Headaches




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.



TABLE 28.1

Differential Diagnosis of Headache





















































Headache Type Genetics Epidemiology Characteristic Features Length Accompanying Symptoms
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

From Digre KB. Headaches and other head pain. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine . Vol. 2. 25th ed. Philadelphia: Elsevier; 2016:2357, Table 398-2.


(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.




FIGURE 28.1


Common location of migraine (A) and tension (B) headaches.

(From Reilly BM. Practical Strategies in Outpatient Medicine. 2nd ed. Philadelphia: WB Saunders; 1991.)



FIGURE 28.2


Periorbital headache.

(From Reilly BM. Practical Strategies in Outpatient Medicine . 2nd ed. Philadelphia: WB Saunders; 1991.)



FIGURE 28.3


Cluster headache.

(From Reilly BM. Practical Strategies in Outpatient Medicine. 2nd ed. Philadelphia: WB Saunders; 1991.)


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 ).



TABLE 28.2

Four Temporal Patterns of Childhood Headache











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.

From Marcdante KJ, Kliegman RM. Headache and migraine. In: Marcdante KJ, ed. Nelson Essentials of Pediatrics . 7th ed. Elsevier; 2015:616-618.


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.



TABLE 28.3

Main and Rare Causes of Thunderclap Headache

































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

From Linn FHH. Primary thunderclap headache. In: Aminoff MJ, ed. Handbook of Clinical Neurology. Vol. 97. New York: Elsevier; 2010: 473-481.


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.



TABLE 28.4

The Headache Diary for Recurring Headaches *








  • Date



  • Time of onset



  • Time of resolution



  • Maximum level of pain (mild, moderate, or severe or according to a visual or numerical pain scale)



  • Triggers:




    • Sleep



    • Foods



    • Activities



    • Medications




  • Modifiers:




    • Response to position changes or Valsalva maneuver



    • Medications used (dose, response)



    • Other modifiers




  • Additional symptoms


* 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 ).



TABLE 28.5

History-Related Red Flags for Secondary Headaches








  • Quality:




    • “Thunderclap” headache or the “worst headache of my life”



    • Recent worsening in severity or frequency



    • Change in quality



    • New-onset symptoms consistent with cluster headache




  • Location:




    • Unilateral without alteration of sides



    • Chronic or recurrent occipital headache




  • Timing:




    • Awakens from sleep



    • Occurs in morning or causes morning vomiting



    • Chronic progressive pattern




  • Positional or activity-related variations:




    • Worsened in the recumbent position



    • Headache experienced with cough or the Valsalva maneuver




  • Associated neurologic history:




    • Altered sensorium during headache



    • Sensory deficits or changes in vision, gait, or coordination



    • Other focal neurologic deficits



    • Seizures or syncope



    • Mental status changes (e.g., confusion or disorientation)



    • Regression in fine or gross motor developmental skills



    • Decline in cognition or school performance



    • Change in behavior or personality




  • Associated general history:




    • Vomiting without nausea



    • Polyuria or polydipsia



    • Preschool or younger age



    • History of head trauma



    • Medical comorbidities



    • Negative family history of primary headache disorders




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.



TABLE 28.6

Physical Examination Red Flags for Secondary Headaches








  • Hypertension



  • Growth failure



  • Increased head circumference or bulging fontanel



  • Meningeal signs with or without fever



  • Evidence of cranial trauma



  • Cranial bruit



  • Frontal bony tenderness



  • Abnormal ophthalmologic findings:




    • Papilledema



    • Abnormal ocular movements



    • Squinting



    • Pathologic pupillary response



    • Visual field defects




  • Abnormal neurologic findings:




    • Impaired mental status



    • Cranial nerve palsy



    • Ataxia



    • Abnormal gait



    • Abnormal coordination



    • Abnormal reflexes



    • Asymmetric motor or sensory examination



    • Hemiparesis



    • Developmental regression




  • Precocious, delayed, or arrested puberty



  • Skin findings:




    • Café-au-lait or ash leaf macules



    • Petechiae or purpura



    • Facial hemangioma



    • Malar rash




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.



TABLE 28.7

Headache Disorders Associated with Neurologic Signs
























































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

A, acute; AR, acute recurrent; CP, chronic progressive.


TABLE 28.8

Headache Disorders with No Neurologic Signs




















































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

A, acute; AR, acute recurrent; CN, chronic nonprogressive; CP, chronic progressive.


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.



TABLE 28.9

Reasons to Obtain Neuroimaging in a Child with Headache








  • Abnormal neurologic findings (including papilledema)



  • Associated confusion, disorientation, or signs of increased intracranial pressure



  • Change in pattern or severity of previously stable headache



  • Cough headache



  • Headache consistent with trigeminal autonomic cephalgia



  • Headache occurring in the morning or waking the child from sleep



  • Headache with known concerning underlying disorder or insult



  • History of neurologic dysfunction (outside of typical aura symptoms)



  • Incompatibility of headache with primary headache disorder or unusual headache in a child



  • Meningeal signs without fever



  • Recent onset of severe headache



  • Recent trauma



  • Seizures



  • “Thunderclap” headache or “worst headache of my life”



  • Ventriculoperitoneal shunt



  • Young age (less than 6 yr) or inability to describe headache



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.


Apr 4, 2019 | Posted by in PEDIATRICS | Comments Off on Headaches

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