Approach

Headache may be primary (due to an underlying headache disorder) or secondary (symptomatic of an underlying systemic or neurologic condition).[10] The initial diagnostic step is considering secondary types of headache.[11] Children with abrupt onset of headache, other neurologic or medical problems, or focal symptoms or signs are at higher risk for having a secondary headache etiology. A detailed history, medical examination, and neurologic examination are required for all patients. Investigations include imaging (CT or MRI), blood tests, and lumbar puncture, and are dependent on clinical assessment.

Patients may benefit from keeping a detailed headache calendar or diary to assess the true frequency and description of headaches. It may also be useful for headache management, and determining whether treatment produces a quantitative change in headache occurrence.[12] Management generally focuses on abortive medications (taken at headache onset; useful for rarer headaches) or prophylactic medications (taken daily even if headache is not present; useful for more frequent headaches or headaches that don't respond well to abortive medications). Psychological comorbidities may coexist and are important to consider. Few studies address treatment modalities in the ER for children experiencing migraine.[13]

History

Time course

  • Recent-onset headache or progressively worsening headache is more concerning for an acute symptomatic etiology for the headache.

  • Abrupt-onset headache is more concerning for acute symptomatic headache and is termed thunderclap headache.

Context

  • Onset with activity is more concerning for an intracranial hemorrhage.

  • Intracranial hemorrhage or arterial dissection must be considered if there is head or neck trauma.

  • Intracranial hypotension must be considered if there is a history of recent back trauma.

  • Important to establish whether the onset was associated with or provoked by a particular stimulus.

Localization

  • Locating the pain (e.g., tension headaches commonly result in mild, posterior, or diffuse pain).

Prior headaches

  • Previous history of similar headache suggests primary headache, unless there is evidence of progressive or intermittent elevated intracranial pressure, suggested by headache worsening when the patient remains in certain positions.

  • Frequent headaches are more concerning for a worsening intracranial etiology.

  • Headaches of increasing frequency are more concerning for a worsening intracranial etiology.

  • Patients with primary headache disorders can also develop other acute symptomatic etiologies for headache that must be considered if this headache is different to that of the patient's typical recurring headaches.

Provocation

  • Change in headache with a position change suggests intracranial hypertension or hypotension.

  • Worse when lying down is suggestive of intracranial hypertension.

  • Worse when standing up is suggestive of intracranial hypotension.

  • Worse following a bowel movement is suggestive of intracranial hypertension worsened with Valsalva.

Associated conditions or medical history

  • Other neurologic symptoms or signs are suggestive of a secondary etiology of headache.

  • Vision changes are suggestive of optic nerve edema related to intracranial hypertension; diplopia due to focal lesions or elevated intracranial pressure; or visual strokes/transient ischemic attacks.

Initial survey

Vital signs

  • Abnormal blood pressure (hypertension suggests possible elevated intracranial pressure or hypertensive encephalopathy).

  • Temperature (fever suggests possible infectious etiology that may be intracranial or extracranial).

  • Cushing triad: hypertension, bradycardia, and irregular breathing pattern (suggests elevated intracranial pressure).

Mental status

  • Abnormality suggests encephalopathic process.

Skin

  • Neurocutaneous lesions (hyper- or hypo-pigmented skin lesions; oro-buccal telangiectasias; axillary or inguinal freckles) may suggest intracranial lesions.

Head and neck examination

Cranial and neck auscultation

  • Bruits suggest vascular malformation or dissection.

Sinuses

  • Tenderness to palpation, inflamed mucosa, and purulent nasal discharge may suggest sinusitis.

Temporomandibular joint (TMJ)

  • Tenderness to palpation at TMJ or temples.

  • Clicks at TMJ when opening and/or closing jaw.

  • Reduced jaw movement: maximal mandibular opening is 35-55 mm.[14] Movement may be reduced to <35 mm.

Oral/dental examination

  • May reveal dental caries, gingival disease, or oral abscess that could cause head pain.

Meningeal signs

  • Meningismus, photophobia.

  • Suggestive of meningeal inflammation due to inflammatory, infectious, or neoplastic disease.

Head circumference (in all children)

  • Abnormal rate of increase in head circumference suggests an intracranial lesion.

Neurologic examination

A full neurologic examination is required for all patients. Identifying abnormal signs suggests an underlying brain lesion and a secondary headache etiology. Focal findings may suggest specific localization of the lesion, based on the examination sign. Nonfocal findings, such as optic nerve edema or bilateral abducens (6th cranial nerve) nerve palsies, may be nonspecific signs of elevated intracranial pressure. Neurologic examination abnormalities generally suggest further evaluation is needed urgently, often beginning with a noncontrast head CT.

Cranial nerve (CN) II: optic nerve

  • Optic nerve edema suggests elevated intracranial pressure.

  • Visual fields may be reduced with elevated intracranial pressure.

Pupil function or asymmetry

  • An abnormally dilated pupil in a bright room suggests herniation syndrome.

  • An abnormally constricted pupil in a dark room suggests Horner syndrome with a carotid artery dissection.

  • Dilated pupils with oculomotor palsy (eye gazes out and down) suggests CN III palsy and compressive lesions including acute herniation.

Extra-ocular movements

  • Deficits such as lateralizing nystagmus, disconjugate gaze, or limitations in horizontal or vertical gaze suggest a brainstem lesion.

  • Eye gazing out and down combined with dilated pupil suggests CN III palsy and possible compressive lesion including acute herniation.

CN IV trochlear nerve palsy

  • Presents with head tilt and chin tuck.

  • May occur with head trauma or brainstem lesions.

CN VI abducens nerve palsy

  • Presents with head turn or horizontal diplopia.

  • Suggests elevated intracranial pressure or brainstem lesions.

Strength, tone, reflex, and sensory asymmetries and abnormalities

  • Suggests focal lesions.

Babinsky sign

  • Upgoing toes (suggests an upper motor neuron lesion in the brain or spinal cord).

Cerebellar signs

  • Suggest a posterior fossa lesion (i.e., tumor, cerebellitis).

Gait evaluation

  • Gait that is worse than baseline in any child, wide based if older than a toddler, or abnormal due to unilateral dysfunction is concerning.

Urgent neuroimaging

Most patients with headache do not require urgent neuroimaging.

Indications for urgent neuroimaging include:

  • A very severe headache with acute onset (worst headache of one's life; e.g., thunderclap headache, primary cough headache)[15]

  • New focal neurologic signs

  • Meningismus

  • Optic nerve edema

  • History suggestive of elevated intracranial pressure (rapidly worsening headache, vomiting, diplopia, optic nerve edema if not acutely elevated)

  • Possible ventriculoperitoneal shunt infection or malfunction

  • Headache with known or possibly metastatic cancer

  • Headache in an immunocompromised patient.

Several individual clinical features, in a review, were found to be associated with a significant intracranial abnormality, and patients with these features were recommended to undergo neuroimaging.[16] These include cluster-type headache, abnormal findings on neurologic examination, undefined headache (i.e., not cluster-, migraine-, or tension-type), headache with aura or vomiting, or headache aggravated by exertion or a Valsalva-like maneuver. No clinical features were useful in ruling out significant pathologic conditions. One review found that, in patients presenting with thunderclap headache, a normal neurologic exam and a normal brain CT within 6 hours of the headache are extremely sensitive in ruling out aneurysmal subarachnoid hemorrhage.[9]

Noncontrast head CT is generally indicated as the initial study because it is rapid, readily available, and sensitive for blood detection. MRI (sometimes with angiography or venography) may be indicated as a subsequent study, especially to detect small tumors, vascular malformation, inflammatory changes, or abnormalities in the posterior fossa and cervical cord.

Nonurgent neuroimaging

Other indications for neuroimaging include:

  • New onset of headaches

  • Chronic-progressive pattern

  • Unvarying headache location

  • Headache that awakens child from sleep or is present on awakening

  • Child who cannot provide a clear history.

If these patents are otherwise well, a CT might be deferred to avoid radiation exposure and an MRI obtained as an outpatient. A practice parameter from the American Academy of Neurology addresses the evaluation of recurrent headache in children and adolescents.[17]

Lumbar puncture

If infection or elevated intracranial pressure is suspected, treatment should not be delayed in order to obtain a lumbar puncture.

Lumbar puncture should be considered for:

  • Acute onset of severe headache

  • Headache associated with fever and/or meningismus, altered mental status, or seizure

  • Immunocompromised patients

  • History suggestive of pseudotumor cerebri (idiopathic intracranial hypertension).

Accompanying studies

  • If elevated intracranial pressure is suspected, neuroimaging should be performed prior to lumbar puncture to avoid the rare but real possibility of herniation.

  • If high or low pressure is suspected, opening pressure should be measured with the patient in a relaxed lateral recumbent position.

  • If a subarachnoid bleed is suspected, examination of the centrifuged supernatant CSF for xanthochromia (yellow coloration) is the most sensitive method and is best identified and quantified in the laboratory, rather than visually. Xanthochromia can persist up to several weeks following a subarachnoid hemorrhage. If this method is not available, sending tubes 1 and 4 for a cell count can also enable differentiation between true subarachnoid blood and blood from a traumatic tap.

Ancillary tests

Additional tests that may help narrow the differential further in specific cases include the following.

Sinovenous thrombosis

  • CBC, PT, and activated PTT to elucidate a clotting problem.

Ventriculoperitoneal shunt dysfunction

  • Shunt series x-rays (imaging of the shunt in the neck, chest, and abdomen may reveal disconnection as the etiology of malfunction).

  • All patients with suspected ventriculoperitoneal shunt dysfunction should be referred for neurosurgical evaluation and possible shunt tapping.

Brain tumor

  • Biopsy to confirm pathology.

Indomethacin-responsive headache

  • These can include trigeminal-autonomic cephalgias, Valsalva-induced headaches, and primary stabbing headache (ice-pick headache or jabs and jolts syndrome).

  • Paroxysmal and continuous hemicranias invariably respond in an absolute manner to indomethacin; Valsalva-induced and ice-pick headaches may respond in an equally dramatic, but less consistent fashion. Hypnic headache may also respond to indomethacin.[18]

Cluster headaches

  • Therapeutic trial of oxygen supplementation: resolution of headache is diagnostic. Cluster headache is rare in children and may be symptomatic of a brain lesion, so MRI is indicated.

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