Pediatric Headache Clinical Presentation

A thorough history should be obtained in any child presenting with headache. The history should describe headache onset, duration, severity, and associated symptoms. A family history of migraines may be helpful in clarifying the diagnosis. A medication history should also be sought.

One study found that a structured interview tool known as the Diagnostic Interview of Headache Syndromes–Child Version (DIHS-C) was reliable and valid for detecting migraine in children and teens in clinical and community settings. ^[34] The DIHS-C had a sensitivity of 98% and a specificity of 61%. ^[35] The study authors suggested that this tool can be used in doctors’ offices as an initial history-gathering method administered by a nonphysician, and the treating physician can then use the information obtained.

Although the minority of headaches in children are due to serious underlying pathology, early recognition is paramount for appropriate diagnosis and management. Structural headaches frequently are caused by space-occupying lesions, inflammation, and/or an increase in intracranial pressure. Frequently, neurosurgical intervention is needed.

No single sign or symptom indicates a structural etiology; however, several signs and symptoms warrant further investigation. Headaches due to increased intracranial pressure may be worse in the morning and improve as the day progresses or may be aggravated by sneezing, coughing, or straining. Headaches persistently localized to the occipital region warrant attention (as do any focal neurologic signs or symptoms with or without headache).

Worsening of headache severity and/or frequency (especially with rapid progression) may also suggest an intracranial pathologic process, as may any significant change in a previously diagnosed headache syndrome. Failure of an adequate trial of headache therapy may imply an incorrect diagnosis.

Children with a prior history of epilepsy may have a generalized or focal headache after a seizure. Headaches may also accompany the aura prior to a seizure.

Besides being classified on the basis of associated symptoms, headaches can, as previously mentioned, also be classified according to their temporal pattern, as follows:

• Acute

• Acute recurrent (episodic)

• Chronic nonprogressive

• Chronic progressive

Acute headache

Acute headache is defined as a recent onset of headache with no prior history of similar episodes. Establishing whether any neurologic symptoms accompany this headache is very important. The differential diagnosis of acute headache includes systemic infection, trauma, CNS infection, and first episode of migraine. Similar headaches that recur as often as several times a month with intervening symptom-free intervals are classified as acute recurrent headaches (usually migraine).

Chronic headache

Nonprogressive

Chronic nonprogressive headaches differ from acute recurrent headaches by their greater frequency and persistence for years with no associated neurologic symptoms or change in headache severity. Chronic nonprogressive headaches may have emotional or behavioral components. A common headache in this category is tension-type headache, but migraine with and without aura can also present this way.

Chronic daily headache (CDH) was first described in adults who reported daily or nearly daily headaches. It was soon recognized that although patients were similar in the number of headaches experienced, the characteristics of their headaches fell on a continuum between migraine and tension-type headache. A similar spectrum has been demonstrated in children. The most common CDH pattern is a progression from episodic migraines to this daily pattern.

Progressive

Chronic progressive headaches occur at least several times a week, but unlike the nonprogressive variety, these headaches increase in frequency and/or severity with time. The changing headache pattern should alert the care provider to the possibility that these headaches are secondary to a structural etiology.

Migraine headache

Symptoms vary according to the type of migraine a patient has. Many children with migraines have a previous history of motion sickness, paroxysmal dizziness, or vertigo. Clinicians should suspect migraine headache in any child who presents with recurrent episodes of incapacitating headaches. Nearly 70% of pediatric patients have a family history of migraine headache.

A possible relationship exists between children who have cyclic vomiting syndrome and migraine headache. A genetic predisposition to develop migraine headaches appears to exist for patients with cyclic vomiting syndrome and their family members. Patients with cyclic vomiting syndrome, their mothers, and grandmothers may have a prevalence of migraine headache that is about twice that of the general population.

Many children with migraine will have some type of premonitory symptoms before the onset of headache. Some of these premonitory symptoms include irritability, fatigue, and changes in facial expression. ^[36] The recognition of these subtle premonitory symptoms is helpful in the initiation of abortive therapy.

A study by Gelfand et al of 154 infant-mother pairs indicated that infants born to mothers with a history of migraine headaches have a 2.6 times greater risk of developing colic. As a result of this finding, the investigators suggested that migraine may manifest early as colic. ^[37]

Common differences between pediatric and adult presentation of migraine include, in children, include lack of throbbing, absence of lateralization, and shorter duration of the attack. ^{[1, 38]}

Headache triggers

Distinguishing between headache causes and triggers is important, since the latter act merely as precipitants of the headache condition, most often migraine. Factors that precipitate migraine headache include the following:

• Stress/anxiety

• Menstruation

• Oral contraceptives

• Physical exertion/fatigue

• Lack of sleep (sleep apnea may also be a primary cause of headache)

• Glare

• Hunger

• Foods/beverages with nitrates, glutamate, caffeine, tyramine, salt

• Reading/refractive error

• Cold foods

• High altitude

• Drugs such as nitroglycerin, indomethacin, and hydralazine

• Chemicals, such as tyramine in cheese, chocolate, nuts, and monosodium glutamate

• Epilepsy - Children with epilepsy are at an increased risk of developing migraine headaches

Migraine with aura

An aura is a sudden and self-limited neurologic dysfunction that generally lasts a few minutes; sometimes several can occur simultaneously or sequentially. The following symptoms can develop:

• Visual symptoms - Scotomas, phosphenes that look like stars, straight or broken lines (fortification spectra), colors, illusions of shape (micropsias, macropsias, dysmorphopsia), and, rarely, hallucinations (more complex pictures)

• Sensory symptoms - Paresthesias, rarely dysmorphopsia (impression one's body is deformed)

• Motor symptoms - Paresis or hemiplegia, especially prominent in familial hemiplegic migraine (FHM)

• Speech or language disturbance - Dysarthria, aphasia

• Other cognitive effects - Confusion or amnesia

All of these symptoms have been attributed to cortical dysfunction. In a few instances, the aura supposedly has a brain-stem origin, although it has never been proven (eg, loss of consciousness, ophthalmoparesis, vertigo).

In cases of migraine with aura, the above symptoms precede a sharply defined headache. Adolescent and adult patients who have migraine with aura are thought to be at a higher risk for ischemic stroke, with this risk being magnified among women, patients younger than 45 years, smokers, and persons using oral contraceptives. ^[32]

Complicated migraine

In complicated migraine, focal or diffuse neurologic deficits may occur with the headache.

Hemiplegic migraine

With hemiplegic or hemisensory migraine, the headache is accompanied by unilateral motor weakness or sensory disturbance (eg, paresthesias) that may persist for several hours after the headache has subsided.

Basilar migraine

With basilar migraine, the pathogenesis involves vasoconstriction of the basilar and posterior cerebral arteries, which is well known to be an epiphenomenon in migraines and is not the cause of the headache. Diplopia, vertigo, tinnitus, or ataxia may also be noted.

Acute confusional states

Acute confusional states refer to an unusual type of migraine headache, characterized by sudden onset of confusion, unresponsiveness, memory disturbances, disorientation, and dysarthria; this type of migraine headache is thought to be more common in boys.

Migraine Disability Assessment Score (MIDAS)

Proper assessment of the patient, besides adequate characterization of migraine attacks, includes an objective determination of the disability imparted by the headache. A helpful adjunct to obtaining the history in such patients is the pediatric adjustment of the migraine disability assessment score (MIDAS), called PedsMIDAS, is an excellent and easy-to-use tool and is recommended by Hershey et al. ^[33] Quality of life is seriously affected by headaches, especially chronic ones, in children and adolescents, from most standpoints: physical, psychological, and social and role function, as observed by Osterhaus et al and Powers et al. ^{[39, 40]}

Tension-type headache

Tension-type headaches are common in children. Differentiating tension headache from migraine headache may be difficult, as many children with migraine headache also complain of neck pain. Distinguishing characteristics of tension headaches include the following:

• Occurring during times of obvious stress

• Involving the neck and occiput

• Continuous pain

• No nausea, vomiting, or abdominal pain

• Family history of migraine is less likely

• In a subgroup of patients with tension headache, some patients have obvious symptoms of depression, such as depressed mood, feelings of worthlessness, anhedonia, or anorexia; in this subgroup, the headaches are relieved when the depression is treated

Cluster headache

In this disorder, headaches occur in groups or clusters. Nasal discharge, congestion, and a watery, red eye are present on the same side of the head as the headache. Pain localizes to one side of the head. Cluster headaches often awaken a patient from sleep and most often occur in middle-aged men. Cluster headaches are rare in children.

Sinus headache

The diagnosis of headache due to sinusitis is suggested by a history of persistent upper respiratory infection (URI) symptoms lasting longer than 10 days. Nasal discharge, congestion, and cough lasting more than 10 days are usually present; fever may be present as well.

Recurrent headaches occur in approximately 15% of children with sinusitis. These patients complain of a throbbing headache that is worse in the morning or that occurs at the same time each day. The pain may vary with changes in head position.

With ethmoid disease, pain may be referred to behind the ipsilateral eye. With frontal sinusitis, pain may occur just above the inner canthi of both eyes.

Acute bacterial sinusitis may present with the following ^[41] :

• Persistent symptoms of nasal congestion/discharge and cough lasting more than 10 days without clinical improvement

• Abrupt onset of severe symptoms/signs of high fever (>39° C), purulent nasal discharge, and facial pain lasting for 3-4 consecutive days

• Onset of worsening symptoms and signs, such as a new fever, headache, or increased nasal discharge 5-6 days after the onset of a typical viral upper respiratory infection

Head trauma-related headache

Headaches frequently follow closed-head trauma. The headache may appear acutely or may be present for months after the initial injury. Acutely, the patient may complain of headache shortly after the injury, which may worsen and may be accompanied by vomiting, lethargy, or seizures; these may be the earliest symptoms of an intracranial hemorrhage. In chronic cases, headache, dizziness, sleep disturbances, and personality changes may be present for months after the initial injury. Headache is a key feature of the postconcussive syndrome.

Intracranial mass-related headache

Distinguishing intracranial causes from extracranial causes of headache may be difficult. Patients with intracranial masses may complain of pain localized to the region of the mass. However, if a diffuse rise in intracranial pressure exists, the headache may be generalized.

Historical features of intracranial masses include the following:

• Severe occipital headache

• Sneezing, coughing, any Valsalva maneuver, or change in head position exacerbates the pain

• Pain is worse in the morning or awakens the patient from sleep

• Projectile vomiting without nausea and focal seizures may occur

However, morning headaches and projectile vomiting, once thought to be hallmarks of raised intracranial pressure, may also occur from etiologies other than intracranial masses.

Benign intracranial hypertension

Benign intracranial hypertension (pseudotumor cerebri) produces headaches similar to those occurring in conditions with raised intracranial pressure. In addition to having greater pain in the morning and vomiting, patients may have vision problems (eg, diplopia) or gait abnormalities (eg, ataxia).

Meningeal irritation

Meningeal irritation due to inflammation, infection, or hemorrhage (eg, malignant hypertension, vascular lesions) results in the acute onset of diffuse, severe headache. Neck pain or stiffness and alteration in consciousness may be present.

Medication-overuse headache

Chronic use of medications to treat headaches, such as analgesics or triptans, can result in medication-overuse headache. The International Classification of Headache Disorders (ICHD) has recognized this entity. It is defined as the development of a different type of headache or worsening of a migraine or tension headache, resulting in chronic daily headaches. It develops after use of medications such as analgesics or the triptans on more than 10 days per month or after the use of over-the-counter (OTC) analgesics for more than 15 days per month for 3 months' duration.

A thorough physical examination often can exclude systemic causes of headache. Attention should be paid to vital signs, especially the presence of fever, elevated blood pressure, or bradycardia. Search the skin for rashes or cutaneous lesions (eg, petechiae, purpura, ash-leaf spots, café-au-lait spots).

A thorough neurologic examination should be performed to assess the level of consciousness and to evaluate cranial nerve dysfunction, hypertonia, hyperreflexia, hemiparesis, or hemiplegia. Also look for nuchal rigidity, and check the head for hematomas or other signs of trauma. Perform funduscopic examination, looking for papilledema or subhyaloid hemorrhage. Other findings can include the following:

• Migraine headache - Most children with migraine headaches have a normal physical examination without focal deficits; some children with complicated migraine, however, may have focal neurologic abnormalities, such as weakness, third-nerve palsy, or ataxia.

• Tension headache - The physical examination findings are usually normal, but pain on palpation of the posterior neck muscles may be noted

• Sinus headache - Physical findings include pale, edematous nasal mucosa; boggy turbinates; clear or yellow nasal discharge; pain on palpation of frontal or maxillary sinuses; and failure of these sinuses to transilluminate

Head injuries

In acute injuries to the head, the child may have an altered level of consciousness, focal neurologic deficits, abnormalities in cranial nerve function (III, VI), and hemiparesis. In chronic injuries, the physical examination findings often are normal.

Intracranial masses

Patients with headaches due to an intracranial mass often have focal neurologic abnormalities, especially if they have had headaches for several months. These abnormalities include papilledema, sixth-nerve palsy, ataxia, spasticity of the lower extremities, and indications of brain dysfunction regarding language, motor control, or vision (depending on the location of the lesion). Early in the course of the mass lesion, the physical examination findings may be normal. Children with intracranial abscesses may have alteration of the level of consciousness only during the acute presentation.

Other diagnostic signs

Other findings in pediatric patients with headache can include the following:

• Intracranial hypertension - These patients usually have papilledema and occasionally other neurologic deficits (eg, sixth-nerve palsy, ataxia, spasticity of the extremities)

• Uncomplicated idiopathic epilepsy - These children have normal physical examination findings

• Seizures - Children with seizures due to metabolic disorders or abnormal brain architecture may have baseline neurologic deficits (eg, hypertonia, hemiparesis).

• Meningeal irritation - In cases of meningeal irritation, fever (meningitis) or hypertension (malignant hypertension) may be present, as well as altered consciousness, nuchal rigidity, or perivenous hemorrhage of the fundus (subarachnoid hemorrhage secondary to hypertension)