Introduction
Headaches are classified as primary or secondary. Primary headaches are benign, are not caused by underlying disease or structural problems, and include migraines, tension-type headaches, and the trigeminal autonomic cephalgias ( Table 28.1 ). While primary headaches may cause significant pain and disability, they are not intrinsically dangerous. Secondary headaches are caused by an underlying disease, such as infection, tumor, intracranial hemorrhage, or a vascular disorder, and may indicate an innocuous etiology or portend a serious illness. Most headaches in children are primary headaches or harmless secondary headaches. History and physical examination guide the diagnosis of primary headache disorders, assess the degree of headache-related disability, and reveal information that may prompt evaluation for secondary headaches. Each subsequent visit allows for assessing the response to therapy and considering secondary headaches, the causes of some of which may be life threatening.
Headache Type | Genetics | Epidemiology | Characteristic Features | Length | Accompanying Symptoms |
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Migraine headache | Complex genetics but usually a family history | More frequent in women | Unilateral, bilateral; throbbing; moderate to severe; worsens with activity | Hours to days | Photophobia, phonophobia, nausea and/or vomiting |
Tension-type headache | Usually a family history | Equal frequency in men and women | Tight bandlike pain; bilateral; pain may be mild to moderate; improves with activity | Hours to days | No nausea or vomiting; small amount of light or sound sensitivity, but not both |
Cluster headache | May have a family history | More frequent in men | Unilateral severe pain in the face | Minutes to hours | Ipsilateral ptosis, miosis, rhinorrhea, eyelid edema, tearing |
Paroxysmal hemicrania | Usually no family history | More frequent in women | Unilateral pain in the face | Minutes | Ipsilateral ptosis, miosis, rhinorrhea, eyelid edema, tearing; responds to indomethacin |
Short unilateral headache with conjunctival injection, tearing | No family history | More frequent in men | Unilateral eye pain; orbit pain | Typically 4 minutes or less | Conjunctival injection, tearing |
Hemicrania continua | No family history | More frequent in women | Unilateral continuous headache with episodic stabbing pains | Continuous | Ipsilateral ptosis, miosis, rhinorrhea, eyelid edema, tearing |
(See Nelson Textbook of Pediatrics, p. 2863.)
History
The specific headache diagnosis is determined by the headache phenotype, which is defined in terms of laterality, location ( Figs. 28.1, 28.2, and 28.3 ), timing, frequency, duration, quality, severity, associated symptoms, and alleviating and aggravating factors. In most cases, a single phenotypic headache is present. If the patient has more than 1 type of headache, the clinician must obtain a specific history for each type. Ideally, the history should be obtained from the child, parent, and any other caregivers, including teachers. Even a young child should be given the opportunity to describe the symptoms experienced with each headache episode and may use drawings to do this.
The laterality and location of the pain should be established (see Figs. 28.1, 28.2, and 28.3 ). If the pain is unilateral, it should be noted whether the pain is always on 1 side or if the side varies. The location may be fairly restricted or more widely distributed; if the location varies from 1 episode to another, this should be noted as well. The timing, frequency, and duration of headaches should be described, as the temporal patterns of headaches are useful in both creating a differential diagnosis and classifying the subtype of a particular headache diagnosis. The temporal categories of headache include acute, acute recurrent, chronic nonprogressive, and chronic progressive ( Table 28.2 ).
Acute: Single episode of pain without a history of such episodes. The “first and worst” headache, which raises concerns for aneurysmal subarachnoid hemorrhage in adults, is commonly due to a febrile illness related to upper respiratory tract infection in children. Regardless, more ominous causes of acute headache (hemorrhage, meningitis, tumor) must be considered. |
Acute recurrent: Recurrent attacks of pain separated by symptom-free intervals. Primary headache syndromes, such as migraine or tension-type headache, usually cause this pattern. Infrequently, recurrent headaches can sometimes also be attributed to certain epilepsy syndromes (benign occipital epilepsy), substance abuse, or recurrent trauma. |
Chronic progressive: Most ominous of the temporal patterns; implies a gradually increasing frequency and severity of headache. The pathologic correlate is increasing intracranial pressure. Causes of this pattern include pseudotumor cerebri, brain tumor, hydrocephalus, chronic meningitis, brain abscess, and subdural collections. |
Chronic nonprogressive or chronic daily: Pattern of frequent or constant headache. Chronic daily headache generally is defined as >4-mo history of >15 headaches/mo, with headaches lasting >4 hr. Affected patients have normal neurologic examinations; psychologic factors and anxiety about possible underlying organic causes are common. |
The severity of a headache does not necessarily correlate with the seriousness of its etiology. Pain caused by brain tumors may initially be mild, whereas the pain of tension-type headaches may be excruciating. Pain is subjective and may be influenced by age, culture, duration, and previous encounters with medical care, leading some patients to unintentionally minimize or exaggerate their pain; as such, pain alone should not be used to narrow the differential diagnosis. An exception to this principle is the thunderclap headache , in which pain onsets suddenly, reaches maximum severity within seconds, and is oftentimes described by patients as the worst headache they have ever had. Such headaches may indicate subarachnoid hemorrhage, arterial dissection, or venous sinus thrombosis, among other causes ( Table 28.3 ). Numerical scales, or visual scales for younger children, are helpful for quantifying pain and determining the efficacy of treatment. In older patients, descriptive phrases, such as mild, moderate, severe, and excruciating , may suffice.
Main Causes | Rare Causes |
---|---|
Vascular Disorders | |
Subarachnoid hemorrhage Intracerebral hemorrhage Cerebral venous thrombosis Spontaneous intracranial hypotension Cervical artery dissection | Pituitary apoplexy, arteritis, angiitis Unruptured vascular malformation, aneurysm Arterial hypertension Cerebral segmental vasoconstriction |
Nonvascular Disorders | |
Greater occipital neuralgia | |
Intermittent hydrocephalus by colloid cyst | |
Infections | |
Meningitis, encephalitis | Erve virus |
Sinusitis | |
Primary Headache Disorders | |
MigrainePrimary thunderclap headachePrimary exertional headachePrimary cough headache | Cluster headacheTension headache, new daily persistent headache |
Associated symptoms such as hemiparesis, ataxia, visual loss, diplopia, scotomas, vertigo, seizure-like activity, confusion, mood or behavioral changes, autonomic symptoms, and hemisensory occurrences may suggest neurologic dysfunction or a migraine-related aura. Any history of fevers, syncope, nausea, vomiting, and appetite changes should also be ascertained. Special note should be made if the pain awakens the patient from sleep, is present upon awakening in the morning, or worsens when recumbent; these findings may indicate increased intracranial pressure. Events associated with the onset or aggravation of headaches, such as trauma, intake of particular foods, or physical exertion, may provide insight into the etiology of headaches, as well as potential triggers to avoid. Alleviation via rest or positional changes should be noted, as should the response of the headaches to particular medications. A thorough medication history is essential for diagnosing analgesic overuse headaches and headaches caused by medication side effects. The use of over-the-counter medication and prescription medications, including medications that have not been prescribed for the patient, should be delineated, as well as any supplements or traditional remedies. Both primary and secondary headaches may respond to medications and such a response is not diagnostic of any particular headache disorder. For example, relief of an acute headache by triptans is not diagnostic of migraine, as triptans may also be effective for other causes of headache. An exception is certain trigeminal autonomic cephalgias, which respond only to indomethacin.
In patients with recurring headaches, the history may be clarified by keeping a headache diary , which can additionally determine headache patterns, identify triggers, aid diagnosis, and assess the efficacy of therapy ( Table 28.4 ). A headache diary may also assist in determining the degree of disability caused by the headache. Disability evaluation may be augmented by school attendance and performance records. For example, headaches that improve with the onset of the summer school holiday may suggest that a child is struggling academically or is being bullied at school. In younger children, where detailed personal descriptions of pain may be more difficult to obtain and record in a diary, videos of the headache episodes may aid diagnosis.
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* If more than 1 type of headache exists, the types should be defined and labeled, and separate data should be recorded for each type.
The past medical history may reveal potentially serious causes of secondary headaches that require prompt evaluation, such as sickle cell disease, thyroid disorders, parathyroid dysfunction, malignancy, hypercoagulability, hypertension, immunodeficiency, congenital heart disease, and arteriovenous malformations. Allergic rhinitis and other atopic disorders are also associated with headaches. Infantile colic, benign paroxysmal torticollis, cyclic vomiting syndrome, and benign paroxysmal vertigo are considered episodic syndromes that may be associated with migraine and may precede the development of typical migraine symptoms later in life. In females, a menstrual history should be obtained, including details of the cycle and the timing of headaches with respect to the menstrual cycle. A history of secondary amenorrhea could suggest pituitary or other central nervous system neoplasms.
The family history should be probed for any genetic predisposition to migraines, aneurysms, other vascular malformations, or brain neoplasms. A negative family history for primary headaches should cause the clinician to be more cautious in assigning the diagnosis of a primary headache disorder. Social history should investigate for psychosocial factors that may influence or be influenced by headaches, such as school performance, the relationships between family members, recent changes in social structure, and substance abuse in the patient or the family. The provider should also screen for indications of neglect or abuse. Detailed psychologic evaluation with screening for symptoms of depression and anxiety may be indicated.
Throughout the history, the clinician should constantly assess for warning signs of serious and sometimes life-threatening causes of secondary headache. The identification of any of these red flag symptoms should cause concern and lead promptly to further investigation ( Table 28.5 ).
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Physical Examination
Abnormalities in the examination may provide clues to the underlying etiology of secondary headaches, and red flags may identify specific diagnoses of concern ( Table 28.6 ). Vital signs assessment may reveal elevated blood pressure, which may be the cause of headache, signal increased intracranial pressure, or herald an underlying renal abnormality. Fever may be a sign of an infectious or inflammatory process. Growth parameters, including height, weight, body mass index, and head circumference should be obtained. Poor weight gain may indicate an underlying chronic illness associated with headaches, such as celiac disease, respiratory disorders, neurofibromatosis type 1, or neglect. Obesity should alert the clinician to assess for symptoms of obstructive sleep apnea or pseudotumor cerebri. Enlarged head circumference associated with signs of headache or other evidence of increased intracranial pressure warrants alarm.
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The general examination starts with assessment of mental status and overall level of distress. The head and neck examination should assess specifically for nasal congestion, sinus tenderness, and signs of allergic rhinitis, such as boggy nasal turbinates. Frontal bone tenderness could be an early sign of Pott puffy tumor, a complication of frontal sinusitis. Tenderness over the mandibular condyle in children with dental malocclusion, or jaw crepitus in patients with arthritis, may indicate temporomandibular joint dysfunction as a cause of headache. Thorough lymphatic, respiratory, cardiac, and abdominal examinations should also be completed. Genitourinary examination should include pubertal stage, as headaches may be associated with endocrine disorders. Skin should be evaluated for petechiae, atopic findings, and lesions associated with neurocutaneous syndromes such as neurofibromatosis or tuberous sclerosis. Signs of trauma should be noted. Neurologic examination should be detailed and include assessments of mental status, cranial nerves, auditory function, sensation, motor strength, reflexes, gait, coordination, and speech. Whenever possible, a thorough ophthalmologic examination should be undertaken, including visual acuity testing and a funduscopic evaluation for papilledema. In a young child, much of the neurologic examination is completed through observation or engaging the child in play to elicit findings. A complete ophthalmologic evaluation may be limited by lack of cooperation or comprehension. If the clinician is unable to complete or interpret the neurologic and ophthalmologic assessments, the support of a neurologist and ophthalmologist may be required. If the results of either examination suggest a structural brain lesion or increased intracranial pressure, neuroimaging is warranted ( Table 28.7 ). However, many causes of headache, including some serious diseases early in their course, do not present with abnormal findings on physical examination or have fluctuating abnormal findings ( Table 28.8 ). A single normal physical examination does not exclude pathology; thus, periodic reassessments are essential if headache persists.
Headache | Pain Profile | Neurologic Sign |
---|---|---|
Complicated migraine | AR | Hemiparesis, aphasia, paresthesia, hemianopsia |
Migraine with brainstem aura | AR | Dysarthria, vertigo, tinnitus, hypoacusis, diplopia, ataxia, decreased level of consciousness |
Acute confusional migraine | AR | Alteration in migraine sensorium, stupor, agitation, fugue state |
Vasculitis | CP, AR | Seizure, changes in sensorium |
Brain neoplasm or mass | CP | Papilledema, focal deficit |
Hydrocephalus | CP, AR | Papilledema, bilateral sixth nerve palsies, increased motor tone, impaired upward gaze and Parinaud syndrome |
Pseudotumor cerebri | CP | Papilledema, constricted visual fields, enlarged blind spot |
Subarachnoid hemorrhage, ruptured aneurysm | A | Changes in sensorium, focal neurologic signs, meningismus |
Subdural or epidural hemorrhage | CP | Focal neurologic signs, papilledema, changes in sensorium |
Sagittal sinus thrombosis | A | Papilledema, focal neurologic deficits, changes in sensorium, seizures |
Meningitis, encephalitis | A | Papilledema, focal neurologic deficits, changes in sensorium, seizures |
Optic neuritis | A | Papillitis, decreased visual acuity, afferent pupillary defect |
Headache Disorder | Pain Profile |
---|---|
Tension-type headache | CN, AR |
Migraine without aura | AR, CN |
Cluster headache | AR |
Hypertension, uncomplicated | AR, CN |
Fever | A |
Anoxia | A |
Medication overuse | CN |
Caffeine withdrawal | A, AR |
Early hydrocephalus or brain mass | CP |
Cough headache, uncomplicated | AR |
Meningitis, uncomplicated | A |
Sinusitis, dental or pharyngeal abscess | AR |
Temporomandibular joint syndrome | CN |
Postconcussive syndrome | CN |
Conversion disorder | CN |
Neuroimaging
Most children do not require neuroimaging for headaches, particularly children with recurrent headaches and normal neurologic examinations. Neuroimaging in the assessment of headaches in children is indicated under the following circumstances: (1) abnormal neurologic findings, (2) headaches occurring early in the morning or waking the child from sleep, (3) associated confusion, disorientation, or signs of increased intracranial pressure, (4) presence of a ventricular shunt, (5) recent trauma, and (6) age less than 3 years. Additional indications for neuroimaging include recent onset of severe headache, incompatibility of headache with a primary headache disorder diagnosis, change in the pattern or severity of a previously stable headache, and a history of neurologic dysfunction beyond typical aura symptoms ( Table 28.9 ). In specific cases, neuroimaging may be considered when there is history of a brain tumor in the family, fear by the patient or the parents of underlying pathology, or an inability to obtain an accurate physical examination due to lack of patient cooperativity.
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Magnetic resonance imaging (MRI) and computed tomography (CT) are the 2 neuroimaging modalities to consider ( Table 28.10 ). CT remains the most sensitive and rapid method for detecting acute intracranial bleeding, and is preferred in emergency situations or when MRI is contraindicated or unavailable. MRI is otherwise the preferred imaging modality, offering superior visualization of soft-tissue contrast and gray-to-white matter differentiation without exposing the patient to the ionizing radiation associated with CT scanning. While gadolinium contrast for MRI is considered safe, it is not usually necessary. MRI may involve the need for sedation, particularly in younger children. Normal neuroimaging and a single normal neurologic examination should not give complete reassurance. Follow-up assessment of ongoing symptoms or for changes in the physical examination remains necessary.