Chief Complaint Review
Headache and Facial Pain: Differential Diagnosis and Treatment Jonathan A. Bernstein, MDa, Roger W. Fox, MDb, Vincent T. Martin, MDc,d, and Richard F. Lockey, MDe Cincinnati, Ohio; Tampa, Fla; and Mount Royal, NJ Headaches affect 90% of the population sometime during their life. Most are benign and fleeting, some are serious and lifethreatening, and others require ongoing medical consultation and treatment. A careful history and physical is necessary to establish a differential diagnosis and to guide the choice of testing to make an accurate diagnosis. The most common types of headaches are discussed in this review. They are divided into primary and secondary headache disorders as classified by the International Headache Society. Primary headache disorders include migraine without and with aura, cluster and tension-type headaches. Secondary headaches are those that occur as a result of some other disorder and include brain tumors, rhinosinusitis, diseases of intracranial and extracranial vasculature, and temporomandibular joint disease. Ó 2013 American Academy a
Professor of Medicine, Director of Clinical Research, Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio b Professor of Medicine, Pediatrics and Public Health, Division of Allergy and Immunology, Department of Internal Medicine, University of South Florida Morsani College of Medicine, Tampa, Fla c Professor of Medicine, Department of Internal Medicine, Division of General Internal Medicine, University of Cincinnati, Cincinnati, Ohio d Associate Editor for Headache, American Headache Society, Mount Royal, NJ e Distinguished University Health Professor, Professor of Medicine, Pediatrics and Public Health, Director, Division of Allergy and Immunology, Joy McCann Culverhouse Chair in Allergy and Immunology, Morsani University of South Florida College of Medicine, Tampa, Fla No funding was received for this work. Conflicts of interest: J. A. Bernstein is on the AAAAI Board of Directors and the AFI board; has received consultancy fees from Dyax, Shire, CSL-Behring, and ViroPharma; is employed by the Bernstein Allergy Group and Bernstein Clinical Research; has received research support from Boehringer Ingelheim, Forrest, Viropharma, CSL Behring, Dyax, Shire, Pharming, and Novartis; has received lecture fees from Shire, Teva, Dyax, ViroPharma, and CSL Behring; has received payment for developing educational presentations from ViroPharma, Shire, and Medscape; and is Editor in Chief of the Journal of Asthma. R. W. Fox declares he has no relevant conflicts. V. T. Martin has received consultancy fees from Allergan, has received research support from GlaxoSmithKline, and has received lecture fees from Allergan, Zogenix. R. F. Lockey is on the World Allergy Organization Board; has received consultancy fees from Merck and ALK-Abelló; is employed by the University of South Florida and the VA Hospital; has provided expert testimony on medicolegal cases for Shook, Hardy & Bacon and Chamberlain & McHaney; has received research support from ALA Pharmaceuticals; has received lecture fees from Merck and AstraZeneca; receives royalties from Informa; and has received travel support from the World Allergy Organization. Received for publication February 12, 2013; revised March 7, 2013; accepted for publication March 8, 2013. Cite this article as: Bernstein JA, Fox RW, Martin VT, Lockey RF. Headache and facial pain: Differential diagnosis and treatment. J Allergy Clin Immunol: In Practice 2013;1:242-51. http://dx.doi.org/10.1016/j.jaip.2013.03.014. Corresponding author: Richard F. Lockey, MD, University of South Florida Morsani College of Medicine, c/o James A. Haley Veterans’ Hospital, 13000 Bruce B. Downs Blvd (111D), Tampa, FL 33612. E-mail: [email protected]. 2213-2198/$36.00 Ó 2013 American Academy of Allergy, Asthma & Immunology http://dx.doi.org/10.1016/j.jaip.2013.03.014
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of Allergy, Asthma & Immunology (J Allergy Clin Immunol: In Practice 2013;1:242-51) Key words: Migraine headache; Tension-type headache; Rhinosinusitis; Rhinitis; Sinusitis; Temporomandibular joint; Diagnosis; Treatment; Primary headache; Secondary headache
PRIMARY HEADACHE DISORDERS Primary headaches refer to headaches without an anatomical or physiological explanation. Although their cause is unknown, they involve the neurovascular system of the brain. Such headaches include migraine without or with an aura and tension and cluster headaches. Patients with primary headache disorders often present to the allergist/immunologist to rule out an allergic cause for their headache and for possible treatment. Patients also present for evaluation and treatment of rhinitis and/or rhinosinusitis, and during their history it is revealed that they also have a primary headache disorder. Therefore, it behooves the allergist to be knowledgeable about the evaluation, diagnosis, and treatment of primary headache disorders. Migraine headache Migraine is a chronic neurologic disorder characterized by recurrent moderate-to-severe headaches often accompanied by associated symptoms such as nausea, vomiting, photophobia, and phonophobia.1 It is one of the most frequent neurologic disorders, affecting up to 12.6% of the population (6% men, 18% women) and costs billions of dollars in direct medical costs as well as indirect costs such as lost productivity at work because of absenteeism.2 More than 30 million Americans, 75% of whom are women, are affected by this kind of headache.3 The World Health Organization considers migraine to be 1 of the top 20 most disabling diseases worldwide.4 Migraines can be subclassified into those with episodic and chronic types, primarily differentiated by frequency. Episodic migraine is defined as having 50% of patients with migraine have affected family members.8 The International Headache Society (IHS) classifies headaches in The International Classification of Headache Disorders (www .ihs-headaches.org). Migraine with aura and migraine without aura are used for diagnosis and coding purposes, not terms such as common migraine or classic migraine. An aura is the complex
J ALLERGY CLIN IMMUNOL: IN PRACTICE VOLUME 1, NUMBER 3
Abbreviations used CRS- Chronic rhinosinusitis CT- Computed tomographic CTTH- Chronic tension-type headache ETTH- Episodic tension-type headache IHS- International Headache Society MOH- Medication overuse headache MRI- Magnetic resonance imaging NSAIDs- Nonsteroidal anti-inflammatory drugs TMJ- Temporomandibular joint TTH- Tension-type headache
of focal neurologic symptoms that initiate or accompany migraine attacks. Seventy percent to 85% of migraineurs have attacks without an aura, some have both, and 15% to 30% experience an aura.9
Migraine without aura. Migraine without aura is an idiopathic, recurring headache disorder that manifests with attacks lasting 4 to 72 hours (Table I). Typical characteristics of this headache include a unilateral and an occasion bilateral headache with a pulsatile quality, of moderate-to-severe intensity, aggravated by routine physical activity, and associated with nausea and photophobia and phonophobia. The prodrome or premonitory phase, which can occur hours to several days before a migraine, with or without an aura, occurs in 60% of patients.10 It may consist of hyperactivity, hypoactivity, depression, craving for certain foods, repetitive yawning, and other atypical symptoms, such as sensitivity to certain smells and stiff muscles, often of the neck. Common triggers include stress, fasting, sleep disturbances, menstrual periods, weather, alcohol, and foods that contain monosodium glutamate, tyramine, and nitrates.11 The mechanism for the headache phase of migraine is poorly understood, but it likely involves release of calcitonin generelated peptide from C fibers of the trigeminal nerve and sensitization of primary and secondary trigeminal afferents as the migraine progresses. Other theories include enhanced excitability of cortical neurons that play a role in the aura symptoms and dysmodulation of inhibitory neurons within the brain stem, leading to the initiation of a migraine attack1 (Figure 1). Migraine with aura. Migraine with aura is an idiopathic, recurrent disorder associated with neurologic symptoms that are localized to the cerebral cortex or brain stem (Table I). The aura gradually develops over 5 to 20 minutes and last fewer than 60 minutes. Headache, nausea, or phonophobia or photophobia usually follow the neurologic aura symptoms or after an asymptomatic period of less than an hour. The headache usually lasts 4 to 72 hours, but it may not even occur after an aura. There are three forms of aura: visual, sensory, and motor auras.12 A visual aura is most common and is often described as flashing light or zigzag lines that surround a central scotoma (eg, blind spot). A sensory aura is the next most common aura and presents with paresthesias or numbness on one side of an upper or lower extremity. The third most common aura is a motor aura that presents with motor weakness of the upper and lower extremities or a dysphasic speech disturbance. The differential diagnoses in patients presenting with possible migraine include temporal arteritis, acute glaucoma, meningitis, transient ischemic attacks, and subarachnoid hemorrhage.13
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If a patient has one or more “red flags” for a secondary headache disorder (see “Secondary Headaches”), then the physician is obligated to do a workup that should include either a magnetic resonance imaging (MRI) or computed tomographic (CT) scan of the head to rule out other structural conditions. Cluster and tension headaches in the differential diagnosis for migraine headaches are discussed below. Migraine treatment involves avoidance of known triggers, lifestyle modifications, and pharmacologic treatment and/or preventative therapies. Recommendations for treatment of an acute migraine attack include nonsteroidal anti-inflammatory drugs (NSAIDs); opiates; barbiturates; ergotamine or its derivative, dihydroergotamine; the combination of dichloralphenazone, isometheptene, and acetaminophen; or one of seven triptans.14 Treatment for nausea is helpful in some cases. The triptans include almotriptan, eletriptan, frovatriptan, naratriptan, rizatriptan, sumatriptan, and zolmitriptan. Rare side effects such as paresthesias, a warm feeling in the neck and chest, flushing, and dizziness may occur. Triptans are contraindicated in patients with coronary artery disease. A triptan, which is essentially a modified serotonin molecule, stimulates the neurotransmitter receptors for 5-hydroxytryptamine (serotonin) located on the trigeminal nerve and trigeminal nucleus caudalis, and thereby reduces neuronal signaling of pain and inhibits the release of calcitonin gene-related peptide. All triptans are equally effective, but unique characteristics of one agent over another seem to benefit some patients more than others.15 Indications for preventative or prophylactic treatment include chronic, frequent recurring migraines (eg, >3 to 5 days per month with migraine), migraines not responding to acute treatment, and migraines preceded by complex auras with hemiplegia, hemisensory loss, or dysarthria. Medications recommended include amitriptyline, diavaproex sodium, sodium valproate, topiramate, propanaolol, timolol, and frovatriptan, the latter for menstrual-related migraines.16 In addition, minerals, vitamin, and herbs are also used as preventatives but are not scientifically documented and include magnesium, riboflavin, coenzyme Q-10, and petasites. Injections of onabotu linum toxin A have shown efficacy in patients with chronic migraine.17
Cluster headaches Cluster headaches are severe, excruciating, unilateral headaches occurring in the orbital or supraorbital and/or temporal areas that last 15 to 180 minutes and occur every other day up to eight times a day. Conjunctiva injection, lacrimation, nasal congestion, rhinorrhea, forehead and facial sweating, myosis, ptosis, and/or eyelid edema may be associated with cluster headaches. Attacks occur in series that last for weeks or months, separated by remission for months or years. Such headaches are rare, with approximately 10% of patients with chronic or recurrent headaches manifesting cluster headaches. No prodrome or aura is associated with cluster headaches. Alcohol, red wine, and nitroglycerin can provoke these headaches during symptomatic periods or in patients with chronic cluster headaches. Pain usually recurs on the same side of the head and is excruciating during the worst attacks. The age of onset is typically in the 20- to 40-year old man who is five to six times more often affected than women.7 These headaches are medically difficult to manage and usually require pain management by a neurologist or headache specialist. Preventative treatments include verapamil, ergots, lithium, and topiramate.
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TABLE I. Diagnostic criteria for migraine without and with aura and tension type headaches* Migraine without aura
Migraine with aura
At least five attacks before seeking medical care
At least two attacks with characteristics of headache lasting 4-72 h
Headache lasting 4-72 h, when untreated or unsuccessfully treated
At least three of the following four characteristics: 1. One or more fully reversible aura symptoms indicating focal cerebral cortical and/or brain stem dysfunction. 2. At least one aura symptom gradually developing over more than 4 min or two or more symptoms occurring in succession. 3. No aura symptoms lasting >60 min. If more than one aura symptom is present, the accepted duration is proportionally increased. 4. Headache after an aura with a free interval of
Headache and Facial Pain: Differential Diagnosis and Treatment Jonathan A. Bernstein, MDa, Roger W. Fox, MDb, Vincent T. Martin, MDc,d, and Richard F. Lockey, MDe Cincinnati, Ohio; Tampa, Fla; and Mount Royal, NJ Headaches affect 90% of the population sometime during their life. Most are benign and fleeting, some are serious and lifethreatening, and others require ongoing medical consultation and treatment. A careful history and physical is necessary to establish a differential diagnosis and to guide the choice of testing to make an accurate diagnosis. The most common types of headaches are discussed in this review. They are divided into primary and secondary headache disorders as classified by the International Headache Society. Primary headache disorders include migraine without and with aura, cluster and tension-type headaches. Secondary headaches are those that occur as a result of some other disorder and include brain tumors, rhinosinusitis, diseases of intracranial and extracranial vasculature, and temporomandibular joint disease. Ó 2013 American Academy a
Professor of Medicine, Director of Clinical Research, Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio b Professor of Medicine, Pediatrics and Public Health, Division of Allergy and Immunology, Department of Internal Medicine, University of South Florida Morsani College of Medicine, Tampa, Fla c Professor of Medicine, Department of Internal Medicine, Division of General Internal Medicine, University of Cincinnati, Cincinnati, Ohio d Associate Editor for Headache, American Headache Society, Mount Royal, NJ e Distinguished University Health Professor, Professor of Medicine, Pediatrics and Public Health, Director, Division of Allergy and Immunology, Joy McCann Culverhouse Chair in Allergy and Immunology, Morsani University of South Florida College of Medicine, Tampa, Fla No funding was received for this work. Conflicts of interest: J. A. Bernstein is on the AAAAI Board of Directors and the AFI board; has received consultancy fees from Dyax, Shire, CSL-Behring, and ViroPharma; is employed by the Bernstein Allergy Group and Bernstein Clinical Research; has received research support from Boehringer Ingelheim, Forrest, Viropharma, CSL Behring, Dyax, Shire, Pharming, and Novartis; has received lecture fees from Shire, Teva, Dyax, ViroPharma, and CSL Behring; has received payment for developing educational presentations from ViroPharma, Shire, and Medscape; and is Editor in Chief of the Journal of Asthma. R. W. Fox declares he has no relevant conflicts. V. T. Martin has received consultancy fees from Allergan, has received research support from GlaxoSmithKline, and has received lecture fees from Allergan, Zogenix. R. F. Lockey is on the World Allergy Organization Board; has received consultancy fees from Merck and ALK-Abelló; is employed by the University of South Florida and the VA Hospital; has provided expert testimony on medicolegal cases for Shook, Hardy & Bacon and Chamberlain & McHaney; has received research support from ALA Pharmaceuticals; has received lecture fees from Merck and AstraZeneca; receives royalties from Informa; and has received travel support from the World Allergy Organization. Received for publication February 12, 2013; revised March 7, 2013; accepted for publication March 8, 2013. Cite this article as: Bernstein JA, Fox RW, Martin VT, Lockey RF. Headache and facial pain: Differential diagnosis and treatment. J Allergy Clin Immunol: In Practice 2013;1:242-51. http://dx.doi.org/10.1016/j.jaip.2013.03.014. Corresponding author: Richard F. Lockey, MD, University of South Florida Morsani College of Medicine, c/o James A. Haley Veterans’ Hospital, 13000 Bruce B. Downs Blvd (111D), Tampa, FL 33612. E-mail: [email protected]. 2213-2198/$36.00 Ó 2013 American Academy of Allergy, Asthma & Immunology http://dx.doi.org/10.1016/j.jaip.2013.03.014
242
of Allergy, Asthma & Immunology (J Allergy Clin Immunol: In Practice 2013;1:242-51) Key words: Migraine headache; Tension-type headache; Rhinosinusitis; Rhinitis; Sinusitis; Temporomandibular joint; Diagnosis; Treatment; Primary headache; Secondary headache
PRIMARY HEADACHE DISORDERS Primary headaches refer to headaches without an anatomical or physiological explanation. Although their cause is unknown, they involve the neurovascular system of the brain. Such headaches include migraine without or with an aura and tension and cluster headaches. Patients with primary headache disorders often present to the allergist/immunologist to rule out an allergic cause for their headache and for possible treatment. Patients also present for evaluation and treatment of rhinitis and/or rhinosinusitis, and during their history it is revealed that they also have a primary headache disorder. Therefore, it behooves the allergist to be knowledgeable about the evaluation, diagnosis, and treatment of primary headache disorders. Migraine headache Migraine is a chronic neurologic disorder characterized by recurrent moderate-to-severe headaches often accompanied by associated symptoms such as nausea, vomiting, photophobia, and phonophobia.1 It is one of the most frequent neurologic disorders, affecting up to 12.6% of the population (6% men, 18% women) and costs billions of dollars in direct medical costs as well as indirect costs such as lost productivity at work because of absenteeism.2 More than 30 million Americans, 75% of whom are women, are affected by this kind of headache.3 The World Health Organization considers migraine to be 1 of the top 20 most disabling diseases worldwide.4 Migraines can be subclassified into those with episodic and chronic types, primarily differentiated by frequency. Episodic migraine is defined as having 50% of patients with migraine have affected family members.8 The International Headache Society (IHS) classifies headaches in The International Classification of Headache Disorders (www .ihs-headaches.org). Migraine with aura and migraine without aura are used for diagnosis and coding purposes, not terms such as common migraine or classic migraine. An aura is the complex
J ALLERGY CLIN IMMUNOL: IN PRACTICE VOLUME 1, NUMBER 3
Abbreviations used CRS- Chronic rhinosinusitis CT- Computed tomographic CTTH- Chronic tension-type headache ETTH- Episodic tension-type headache IHS- International Headache Society MOH- Medication overuse headache MRI- Magnetic resonance imaging NSAIDs- Nonsteroidal anti-inflammatory drugs TMJ- Temporomandibular joint TTH- Tension-type headache
of focal neurologic symptoms that initiate or accompany migraine attacks. Seventy percent to 85% of migraineurs have attacks without an aura, some have both, and 15% to 30% experience an aura.9
Migraine without aura. Migraine without aura is an idiopathic, recurring headache disorder that manifests with attacks lasting 4 to 72 hours (Table I). Typical characteristics of this headache include a unilateral and an occasion bilateral headache with a pulsatile quality, of moderate-to-severe intensity, aggravated by routine physical activity, and associated with nausea and photophobia and phonophobia. The prodrome or premonitory phase, which can occur hours to several days before a migraine, with or without an aura, occurs in 60% of patients.10 It may consist of hyperactivity, hypoactivity, depression, craving for certain foods, repetitive yawning, and other atypical symptoms, such as sensitivity to certain smells and stiff muscles, often of the neck. Common triggers include stress, fasting, sleep disturbances, menstrual periods, weather, alcohol, and foods that contain monosodium glutamate, tyramine, and nitrates.11 The mechanism for the headache phase of migraine is poorly understood, but it likely involves release of calcitonin generelated peptide from C fibers of the trigeminal nerve and sensitization of primary and secondary trigeminal afferents as the migraine progresses. Other theories include enhanced excitability of cortical neurons that play a role in the aura symptoms and dysmodulation of inhibitory neurons within the brain stem, leading to the initiation of a migraine attack1 (Figure 1). Migraine with aura. Migraine with aura is an idiopathic, recurrent disorder associated with neurologic symptoms that are localized to the cerebral cortex or brain stem (Table I). The aura gradually develops over 5 to 20 minutes and last fewer than 60 minutes. Headache, nausea, or phonophobia or photophobia usually follow the neurologic aura symptoms or after an asymptomatic period of less than an hour. The headache usually lasts 4 to 72 hours, but it may not even occur after an aura. There are three forms of aura: visual, sensory, and motor auras.12 A visual aura is most common and is often described as flashing light or zigzag lines that surround a central scotoma (eg, blind spot). A sensory aura is the next most common aura and presents with paresthesias or numbness on one side of an upper or lower extremity. The third most common aura is a motor aura that presents with motor weakness of the upper and lower extremities or a dysphasic speech disturbance. The differential diagnoses in patients presenting with possible migraine include temporal arteritis, acute glaucoma, meningitis, transient ischemic attacks, and subarachnoid hemorrhage.13
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If a patient has one or more “red flags” for a secondary headache disorder (see “Secondary Headaches”), then the physician is obligated to do a workup that should include either a magnetic resonance imaging (MRI) or computed tomographic (CT) scan of the head to rule out other structural conditions. Cluster and tension headaches in the differential diagnosis for migraine headaches are discussed below. Migraine treatment involves avoidance of known triggers, lifestyle modifications, and pharmacologic treatment and/or preventative therapies. Recommendations for treatment of an acute migraine attack include nonsteroidal anti-inflammatory drugs (NSAIDs); opiates; barbiturates; ergotamine or its derivative, dihydroergotamine; the combination of dichloralphenazone, isometheptene, and acetaminophen; or one of seven triptans.14 Treatment for nausea is helpful in some cases. The triptans include almotriptan, eletriptan, frovatriptan, naratriptan, rizatriptan, sumatriptan, and zolmitriptan. Rare side effects such as paresthesias, a warm feeling in the neck and chest, flushing, and dizziness may occur. Triptans are contraindicated in patients with coronary artery disease. A triptan, which is essentially a modified serotonin molecule, stimulates the neurotransmitter receptors for 5-hydroxytryptamine (serotonin) located on the trigeminal nerve and trigeminal nucleus caudalis, and thereby reduces neuronal signaling of pain and inhibits the release of calcitonin gene-related peptide. All triptans are equally effective, but unique characteristics of one agent over another seem to benefit some patients more than others.15 Indications for preventative or prophylactic treatment include chronic, frequent recurring migraines (eg, >3 to 5 days per month with migraine), migraines not responding to acute treatment, and migraines preceded by complex auras with hemiplegia, hemisensory loss, or dysarthria. Medications recommended include amitriptyline, diavaproex sodium, sodium valproate, topiramate, propanaolol, timolol, and frovatriptan, the latter for menstrual-related migraines.16 In addition, minerals, vitamin, and herbs are also used as preventatives but are not scientifically documented and include magnesium, riboflavin, coenzyme Q-10, and petasites. Injections of onabotu linum toxin A have shown efficacy in patients with chronic migraine.17
Cluster headaches Cluster headaches are severe, excruciating, unilateral headaches occurring in the orbital or supraorbital and/or temporal areas that last 15 to 180 minutes and occur every other day up to eight times a day. Conjunctiva injection, lacrimation, nasal congestion, rhinorrhea, forehead and facial sweating, myosis, ptosis, and/or eyelid edema may be associated with cluster headaches. Attacks occur in series that last for weeks or months, separated by remission for months or years. Such headaches are rare, with approximately 10% of patients with chronic or recurrent headaches manifesting cluster headaches. No prodrome or aura is associated with cluster headaches. Alcohol, red wine, and nitroglycerin can provoke these headaches during symptomatic periods or in patients with chronic cluster headaches. Pain usually recurs on the same side of the head and is excruciating during the worst attacks. The age of onset is typically in the 20- to 40-year old man who is five to six times more often affected than women.7 These headaches are medically difficult to manage and usually require pain management by a neurologist or headache specialist. Preventative treatments include verapamil, ergots, lithium, and topiramate.
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J ALLERGY CLIN IMMUNOL: IN PRACTICE MAY/JUNE 2013
TABLE I. Diagnostic criteria for migraine without and with aura and tension type headaches* Migraine without aura
Migraine with aura
At least five attacks before seeking medical care
At least two attacks with characteristics of headache lasting 4-72 h
Headache lasting 4-72 h, when untreated or unsuccessfully treated
At least three of the following four characteristics: 1. One or more fully reversible aura symptoms indicating focal cerebral cortical and/or brain stem dysfunction. 2. At least one aura symptom gradually developing over more than 4 min or two or more symptoms occurring in succession. 3. No aura symptoms lasting >60 min. If more than one aura symptom is present, the accepted duration is proportionally increased. 4. Headache after an aura with a free interval of
