Headaches

History

The specific headache diagnosis is determined by the headache phenotype, which is defined in terms of laterality, location ( Figs. 34.1, 34.2, and 34.3 ), 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 one 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. 34.1, 34.2, and 34.3 ). If the pain is unilateral, it should be noted whether the pain is always on one side or if the side varies. The location may be restricted or more widely distributed; if the location varies from one episode to another, this should be noted as well. Unusual locations in pediatrics may include the occipital region. This location should raise index of suspicion for intracranial pathology if there are abnormal neurologic findings or the headache does not meet criteria for a primary headache disorder.

The timing, frequency, and duration of headaches should be described, as the temporal patterns of headaches are useful in creating a differential diagnosis, identifying the need for work-up, and classifying the type of headache. The temporal categories of headache include acute, acute recurrent, chronic nonprogressive, and chronic progressive ( Table 34.2 ).

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 TTHs 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 the onset of pain is sudden, reaches maximum severity within seconds, and is often 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 34.3 ). 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.

Associated symptoms such as hemiparesis, ataxia, visual loss, diplopia, scotomata, 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 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 the use of any supplements or traditional remedies. Both primary and secondary headaches may respond to medications and such a response is not diagnostic of any specific 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.

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 34.4 ). 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. 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.

The past medical history may reveal a risk for 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, autoimmune disorders, 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, early-onset strokes, 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.

Some historical components are classic features of primary headaches, while others are concerning for secondary headaches ( Table 34.5 ). 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.

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 34.6 ). 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 idiopathic intracranial hypertension (IIH). Enlarged head circumference associated with signs of headache or other evidence of increased intracranial pressure warrants alarm.

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 or vasculitis 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 ( Fig. 34.4 ). 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 an ophthalmologist may be required. If the results of examination suggest a structural brain lesion or increased intracranial pressure, neuroimaging is warranted ( Table 34.7 ). 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 34.8 ). A single normal physical examination does not exclude pathology; thus, periodic reassessments are essential if headache persists.

Neuroimaging

Most children do not require neuroimaging for headaches, and it is rarely indicated in children with recurrent headaches and a normal neurologic and ophthalmologic exam. Neuroimaging should be considered when the headache history or symptomatic progression is incompatible with a primary headache disorder or if there is no family history of primary headache disorder. Furthermore, neuroimaging in the assessment of headaches in children is indicated when the following features are present: abnormal neurologic exam or historical findings; new onset of afebrile seizures or alteration in seizure type or frequency; new severe headache; change in frequency or severity of headaches; headache consistent with increased intracranial pressure; association of headache with cough or bending over; concerning past medical history components, including trauma or presence of a ventriculoperitoneal shunt; and age younger than 6 years ( Table 34.9 ). 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 inability to obtain an accurate physical examination due to lack of patient cooperation.

MRI and CT are the neuroimaging modalities to consider ( Table 34.10 ). 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. An MRI contrast study should be considered when there is concern for infection or if the noncontrast study is abnormal. 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.

Laboratory Investigations

Routine blood work is not indicated when history suggests a primary headache disorder and physical and neurologic examinations are normal. Findings in the history, physical examination, or neuroimaging that dictate directed laboratory evaluation are listed in Table 34.11 .

Classification of Headaches

Headaches are classified broadly as primary or secondary. The acuity or chronicity of the headache helps to guide the development of the differential diagnosis ( Fig. 34.5 ).