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A healthy 16 year old female is taken to a neurologist for evaluation of almost daily headaches over the last 9 months. She reports headaches occurring on both weekdays and weekends. She has been using ibuprofen and acetaminophen with little relief. During her headaches, she is essentially bedridden and frequently has to leave school due to the discomfort. She complains of unilateral, pulsating pain on the right. She reports intense nausea with some episodes of vomiting during the event. She is not able to eat anything during the headaches and will often skip meals. She typically lies in her bed with the lights off due to intense noise and light sensitivity. The symptoms usually last less than 2 hours, but she had three episodes which lasted a day. She denies any head injuries or concussions. The headaches are independent of her fluid intake, which she increased this month after her headaches started. The headaches do not correlate with her menstrual cycle, although she reports the pain is often worse when she has her menses.
PMH is negative for headaches prior to this, head injury, seizures, or other serious medical conditions. She is an A student, and also plays lacrosse for her high school team. Her mother has a history of migraine headaches. No family history of seizures, brain tumors, or strokes.
Exam: Vital signs normal. She is alert, oriented, sitting on the exam table. She is appropriate and answers all questions. Her head is normal and without evidence of trauma. Neck is supple with full range of active and passive motion. Pupils are round, equal, and reactive to light. Extra-ocular movements are intact without nystagmus. No facial asymmetry seen. Heart is regular without murmur. Lungs are clear. Abdominal exam is unremarkable. Skin is free of neurocutaneous stigmata. Neurologic evaluation reveals grade 5/5 strength throughout bilateral upper and lower extremities. She has normal muscle bulk and tone. Deep tendon reflexes are 2+ throughout. No Babinski sign present on either side. Coordination is intact bilaterally. Gait is normal with normal toe, heel, and tandem gait also observed. Standard mini-mental state examination score is 30/30. Normal fundi visualized without papilledema.
Previous CBC and electrolytes obtained by her primary care physician were normal. Head CT done by the PCP was also normal.
Headaches are becoming a more widely recognized problem in children and adolescents. It is a common cause of visits to emergency rooms and primary physicians. Most headaches seen in children are the result of a primary headache disorder, such as migraine, or secondary to a benign process, such as infection. The overall prevalence of headaches in children is 58%, with studies showing large variation in incidence with 17% to 90% children reporting headaches. Headaches are more common in young boys than girls, with an increased prevalence in both sexes as they age. The increase in prevalence is much sharper in girls during adolescence and young adulthood, with a higher prevalence of headaches in women than men. In adolescence, 27% of girls and 20% of boys report frequent or severe headaches. This past year, 8% of girls and 5% of boys report migraines (1).
Headaches can be divided into four basic patterns: 1) acute, 2) acute recurrent/episodic, 3) chronic progressive, and 4) chronic nonprogressive. Thorough history and exam are essential to help classify and manage headaches. Acute recurrent and chronic non-progressive headaches are usually due to a primary headache disorder. Chronic progressive headaches are the most worrisome type of headaches in children and require thorough evaluation, usually involving neuroimaging. Single acute headaches are usually benign, typically triggered by a primary headache disorder or viral infection (1).
The prevalence of childhood migraine is 1% to 3% in children aged 3 to 7 years and 8% to 23% in adolescence, more commonly in girls than in boys. Migraine can be divided into two types: with and without aura.
Migraine without aura must meet the following criteria: at least five attacks lasting 4 to 72 hours in duration, with two of the following: 1) unilateral location (although can be bilateral or frontal), 2) pulsating, 3) moderate to severe intensity, or 4) aggravated by physical activity; plus at least one concomitant symptom including 1) nausea and/or vomiting, or 2) photophobia and phonophobia.
Migraine with aura must have at least two attacks with an aura consisting of at least one of the following: 1) fully reversible visual symptoms including positive (e.g., scotomatas, flickering lights) or negative (e.g., loss of vision) features, 2) fully reversible sensory symptoms including positive (e.g., pins and needles) or negative (e.g., numbness) features, or 3) fully reversible dysphagic speech disturbances. In addition to the above, there must be at least two of the following: 1) homonymous visual symptoms and/or unilateral sensory symptoms, 2) at least one aura symptom develops gradually over 5 or more minutes with different aura symptoms occurring in succession over greater than 5 minutes, or 3) each symptom lasts greater than 5 minutes but less than 60 minutes. Headaches meeting these criteria must appear during the aura or follow the aura within 60 minutes (1,4).
The pathophysiology of migraine in children is assumed to be similar to that of adults. The mechanisms are based on complex interactions between vascular and neural systems, including spreading cortical depression, abnormal neuronal excitability, serotonin activity, inflammatory response, and trigeminal neurovascular activation with signal transmission through the thalamus to the cortex (1). Migraine is multifactorial in nature, with a strong genetic component, but no clear heritability pattern has been identified, with different clinical manifestations often seen in different family members. This has led many to believe there is a strong environmental component to migraine. Children often have a shorter migraine duration than adults, with headaches lasting 30 to 60 minutes. Children often go to their rooms, searching for a dark quiet place due to photophobia or phonophobia. They often have anorexia, nausea, and vomiting. Sleep can help relieve the symptoms. Some children will report dizziness, blurry vision, stomach pain, sweating, pallor, and flushing, among many other symptoms. Children frequently have difficulty describing their pain or symptoms related to their headaches, making history important, as their actions can help lend light to their symptoms. Having children draw their headaches can help define the headache. Common triggers include stress, stress "let-up", fatigue, poor sleep, illness, fasting, and dehydration. No obvious food triggers have been identified in migraine, but can be a factor for some children (1,3,4).
Migraine with aura is much less common in children than adults. Auras usually occur less than 30 minutes prior to the headache and last 5 to 20 minutes. Symptoms of auras are completely reversible, usually last less than 30 minutes, and are recurrent over time. Patients with neurologic symptoms that are prolonged, not related to headache, or not completely reversible should be evaluated to rule out an underlying condition (1,3,4).
Tension type headaches are the most common headache type in children, but are much less disabling than migraine. The pain is typically mild to moderate and lasting 1 hour to several days. The pain is often described as "band-like", pressure, and tightening. Triggers may be similar to migraine triggers. Most common triggers include stress, fatigue, and illness, but also include muscle pain and tension, usually in the neck and shoulders. Tension headaches can be episodic (<15 days in a month) or chronic (> 15 days per month). It is important to use the history to identify stressors, depression, or other factors affecting headaches. The pathophysiology of tension-type headaches is not known at this time. There is some belief that nociceptive input from the cranial/cervical myofascial components triggers the headaches initially and if the input is sustained, central sensitization can occur. With time, the individual becomes more sensitive to the impulses and develops chronic headaches (1,2).
Chronic daily headache is defined as 15 or more headache days in a month. In children these include chronic migraine, chronic tension-type headaches, and new daily persistent headache (NDPH). Chronic migraine is common in adolescents. These children usually have a history of episodic migraine that slowly become more and more frequent, often having few or no headache free days. Symptoms such as vomiting and severe head pain often diminish as the headaches become more frequent, although spikes with more severe symptoms can occur. Chronic tension-type headaches share many similarities with chronic migraine. It can be difficult to distinguish tension-type from migraine headache in these children. Children with chronic tension-type headaches will not have a history of episodic migraine prior to the increased frequency of headache. Preventative treatment should be considered when the child is having 4 or more disabling headaches a week. It is critical to assess lifestyle issues first, such as sleep, food or caffeine intake, exercise habits, and poor hydration. Depression and anxiety are common comorbid issues for patients with chronic headaches and may worsen the headaches. NDPH is characterized by new headache that becomes daily within 3 days of onset and is not caused by another disorder. The onset is abrupt, so children with these should be worked up for secondary causes of headaches. NDPH is often triggered by a viral illness, but can be due to mild head injury or surgery (1).
Cluster headaches are uncommon in children. They occur more often in boys than girls and are rarely are seen in other family members. These are seen almost exclusively over the age of 10 years. Cluster headaches recur over periods of weeks or months often separated by intervals of 1 to 2 years. Clusters of daily attacks lasting 4 to 8 weeks may occur once to twice yearly, with absence of headaches in the interim. These headaches often begin during sleep, lasting 30 to 90 minutes, and repeat two to six times daily. It is always unilateral and affects the same side of the head with each headache. Pain begins behind and around one eye before spreading to the rest of the hemicranium. Pain is usually throbbing and constant and the scalp can feel edematous and tender. Patients often pace during the episodes and report they feel unable to sit still due to pain. Horner syndrome, sweating, flushing, and congestion can develop ipsilateral to the headache (2).
Secondary headaches have a variety of causes, including abnormal intracranial pressure, idiopathic intracranial hypertension, infection, vascular disorders, and structural disorders to name a few. Red flags for secondary headaches include progressive pattern of the headache with increased frequency or severity; increased headache with straining, coughing, or sneezing; explosive or sudden onset; systemic symptoms; neurologic signs or symptoms (including altered mental status, papilledema, abnormal eye movements, etc.); secondary risk factors (immunosuppression, genetic disorders, rheumatologic disorder, etc.); new or different severe headache; change in attack frequency, severity, or clinical features; or sleep related headaches, which wake the patient from sleep or which is always present in the morning. Substances in the environment (such as carbon monoxide) and substance abuse are another important topic to assess in the history, especially in adolescents. Trauma is another common cause which can be assessed with a good history and physical (1,2).
In patients with chronic headaches, analgesic rebound headaches should always be considered. Overuse of medications to treat headaches, especially analgesics, caffeine, opioids, ergotamines, and 5-hydroxytryptamine receptor agonists (triptans) can lead to worsening headaches. These substances can transform episodic headaches into chronic headaches. These medications can trigger headaches through their primary mechanism of action, an idiosyncratic response to the medication, or medication withdrawal (1).
Workup for headaches is usually minimal in most cases. Diagnosis is made from history in most cases, with testing used to rule out secondary causes of the headache. The American Academy of Neurology (AAN) practice parameter has the following statement on neuroimaging in children with headaches (5):
. . . . . 1) Obtaining a neuroimaging study on a routine basis is not indicated in children who have recurrent headaches and normal results on neurologic examination.
. . . . . 2) Neuroimaging should be considered in children who have abnormal results on neurologic examination, the coexistence of seizures, or both.
. . . . . 3) Neuroimaging should be considered in children whom there are historical features to suggest the recent onset of severe headaches, change in their type of headaches, or if there are associated features that suggest neurologic dysfunction (3,5).
Management of primary headache syndromes should start with patient and family education. Many families need reassurance that there is no underlying or secondary disorder. It is also important to have the patients and their families on the same page about the treatment strategy and goals of therapy. It is helpful to initially start with the patient keeping a headache diary. This will allow physicians to analyze the patterns and possible triggers of the headaches and after therapy is initiated, assess its effectiveness. There are four major aspects of headache management: 1) lifestyle modification, 2) acute headache management, 3) complementary treatment, and 4) preventive treatment. Many patients will only require recognition of headache triggers and appropriate acute headache management. The SMART mnemonic can help with lifestyle modifications. SMART in this case stands for Sleep, Meals, Activity, Relaxation, and Trigger avoidance. Regular and sufficient sleep, adequate meals (for many kids this means adding breakfast and sufficient hydration), and aerobic exercise are easy lifestyle modifications which can have huge impacts on children’s headaches. Stress reduction and relaxation mechanisms can also influence children’s headaches greatly, and acknowledging and helping to alleviate stressors can greatly improve headaches. Caffeine intake should also be closely monitored since intake of more than 2 to 3 times per week may cause rebound or medication overuse headaches (1).
Acute treatment of headaches should focus on early intervention for significant headaches, as early treatment has been shown to be more effective. Patients should be warned not to use acute therapies for more than 2 to 3 days per week. It is suggested to not use NSAIDs more than 15 days in a month and not to use triptans or caffeine more than 10 days per month. If patients are using acute medications more frequently than what is recommended, then this indicates the need for preventive medication. It has been shown that over the counter medications, such as ibuprofen, acetaminophen, and naproxen, can be effective in children for the management of migraine. For severe headaches, a dose of a NSAID can be combined with caffeine less than 9 days per month, as long as the child does not regularly take in caffeine. If NSAIDs are not effective in alleviating the headache, triptans can be used. Only rizatriptan has been approved for migraine treatment of children aged 6 to 17 years and almotriptan has been approved for use in adolescents. There is limited data supporting the use of intranasal and subcutaneous sumatriptan and rizatriptan in children younger than 6 years. Zolmitriptan has been shown to be effective in oral and nasal forms for children over 12 years of age. The use of triptans in children is not considered unsafe, only that it is not adequately studied at this time. Opiates and barbiturates are not indicated for treatment of primary headaches in children. Antiemetics can be used in conjunction with NSAIDs or triptans in patients with significant nausea or vomiting associated with their headaches. Intravenous Compazine and Toradol have been shown to be effective as an abortive for children with migraine in the emergency room setting (1,6).
Daily preventive medications should be considered in children with 4 or more disabling headaches per month. Before starting preventative medications it is important to discuss expectations with families because therapies can take 8 to 12 weeks to show recognizable effects. Switching preventative medications every few weeks due to presumed ineffectiveness without an adequate trial will lead to frustration for all. Several studies have shown topiramate is more effective than placebo for management of migraine in children and is usually the initial medication tried. Valproic acid and gabapentin are other anticonvulsants which have shown effectiveness at doses lower than those used for seizure therapy. Beta-blockers, such as propranolol, and tricyclic antidepressants, such as amitriptyline or nortriptyline, have also been used for migraine prevention. Nightly doses of cyproheptadine can be helpful in children with environmental allergies and migraine. Preventive prescription medications should be started at a low dose and increased to the goal or effective dose. The choice of preventive medications should be based on comorbid factors. Side effects of medications should always be taken into account. For example, teenage girls should not be started on valproic acid as a first time treatment due to its known toxic effects and teratogenic nature, and beta-blockers should be avoided in children with asthma and migraine (1,2,3).
Complementary therapies have also shown effectiveness in recurrent and chronic headaches. Behavioral techniques including biofeedback, relaxation techniques, hypnosis, acupuncture, and coping mechanisms have all been successful. Physical therapy and massage therapy can be helpful especially for children with tension or muscle pain associated with their headaches (1).
1. Explain the criteria for diagnosis of migraine headaches, with and without aura.
2. Which of the following are included in initial management of headaches?
. . . . . a. good sleep hygiene
. . . . . b. regular meals
. . . . . c. adequate fluid intake
. . . . . d. stress reduction
. . . . . e. all of the above
3. Which of the following would be a reason to obtain an MRI in a child with recurrent headaches?
. . . . . a. Under 5 years of age
. . . . . b. Unilateral headache
. . . . . c. Slight limp detected on exam
. . . . . d. Male gender
1. Blume HK. Pediatric Headache: A Review. Pediatrics in Review. 2012;33:562-576.
2. Fenichel GM. Chapter 3: Headache. Clinical Pediatric Neurology: A Signs and Symptoms Approach, 5th edition. 2005, Philadelphia: Elsevier Saunders, pp 77-90.
3. Olness KN, MacDonald JT. Recurrent Headaches in Children: Diagnosis and Treatment. Pediatrics in Review. 1987;8:307-311.
4. Singer HS. Migraine Headaches in Children. Pediatrics in Review. 1994;15:94-101.
5. Lewis DW et al. Quality Standards Subcommittee of the American Academy of Neurology; Practice Committee of the Child Neurology Society. Practice parameter: evaluation of children and adolescents with recurrent headaches: report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2002;59(4):490-498.
6. Aukerman G, Knutson D, Miser WF. Management of the Acute Migraine Headache. American Family Physician. 2002;66(11):2123-2131.
Answers to questions
1. Migraine without aura criteria:
. . . . . a. At least five attacks lasting 1 to 72 hours fulfilling the criteria.
. . . . . b. Two out of four characteristics: unilateral, pulsating, moderate or severe pain, and aggravated by physical activity.
. . . . . c. One of two concomitant features: nausea and/or vomiting or photophobia and phonophobia.
Migraine with aura criteria:
. . . . . a. at least two attacks fulfilling the criteria
. . . . . b. An aura with at least one of the following: 1) fully reversible visual symptoms including positive (e.g., scotomatas, flickering lights) or negative (e.g., loss of vision) features, 2) fully reversible sensory symptoms including positive (e.g., pins and needles) or negative (e.g., numbness) features, or 3) fully reversible dysphagic speech disturbances.
. . . . . c. At least two of the following: 1) homonymous visual symptoms and/or unilateral sensory symptoms, 2) at least one aura symptom develops gradually over 5 or more minutes with different aura symptoms occurring in succession over greater than 5 minutes, or 3) each symptom lasts greater than 5 minutes but less than 60 minutes. Headaches meeting these criteria must appear during the aura or follow the aura within 60 minutes
2. All of the above. Lifestyle changes are key in the treatment of headaches. They include getting adequate sleep with good sleep hygiene, eating regular and sufficient meals with adequate daily fluid intake, regular exercise, stress reduction and relaxation, and avoidance of triggers.
3. Slight detection of a limp on exam would be reason to obtain neuroimaging, as this an abnormality in the neurologic exam. According the AAN’s practice parameters, this would be cause for imaging.