratory tests, can usually guide the practitioner to the correct diagnosis.
The classification of headache is based on the presumed location of the abnormality, its origin, its pathophysiology, or the symptom complex with which the patient presents. The International Headache Society has provided one widely used classification.3 I have found it useful to classify headaches according to their Address reprint requests to: A. David Rothner, MD. Section of Child Neurology, Cleveland Clinic Foundation, 9500 Euclid kenue, Cleveland, OH 44195, USA. 0 U.S. Cancer Pain Relief Committee, MB Published by Elsevier, New York, New York
temporal pattern. 4 By plotting the severity of the headache over time, one of four sequences result: acute, acute and recurrent, chronic and progressive, and chronic and nonprogressive (Figure 1). The mixed-headache syndrome combines features of acute-recurrent headache and chronic-nonprogressive headache.
in When evaluating headache, a properly ob tained history is the most important factor in determining the correct diagnosis.The clinician should s*uspectthe etiology of the headache at the conclusion of the history. Questions should be directed to both the child and the parents. Even a young child can provide surprismgly useful information. The younger the child, however, the less specific and reliable the information. In adolescents, a private interview may prove useful as well. Answers to the questions listed in Table 1 will provide a data base from which to formulate a diagnosis. Additional questions concerning specific neurologic symptoms, such as ataxia, lethargy, seizures, vomiting, visual disturbances, weakness, and personality change, should aIs0 be 08853924/93/$6.OfJ
Vol. 8 No. 2 February 1993
Rothner
82
ACUTE PECURRENT
CHRONIC OGRESSWE
CHRONIC NPROGRESSIVE
Fig. 1. Headache types.
obtained, The standard pediatric history regarding pregnancy, labor and delivery, growth and development, academic function, behavior, previous encephalopathic events, and the systems review may provide information pertinent to the headache evaluation. Problems such as hypertension, chronic sinus disease, recurrent abdominal pain, previous emotional disorders, trauma, and the use of medications for other conditions may add to a better understanding of the headache. During the interview, it is important to pay attention to the &iect of the child and the parents, as well as the interaction between them. These observations may provide useful clues concerning subjects that are not directly discussed, such as depression, anxiety, familial conflict, and hostility. The general physical examination must include the measurement of blood pressure, as this may be the only indication of hypertension. Each organ system must be examined carefully. Height and weight must be plotted on a growth chart. If either are signi%icantly above or below normal, a hypothalamic or pituitary disorder could exist. A complete skin examination may reveal evidence of petechiae, striae, cafe au lait spots, hypopigmented lesions, or adenoma sebaceum. A thorough neurologic examination begins with the measurement of cranial circumference and comparison with normal values.5 If the head is significantly enlarged and this is not a familial trait, hydrocephalus or an occult tumor is possible. An underlying vascular malformation may be present if auscultation of
the cranium reveals an asymmetrical machinerylike bruit. The presence of tenderness of the scalp may indicate occult trauma, as seen in the battered-child syndrome. The funduscopic examination, which may be difficult in a small child, is best left until the end of the examination to avoid losing the cooperation of the child. Findings such as optic atrophy, hemorrhages, or papilledema may be clues to underlying systemic or central nervous system disorders. The presence of a sixth-nerve palsy may indicate increased intracranial pressure. Any combination of lower cranial nerve abnormalities, cerebellar dysfunction, or long-tract signs may be indicative of a brainstem or posterior fossa lesion. In the normal patient, strength, muscle bulk, muscle tone, and reflexes should be symmetric. If asymmetry is found at any level of the neuraxis, a pathologic process is likely. The pattern of the abnormality will allow localization within the nervous system. Throughout the history and physical examination, the patient’s mental status, language, affect, and level of consciousness should be monitored, as abnormalities may indicate the presence of diverse problems, such as encephalitis or a thought disorder. The clinician’s choice of laboratory tests depends on the differential diagnosis. Laboratory tests may not be indicated in otherwise uncomplicated migraine patients. I rarely find skull roentgenogram useful in localizing abnormalities in children with headache. When neurologic disease is present, most films show either no abnormality or nonspecific changes.
Vol. 8 No. PPkbmaly I993
Headaches in @hikeren and Aa!&scents
Tabk I
eadachedata base 1. Do you have more tiIdn one type of headache? 2. How did the headache begin? Trauma? Infection? 3. How long has it been present? 4. Are the symptoms worsening or staying the same? 5. Howoften do they occur? 6. Howlong do they last? 7. Do they occur at any special time or under specific circumstances? 8. Are they preceded by warning signs? 9. Where does it hurt? 10. What is the qualitv of the pain? Pounding? Sharp? 11. Do you have associated symptoms during the headache? Abdominal pain? Nausea, vomiting? 12. Do you stop what you are doing duringthe
headache? 13. Do you have any other medical problems? 14. Are you taking any medications regularly? 15. Are there any activities that make the headache
worse? 16. Does any particular medication make the headache better? 17. Does anyone else in your family have headaches? 18. What do you think is causing your headaches?
Likewise, the electroencephalogram is of limited value in the routine evaluation of children with chronic headaches. Nonspecific abnormalities are frequently found, not only in children with central nervous system disorders, but in many normal children as well. In acute disorders, persistent focal slowing or generalized slowing of a significant degree must be evaluated with more specific diagnostic tests. Epileptiform abnormalities occur to a slightly greater degree in migraine patients, but their clinical signiIIcance and relevance to therapy rarely are important6 Both computed tomography (CT) and magnetic resonance imaging (MRI) are safe and accurate methods of evaluating inuacrauial contents. They are useful in the assessment of many conditions, including malformations, infections, injuries, neoplasms, and degenerative and vascular disorders. They have essentially replaced skull roentgenogram, angiograms, pneumoencephalogmms, and isotope scans as the most valuable tests in evaluating patients with headache who are suspected of harboring an intracranial disorder. Cranial imaging is mandatory to eliminate the possibility of a structural abnormality in any patient who has a focal symptom, a progressive disorder, symptoms of increased inuacranial xpressure, or a neurologic examination that reveals
83
abnormality. Lhuing CT scanning, intravenous injection of contrast material increases the likelihood of visualizingvascular abnormalities or neoplasms. Magnetic resonance imaging has proven even more valuable when looking for abnormalities in the vicinityof the sella turcica, temporal lobes, posterior fossa, and cervicomedullaryjunction. This study may show white matter abnormalities, vascular problems, and neoplasms not visible on a CT scan. Lumbar puncture ic effective in determining the presence of an infectious process or increased intracranial pressure, as seen in pseudotumor cerebri. If an intracranial spaceoccupying lesion is suspected, however, lumbar puncture is associated with a riik of hemiation and is contraindicated without prior neuroimaging. Psychologic tests are useful in patients with “functional” headaches, as well as in those with migraine headaches thought to be precipitated by stressful circumstances.
A&4? An isolated, acute headache may represent a difficult diagnostic problem. Many children with systemic illnesses associated with fever have a generalized nonspecific headache. In the absence of nuchal rigidity or other abnormalities, symptomatic treatment of the headache with over-the-counter medications is indicated. If the patient is seriously ill, however, consideration must be given to the diagnoses of meningitis and subarachnoid hemorrhage. Other problems encountered in the emergency room include trauma, migraine, hypertension, and tension headaches. An unusual form of acute headache related to exertion bears mention here. These headaches occur acutely during such activities as weight lifting, running, playing football, and sexual intercourse. They are generalized, severe, and throbbing, and may last from a few minutes to several hours. At the time of initial presentation, and when accompanied by transient neurologic deficits, laboratory evaluation, including imaging, is indicated. Once a serious neurologic problem has been ruled out, exertional headache may respond to pretreatment with indomethacin. Localized acute headaches may be due to
84
sinusitis, oti&,, dental disorders, or head trauma. Headaches are a prominent symptom in up to 13% of patients with acute or chronic sinusitis. patients with sinusitis usually have frontal headache, rhinorrhea, chronic allergies, and cough. A liauted CT scan of the sinuses confirms the diagnosis.’ Astigmatism,refractory errors, eye strain, and squint are rarely responsible for headaches in children. However, an ophthalmologic examination is required if the headache is localized to the frontal area and is precipitated by watching television, reading, or doing schoolwork, and is relieved by stopping the ac tivlty. Dental disorders usually are diagnosed by localized pain in the jaw or teeth. Temporomandibular joint dysfunction is uncommon in children, but when present, willproduce unilateral pain in the ear, jaw, or mouth rather than the head. Headache associated with trauma frequently is related to the specific area of trauma and resolves within a few days. If the headache is protracted over months and the neurologic evaluation is negative, reassurance and simple analgesics usually improve the situation. If symptoms change, however, a reevaluation is indicated.
Acute-RecurrentHeadache Migraine is a specific genetic disorder characterized by episodic, periodic paroxysmal attacks of vasoconstriction and vasodilation. Five percent of children will have experienced migraine by the age of 15 yr. Prior to adolescence, migraine is more common in boys than girls, but occurs more frequently in girls after puberty. The clinical features of children with migraine include paroxysmal headache in 100%; relief after sleep in 94%; nausea, vomiting, and abdominal pain in 90%; a positive family history of migraine in 69%; a throbbing, pounding headache in 58%; unilateral headache in 36%; and an aura in 17%.s Phonophobia and photophobia are also quite common. Episodes may be triggered by anxiety, minor head trauma, exercise, menses, and travel. In a few patients, dietary factors such as chocolate, cola drinks, preserved meats (nitrates), and monosodium glutamate may play a role.8 Migraine can be classified into four groups: classic, common, complex, and variant forms. In children, common migraine is encountered most frequently.This headache may begin
Rdhner
&l. 8 No. 2 February I993
with premonitory pallor, malaise, fatigue, and irritability, which are followed by a bifrontal headache, nausea, and vomiting. The child will generally avoid light and noise and will try to sleep. The usual attack resolvesin 2-8 hr. Classic migraine usually begins with a visual aura, which is followed by a throbbing headache contralaterally. The remaining symptoms are similar to those of common migraine. Any child who presents with neurologic symptoms-generalized, focal, or progressive-should be suspected of having an organic process, such as a tumor or arteriovenous malformation. The diagnosis of complex migraine should be reserved for those patients whose diagnostic workup proves negative. Hemiplegic migraine is defined by hemiparesis that precedes, accompanies, or follows the headache. Neuroimaglng is indicated in all cases. If the attacks are frequent, prophylactic medications, such as cyproheptadine or propmnolol, are indicated. Calcium-channel blockers or anticonvulsants should be tried when the usual agents are unsuccessful. Ophthalmoplegic migraine involves eye pain and an incomplete or complete third-nerve palsy. Pupillary dilation, outward deviation of the eye, and ptosis are most common. Neuroimaging is needed to exclude a possible vascular abnormality or other intracranial process. The use of steroids is indicated during the acute processp and prophylactic medications are indicated when attacks are recurrent and frequent. Migraine variants are more common in the pediatric population than in adults. They include confusional migraine, basilar artery migraine, paroxysmal vertigo, and the periodic syndrome, also known as cyclic vomiting. Confusional migraines are episodes of altered consciousness, agitation, and receptive or expressive aphasia along with headache. To establish the diagnosis, other causes of acute encephalopathy, including drug abuse and encephalitis, must be ruled out. Basilar artery migraine is defined by symptoms referable to the brainstem and cerebellum. Symptoms include occipital headache, nausea, vomiting, vertigo, tinnitus, and facial weakness. Episodes usually clear completely. Paroxysmal vertigo occurs in young children between the ages of 2 and 6 yr. Episodes are brief and sudden and cause vertigo with difficulty maintaining pasture. Headache, nausea, and vomiting usually are not seen. Finally, episodes of cyclic vomit-
Vol. 8 No. 2 February 1993
Headaches in CMdm. and AdoIments
ing with abdominal pain, which are paroxysmal and result in acidosis and dehydration, may represent a migraine variant. Bnce structural disorders of the gastrointestinal tract and metabolic diseases have been excluded, migraine prophylaxis may be initiated. When treating pediatric patients with migraine, the age of the patient, his or her reliability, and the frequency and severity of attacks should be considered. The family’s attitude toward the chronic use of medication also should be noted. In younger patients with infrequent attacks, reassurance regarding the absence of underlying neurologic disease is important, and dietary and other triggers should be removed. Treatment is symptomatic and includes sedatives, analgesics, and antiemetics. If a young child has frequent and recurrent attacks, daily cyproheptadine or propranolo1 can be utiliied. In the older adolescent with infrequent auacks, especially if accompanied by an aura, the use of, for example, Midrin or ergot preparation (e.g., Cafergot) can be considered. The older adolescent with frequent attacks may benefit from cyproheptadine, propmnolol, or, at times, amitriptyline.8
Chronic-Progressive Headache A pathologic process within the cranial vault that increases irmacranial pressure will cause headaches that are progressive in severity and frequency_ The most common causes are brain tumors, pseudotumor cerebri, hydrocephalus, brain abscess, and subdural hematoma. The rapidity with which the process progresses determines the speed of evaluation. In addition to headache, symptoms of increased intracranial pressure include nausea, vomiting, weakness, ataxia, lethargy, personality change, visual disturbances, and seizures. The physical examination may be normal or may show papilledema and a sixth-nerve palsy, both of which are nonlocalizing signs of increased intracranial pressure. The type of pathologic process and its location may result in varying combinations of cortical, pyramidal, exttapyramidal, cerebellar, and cranial nerve abnormalities. If a structural abnormality of the central nervous system is suspected, an MRI or enhanced CT scan are the diagnostic tools of choice. At least ‘78% of children with brain tumors have headache as a presenting symptom. The headache is difhrse in 50% of the patients and awakens them or is present on awakening in
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approximately 65% of the patients. Headaches caused by brain tumor may be caused by the mass ikelf or by hydrocephalus secondary to obstruction of cerebrospinal fluid flow. The location of the headache has limited localizing value. Exertion, change in position, coughing, and defecation may exacerbate the pain. The quality of the pain is variable and not diagnostic. The headache may be more severe in the morning and may be associated with and relieved by vomiting. Treatment depends on -the type and location of the neoplasm. Pseudotumor cerebri is a syndrome consisting of increased intracranial pressure without evidence of infection, mass lesion, or hydrocephalus. The usual signs are papihedema and sixth-nerve palsy. Visual field testing may show an enlarged blind spot. Neuroimaging is normal and should precede a lumbar puncture, which usually demonstrates normal chemistries and elevated pressure. Obesity, menstrual irregularity, chronic otitis, and withdrawal from corticosteroid therapy are among the many associated causes of this condition. Treatment involvesrepeated lumbar punctures to remove enough cerebrospinal fluid to normalize pressure. If necessary, diuretics and corticosteroids may be prescribed. Surgical decompression of the optic nerve sheath is indicated if vision is compromised. Subdural hematoma usuallyfollows a known instance of head trauma. It should be noted, however, that spontaneous rupture of a blood vessel or hemorrhage secondary to a blood dyscrasia are also causative. Symptoms and signs of increased intracranial pressure usually are present. Neuroimaging confirms the diagnosis and therapy involves drainage of the subdural collection by repeated subdural taps, external drainage, or shunting procedures. Brain abscesses are rare in children and adolescents, but may be found in association with cyanotic congenital heart disease, chronic sinusitis or otitis, or in an immunosuppressed child. The abscess may be single or multiple. Fever and the usual symptoms of increased intracranial pressure are present. Seizures and/ or focal neurologic deficits may be present, depending on the location of the abscess. Neuroimaging is diagnostic, and treatment involves antibiotics and surgical drainage. Hy&ocephdus can result from obstruction Ofthe normal flow of cerebrospinal fluid anywhere from its point of origin to its site of absorption.
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There is an increased quantity of cerebrospinal fluid under increased pressure. In young children, macrocephaly, superficial cranial venous dilation, and the “sunset sign” may accompany symptoms of increased intracranial pressure. Neuroimaging reveals dilated ventricles. A shunting procedure is the treatment of choice.
Chronic-Nonjrrogressive Headache If a headache has persisted on a daily or almost da.ily basis for several months in the absence of neurologic symptoms and abnormalities on neurologic exam, the diagnosis usually is nonorganic headache.g Included in this category are patients with muscle contzzction headaches, headaches that are depressive equivalents, and conversion headaches. Chronic-nonprogressive headaches are much more common in adolescents than in children under 10 yr. In fact, stress-related headaches are the most common headache we in adolescents. In most instances, there is no aura, and the pain is bilateral and frontal, or may extend around the head in a circumferential pattern. Associated nausea is rare, although dizziness and fatigue are frequent. The pain may be daily or several times per week; often it is constant. The syndrome is more frequent in young women. Secondary gain in the form of frequent and prolonged absence from school is common, despite excellent academic abilities (the “valedictory” syndrome). In the assessment of the headache, it is important to ask specific questions about headaches in other family members, alcoholism or divorce in the home, absence of a sibling or parent from the home, and recent death of a relative. In cases of functional headache, the results of the general and neurologic examinations are normal. A neuroimaging study that includes the sinuses is helpful, as pansinusitiscan cause chronic headache.’ The diagnosis of headache secondary to stress should be discussed freely and openly with the patient and his or her parents. Treatment modalities include family counseling, individual counseling, or biofeedback alone or in conjunction with pharmacologic agents. We have found amitriptyline to be especially useful.
Mixed Headache The combination of daily headaches without neurologic symptoms, which are secondary to stress, and acute-recurrent headaches with
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nausea and vomiting, which are migrainous in nature, is not uncommon. The patient has no difficulty differentiating between the milder daily headaches and the intermittent, less frequent, severe headaches. In these patients, stress is a common contributor to both the chronic daily headaches and the episodic migrainous headaches. The general physical examination and neurologic examination are normal. Laboratory testing generally is unrevealing. A thorough psychosocial evaluation may support the diagnosis. Treatment modalities include counseling, biofeedback, and antidepressant medication.
c0?&si0?2 Even the experienced clinician may have difficulty determining whether a child’s headache is organic or functional. Highly specific questioning during the patient interviewusually will tell the practitioner all he or she needs to know about the headache syndrome. This information, coupled with a careful physical and neurologic exam, will guide the clinician in selecting laboratory tests and specific treatment.
w
erences
1. Bille BS. Migraine in school children. Acta
Paediatr Stand 1962;51 (suppl 136):1-51.
2. Koch C, Melchoir JD. Headache in childhood. Dan Med Bull 1969;16:109-114. 3. Olesen J_ Classification and diagnostic criteria of headache disorders, cranial neuralgias, and facial pain. Cephalagia 1988;8:1-96. 4. Rothner AD. Headaches in children: a review. Headache 1978;18:169. 5. Paine RS, Oppe TE. Neurologic examination of children (Clinics in developmental medicine, vols 20 and 21). Lavenham, UK: Spastics International Medical, 1966. 6. Kinast M, buders H, Rothner AD, et al. Benign focal epileptiform discharges in childhood migraine (BFEDC). Neurology 1982;32:1309-1311. 7. Faleck H, Rothner AD, Erenberg G, Cruse RP. Headache and subacute sinusitis in children and adolescents. Headache 1988;28:96-98. 8. Rothner AD. The migraine syndrome in children and adolescents. Pediatr Clin North Am 1986;2:121126. 9. Ling W, Oftedal G, Weinberg W. Depressive illness in childhood presenting as a severe headache. Am J Dis Child 1970:120:122-124.