Approach
Migraine remains a purely clinical diagnosis based on suggestive history and supported by normal neurologic exam. There are no biomarkers for migraine. Although some pediatric patients fulfill the International Headache Society's diagnostic criteria for migraine, others do not.[1] Moreover, there are certain characteristics specific to pediatric migraine. Those differences should not deprive the pediatric patient of proper treatment or inclusion in clinical trials. The main differences in clinical presentation between pediatric and adult migraine are duration (pediatric migraine attacks can be as short as 20 minutes in duration) and location (pediatric migraine headache is often reported as bilateral). Pediatric patients tend to under-report symptoms associated with their headache (photophobia, phonophobia, and nausea), symptoms related to aura, or symptoms related to prodrome and postdrome phases.[25]
Clinical history
A history of recurrent, severe, often disabling headaches with a total return to normal in between attacks is suggestive of migraine.[1] At least some of the migraine attacks are disabling and interfere with the ability to function. The other typical features of migraines should be sought as their presence will strengthen the diagnostic certainty.
Elements of history should be obtained from the patient and supplemented by a parent whenever possible.
A complete history will collect information about premonitory and aura symptoms; headache characteristics (severity, location, character, duration); associated symptoms (nausea, vomiting, light sensitivity, and noise sensitivity); headache onset, course, duration, and frequency; and pain-related disability.[26] History should include a detailed enquiry about potential triggers and the patient's routine acute headache management. A review of both abortive and preventive drugs used thus far and the frequency of abortive drug use should follow. An enquiry into patient sleep habits and various sources of stress will often provide additional interventional avenues. A headache diary or calendar may assist with this.[25] Enquire about symptoms suggestive of periodic syndromes associated with migraine, such as benign paroxysmal torticollis, benign paroxysmal vertigo of childhood, abdominal migraine, and cyclical vomiting.[23]
Triggers are hard to identify, especially in younger patients. Lack of sleep, skipping meals, excessive noise, certain smells, stress (of any type), certain foods, and weather changes are some of the more frequent triggers and should be sought.[27]
While adolescents easily recognize photophobia and phonophobia, this is often not the case for younger children. In those circumstances, photophobia, phonophobia, and other symptoms can be inferred from the child's behavior (the child prefers the darker parent's bedroom, asks to turn the TV off, turn the light off, etc.). Vomiting is often reported, while nausea might be harder to recognize by the younger patient.
Apart from the aura symptoms, a migraine with aura has a similar presentation to migraine without aura. Adolescents report aura symptoms such as visual and sensory changes, but younger children probably under-report these. Less common aura symptoms such as aphasia and hemiparesis often lead to emergency department visits.
Attention to various red flags is paramount to avoid missing a more serious etiology. Such red flags include:[26]
Nocturnal headache
Headache triggered by the Valsalva maneuver (coughing, straining)
Headache triggered by exercise
Double vision
Changes in gait pattern or falls
Recent onset of behavioral issues
Acute or subacute, unexpected changes in frequency or character of the headache
Positional aggravation
Migraine with brainstem aura and hemiplegic migraine are two less common types of migraine that warrant special attention:
Migraine with brainstem aura[1]
Typical migraine headache is associated with aura symptoms that localize to the brainstem, including dysarthria, vertigo, tinnitus, hyperacusis, diplopia, ataxia, and decreased level of consciousness.
This type of migraine may coexist with other migraine types.
Headache can be occipital in location.
Not infrequently, this type of migraine leads to emergency department investigations, including brain imaging.
Hemiplegic migraine[1]
The hallmark of this type of migraine is the development of unilateral weakness associated with otherwise typical migraine headaches and typical visual and sensory aura symptoms.
The weakness is reversible.
Weakness typically lasts less than 72 hours; some patients can display persistent symptoms for weeks.
There are sporadic and familial forms.
Unless there are previous similar episodes, hemiplegic migraine is a diagnosis of exclusion.
Physical examination
Normal neurologic exam is typical for patients with migraines.
Occasionally, and primarily in an emergency department, signs related to patient aura, such as scotoma, sensory changes, aphasia, and hemiplegia, might be encountered. Abnormal neurologic findings should lead to urgent further investigations, such as brain imaging and lumbar puncture, depending on the circumstances.
The following findings associated with acute and subacute headache suggest alternative diagnoses and should be specifically sought and excluded on exam:
Fever
Neck stiffness
Acute behavioral and mental status changes
Hypertension associated with bradycardia
Seizures
Large head, prominent scalp veins, "sun-setting" sign
Papilledema
Abnormal extraocular movements
Cranial nerve deficits
Weakness
Abnormal gait
Diagnostic investigations
No further testing is needed for a child with a history suggestive of migraine, normal neurologic exam, and absent red flags.
Several diagnostic tests are available to rule out serious medical conditions in less-than-typical cases, abnormal neurologic exams, and/or the presence of red flags. There are no confirmatory tests for migraine.
Brain imaging
Brain imaging is not recommended for typical cases of pediatric migraine.[28][29]
Brain magnetic resonance imaging (brain MRI) with and without contrast is the preferred imaging modality to evaluate atypical headaches.
Head computed tomography (head CT) is indicated when intracranial bleeding is suspected in acute settings.
Occasionally, when cerebral venous thrombosis is suspected, vascular imaging (CT angiography or magnetic resonance angiography, venography, or conventional angiography) can be employed.
The first episodes of hemiplegic migraine or brainstem migraine are considered diagnoses of exclusion, and brain imaging is recommended. Similarly, brain imaging should be carefully considered when a patient presents with less common aura symptoms or when examination is difficult due to age. An atypical acute or subacute course, or a sudden change in the character or frequency of the headaches, should prompt consideration for further investigations and brain imaging.
Electroencephalogram (EEG)
EEG is not indicated in patients with otherwise typical migraine.[30]
An EEG is recommended when the clinical picture suggests late-onset occipital epilepsy. In this condition, children present with visual phenomena lasting for a few minutes, mimicking visual aura, followed by headaches with migrainous features. The visual phenomena tend to be described as colorful geometric shapes representing occipital seizures, making the differentiation between this entity and migraine with aura difficult.
Lumbar puncture
A lumbar puncture is not recommended for otherwise typical pediatric migraine.
A lumbar puncture is indicated if a central nervous infection is suspected, papilledema is present (only after brain imaging has been obtained), or intracranial hypotension is a concern.
Laboratory testing
Laboratory tests are not indicated when the clinical picture suggests pediatric migraines.
Whenever an inflammatory condition such as vasculitis is suspected, inflammatory markers (erythrocyte sedimentation rate [ESR], C-reactive protein [CRP]) can be useful as initial screening tests.
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