Diagnosis and Management of Headaches in the Emergency Department (ED) in Adults and Children
Correspondence Address: Source of Support: None, Conflict of Interest: None DOI: 10.4103/0028-3886.315977
Source of Support: None, Conflict of Interest: None
Keywords: Adult, emergency department, headache, migraine, pediatric, secondary headache, thunderclap headache
Headaches have been prevalent for long, but only after the systematic epidemiological studies since 1990, did its magnitude became apparent, with an estimate of three billion adults currently suffering from migraine or tension-type headaches. In children and adolescents under twenty years of age, the world-wide prevalence is about 60% over a period of three months to lifetime, with females 1.5 times more likely than males to have a headache. 8% of children and adolescents have migraines, with a higher incidence in those above 14 years. Despite the widespread prevalence in both adults and children alike, there has been little improvement in the years lived with disability of patients with headaches. Hence it is unsurprising that patients with headaches account for a high proportion of cases presenting at the ED.
In various ED centers globally, headaches account for approximately 1–9% of visit.,,, There is a high societal and economic cost of headaches,,, owing to recurrent ED and clinic visits, unnecessary diagnostic tests, work, and school absenteeism. Although most of the headache cases visiting ED have either a primary or benign type of headache, life-threatening causes can be seen in 4–10% of cases.,,,,In such cases, the emergency physicians' preliminary clinical assessment and evaluation play a critical role in identifying those patients who may need further investigations or admission for monitoring. In this article, we will review the diagnostic approach for children and adults who present with headache to the ED, detail key features that will help distinguish the primary from the secondary causes and review specific ED management strategies for these groups of patients.
History is an important aspect of clinical assessment in evaluating the symptoms of headaches in any clinical setting. In the ED, enquiring about and understanding the patients' motivations for the emergency visit constitutes an important first step in identifying disconcerting signs of more severe headaches such as the history of trauma, new onset of debilitating headaches including thunderclap headaches and persistent headaches which cannot be alleviated by analgesia. The headache history should be characterized by the temporal progression, intensity, quality of pain, location, alleviating, and aggravating factors. Associated features such as nausea, vomiting, photophobia, phonophobia, or autonomic features frequently accompany primary headaches and give diagnostic clues regarding the specific type of primary headache. Red flags in history and examination should be identified for possible secondary headaches [Table 1]. The identification of red flags can aid in more targeted physical examinations and investigations, which will in turn provide further insights on headache etiology.
Typically, the examination of a patient begins with a general inspection, evaluating the patient's mental status and conscious level. In the context of headaches related to trauma, signs of periorbital or retro-auricular ecchymosis may indicate underlying skull base fracture which necessitates the use of computed tomography (CT) for further evaluation. A toxic-looking patient may indicate a systemic or central nervous system (CNS) infection and the physician should assess for signs of meningismus. Classical signs of meningism include Kernig's sign, Brudzinski's sign, and nuchal rigidity. Jolt accentuation of headache is a simple and quick test that can be used to pick up subtle signs of meningismus. It is considered as positive if the horizontal rotation of the neck with a frequency of 2-3 times per second exacerbates the headache. A proper neurological examination of the motor, sensory, and cerebellum helps to identify focal neurological deficits that are indicative of secondary headaches. In addition, physical examination for certain specific headache etiologies includes palpation for temporal artery tenderness (in an elderly patient for temporal arteritis), sinus tenderness (for acute sinus headache), and temporomandibular joint (TMJ) tenderness (for TMJ disorders). A fundoscopy is essential to determine the presence of raised ICP. If the suspicion for secondary headache is high after clinical assessment, the ED physician should proceed to consider further diagnostic testing.
In cases of suspected giant cell arteritis (GCA), a positive test of erythrocyte sedimentation rate (ESR) ≥50 mm/h, platelet ≥300 and C-Reactive Protein (CRP) ≥20 are useful serology tests in the prediction of GCA., (positive tests of high platelet and CRP have a specificity of 84%). Temporal artery biopsy, however, remains the gold standard for diagnosis. A urine pregnancy test should be done in women of child-bearing age to exclude pregnancy, as a positive pregnancy test will influence the choice of neuroimaging and headache treatment due to potential fetal risk. Electrolytes should be assessed in patients with migraine presenting with intractable vomiting and signs of dehydration.
Neuroimaging is the most widely used investigation in the ED for headache, with a preference for computed tomography (CT),, due to its quick performance time, low cost, and availability in the emergency department. MRI has a longer assessment time, higher cost, and is less commonly available in the ED. Nevertheless, it offers the advantages of no ionizing radiation and has better tissue evaluation, especially in the posterior cranial fossa. The American College of Radiology recommends non-contrasted CT in the evaluation of worst headache of one's life, new headaches with papilledema or new, progressive headaches with red flags as highlighted in [Table 1]. MRI on the other hand is useful in suspected trigeminal autonomic cephalalgias or chronic headache with worrisome features. Focused areas of imaging also need to be considered, for example, computed tomography angiography (CTA) for suspected cervical artery dissection, imaging of the TMJ for suspected TMJ-related headache disorders or sinus CT in sinus headache.
Lumbar puncture (LP), albeit difficult in the ED setting, is useful for cerebrospinal fluid (CSF) and intracranial pressure analysis for suspected cases of intracranial infection or pressure-related headaches. LP in the ED setting can also be therapeutic for patients with raised intracranial pressures, cryptococcal meningitis, and idiopathic intracranial hypertension.
Etiologies for secondary headache can be broadly classified by ICHD into headaches related to trauma, vascular disorders, non-vascular intracranial disorders, substance usage or its withdrawal, infection, homeostasis, or surrounding structure disorders. Subarachnoid (SAH) or intraparenchymal hemorrhage and bacterial meningitis presents with only a prevalence up to 3%, but can be life-threatening. However, from an ED perspective, thunderclap headaches are the most important.
Patients with thunderclap headache onset frequently present to the ED due to the intensity of onset of headache, peaking to maximum intensity within seconds to minutes from the onset. Differentials for thunderclap headache are wide, of which subarachnoid hemorrhage is the most common cause. In the evaluation of thunderclap headaches, Ottawa subarachnoid hemorrhage rule can be used to discern headaches that are suspicious and necessitate further investigations. Some of the commonly noted characteristics of patients with SAH are age of >/= 40, neck stiffness or pain, limited neck flexion on examination, loss of consciousness, onset during exertion, and thunderclap headache with peaking within one second., The combination of performing a non-contrast CT brain followed by LP has a sensitivity of 100%. A negative CT and LP study is sufficient to rule out SAH.
In patients with headache, fever, altered mental status and neck stiffness, bacterial meningitis should be suspected, with up to 95% of patients presenting with at least two out of the four symptoms. Due to high mortality rate in bacterial meningitis, there is an advocation for early antibiotic treatment, and if possible, CSF culture should be obtained prior to initiation of treatment, without the need for CT brain. In the absence of other neurological symptoms and signs, a CT brain delays the initiation of treatment and affects the mortality rate in this group of patients.,,
Amongst the various types of primary headaches, cluster headache patients may present disproportionately frequently to the ED despite its low prevalence, due to the high intensity of the headache and the need for emergent relief. Between tension-type headaches (TTH) and migraines, the latter tends to cause more disability with a higher pain intensity and occasional status migrainosus. As a result, migraineurs present to the ED more frequently. Despite the clear diagnostic criteria laid down by ICHD-3, migraine remains under-diagnosed in ED, with a high proportion of emergency physicians discharging patients with a diagnosis of headache not otherwise specified. One study noted a significant discrepancy between the headache diagnosis given by the ED physician with that of the final diagnosis given by the neurologist. This delay of an accurate diagnosis often means specific treatment for migraine may also be delayed in these patients.
Following comprehensive evaluation and diagnostic tests to exclude secondary causes of headaches, providing timely and effective control of symptoms remains the goal of management. Standardized ED headache protocols would ideally provide evidence-based guidance to the selection of pharmacologic agents and facilitate coordination of care. Based on the suspected headache disorder, the physician can tailor the treatment based on patient-specific factors [Table 2]. The majority of patients presenting to the ED would have tried at least one oral analgesic without satisfactory relief. Hence, pharmacologic agents available in alternative routes of administration are often required.
Tension-type headache is usually associated with only mild to moderate pain. Most patients are successfully treated with first-line oral agents such as acetaminophen, ibuprofen, naproxen sodium, ketoprofen, or diclofenac. Those who present to the ED may be the ones who have insufficient relief with maximum doses of the oral agents or experience intolerance to existing drugs. There are limited good quality data that evaluated the effectiveness of parenteral agents in TTH. Current literature demonstrated treatment efficacy with the use of intramuscular ketorolac, intravenous chlorpromazine and intravenous metoclopramide.,
Treatment of CH comprises of three phases: a fast-acting abortive treatment, transitional preventive, and maintenance preventive treatment. To date, there are limited drugs that have demonstrated efficacy for acute treatment. Guidelines have endorsed subcutaneous sumatriptan to be the most effective agent for abortive treatment., Randomized controlled trials (RCTs) have demonstrated that subcutaneous sumatriptan 6 mg provided pain relief in 75% of patients within 15 minutes and pain freedom in one-third. Studies that explored the use of subcutaneous sumatriptan at higher (12 mg) or lower doses (<6 mg) did not provide conclusive evidence of equivalence in terms of treatment efficacy or incidence of adverse effects., Sumatriptan and zolmitriptan administered through the nasal route are alternatives for patients who are unable to tolerate injections or have attacks that are protracted (>1 hour). Compared to the subcutaneous route, treatment effect with nasal spray is expected to be slower as it involves resorption of the drug through the mucosa. Intranasal sumatriptan 20 mg provided pain relief in 57% of patients and about half achieved pain freedom after 30 min., Similarly, in the two RCTs that investigated intranasal zolmitriptan, pain relief was observed in 40-50% (5 mg) and about 60% (10 mg) of patients, 30 min after administration., Triptans are contraindicated in patients with significant vascular risks (e.g., ischemic stroke, ischemic heart disease). In addition, there are limitations on daily usage to prevent tachyphylaxis and rebound.
High-flow oxygen therapy (100% oxygen 7-12 L/min for 15 minutes) is another first-line option in the management of CH attacks. Treatment efficacy is demonstrated in RCTs, with significant pain relief achieved in 75% of patients., The main advantages of oxygen therapy include a lack of established adverse effects, ease of usage in tandem with other treatments, and potential for daily repeated doses. Other agents that have been investigated as abortive treatment in CH, such as lidocaine nasal spray, somatostatin, octreotide, and intranasal dihydroergotamine spray, have yet to demonstrate definitive efficacy data to support routine use in ED. Patients who are newly diagnosed with CH should be referred to headache specialists so that preventive treatment can be promptly initiated.
Migraine patients who require medical attention at the ED usually have persistent symptoms despite existing analgesics or have associated nausea and vomiting that preclude oral agents. Ideally, the medication should have a rapid onset of action and provide sustained headache relief. In addition, it should be devoid of adverse effects and able to facilitate patients' transit back to their normal routine in the shortest time. However, data from clinical trials showed that less than one-quarter of patients have sustained headache freedom after their treatment of acute migraine at the ED. The American Headache Society (AHS) and the Canadian Headache Society (CHS) have published treatment guidelines on the management of migraine that are relevant to the ED setting., Despite the availability of these guidelines, evidence-based treatment is not always utilized, possibly due to a lack of familiarity.
The current body of evidence supports the use of dopaminergic antagonists (prochlorperazine, metoclopramide, chlorpromazine), subcutaneous sumatriptan, and parenteral NSAIDs as first-line agents for the emergent treatment of migraine [Table 3].,,, Amongst the phenothiazines, intravenous prochlorperazine has demonstrated better treatment efficacy against placebo, intravenous metoclopramide (10 mg), subcutaneous sumatriptan, intravenous valproic acid, and intravenous promethazine.,,,, Intravenous phenothiazines however can produce extrapyramidal side effects. Intravenous metoclopramide (20 mg) has demonstrated better efficacy than placebo and subcutaneous sumatriptan and has comparable efficacy and adverse effects as intravenous prochlorperazine intravenous haloperidol, intravenous ketorolac, and intravenous magnesium.
Subcutaneous sumatriptan has consistently demonstrated efficacy over placebo and is recommended for acute treatment of migraine.,,, However, it appears to be inferior to intravenous prochlorperazine and intravenous metoclopramide. When compared against subcutaneous dihydroergotamine, subcutaneous sumatriptan is reported to demonstrate earlier treatment efficacy and lower incidence of side effects. Amongst the NSAIDs, parenteral ketorolac has stronger evidence of efficacy than intramuscular diclofenac and may be the preferred agent if available. Other parenteral agents such as valproic acid, ketamine, propofol, magnesium, and dexamethasone either lack quality data or have provided conflicting results for efficacy and therefore should not be offered as the initial treatment.
The evidence for the use of intravenous fluid use in patients who presented to the ED for headache has been systematically evaluated in the National Hospital Ambulatory Medical Care Survey (NHAMCS) study and a post-hoc meta-analysis of four ED-based clinical trials., The prevalence of such practice was estimated to be about 40% from the NHAMCS database, with similar proportions in migraine patients and those with nonspecific headaches. Those patients who received intravenous fluids had a longer ED length of stay than those who did not receive intravenous fluids. Intravenous fluids also did not affect pain improvement nor sustained headache relief. Until more definitive data is made available, intravenous fluids should only be given to patients with clinical signs of dehydration.
Another common practice in ED is the use of intravenous opioids for the acute treatment of migraine, despite recommendations for using the migraine-specific treatment., A randomized, double-blind, ED-based study demonstrated that intravenous prochlorperazine with diphenhydramine was substantially more effective than hydromorphone in treating acute migraine. In addition, this study also provided evidence that inadequately treated migraine, rather than opioid addiction, leads to the cycle of return visits.,, Given the availability of evidence-based treatment alternatives and demonstrated lack of efficacy, the prevalent use of intravenous opioids should be discouraged.
Childhood headache is also one of the most common neurological complaint prompting ED visits., The majority of these are due to benign causes of which upper respiratory tract infections account for the vast majority of cases, followed by primary headaches and sinusitis. Potential life-threatening causes requiring emergent treatment are similarly less common, making up 2–15% of cases.
An accurate and thorough pediatric headache history requires careful questioning of not just the child, but the caregiver as well. Similar to the adult patient, the emergency physician should obtain a clear description of the headache (encompassing onset, prodrome, duration, severity, quality, triggering, aggravating, and alleviating factors), and screening for risk factors for potentially life-threatening causes. History of trauma, existing medical conditions, medication use and associated symptomatology should also be obtained from the caregiver [Table 4]. A quick screen on psychosocial, academic and family background would be helpful in determining subsequent referral and follow up.
'Red flags' in childhood headaches [Table 1] have been identified by various studies and will necessitate further investigation for more sinister causes.,,, Traditionally, a headache that causes night or early morning awakening is a cause of concern. However, a recent study of children with night awakening, but are clinically well and with normal neurology, demonstrated normal neuroimaging in 95%, with the remaining having a non-significant abnormality that would not have contributed to their symptoms. There is also debate on whether occipital headache heralds more significant etiology. Some report that as a risk factor requiring further evaluation, but others show no difference in underlying diagnosis compared to other sites of pain. Often a secondary headache can present with intense pain, and it is helpful to know that impairment of a child's activities during the headache is a more sensitive indicator of severity than a pain score.
A diagnosis of primary headache can be made if the criteria set by the International Headache Society (IHS) are met. Lewis et al. proposed classifying headaches based on four temporal patterns: acute, recurrent acute, chronic progressive, and chronic non-progressive. Acute headaches with sudden onset need careful evaluation to exclude organic causes. Fever during upper respiratory tract infection and sinusitis are the most frequent causes, however, meningitis, intracranial bleeds, stroke, medication, or intoxication can present similarly. Chronic progressive headaches are also particularly concerning as they can be a manifestation of increased intracranial pressure caused by mass lesions (brain tumor, abscess, and vascular malformations), hydrocephalus, or idiopathic intracranial hypertension (IIH). The patterns of acute recurrence with symptom-free intervals and chronic non-progressive headaches mostly occur in migraine, tension-type headache, cluster headaches, and the other episodic syndromes (cyclic vomiting, abdominal migraine, benign paroxysmal vertigo, benign paroxysmal torticollis). Seizures should also be considered if headaches precede stereotypical repetitive movements and/or impaired awareness.
Examination of a young child can be difficult, but care should be taken to ensure a complete neurologic examination is carried out regardless. This includes a thorough assessment of the child's level of consciousness, cognition, head circumference, cranial nerves (including the optic discs via fundoscopy), gait, and coordination. A relevant systemic review of other systems of the body may also be indicated depending on the presenting symptoms. Encephalopathy, meningeal signs, papilledema, speech abnormalities, focal deficits and ataxia all point to serious conditions, as are altered vital parameters [Table 5]. Conversely, a normal neurological examination has been shown to highly correlate with the absence of relevant intracranial processes.,
While history and physical examination have been shown to have good sensitivity in determining etiology, there are some caveats to bear in mind. In younger children who may not cooperate so readily, signs can be easily missed. Also, the accuracy of the assessment is dependent on the clinician and will inevitably be influenced by their experience and acumen. In specific areas like fundoscopy, a junior doctor may not be able to pick up papilledema and should seek the assistance of a more experienced physician when in doubt. Lastly, symptoms and signs can fluctuate over time and may not be present at the initial ED visit. Thus, when in doubt, follow up re-evaluations or an overnight admission should be scheduled for serial examination and monitoring.
A CT brain is indicated if the history and physical examination point to an underlying secondary cause of headache. It is quick and can diagnose intracranial bleeds and tumors, which will require emergent management. Otherwise, magnetic resonance imaging (MRI) of the brain remains the imaging modality of choice due to the potential risk of ionizing radiation in this population of patients. It is, however, not as readily available in the ED and if indicated, admission to the hospital or referral to the outpatient clinic should be made. In general, routine neuroimaging is not indicated if the history of headaches is long-standing with features consistent with a primary headache and normal neurological exam., Unwarranted imaging may lead to incidental benign abnormalities which may in turn, cause undue anxiety. A retrospective study found that about 20% of pediatric headache patients with neuroimaging had benign abnormalities which did not result in a change of management, and only 1.2% of them had abnormal imaging with a normal examination. The most common benign findings are sinus disease, Chiari I malformations, and non-specific white matter abnormalities.
However, there are caregivers and/or patients who are less likely to be reassured by clinical assessment alone. Some may cite a family history of malignancy as a source of concern. In fretful young patients, the neurological examination may also be limited. In these selected cases, neuroimaging may be offered with adequate counseling. A randomized controlled trial studying the role of MRI brain scan in children to provide reassurance showed that patients were temporarily assured, and more importantly, it reduced service cost as they were less likely to seek repeat consultations.
Aside from MRI brain imaging, other diagnostic options such as blood investigations and CSF studies can be considered in the pediatric patient depending on the suspected etiology. Most are not required if the neurological examination is normal. Electroencephalography usually has no role in the work-up of headaches. Lumbar puncture is diagnostic if meningitis or encephalitis is suspected, or in cases of idiopathic intracranial hypertension.
In the recent update by the American Academy of Neurology (AAN) and the American Headache Society (AHS) on the acute treatment of migraine in children and adolescents, ibuprofen oral solution (OS) (10 mg/kg) is recommended as the initial option to reduce pain in children and adolescents with migraine. For adolescents with migraine, the recommended options include sumatriptan/naproxen oral tablet (OT) (10/60, 30/180, 85/500 mg), zolmitriptan nasal spray (NS) (5 mg), sumatriptan NS (20 mg), rizatriptan oral disintegrating tablet (ODT) (5 or 10 mg), or almotriptan OT (6.25 or 12.5 mg). If migraine is incompletely responsive to a triptan, ibuprofen or naproxen may be added to provide greater relief. The treatment effects for other migraine-associated symptoms (nausea, vomiting, photophobia, phonophobia) were less pronounced. Zolmitriptan NS and sumatriptan/naproxen have demonstrated some efficacy for photophobia and phonophobia, but none of the agents were helpful for nausea or vomiting. Antiemetics that are often used to treat nausea and vomiting in other pediatric conditions may be of benefit.
Parenteral dopaminergic antagonists (prochlorperazine, promethazine, metoclopramide) were evaluated in the treatment of pediatric migraine in a small retrospective cohort study. Among the three agents, promethazine was associated with a higher chance of treatment failure, with poorer pain control and the need for opioid administration. The authors proposed using prochlorperazine or metoclopramide, instead of promethazine for pediatric migraine treatment in the ED setting. Intravenous valproic acid and intravenous dihydroergotamine have also been explored for use in ED acute migraine treatment in retrospective cohort studies but the evidence is inconclusive.
Protocols have been advocated by the Emergency Nurses Associated (ENA) and American College of Emergency Physicians (ACEP) to promote patient-centric practice, reduce care variation and facilitate the adoption of evidence-based practice. Standardized headache protocols that include order sets, procedures, and standing orders can be considered to improve overall coordination of care.
Headache may recur after ED discharge, and in one study up to 64% of patients have reported return of their migraine headache. Once patients are assessed to be fit for discharge from ED, adequate and treatment-specific analgesia should be prescribed for patients as an interim care till they return for an outpatient neurology review. Setting up collaborative arrangement to allow for rapid referral to neurologist or headache clinic can also achieve early definitive diagnosis and appropriate treatment.
In pediatric and adult headache patients alike, non-pharmacological measures are an essential part of long-term management. Measures such as good sleeping habits, adequate hydration, regular meals, and physical activity should be emphasized., Brief explanation on these strategies and reassurance may empower patients and caregivers even in the ED setting.
In the ED, it is paramount that focus is placed on identifying key factors that would distinguish the common headache disorders from the life-threatening ones. A comprehensive history and directed clinic-neurological examination are most sensitive in determining the underlying etiology. This would better streamline the process of evaluation, and determine astutely the need for further diagnostic tests, which would facilitate the formulation of appropriate management plans for dedicated headache diagnosis. Utilizing effective pharmacological treatment that is evidence-based would provide better symptom control and help reduce unnecessary ED revisits. Finally, establishing collaborative networks between ED and headache clinics would improve care transition and optimize patient management.
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Conflicts of interest
There are no conflicts of interest.
[Table 1], [Table 2], [Table 3], [Table 4], [Table 5]