Headache

Recurrent headache is one of the most common neurologic conditions in childhood and is one of the most frequent symptoms evaluated by child neurologists, in both emergency and outpatient settings. Primary headache disorders, specifically tension-type headaches and migraines, are the most common recurring headache patterns seen in children. The International Headache Society provides clinical criteria for diagnosing a number of headache disorders in the International Classification of Headache Disorders, 3rd edition (ICHD-3).

In this section, we cover the following topics:

• Evaluation of Headaches

• Primary Headache Disorders (tension-type and migraine headaches)

• Treatment of Primary Headache

• Idiopathic Intracranial Hypertension

Evaluation of Headaches

The main goal of evaluating a child with headache is to determine if the headaches are a primary headache disorder or, in rare cases, a secondary headache due to a pathological intracranial process.

• Primary headache disorders include:

  • tension-type headaches (discussed below)

  • migraine headaches (discussed below)

  • trigeminal autonomic cephalgias (relatively rare headaches characterized by unilateral trigeminal distribution pain in association with prominent ipsilateral cranial autonomic features; include cluster headache, paroxysmal hemicrania, and short-lasting, unilateral, neuralgiform headache attack)

  • other rare primary headache disorders

• Secondary headaches (often resulting from structural changes inside the skull) include:

  • aneurysm

  • hematoma: subdural, epidural (see Trauma in the Pediatric Emergency Medicine rotation guide)

  • hemorrhage: parenchymal, subarachnoid (see Neurologic Conditions in the Neonatal Care rotation guide)

  • idiopathic intracranial hypertension (IIH; discussed below)

  • infection: abscess, meningitis, encephalitis (see Pediatric Infectious Diseases)

  • neoplasm

  • traumatic brain injury/concussion (see Pediatric Emergency Medicine)

  • vascular malformation

  • vasculitis (see Pediatric Rheumatology)

  • stroke (see Weakness Disorders in this rotation guide)

Headache History

To classify and accurately diagnose primary headache disorders, the following information should be obtained:

• location of pain

• duration (typical length of time from beginning to end of headache)

• quality of pain (e.g., sharp, throbbing)

• frequency

• lifestyle assessment: sleep hygiene, hydration, exercise, diet (particularly meal skipping), frequency of abortive medication usage

• family history of migraines

Neuroimaging

Diagnosis of headache disorders in children should be guided by history and physical examination. Neuroimaging and cerebrospinal fluid (CSF) studies are rarely needed but should be considered if any of the following red-flag features of secondary headache are noted:

• Red-flag features for secondary headache include:

  • age <6 years

  • aura lasting >one hour

  • awakening during sleep

  • diplopia/acquired ocular malalignment

  • headache quality significantly changed

  • stabbing quality

  • visual loss

  • worsening with lying down

  • worsening with Valsalva maneuver

  • reported worst headache ever

  • change in usual headache symptoms (e.g., location, quality)

Neuroimaging should also be performed in any child with headache and a neurologic examination that reveals new abnormalities. When performing a neurologic examination in children with headache, it is crucial to perform a funduscopic examination to evaluate for papilledema (optic-nerve swelling caused by increased intracranial pressure of any cause). Neuroimaging and lumbar puncture should be performed in children in whom meningitis or IIH are suspected.

Primary Headache Disorders

The following discussion of primary headache disorders covers diagnostic criteria for tension-type and migraine headaches, followed by information on the treatment of primary headache. Tension and migraine headaches are distinguished by their clinical characteristics.

Tension-Type Headaches

Clinical features: The clinical features of tension-type headaches are as follows:

• typically, bilateral

• pressing- or tightening-quality headaches of mild-to-moderate intensity

• can last minutes, hours, or days

• either photophobia or phonophobia may be present but not both

• no associated nausea

• do not worsen with physical activity

Subtypes: Tension-type headaches are subdivided by frequency, as follows:

• infrequent: less than once a month

• frequent: 1-14 days per month

• chronic: 15 or more days per month

Migraines

Clinical features: Clinical characteristics of migraine headaches are as follows:

• typically localized as unilateral in adults and older adolescents but may be bilateral in children

• pulsating quality of moderate-to-severe intensity

• may be exacerbated by routine physical activity

• duration of an untreated migraine should be at least 2 hours in children (as compared to 4 hours or more in adults)

• associated with photophobia and photophobia and/or nausea, sometimes with vomiting

Migraine with aura: Migraines may be associated with an aura. Migraine with aura is characterized by transient focal neurologic symptoms that usually precede and sometimes accompany the headache. Examples include visual scotomata such as flashing lights. Visual auras are the most common type of auras, accounting for more than 90% of auras in migraine with aura. Migraine aura may occur without headache.

Chronic migraine and chronic daily headache: Children with frequent or chronic headaches often have both tension-type headaches and migraine headaches. Chronic migraine is defined as headache that occurs on 15 or more days per month with migraine features on 8 or more days. Chronic headache can be quite debilitating and can result in many missed days of school.

Other paroxysmal attacks that affect children with chronic headache and are considered migraine-related phenomena include:

• cyclic vomiting syndrome: recurrent episodic attacks of intense nausea and vomiting

• abdominal migraine: recurrent attacks of moderate-to-severe midline abdominal pain associated with vasomotor symptoms, nausea, and vomiting

• benign paroxysmal vertigo: recurrent brief attacks of vertigo, occurring without warning and resolving spontaneously

• benign paroxysmal torticollis (in infants): recurrent episodes of head tilt to one side

Status migrainosus: Status migrainosus is a debilitating migraine lasting for longer than 72 hours. Children with status migrainosus often require inpatient or emergency department treatment.

Treatment of Primary Headache

Headache treatment can be divided into acute abortive treatment and chronic treatment. Acute treatments may be administered at home or school or in the emergency department or hospital. Preventive, chronic treatment includes medications and lifestyle modifications.

Acute treatment: Acute treatment should be given at the onset of the headache (or onset of aura, if present) to prevent the headache from worsening or becoming refractory. No acute headache treatment should be taken more than three times per week to avoid medication overuse headache.

• Nonsteroidal anti-inflammatory drugs (NSAIDs): Initial home treatment of acute headache typically includes a weight-appropriate dose of an NSAID (e.g., ibuprofen or naproxen sodium, or acetaminophen taken with ample fluids). If headache does not abate within 3-4 hours, the dose can be repeated.

• Triptans: Triptans are designed specifically for treatment of acute migraine and are often prescribed for acute treatment. If a child does not respond to one triptan they may respond to a different triptan and can be offered an alternative. These medications come in oral, intranasal, and subcutaneous formulations. Triptans can be taken up to six times per month and can be readministered after 2 hours if significant pain persists despite the initial dose.

  • Triptans can cause vasoconstriction of coronary and limb arteries and are contraindicated in patients with known history of myocardial infarction, coronary artery disease, cerebrovascular accidents, hemiplegic migraines, uncontrolled hypertension, or peripheral vascular disease.

  • Of the seven triptans currently available, four are approved for use in adolescents and one is approved for use in children.

    • Triptans approved for use in adolescents >12 years:

      • almotriptan oral tablets

      • rizatriptan oral tablets

      • sumatriptan/naproxen combination tablets

      • zolmitriptan nasal spray

    • Triptans approved for use in children >6 years:

      • rizatriptan oral tablets

• Ditans: This newer class of drugs was approved by the U.S. Food and Drug Administration in 2019 for treatment of migraines in adults. Ditans target 5-HT_1F receptors without the vasoconstriction caused by triptans.

• Nonpharmacologic treatment: Evidence is emerging for the efficacy of nonpharmacologic treatment of acute headache in the pediatric population. Neurology consultation is advised if these treatments are being considered. The FDA has cleared four nonpharmacologic treatment devices:

  • acute abortive treatment: remote electrical neuromodulation

  • preventive treatment: external trigeminal nerve stimulation, noninvasive vagal nerve stimulation, single-pulse transcranial magnetic stimulation

Emergency department (ED) and hospital treatment: Sometimes headaches are not responsive to home treatment, particularly if treatment is not initiated at the start of a headache. In this case, children often present to the ED with headache.

• First-line treatment in the ED: A combination of intravenous (IV) fluid boluses, ketorolac, and a dopamine 2 (D2)-receptor antagonist (e.g., prochlorperazine or metoclopramide).

• If the child’s headache is not responsive to initial treatment, other treatments can be used, with varying levels of evidence to support them:

• Sodium valproate or dihydroergotamine (DHE): A single dose of DHE can be effective in aborting headache in the ED setting.

  • Occasionally, children are admitted to the hospital for continued treatment with DHE if headache continues to be refractory to treatment. DHE has been prescribed for the treatment of migraines for decades. This medication acts via central vasoconstriction. Several DHE protocols exist for inpatient treatment, but treatment typically involves 0.5-1.0 mg of DHE (depending on age and tolerability) every 8 hours until headache freedom, with a maximum of 10 doses. After headache freedom is obtained, an additional 2 doses may be given to prevent headache recurrence. Hospital admission is usually 2-3 days. Adverse effects are common and most often include initial worsening of headache, nausea, vomiting, and temporary increase in blood pressure.

• Magnesium: Used for treatment of acute headache, particularly in patients with migraine with aura.

• Methylprednisolone followed by a methylprednisolone dose pack: If headache resolves after treatment and the child has frequent headaches, a methylprednisolone dose pack with preset tapering of daily doses may be prescribed to prevent rebound headache.

View the algorithm for acute headache treatment at the Cincinnati Children’s Hospital Medical Center Emergency Department. This is a single center’s approach to acute migraine treatment based on available evidence; other centers may have different approaches.

Preventive treatment:

• Lifestyle modifications: For children with chronic daily headaches, or less frequent but highly debilitating headaches, behavioral training for pain management and daily pharmacologic treatment may be helpful. Lifestyle measures that can help prevent migraine include:

  • adequate amount of sleep

  • maintaining a regular sleep routine

  • maintaining adequate hydration

  • avoiding or limiting caffeine

  • avoiding skipped meals

  • regular exercise

  • eating balanced meals

• Cognitive behavioral therapy (CBT): CBT has also been shown to be effective for treatment of headache. A relatively short course of therapy is usually needed to attain benefit.

• Supplements: Vitamin and enzyme deficiencies may contribute to headaches, and some physicians advocate for checking folate, vitamin B₂, vitamin D, and, coenzyme Q10 levels.

• Pharmacologic prophylaxis: A diverse group of medications are used to prevent migraine attacks, including antidepressants, antiepileptics, antihistamines, and antihypertensive agents, yet there remains a lack of controlled studies on drug therapies for pediatric migraine. Therefore, data from adult studies are often extrapolated and applied to the pediatric patient.

  • Amitriptyline is the most commonly prescribed medication for headache prevention in children. However, it should be used with caution in children and adolescents prescribed selective serotonin-reuptake inhibitors (SSRIs) or selective norepinephrine-reuptake inhibitors (SNRIs).

    • In a large, randomized, controlled trial, researchers compared preventive treatment with amitriptyline, topiramate, and placebo and found no difference among treatment groups, with a high placebo response rate of 60%. Adverse events were more common in the topiramate and amitriptyline groups. Nevertheless, physicians often prescribe preventive medication because patients may receive some benefit from the routine of taking a medication every day.

  • Sodium valproate, propranolol, verapamil, and botulinum toxin injections. have been shown to be effective migraine preventive treatments in adults and can be considered for treatment in children.

  • Cyproheptadine is an antihistamine that can also be used as a preventive headache medication in younger children (typically, younger than 6 years) following a thorough workup to exclude secondary causes of headache. Adverse effects include sedation and weight gain.

  • Calcitonin gene-related peptide (CGRP)-receptor monoclonal antibodies and antagonists are a new class of medications that have recently become available for prevention of migraine in adults. Although they are not approved for use in adolescents or children, they may be considered in adolescents with refractory migraines.

Idiopathic Intracranial Hypertension

Idiopathic intracranial hypertension (IIH; previously named pseudotumor cerebri) is a headache syndrome characterized by signs and symptoms of elevated intracranial pressure and elevated cerebrospinal fluid (CSF) opening pressure on lumbar puncture, normal CSF content, and no explanation for increased intracranial pressure on MRI.

Clinical Features

Headache is the most common presenting symptom of IIH, although children may also present after routine ophthalmologic examination reveals papilledema. Headaches in IIH are frequently bilateral, frontal, retro-orbital, pressure-type headaches. IIH can be associated with obesity and with some medications, including tetracycline and isotretinoin used in the treatment of acne. Both glucocorticoid use and withdrawal have also been associated with IIH.

Similar to other headaches induced by elevated intracranial pressure, headaches associated with IIH are characterized by the following:

• worsen with lying down or with Valsalva maneuver

• worsen on awakening

• nausea and vomiting, photophobia and phonophobia

• visual symptoms: common and concerning in children with IIH

  • transient visual obscurations (brief [lasting seconds] episodes of unilateral or bilateral partial or complete vision loss, commonly occurring with change in posture)

  • diplopia and blurred vision

• pulsatile tinnitus: occurs in about half of patients with IIH

Physical Examination

Papilledema (optic disc swelling) is the hallmark physical examination finding in IIH. The papilledema grading system (Frisén scale) is depicted in the following images.

Papilledema Grading System

(Source: Papilledema Grades. University of Iowa Health Care 2010.)

Papilledema, particularly grade I papilledema, may be difficult to see in children, especially younger children who are less cooperative during funduscopic examination. In children with suspected IIH, ophthalmology consultation early in the diagnostic evaluation is advised.

Visual field deficits can be experienced by patients with IIH. Therefore, it is important to check visual fields at bedside. In older children who can participate, formal visual field testing should be performed by ophthalmology.

Cranial nerve palsies are common, particularly cranial nerve VI (impaired eye abduction), although cranial nerve III, IV, and VII palsies may also be seen. The following image illustrates the cranial nerves tested for specific ocular movements during the classic “H” test.

(Source: A Guide to the Cranial Nerves. Stepwards 2016.)

Diagnosis

Diagnosis of IIH requires lumbar puncture and magnetic resonance imaging and venography (MRI/MRV). Exclusion of cerebral venous sinus thrombosis is important for the diagnosis of IIH. The modified Dandy criteria are widely used in the diagnosis of IIH in adults.

Treatment

Recognizing and treating IIH quickly is important to prevent vision loss. Therefore, it is essential to comanage and refer a pediatric patient with IIH to pediatric neurology and ophthalmology.

• Discontinuing medications that might contribute to IIH is the first step in treatment.

• Weight loss in children with IIH who are overweight or obese is the most important long-term intervention.

• Acetazolamide is the first-line medication for treatment of IIH in children. It is a carbonic anhydrase inhibitor that decreases CSF production. Common adverse effects include paresthesias (which can be treated with bicarbonate substitution), metallic taste, and loss of appetite. Duration of treatment is based on resolution of papilledema, visual field abnormalities, and headache. Typical treatment is 2-9 months. In the IIH Treatment Trial, acetazolamide plus a weight-loss diet was associated with modest improvement in visual field function compared to diet and placebo, in adults with IIH and mild visual loss.

• Furosemide is generally the second-line medical treatment.

• Topiramate also has carbonic-anhydrase-inhibitor effects and may be beneficial. An additional benefit of topiramate in overweight patients with IIH is that it sometimes suppresses appetite.

• Headache treatment is often needed for persistent headache after IIH has resolved. These headaches are often debilitating and should be treated with typical headache medications (see Primary Headache Disorders above).

• Neurosurgical consultation for consideration of surgical management is indicated in patients with fulminant vision loss at presentation or progressive vision loss despite maximum medical management. Optic-nerve-sheath fenestration and CSF shunting, either by ventriculoperitoneal shunt or lumboperitoneal shunt, are the most commonly performed surgeries for IIH. Transverse sinus stenting may also be performed.

• Bariatric surgery is recommended in some obese patients with IIH.