Pediatric Headache (Migraine Without Aura) Case File

Pediatric Headache (Migraine Without Aura) Case File

Eugene C. Toy, MD, Ericka Simpson, MD, Pedro Mancias, MD, Erin E. Furr-Stimming, MD

CASE 46

A 13-year-old right hand–dominant adolescent girl has increasingly frequent headaches over the past year. She has “always” had headaches, but these became more bothersome approximately 3 years ago in association with onset of menses and decreased sleep. Her typical headache begins with a sense of slowed thinking and malaise followed soon after by a throbbing pain over the left side of her head, the right side of her head or, at times, over her forehead. The pain increases to its maximum severity of 8 to 9 out of 10 over the course of approximately 1 hour and will last for “many hours” if untreated. The patient reports that even light touch over the affected part of her head causes pain, and she is sensitive to bright lights and loud sounds. She typically feels nauseous and will occasionally have emesis. Acetaminophen and ibuprofen seem to help, but the best pain relief comes with sleeping in a dark room. After the pain resolves, she feels cognitively slow and “out of sorts” for up to a full day. Over the past year, however, the incidence of such attacks has increased to once every 2 to 3 weeks, leading to frequent missed days in school and a drop in school performance. These seem to be associated with menses, strong odors, and poor sleep. Her physical examination and neurologic examination are completely normal. She consistently has had motion sickness “for as long as she can remember.” Neurodevelopmentally she met all milestones. The patient’s mother had “bad headaches” as a teenager and young adult, and she has a maternal aunt who was diagnosed with migraines at approximately 20 years of age. No other neurologic diseases are noted in the family.

▶ What is the most likely diagnosis?

▶ What is the next diagnostic step?

▶ What is the next step in therapy?

ANSWERS TO CASE 46:

Pediatric Headache (Migraine Without Aura)                                            

Summary: This 13-year-old right-handed healthy adolescent girl presents with a history of recurrent hemicranial headaches that are throbbing with moderate to moderately severe pain in a crescendo-decrescendo pattern associated with nausea and occasional emesis. She also reports photophobia and phonophobia. The headaches will last for many hours if untreated, improve somewhat with low doses of acetaminophen, and resolve if the patient can get to sleep. There is a brief prodrome of malaise and a more prolonged postdrome of cognitive dulling. The only noted triggers are sleep deprivation and strong odors, and she has noted an association with her menstrual cycle. Her neurologic examination is completely normal, and her family history is significant for two people with probable migraines.

• Most likely diagnosis: Migraine without aura (common migraine).
• Next diagnostic step: No diagnostic workup necessary at this point.
• Next step in therapy: Trial of appropriately dosed nonsteroidal anti-inflammatory drugs (NSAIDs) followed by a trial of triptans if necessary. Consider prophylactic therapy given headache frequency.

ANALYSIS

Objectives

1. Understand the difference between primary and secondary headaches.
2. Know the clinical criteria for pediatric migraine headaches.
3. Understand the role of neuroimaging in evaluating headaches.
4. Know the different options available for acute abortive therapy for pediatric migraines.
5. Recognize when daily prophylactic therapy is warranted in migraine treatment and what possible options exist.

Considerations

This otherwise healthy and neurodevelopmentally normal 13-year-old adolescent girl is brought in for evaluation of frequent headaches. Because she is currently headache-free with a normal neurologic examination, attention can be turned to classifying her headache disorder, which will aid in dictating any necessary workup and intervention. Headaches in children can be primary headache disorder (ie, migraine, tension-type headache, trigeminal autonomic cephalalgias) or a secondary headache disorder that develops due to underlying disorders (such as upper respiratory tract infection [URI], influenzae, head trauma, central nervous system [CNS] infections, or space-occupying lesions). Most common primary headaches in children are migraine and tension-type headache. Trigeminal autonomic cephalalgias (cluster headache) is uncommon in children younger than 10 years. Most common causes of secondary headaches in children are URI and sinusitis. Less than 10% of children are diagnosed with serious conditions like space-occupying lesions as secondary case of headache. Headache due to visual refractive errors are included in secondary causes of headache. However, evidence is lacking that refractive errors can cause headache.

In general, secondary headaches are defined by the underlying principal problem and require a more extensive and prompt evaluation. Primary headaches, however, are generally defined by their clinical symptoms and can require no workup if clinical criteria are met. The history in this scenario is classic for migraine with the unilateral aspect, throbbing, family history, and triggers.

APPROACH TO:

Pediatric Headache                                                    

Head pain in children and adults can be divided into primary and secondary headaches. It can also be useful to consider the pattern of the patient’s headaches:

(1) acute recurrent—episodic head pain with pain-free intervals in between, 

(2) chronic progressive—gradually worsening head pain with no pain-free intervals,

(3) chronic daily headache—a persistent headache that neither worsens nor remits, and 

(4) a mixed headache—a chronic daily headache with episodic exacerbations. Chronic progressive headaches raise the possibility of increasing intracranial pressure and require further evaluation with neuroimaging. Chronic daily headaches can be a secondary headache caused by cerebral venous sinus thrombosis or can arise from a primary headache disorder. This condition as well as mixed headaches can require referral to a headache specialist.

In 2004, the International Headache Society defined the following criteria for pediatric migraine:

A. Headache attack lasting 1 to 72 hours

B. Headache has at least two of the following four features:

1. Either bilateral or unilateral (frontal/temporal) location
2. Pulsating quality
3. Moderate to severe intensity
4. Aggravated by routine physical activities

C. At least one of the following symptoms accompanies headache:

1. Nausea and/or vomiting
2. Photophobia and phonophobia (can be inferred from their behavior)

D. Five or more attacks fulfilling the above criteria

The mean age of onset for pediatric migraine is approximately 7 for boys and 11 for girls. With regard to prevalence, 8% to 23% of children meet criteria for migraines in the second decade of life, making such primary headaches a very common problem. Although migraines can be seen in children as young as 3 years, the prevalence is less than 3%. This is likely an underestimate, however, given the difficulty of making the diagnosis in very young children. Migraines commonly “run in families” and have a significant genetic component, although only relatively rare migraine syndromes have been directly linked to a single gene mutation. Many cases of familial hemiplegic migraine, for example, have been linked to a CACNA1A (calcium channel) gene mutation that encodes a voltage-gated P/Q-type calcium channel. One interesting association with migraine is that many patients report having motion sickness (ie, “carsickness”) as children. Although this clinical finding is useful if present, its absence in no way diminishes the possibility of migraine.

Migraines can be a chronic and disabling disorder and have a significant effect on the child’s quality of life. It contributes to school absenteeism and affects social interactions. Population studies have shown that over 130,000 school days are missed every 2 weeks and 3 million bedridden days occur per month as a result of migraine headaches in the United States. Since migraines commonly start in childhood, early recognition, establishment of a treatment plan, and implementation of lifestyle changes can alter disease progression and ultimately improve the child’s quality of life.

As in adults, migraines in children often begin with a prodromal premonitory phase with neurologic or constitutional symptoms lasting for hours or days before the headache. These “warning signs” can slowly increase over time or remain constant. Some patients develop an aura prior to the onset of pain that consists of a stereotyped focal symptom usually preceding the headache by no more than an hour. Visual auras are the most common type and can involve a variety of visual aberrations such as scotomata, flashes, or geometric forms. Motor, sensory, and cognitive auras can also be seen. The pattern of the pain is typically crescendo in onset and decrescendo in offset and is certainly not maximal from the beginning. As the pain continues, the patient often develops cutaneous allodynia, which means that normally nonnoxious stimulation is perceived as painful during the headache.

Associated elements such as nausea, photophobia, phonophobia, vertigo, and nasal congestion are common. Following the headache, most patients experience a postdromal phase with symptoms such as difficulty concentrating, particular food cravings, and fatigue. Triggers commonly associated with migraine headaches include strong smells, particularly if noxious, exercise, sleep deprivation, missing meals, and mild head trauma. Many patients associate certain foods with the onset of their migraines, but this can at times be difficult to distinguish between food-cravings occurring during the prodromal phase. Women with migraines are more likely to experience headaches around the time of menses.

EVALUATION

A careful history and physical examination are the most important aspects of the evaluation. The neurologic examination is the most sensitive indicator of needing further evaluation such as lumbar puncture (LP) and neuroimaging. History should be obtained from children but confirmed with parents. If children are not able to explain the characteristics of headache, asking them to draw the pattern can be helpful as well. It is important that the neurologic examination include an assessment of head circumference, visualization of the optic discs, assessment for nuchal rigidity, and palpation of the sinuses in order to carefully screen for underlying causes.

When the history is unequivocally consistent with migraine and the neurologic examination is completely normal, no further workup is needed. In particular, neuroimaging is unnecessary, and the yield is low. However, an abnormal neurologic examination or worrisome feature on history necessitates brain imaging, preferably a magnetic resonance imaging (MRI) scan. Although most parents fear the presence of a brain tumor, more than 98% of patients with intracranial masses have abnormalities on their neurologic examination. Electroencephalography (EEG) is not routinely indicated in the evaluation of headaches. Patients with epilepsy often have postictal headaches, but it would be quite unusual for the headache to be the primary presenting complaint. LP is essential if head pain is thought to be caused by a CNS infection and is part of the evaluation of subarachnoid hemorrhage (if a computed tomography [CT] scan is unrevealing). LP, however, has no routine role in the evaluation of primary headache disorders.

TREATMENT

Treatment of migraine focuses on two concepts: acute pain relief (abortive therapy) and headache prevention (prophylactic therapy). There is an ever-increasing number of available medications that can be used for abortive therapy with few controlled trials to help guide decision-making.

Lifestyle measures: Lifestyle measures are beneficial for controlling migraine headache. Good sleep hygiene, routine meal schedules, drinking adequate amounts of water, maintaining a headache diary, and avoiding the known triggers for headache is an important aspect of care.

Pharmacologic treatment:

a. Preventive therapy: Propranolol is the first-line treatment of migraine prevention. It is started at 1 mg/kg/d and can be titrated up to 3 mg/kg/d. Heart rate and blood pressure should be monitored closely after increasing the dose. It can also be used for prevention of abdominal migraine. Amitriptyline is another drug used for prevention of migraine. Amitriptyline 0.25 to 0.5 mg/kg/d in a single dose at bedtime is effective in prevention. One of the common side effects can be prolongation of QT syndrome, so electrographic monitoring should be obtained with higher doses. In 2014, topiramate became the first drug approved for migraine prevention in children of 12 years or older. Dose range can be 1 to 2 mg/kg/d. Half-life is up to 21 hours, so a single dose may be appropriate. For young children who cannot swallow tablets, an oral solution of cyproheptadine can be prescribed. It is an antihistamine with anticholinergic and calcium channel–blocking properties used for migraine prevention. Cyproheptadine 2 to 16 mg at bedtime prevents daytime sleepiness. Riboflavin (vitamin B2) is considered as an effective agent for migraine prevention. There is limited evidence about mechanism of action, but 25 to 400 mg daily dosing is found to be effective in migraine prevention. M

enstrual cycle can be one of the triggering factors. Short-term preventive therapy with naproxen sodium 550 mg twice daily during the perimenstrual period is found to be effective.

b. Abortive therapy: Many patients will already have tried such medications prior to coming to see their physicians, but they often have been underdosed or given late in the progression of the headache, which renders them as much less effective. In such patients, it is worth a trial of adequately dosed ibuprofen (10 mg/kg) or acetaminophen (15 mg/kg) given as soon as possible after the onset of the pain. If these medications prove ineffective, then a trial of 5-hydroxytryptamine receptor agonists (the triptans) is indicated. The 2004 AAN Practice parameter on the pharmacologic treatment of migraine headache in children and adolescents concluded that sumatriptan nasal spray (NS) is effective and should be considered for the acute treatment of migraine in adolescents (Level A). Subsequently, three triptans and a fourth triptan/NSAID combination have been approved by the Food and Drug Administration (FDA) for acute migraine therapy in the pediatric population: almotriptan in 12- to 17-year-olds, rizatriptan in 6- to 17-year-olds, zolmitriptan NS in ages 12 to 17, and sumatriptan combined with naproxen in ages 12 to 17.

Children or adolescents with intractable or treatment refractory headaches (>15 headache days/month for 3 consecutive months) may be amenable to Onabotulinum toxin A, which was approved by the FDA for use in chronic migraine in adults in 2010. Although there is limited experience in pediatric patients, small unrandomized and/or retrospective studies showed benefit.

CASE CORRELATION

• See also Case 53 (Metastatic Brain Tumor)

COMPREHENSION QUESTIONS

46.1 Which of the following would be classified as a secondary headache?

A. Migraine with aura

B. Cluster headaches

C. Chronic sinusitis

D. Migraine without aura

E. Tension-type headaches

46.2 Which of the following is a criterion for pediatric migraine?

A. A visual aura preceding the onset of head pain

B. Pain improved by physical activity

C. Moderate to severe intensity of head pain

D. A family history of migraine

E. Response to nonsteroidal anti-inflammatory medication

46.3 Which of the following patients should have neuroimaging as part of the evaluation of their headache?

A. An 18-year-old woman who was found unconscious at home and is now in the emergency room with the worst headache of her life

B. A 14-year-old adolescent boy with acute recurrent attacks of moderate-intensity throbbing hemicranial pain associated with nausea and photophobia

C. A 12-year-old straight-A student who is healthy and neurodevelopmentally normal, but who complains of mild squeezing head pain when he is studying for tests

D. A 17-year-old adolescent boy who develops a moderate global headache 1 day after he decides to quit drinking coffee “cold turkey”

46.4 A 9-year-old girl is newly diagnosed with pediatric migraine headaches. Which of the following is the best initial choice for abortive therapy for this patient?

A. Topiramate

B. Naproxen

C. Rizatriptan

D. Ibuprofen

E. Amitriptyline

ANSWERS

46.1 C. A headache caused by a medical disorder such as sinusitis, or subarachnoid hemorrhage would be classified as a secondary headache disorder. All of the other listed possibilities are primary headaches.

46.2 C. To meet criteria, the patient must have had five or more headaches with certain characteristics including moderate-to-severe pain. A family history of migraines, while common and helpful, is not required for the diagnosis.

46.3 A. A history of “worse headache of my life” is troubling. This history is very concerning for a subarachnoid hemorrhage and requires an emergent CT scan.

46.4 D. A trial of ibuprofen at an adequate dose (10 mg/kg) would be the best initial choice. There is more evidence for the effectiveness and safety of ibuprofen and acetaminophen for pediatric migraine headaches versus other medications.

    CLINICAL PEARLS    

▶ Studies support that migraineurs are in a state of mitochondrial energy depletion and supplementation with mitochondrial energy stores such as coenzyme Q10 and riboflavin may have clinical benefit. ▶ It is not uncommon for patients with migraines to experience vertigo in association with their headaches. If associated without headache, it is termed a migraine equivalent. ▶ Although migraine headaches are classically described as unilateral (hemicranial), this is actually only true in approximately 60% of all headaches. It is quite common for migraines to be bifrontal. ▶ Asking what the patient does during a headache is a key clinical question. Patients with migraines generally report wanting to lay still in a darkened room and wanting to go to sleep. ▶ Although not all migraine headaches are severe, headaches that do not interrupt a patient’s activities are unlikely to be migrainous.

REFERENCES

Damen L, Bruijn J, Verhagen A, et al. Symptomatic treatment of migraine in children: a systematic review of medication trials. Pediatrics. 2005;116:295-302. 

Kacperski J. Prophylaxis of migraine in children and adolescents. Pediatri Drugs. 2015;17:217-226. 

Lewis D. Headaches in children and adolescents. Am Fam Physician. 2002;65:625-632. 

Lewis D, Ashwal S, Hershey A, et al. Practice parameter: pharmacological treatment of migraine headache in children and adolescents: report of the American Academy of Neurology Quality Standards Subcommittee and the Practice Committee of the Child Neurology Society. Neurology. 2004;63:2215-2224. 

Patniyot IR, Gelfand AA. Acute treatment therapies for pediatric migraine: a qualitative systematic review. Headache. 2016;56:49-70. 

Young W, Silberstein S. Migraine: spectrum of symptoms and diagnosis. Continuum Headache. 2006;12(6):67-86.

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