Chapter 55: Headache

• Most headaches in children are benign.

• Headaches can be classified as primary or secondary

• Brain tumor headaches in children are associated with neurologic findings such as papilledema, ataxia, behavior change, altered mental status, and weakness

• Pseudotumor cerebri causes headache in the absence of a mass lesion.

Headaches are common in childhood. As many as 90% of children experience a headache by the age of 18 years.^1,2 Although they usually do not result from serious disease, headaches are sometimes the manifestation of life-threatening illness. It is incumbent on the emergency physician to distinguish those headaches that result from benign, self-limited processes from those that can result in serious morbidity or mortality.

Headaches can be classified as primary or secondary. Primary headaches include migraine, tension-type, and cluster headaches, and are based on criteria defined by the International Headache Society and the International Classification of Headache Disorders.³ Secondary headaches have identifiable etiologies based on history and physical examination. These include headaches attributed to head or neck trauma, infection (influenza, viral illness, meningitis, brain abscess, encephalitis), a vascular disorder such as bleed or stroke, a nonvascular intracranial disorder such as elevated intracranial pressure or a neoplasm, or a medication or toxin such as carbon monoxide, cocaine, medication overuse.^1–5 Facial pain, sinus, eye and dental problems can also cause headaches. Some patients with psychiatric disorders will complain of headache. The brain itself is not sensitive to pain, but there are pain-sensitive structures in the skin, the muscles, the vascular sinuses, the intracranial blood vessels, and the meninges at the base of the brain. Inflammation, dilation, irritation, and displacement of the pain-sensitive areas can result in a headache.⁴

The evaluation of a child for a headache includes information on the headache history. Based on this information, the headache can be classified as acute (sudden, first), acute and recurrent (episodic), chronic and progressive (steadily worsening), or chronic and nonprogressive.^1,2 One way to help determine headache etiology is demonstrated in Table 55-1.

The following information should be obtained: age at onset, frequency and duration (minutes, days), time of onset (day, night, school days only), location (frontal, temporal, occipital), quality of pain (stabbing, pressure, pounding), change in frequency of headache, associated symptoms (nausea/vomiting, photophobia), warning signs or aura (blurred vision, vertigo, nausea, weakness), precipitating factors (stress, coughing, certain foods), relieving factors (sleep), recent trauma, change in school or home environment, response to treatment at home, and family history of migraines.^1,2,4,5

The physical evaluation includes general appearance, blood pressure and temperature, height, weight, and head circumference. The eyes are assessed for extraocular nerve palsies or nystagmus. A funduscopic examination evaluates the possibility of papilledema. Examination of the head assesses the temporomandibular joints and sinuses. The neck is auscultated for bruits that would indicate an arteriovenous malformation, and assessed for the presence of meningismus or rigidity. The skin is examined for café-au-lait spots, neurofibromas, and ash-leaf spots. The neurologic examination includes strength testing, deep tendon reflexes, Romberg test, gait testing, cerebellar tests, Brudzinskis and Kernig's sign.^1,2,5

The main concern for a physician is whether intracranial pathology exists. Risk factors/red flags for this include sleep-related headache, absence of family history of migraine, vomiting, absence of visual symptoms, worsening headache (more severe, more frequent), change in headache type, headache less than 6 months, confusion, exclusively occipital headaches, and an abnormal neurologic examination.^2,6 Findings associated with brain tumor headaches in children include papilledema, ataxia, behavior change, altered mental status, and weakness.^2,6 Other life-threatening causes of headache that should not be missed include bacterial meningitis, orbital or cerebral abscess, viral encephalitis, hydrocephalus, intracranial hemorrhage, hypertensive encephalopathy, and carbon monoxide poisoning.

Laboratory studies should be performed based upon the suspected etiology of the headache. If the blood pressure is elevated, electrolytes, BUN, creatinine, and urinalysis are appropriate. For a child with a fever, CBC, blood cultures, and cerebrospinal fluid (CSF) studies, if a lumbar puncture is performed, are appropriate.^1,6,7

Neuroimaging should be performed for a child with signs of increased intracranial pressure, focal symptoms, an abnormal neurologic examination, seizures, skin lesions suggestive of a neurocutaneous syndrome, recent head trauma, and a progressive neurologic disorder (Fig. 55-1).^1,5,7 While a computed tomography (CT) scan is usually adequate to see a space-occupying lesion, bleed, hydrocephalus, and abscess, a magnetic resonance imaging (MRI) may be needed to demonstrate sellar lesions, some small posterior fossa lesions, white-matter abnormalities, and congenital anomalies. However, MRI may not be immediately available, and frequently requires the child be sedated for the procedure.^1,5,6

The American Academy of Neurology and the Child Neurology Society developed a practice parameter for the evaluation of children and adolescents with recurrent headaches. They stated that routine neuroimaging is not recommended in children with recurrent headaches and a normal neurologic examination. Neuroimaging should be considered in the child with headache who has an abnormal neurologic examination consisting of focal findings, signs of increased intracranial pressure, altered level of consciousness, or coexistent seizures or both. Neuroimaging should be considered in children with historical features to suggest recent onset of severe headache, change in the type of headache, or features that suggest neurologic dysfunction.⁷

A child suspected of having meningitis/encephalitis requires a lumbar puncture (LP). If there is concern for a subarachnoid hemorrhage, an LP can be diagnostic if the CT was inconclusive. In addition, if idiopathic intracranial hypertension (pseudotumor cerebri) is being considered, an LP with an opening pressure is required (>20 cm H₂O, with normal CSF findings is diagnostic).

The characteristics of primary headaches are described in Table 55-2.

Migraine Headaches

Migraine headaches are an example of recurrent headaches. The mean age of onset is 7.2 years for males and 10.9 years for females, but the prevalence increases from 3% in children aged 3 to 7 years, to 8% to 23% for children aged 11 to 15 years.^2,8,9 In the younger age group, males are more affected than females, but this reverses by the older age group.^2,9,10 The headache tends to be unilateral, throbbing or pulsating, lasts 1 to 48 hours, is often associated with nausea and vomiting, and is relieved with sleep.^9,10 In younger patients, the symptoms include pallor, vomiting, and decreased activity.¹⁰ There is a genetic predisposition to migraines, with a positive family history in most cases.^2,10

Several theories exist as to the pathophysiology of migraines. It is a primary neuronal process associated with intracranial and extracranial changes that have a genetic predisposition.^9,10

Antidromic stimulation of the trigeminal nerve releases substance P, calcitonin generated peptide, and other vasoactive polypeptides that cause pain and vasodilatation (neurogenic inflammation). There is also a cortical spreading depression of Leao, which is neuronal and glial hyperpolarization followed by depolarization that causes the aura of migraine, activates trigeminal afferents, and alters blood–brain barrier permeability, resulting in the headache.^9,10

The classification of migraines includes migraine with aura, migraine without aura, and childhood periodic syndromes that are commonly precursors of migraine (abdominal migraines, benign paroxysmal vertigo of childhood, and cyclic vomiting).^3,9,11 The IHS classification of pediatric migraine without aura, which accounts for 60% to 85% of cases, includes the following:

1. At least five attacks fulfilling criteria B to D.

2. Headache attacks lasting 1 to 72 hours.

3. Headache has at least two of the following: either bilateral or unilateral location (frontal/temporal, but not occipital), pulsating quality, moderate-to-severe intensity aggravated by routine physical activities such as walking.

4. At least one of the following during the headache: nausea and/or vomiting, photophobia, and phonophobia (may be inferred from behavior).^2,3,8,9

Pediatric migraine with aura occurs in 15% to 30% of patients, with the aura occurring before the headache.² The diagnostic criteria includes the following:

1. At least two attacks fulfilling criteria B to D.

2. An aura consisting of at least one of the following¹: fully reversible visual symptoms such as flickering lights or spots,² fully reversible sensory symptoms such as pins and needles or numbness, or³ fully reversible dysphasic speech disturbances. Motor weakness is not a criteria.

3. At least two of the following¹: homonymous visual symptoms or unilateral sensory symptoms,² at least one aura symptom develops gradually over ≥5 minutes or different aura symptoms occur in succession ≥5 minutes,³ each symptom lasts ≥5 minutes and ≤60 minutes.

4. Symptoms are not attributable to another disorder.^2,3,9

Basilar-type migraines are a subgroup of migraines with aura, and accounts for 3% to 19% of childhood migraines, and the headache is often occipital.^3,9 Therefore a basilar-type migraine must fulfill the criteria for a migraine with aura, but is accompanied by two or more of the following symptoms: vertigo, ataxia, diplopia, tinnitus, hyperacusis, dysarthria, bilateral paresthesias, decreased level of consciousness, decreased hearing.^2,3,9

Hemiplegic migraines have motor weakness or hemiplegia as part of the aura.^9,11 It can be familial, with an autosomal dominant inheritance and due to a mutation in a calcium channel gene.^2,3,9,11

The childhood periodic syndromes that are commonly precursors of migraines include of childhood benign paroxysmal vertigo, cyclic vomiting, and abdominal migraine.^3,9,11 Benign paroxysmal vertigo occurs in children aged 2 to 6 years and consists of sudden, brief episodes of ataxia or unsteadiness, when the child cannot stand upright without support. There is no loss of consciousness, but nystagmus or pallor often occurs. The episode lasts for several minutes, and then the child recovers completely.^9,11

Cyclic vomiting syndrome is recurrent episodes of severe vomiting that last for a few hours to days, separated by symptom-free periods. Treatment includes IV fluids with glucose, antiemetic medication, and sometimes sedation.^9,11

Abdominal migraine is recurrent episodes of abdominal pain lasting 1 to 72 hours, usually vague, and midline or periumbilical in location. It may be accompanied by nausea, vomiting, anorexia, or pallor, but there is no fever or diarrhea.^9,11

Treatment

Reassurance and patient, and parental education are the first steps. Patients should keep a headache diary in order to see if there are any precipitating factors. These include emotional stress, anxiety, menstruation, missing a meal, lack of sleep, and environmental factors such as bright lights, loud noises, and perfumes. Certain foods such as those that contain tyramine (aged cheese), sodium nitrite (hot dogs, smoked meats), or monosodium glutamate (Chinese food) can precipitate migraines, as can caffeine-containing beverages, chocolate, cheese, citrus fruits, and certain drugs (such as oral contraceptives, antihypertensive medications, cimetidine, and H₂-blockers).^1,4,9

ED treatment of migraines consists of providing analgesia and treating associated symptoms such as nausea during the acute attack. Analgesics such as acetaminophen (15 mg/kg), ibuprofen (10 mg/kg), or naproxen (5 mg/kg) are often effective.^2,9,12 If the child is unable to tolerate oral NSAIDs, intravenous ketorolac can be used.¹²

Triptans are serotonin agonists that inhibit the release of vasoactive peptides, promote vasoconstriction and block pain pathways. Unfortunately most have not been approved for pediatric use, so although studied and used, this is done off-label.¹² Sumatriptan is a selective 5-HT agonist that can be given subcutaneously (6 mg) (although not FDA approved for children <16 years) or as a nasal spray for children older than 12 years.^8,9,12 Nasal zolmitriptan has also been used in older children.^8,9,12 Oral sumatriptan has been used successfully in adolescents.¹²

For children older than 10 years who present to the ED with a migraine headache, dihydroergotamine mesylate (DHE) 0.25 to 1.0 mg over 3 minutes intravenously may be beneficial, especially if given with metoclopramide. For nausea, antiemetics such as ondansetron, promethazine, metoclopramide, or prochlorperazine have been used.^2,12

Preventive treatments include cyproheptadine, β-blockers (propranolol), antidepressants (amitriptyline, nortriptyline), anticonvulsants (topiramate, gabapentin, valproic acid, levetiracetam), and calcium channel blockers (verapamil), but conclusive data are lacking regarding their use in children and adolescents.^2,8,9,12 Methods such as relaxation therapy, biofeedback, cognitive therapy/stress management, acupuncture, and dietary measures with supplements such as riboflavin (Vitamin B₂), magnesium, melatonin or Coenzyme Q10, may have some benefit.^2,9 Those children who have frequent headaches and those with headaches that are unresponsive to abortive measures should be placed on prophylactic medications after consultation with a neurologist.

Tension-Type Headaches

Tension-type headaches (formerly called muscle contraction or stress headaches) tend to be chronic and nonprogressive in nature, with the pain described as band-like, bilateral, or generalized. There is no accompanying aura, and nausea is rare. The headache can last for 30 minutes to days and can be accompanied by photophobia or phonophobia but is not aggravated by physical activity.^1,13 There are three subtypes of tension-type headache: infrequent, occurring less than 1 day a month; frequent episodic, occurring 1 to 14 days a month; and chronic, occurring 15 or more days a month.^2,3,13

The pathophysiology of tension-type headache is unknown but may be due to heightened sensitivity of pain pathways.¹³

The diagnosis of tension-type headache is usually based upon clinical criteria, but the differential diagnosis includes infection, increased intracranial pressure, Chiari I malformation, analgesic rebound headache, and chronic sinus infection.¹³ Of note, the physical and neurologic examination of a child with tension-type headaches is normal.

Tension-type headaches are generally managed with emotional support, and mild analgesics, such as acetaminophen and ibuprofen. Reassuring the family that the problem is not organic and advising the patient to avoid precipitating factors, such as stress, is an important part of therapy. Behavioral techniques, such as biofeedback, and relaxation exercises may be useful.¹³

Cluster Headaches

Cluster headaches are uncommon in children younger than 10 years of age. The headache is unilateral, occurs in the frontal or periorbital region, often beginning behind or around the eye, and always occurs on the same side.^1,4 The headache lasts 15 minutes to 3 hours, and is associated with ipsilateral lacrimation, redness of the eye, and ipsilateral nasal congestion; the cheek may become flushed and warm. The patient may develop Horner syndrome—miosis, ptosis, and facial anhidrosis—on the side of the headache.^1,4 The headache tends to occur at the same time each day during a cluster. Patients are unable to lie down or rest because of the pain.⁴ Abortive treatment consists of 100% oxygen at 8 to 10 L/min.⁴ Recurrences may be managed with prednisone (1 mg/kg) for 5 days followed by a 2-week taper.⁴

Brain Tumors and Hydrocephalus

The presence of a headache that is made worse by lying down, or that comes on with coughing, sneezing, or straining at stool and then disappears, suggests increased intracranial pressure.¹ Headaches associated with disorders related to increased intracranial pressure are of a progressive nature. Papilledema is often found on funduscopic examination. A complete neurologic examination may disclose other abnormalities, such as ataxia with a cerebellar tumor, or cranial nerve findings with hydrocephalus. Differentiation of these disorders is by CT scan or MRI, with appropriate consultation if hydrocephalus or a tumor is found.

Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

Idiopathic intracranial hypertension or pseudotumor cerebri is a condition associated with increased intracranial pressure in the absence of a mass lesion or other obvious etiology. It results from impaired reabsorption of CSF. While many cases are idiopathic, it is associated with obesity, pregnancy, high doses of vitamin A, birth control pills, retinoic acid, tetracycline, infections including mastoiditis and otitis media, endocrinopathies, systemic lupus erythematosus, and steroid withdrawal.¹⁴ Patients have a headache, and may complain of blurred vision or diplopia. Physical examination reveals visual field loss, papilledema, and an abducens (sixth cranial nerve) palsy.¹⁵A neuroimaging study, preferably an MRI is done to exclude other causes of increased intracranial pressure.¹⁵ LP should be performed in the lateral decubitus position with the legs extended, and will reveal an opening pressure >20 cm H₂O, with normal protein, glucose, and cell count.¹⁵ Therapy includes treating the underlying cause, serial LPs to relieve acute symptoms, and acetazolamide 15 mg/kg given four times a day to reduce the formation of CSF.¹⁶

Hypertensive Encephalopathy

Severe elevation in blood pressure can cause headache and if untreated can result in the development of encephalopathy and seizures. This should be suspected in a patient with a severe headache whose diastolic blood pressure is greater than the 95th percentile for age. In young children, the development of hypertension is often secondary to an acute illness, such as fulminant glomerulonephritis. It can also be secondary to acute exacerbations in patients with known hypertension. In severe cases, hypertension can result in cardiac as well as neurologic dysfunction. Since other causes of increased intracranial pressure can also occasionally result in hypertension, it is essential that they be excluded. This includes coarctation of the aorta, so blood pressure should be checked in all extremities.

Acute therapy in the ED is individualized, based on complete physical examination findings, degree of hypertension, and past history of hypertension. Patients with encephalopathy and seizures require rapid reduction of blood pressure with an agent such as nitroprusside. The patient is admitted for blood pressure control and complete evaluation.

Acute Hemorrhage

The child presenting with a severe headache of sudden onset, with or without neck or back pain, may have suffered an intracranial hemorrhage. The patient's mental status can range from normal to coma. Focal findings and seizures may or may not be present. Spontaneous intracranial hemorrhage usually results from either a ruptured aneurysm or arteriovenous malformation. It can also occur in association with coagulopathies. The diagnosis and management are further discussed in Chapter 57 on cerebrovascular syndromes.

Meningitis/Encephalitis/Brain Abscess

The association of headache with a fever and stiff neck implies an infectious etiology, such as bacterial meningitis, encephalitis, or brain abscess. A brain abscess can result from orbital cellulitis, extension from sinusitis, or in children with congenital heart disease caused by right-to-left intracardiac shunt.⁵ Viral encephalitis can present with mild neck stiffness along with a change in behavior, fever, and headache. Specific etiologies to consider include herpes simplex and West Nile virus.⁵ If there are no focal findings or signs of increased intracranial pressure on physical examination, an LP is performed and will provide the diagnosis. CSF is sent for culture, cell count, protein, and glucose, with bacterial and viral cultures. If there are focal neurologic abnormalities or signs of increased intracranial pressure, a brain abscess is possible and a CT scan or MRI of the brain with and without contrast is performed prior to an LP to avoid the potential for herniation. Hospital admission is required for children with these problems; the neurologic examination, serum electrolytes, and fluid status need to be monitored closely. Antibiotics are required for bacterial meningitis and brain abscess; acyclovir is the treatment for herpes simplex virus infection.

Included in this group are problems that originate outside the calvarium but that can result in headache, either directly or through referred pain. They include sinusitis, otitis media, dental caries or abscess, pharyngitis, temporomandibular joint abnormalities, postconcussion or posttraumatic syndrome, and ophthalmologic problems, such as refractive errors or astigmatism. It can also include systemic infections, such as influenza, or streptococcal throat infections. Toxic exposures, especially to carbon monoxide, can also cause headache.^1,2,4,5

Psychiatric-related or psychogenic headaches tend to be chronic and nonprogressive and are characterized by vague complaints and nonspecific symptoms.⁴ They may result from stress, adjustment reactions, conversion reactions, depression, and malingering. In the ED, they are a diagnosis of exclusion.