Vascular and Nonvascular Intracranial Causes of Orofacial Pain










Key Points
T
his chapter reviews both vascular and nonvascular intracranial
sources of orofacial pain, many of which may be life-threatening
or associated with signicant morbidity if left untreated
(Box 4-1). The codes from International Classication of Headache
A systematic approach to patient history and clinical ex-
amination is critical to identifying patients with secondary
etiologies of headache.
Intracranial causes of head, neck, and orofacial pain are
numerous and may lead to disability or death if not man-
aged promptly.
Rapid neurologic referral is warranted if patients present
with neurologic symptoms and ndings.
Diagnoses of intracranial causes of head, neck, and oro-
facial pain rest primarily on a careful history, a healthy re-
spect for red ags, and a willingness to rapidly evaluate
further.
The SNOOP4 acronym is a useful mnemonic device for
screening each patient.
Vascular and
Nonvascular
Intracranial
Causes of
Orofacial Pain
4

62
Vascular and Nonvascular Intracranial Causes of Orofacial Pain
4
Disorders, third edition (beta version) (ICHD)
1
are provided for each disorder.
Preliminary Investigation
Although only a minority (approximately 1%)
of patients presenting to the clinic with a
headache will ultimately be found to have a
signicant abnormality on neuroimaging, the
consequences of a missed diagnosis can be
catastrophic. In the emergency room setting,
the probability of a signicant abnormality on
neuroimaging increases to up to 8%. Predic-
tive factors include acute-onset headache,
age above 50 years, and an abnormal neuro-
logic examination.
2
Though neuroimaging is
an important diagnostic tool, many secondary
headache syndromes may be overlooked with
neuroimaging alone, including giant cell arteri-
tis (GCA), idiopathic intracranial hypertension,
and cardiac cephalalgia. The decision to treat
or investigate begins with a careful history and
physical examination, which can be aided by
the mnemonic SNOOP4 to screen for red-ag
features
3
(Box 4-2). Because many intracranial
structures are insensitive to pain (Box 4-3), in-
tracranial pathologic processes must be diag-
nosed according to other concomitant symp-
toms or historic facts.
4
Systemic symptoms or disease
Pain in addition to systemic features such as
fever, weight loss, arthralgia, stiff neck, or rash
could indicate meningoencephalitis, bactere-
mia/sepsis, vasculitis (eg, GCA), or neoplastic
processes. Preexisting risk factors, such as
human immunodeciency virus (HIV), cancer,
or chronic treatment with immunotherapy pre-
dispose patients to systemic disease. HIV is
associated with both intracranial infections and
cancer.
Neurologic signs or symptoms
Neurologic signs or symptoms, ranging from
mild confusion or sedation to frank neurologic
decits such as aphasia or hemiparesis, typi-
cally indicate more than a primary pain disor-
der. At times, migraine attacks can be accom-
panied by neurologic changes such as aphasia,
hemisensory symptoms, visual eld defects,
and/or hemiparesis (ie, aura), but a diagnosis
rst requires exclusion of secondary causes.
For example, the temporal prole of aura is
typically gradual and slowly progressive and is
associated with positive features (eg, scintil-
lating scotoma), whereas stroke is associated
with an abrupt, static onset of negative fea-
tures (eg, vision loss). Focal neurologic signs
and symptoms such as hemiparesis, visual
obscuration, diplopia, dizziness/vertigo, imbal-
ance, or numbness warrant further investiga-
tion to rule out vascular, infectious, inamma-
tory, and neoplastic disease of the brain and/
or meninges. Patients may ultimately be diag-
nosed with a primary headache disorder such
as migraine with aura or an uncommon vari-
ant such as migraine with brainstem aura or
hemiplegic migraine.
Onset sudden (thunderclap)
A sudden-onset, severe, and generalized or lo-
calized headache must be considered serious
until proven otherwise. The differential diagno-
sis for life-threatening acute-onset headaches
includes (1) vascular disorders such as sub-
arachnoid hemorrhage (SAH), cerebral infarc-
tion, cerebral venous thrombosis, and cervical
artery dissection and (2) nonvascular disorders
such as pituitary apoplexy and central nervous
system infections. To rule out SAH in a patient
presenting with sudden-onset headache, the
American College of Emergency Physicians
recommends a diagnostic approach that com-
prises the use of a computed tomography
(CT) image of the head followed by a lumbar
puncture.
5
Other testing, such as noninvasive

63
Box 4-1 Life-threatening secondary
causes of orofacial pain
Vascular disorders
Ischemic cerebrovascular disease
(ICHD 6.1)
Traumatic intracranial hemorrhage
(ICHD 5.5)
Nontraumatic intracranial
hemorrhage (ICHD 6.2)
Unruptured vascular malformation
(ICHD 6.3)
Arteritis (ICHD 6.4)
Carotid or vertebral artery pain
(ICHD 6.5)
Venous thrombosis (ICHD 6.6)
Nonvascular disorders
High cerebrospinal fluid pressure
(ICHD 7.1)
Low cerebrospinal fluid pressure
(ICHD 7.2)
Intracranial noninfectious
inflammation (ICHD 7.3)
Intracranial neoplasm (ICHD 7.4)
Intracranial infection (ICHD 9.1)
Box 4-3 Structures sensitive or insensitive to pain
Sensitive to pain
Intracranial
Dura mater
Venous sinuses and their
tributaries
Intracranial arteries (proximal
portions)
Neural structures:
–Trigeminal nerve (V)
–Facial nerve (VII)
–Glossopharyngeal nerve (IX)
–Vagus nerve (X)
–Upper cervical nerves
Extracranial
Carotid, vertebral, and
basilar arteries
Blood vessels within the
scalp and skin
Skin
Mucosa
Muscles
Fascia
Synovium within the TMJ
Teeth
Periosteum
Insensitive to pain
Intracranial
Brain parenchyma
Pia mater
Arachnoid membrane
Ependyma
Choroid plexus
Extracranial
Skull
Cervical vertebrae
Box 4-2 SNOOP4: Acronym for signs and
symptoms of concern
3
Systemic symptoms or disease
Fever, weight loss, HIV, systemic
cancer
Neurologic signs or symptoms
Horner sign, confusion, clumsiness,
weakness, aphasia, visual problems
Onset sudden
Thunderclap
Onset after age 50 years
Vascular (temporal arteritis), tumor,
infection
Pattern change
Any new or changed headache pattern
or quality or increase in frequency or
intensity
Postural headache
Aggravation by either lying flat or
standing up
Papilledema
Precipitants
Valsalva maneuver (ie, cough, exertion)
Preliminary Investigation

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Key PointsThis chapter reviews both vascular and nonvascular intracranial sources of orofacial pain, many of which may be life-threatening or associated with signicant morbidity if left untreated (Box 4-1). The codes from International Classication of Headache ◊ A systematic approach to patient history and clinical ex-amination is critical to identifying patients with secondary etiologies of headache.◊ Intracranial causes of head, neck, and orofacial pain are numerous and may lead to disability or death if not man-aged promptly.◊ Rapid neurologic referral is warranted if patients present with neurologic symptoms and ndings.◊ Diagnoses of intracranial causes of head, neck, and oro-facial pain rest primarily on a careful history, a healthy re-spect for red ags, and a willingness to rapidly evaluate further. ◊ The SNOOP4 acronym is a useful mnemonic device for screening each patient. Vascular and Nonvascular Intracranial Causes of Orofacial Pain4 62Vascular and Nonvascular Intracranial Causes of Orofacial Pain4Disorders, third edition (beta version) (ICHD)1 are provided for each disorder.Preliminary InvestigationAlthough only a minority (approximately 1%) of patients presenting to the clinic with a headache will ultimately be found to have a signicant abnormality on neuroimaging, the consequences of a missed diagnosis can be catastrophic. In the emergency room setting, the probability of a signicant abnormality on neuroimaging increases to up to 8%. Predic-tive factors include acute-onset headache, age above 50 years, and an abnormal neuro-logic examination.2 Though neuroimaging is an important diagnostic tool, many secondary headache syndromes may be overlooked with neuroimaging alone, including giant cell arteri-tis (GCA), idiopathic intracranial hypertension, and cardiac cephalalgia. The decision to treat or investigate begins with a careful history and physical examination, which can be aided by the mnemonic SNOOP4 to screen for red-ag features3 (Box 4-2). Because many intracranial structures are insensitive to pain (Box 4-3), in-tracranial pathologic processes must be diag-nosed according to other concomitant symp-toms or historic facts.4Systemic symptoms or diseasePain in addition to systemic features such as fever, weight loss, arthralgia, stiff neck, or rash could indicate meningoencephalitis, bactere-mia/sepsis, vasculitis (eg, GCA), or neoplastic processes. Preexisting risk factors, such as human immunodeciency virus (HIV), cancer, or chronic treatment with immunotherapy pre-dispose patients to systemic disease. HIV is associated with both intracranial infections and cancer.Neurologic signs or symptomsNeurologic signs or symptoms, ranging from mild confusion or sedation to frank neurologic decits such as aphasia or hemiparesis, typi-cally indicate more than a primary pain disor-der. At times, migraine attacks can be accom-panied by neurologic changes such as aphasia, hemisensory symptoms, visual eld defects, and/or hemiparesis (ie, aura), but a diagnosis rst requires exclusion of secondary causes. For example, the temporal prole of aura is typically gradual and slowly progressive and is associated with positive features (eg, scintil-lating scotoma), whereas stroke is associated with an abrupt, static onset of negative fea-tures (eg, vision loss). Focal neurologic signs and symptoms such as hemiparesis, visual obscuration, diplopia, dizziness/vertigo, imbal-ance, or numbness warrant further investiga-tion to rule out vascular, infectious, inamma-tory, and neoplastic disease of the brain and/or meninges. Patients may ultimately be diag-nosed with a primary headache disorder such as migraine with aura or an uncommon vari-ant such as migraine with brainstem aura or hemiplegic migraine.Onset sudden (thunderclap)A sudden-onset, severe, and generalized or lo-calized headache must be considered serious until proven otherwise. The differential diagno-sis for life-threatening acute-onset headaches includes (1) vascular disorders such as sub-arachnoid hemorrhage (SAH), cerebral infarc-tion, cerebral venous thrombosis, and cervical artery dissection and (2) nonvascular disorders such as pituitary apoplexy and central nervous system infections. To rule out SAH in a patient presenting with sudden-onset headache, the American College of Emergency Physicians recommends a diagnostic approach that com-prises the use of a computed tomography (CT) image of the head followed by a lumbar puncture.5 Other testing, such as noninvasive 63Box 4-1 Life-threatening secondary causes of orofacial painVascular disorders• Ischemic cerebrovascular disease (ICHD 6.1)• Traumatic intracranial hemorrhage (ICHD 5.5)• Nontraumatic intracranial hemorrhage (ICHD 6.2)• Unruptured vascular malformation (ICHD 6.3)• Arteritis (ICHD 6.4)• Carotid or vertebral artery pain (ICHD 6.5)• Venous thrombosis (ICHD 6.6)Nonvascular disorders• High cerebrospinal fluid pressure (ICHD 7.1)• Low cerebrospinal fluid pressure (ICHD 7.2)• Intracranial noninfectious inflammation (ICHD 7.3)• Intracranial neoplasm (ICHD 7.4)• Intracranial infection (ICHD 9.1)Box 4-3 Structures sensitive or insensitive to painSensitive to painIntracranial• Dura mater• Venous sinuses and their tributaries• Intracranial arteries (proximal portions)• Neural structures:–Trigeminal nerve (V)–Facial nerve (VII)–Glossopharyngeal nerve (IX)–Vagus nerve (X)–Upper cervical nervesExtracranial• Carotid, vertebral, and basilar arteries• Blood vessels within the scalp and skin• Skin• Mucosa• Muscles• Fascia• Synovium within the TMJ• Teeth• PeriosteumInsensitive to painIntracranial• Brain parenchyma• Pia mater• Arachnoid membrane• Ependyma• Choroid plexusExtracranial• Skull• Cervical vertebraeBox 4-2 SNOOP4: Acronym for signs and symptoms of concern3Systemic symptoms or diseaseFever, weight loss, HIV, systemic cancerNeurologic signs or symptomsHorner sign, confusion, clumsiness, weakness, aphasia, visual problemsOnset suddenThunderclapOnset after age 50 yearsVascular (temporal arteritis), tumor, infectionPattern changeAny new or changed headache pattern or quality or increase in frequency or intensityPostural headacheAggravation by either lying flat or standing upPapilledemaPrecipitantsValsalva maneuver (ie, cough, exertion)Preliminary Investigation 64Vascular and Nonvascular Intracranial Causes of Orofacial Pain4angiography and magnetic resonance imaging (MRI) may be considered, noting the incidence of incidental intracranial aneurysm to be 3% of the general population.5 The differential diag-nosis of a thunderclap headache is summa-rized in Box 4-4. Onset after age 50 yearsIn patients older than 50 years, new-onset and/or progressive or changing headaches are worrisome and warrant evaluation. Migraines may begin at any age, but the vast majority of individuals will have onset by the time they are 30 years old. Headache in older patients with no prior history is inherently concerning and likely has a secondary cause. Contingent on the ndings from the history and physi-cal examination, the clinician should consider Box 4-4 Differential diagnosis of acute-onset secondary headache5Vascular• Subarachnoid hemorrhage (ICHD 6.2.2)• Saccular aneurysm (ICHD 6.3.1)• Arteriovenous malformation (ICHD 6.3.2)• Carotid or vertebral artery dissection (ICHD 6.5.1)• Cerebral venous thrombosis (ICHD 6.6)• Pituitary apoplexy (ICHD 6.7.4)Nonvascular• Acute hypertension (ICHD 7.1)• Benign intracranial hypertension (ICHD 7.1.1)• Intermittent hydrocephalus (ICHD 7.1.3)• Intracranial infection (ICHD 9.1)• Pheochromocytoma (ICHD 10.3.1)• Acute glaucoma (ICHD 11.3.1)• Acute mountain sickness (at altitude)• Acute optic neuritisan MRI study of the brain with and without contrast. In addition, hematologic tests may be necessary, such as erythrocyte sedimenta-tion rate (ESR) and C-reactive protein (CRP) to rule out GCA, complete blood count to rule out anemia and blood dyscrasia, free thyroxine and thyroid-stimulating hormone levels to rule out thyroid disease, and a Lyme titer to rule out Lyme disease. Pattern changeWhen patients experience their rst headache or the worst headache of their life, or if there is a change in attack frequency, severity, as-sociated symptoms, or quality, the clinician should review the case for other red ags and seriously consider further investigations. Any migraine can be considered the worst headache of a person’s life, but patients with migraine may also develop secondary head-aches. Chronic and recurrent headaches typi-cally present with similar features over time, but patients should be encouraged to note and report changes in headache pattern. Progres-sively worsening headaches at any age need to be carefully assessed.Postural headacheMigraines characteristically improve with lying down and are aggravated by routine physi-cal activity. If the headaches are precipitated by sitting upright or standing, with improve-ment on lying down, this can be a sign of a low-pressure headache due to a cerebrospi-nal uid (CSF) leak. In contrast, headaches on lying down can be caused by increased intracranial pressure related to an intracranial mass, cerebral venous thrombosis, meningeal process, or idiopathic intracranial hyperten-sion. Headaches presenting only on awaken-ing may be associated with obstructive sleep apnea, substance overuse or withdrawal, brain tumor, or oral parafunctional habits such as bruxism. Headaches that actually awaken 65a patient from his or her usual sleep pattern need to be investigated further to rule out in-creased intracranial pressure, although other disorders such as cluster headache, migraine, cervicogenic headache, and GCA should also be considered. PapilledemaPapilledema is one of the most important neurologic signs to identify in patients with headaches because it immediately directs the examiner toward evaluation of secondary causes. Papilledema indicates swelling of the optic nerve head, which can arise in the con-text of elevated intracranial pressure.PrecipitantsHeadaches precipitated by a cough, a sneeze, straining, or exertion, although possibly be-nign, warrant an MRI scan of the brain to ex-clude an intracranial structural lesion such as a tumor or Arnold-Chiari malformation. This clini-cal history should be carefully distinguished from headaches that are only worsened by a Valsalva maneuver, which are typically benign.Headache Attributed to Cranial or Cervical Vascular Disorder (ICHD 6)Ischemic stroke (ICHD 6.1; ICD-10 I63) or transient ischemic attack (ICHD 6.1; ICD-10 G45)Headache associated with acute ischemic cerebrovascular disease (ICHD 6.1) is well recognized but poorly understood.6,7 The eti-ology should be considered based on the ac-companying neurologic decits that present in an acute fashion, often in a person older than 50 years, and seem temporally related to the head pain. The headache is typically continuous with tension-type features and is only rarely thunderclap in progression.7 Posterior circu-lation dysfunction in the vertebral and basilar artery distribution is more likely than anterior circulation disease to cause headache. Neu-rologic decits reect the region of the brain affected and may include vision loss, diplopia, weakness, sensory loss, aphasia, and ataxia. Transient ischemic attacks present in a simi-lar fashion, but the decits resolve within 24 hours, frequently within 20 minutes. Signs or symptoms consistent with cerebral ischemia warrant referral to the emergency room.Headache secondary to ischemic stroke typically affects older individuals with risk fac-tors such as hypertension, atrial brillation, and tobacco use. However, stroke can occur at any age. Patients presenting with stroke should undergo a CT scan of the head to rule out an intracranial hemorrhage. An MRI study with diffusion-weighted imaging has a higher sensi-tivity and specicity for detection of acute ce-rebral ischemia and tissue damage. The differ-ential diagnosis for a patient with headache and neurologic symptoms must include migraine. However, an older migraineur who develops a headache with new neurologic signs or symp-toms should be considered to have vascular compromise until proven otherwise. Treatment for the headache must be individualized, but this pain typically lasts a few days at most and vasoactive drugs are contraindicated.7Traumatic and nontraumatic intracranial hemorrhageHeadache secondary to traumatic intracranial hematoma may be due to an epidural hema-toma (ICHD 5.5.1; ICD-10 S06.4) or a subdural hematoma (ICHD 5.5.2; ICD-10 S06.5). Head-ache secondary to nontraumatic intracranial hematoma may be due to an intracerebral hemorrhage (ICHD 6.2.1; ICD-10 I61) or an SAH (ICHD 6.2.2; ICD-10 I60).6 Similar to pain associated with ischemia, pain stemming from hemorrhage is best diagnosed according to its accompanying symptoms. Headache Attributed to Cranial or Cervical Vascular Disorder 66Vascular and Nonvascular Intracranial Causes of Orofacial Pain4Traumatic intracranial hematoma (ICHD 5.5, ICD-10 S06)An epidural hematoma (ICHD 5.5.1; ICD-10 S06.4) is most often caused by severe blunt trauma to the skull and rupture of the middle meningeal artery.6 A lucid interval may be ob-served wherein the patient appears to mostly recover after head trauma only to become somnolent and then comatose shortly there-after. This lucid interval is more the exception than the rule; more frequently, patients will continue to have severe pain followed by a change in cognition. Rapid surgical drainage of epidural hematomas is necessary to prevent mortality, necessitating a high index of suspi-cion after head trauma.A subdural hematoma (ICHD 5.5.2; ICD-10 S06.5) may present acutely, subacutely, or even with chronic symptoms.6 The subdural space lls with blood after the bridging veins coursing through it rupture, usually after a fall or other type of head trauma. Older patients and alcoholics are at greater risk because they are more likely to have gait instability and their bridging veins are not as resistant to trauma. There is often a history of minimal trauma, like a minor motor vehicle accident with-out direct head trauma in which neurologic symptoms begin several hours or even days later. Patients on anticoagulation therapy and those taking frequent aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs) are par-ticularly at risk, even with seemingly minimal head trauma. Pain is not always the chief com-plaint. Symptoms may include any neurologic decit, such as gait disturbance, personality change, somnolence, visual disturbances, or focal changes such as hemiparesis, hemisen-sory changes, and visual eld defects. Surgical evacuation of the hematoma may be neces-sary, but careful observation of a small subdu-ral hematoma without brain shift or pressure can be an effective treatment.Nontraumatic intracranial hemorrhage (ICHD 6.2, ICD-10 I62)Head pain associated with a nontraumatic in-tracerebral hemorrhage (ICHD 6.2.1; ICD-10 I61) most typically presents with acute neuro-logic decits.6 Intracerebral hematoma refers to a hematoma within the brain parenchyma and may be caused by many etiologies (eg, hypertension, neoplasm, arteriovenous mal-formations). If it is large enough, this hema-toma can extend into a ventricle. Of course, this may quickly lead to coma or even death contingent upon the cause and the volume of blood, which produces concomitant destruc-tion of cerebral tissue, mass effect, and pos-sibly herniation of the mesial temporal lobe, compressing the third cranial nerve. Headache due to an SAH (ICHD 6.2.2; ICD-10 I60) is fairly characteristic.5 Patients with an SAH typically present with a very sudden–onset thunderclap headache. This pattern of headache reaches its maximal intensity in less than 1 minute, often within seconds. Asking whether a patient was experiencing “the worst headache of their life” is not as useful as asking questions that get at the sudden nature of the onset with dramatic rise to peak pain within seconds. In a series of patients eventually di-agnosed with SAH, headache intensity reached its peak between 1 to 5 minutes in almost 20% of the cohort.8 SAHs commonly present with nausea and vomiting, a stiff neck, or rapid loss of consciousness. A ruptured saccular an-eurysm is the most common cause of a spon-taneous SAH. Whenever an SAH is suspected, the patient should be immediately sent to an emergency room, preferably by ambulance, where a detailed neurologic examination can be performed followed by a head CT scan. In the case of a thunderclap headache with a nor-mal head CT, a lumbar puncture is warranted to rule out microscopic evidence of bleeding or xanthochromia (which may take several hours to develop) as well as to rule out other worrisome etiologies of sudden headache, like 67meningitis. In addition, further testing such as MRI studies could be considered to evaluate for other potential etiologies.5 Key aspects of treatment include maintaining hemodynamic stability, neurosurgical consultation for either aneurysmal clipping or endovascular coiling, and treatment for avoidance of vasospasm.5 Unruptured vascular malformation (ICHD 6.3)Unruptured vascular malformations include saccular aneurysms (ICHD 6.3.1; ICD-10 Q28.3), arteriovenous malformations (ICHD 6.3.2; ICD-10 Q28.2), dural arteriovenous s-tulae (ICHD 6.3.3; ICD-10 I67.1), and venous and cavernous angiomas (ICHD 6.3.4; ICD-10 D18.0).6 These headaches may be silent until they rupture, or they may present with non-specic head pain. Patients can present at any age, and some may complain of pulsatile tin-nitus, though this is not always a sign of omi-nous pathology. A family history is often posi-tive. A magnetic resonance angiogram (MRA) or computed tomography angiography (CTA) may be helpful in the diagnostic process.Saccular aneurysms may be incidental and asymptomatic, and they are present in 3% of the population.5 Therefore, assigning causal-ity to these entities as a source for headache can risk inappropriate intervention. Screening with CTA or MRA should be offered to patients who have at least two family members with known aneurysm or prior SAH.9 Aneurysms that are small (< 7 mm) and in the anterior circulation are unlikely to rupture, whereas large, posterior circulation aneurysms involve a substantially greater risk.9 While patients may experience improvements in headache following repair, a subset of patients will have headaches that worsen following aneurysmal repair. This is especially likely in patients with severe headache and trait anxiety.10 Arteritis (ICHD 6.4, ICD-10 M31)Arteritis typically presents with systemic symp-toms and/or neurologic decits, but at times it may present initially with throbbing unilateral pain in the temple. The pain associated with GCA or temporal arteritis (ICHD 6.4.1; ICD-10 M31.6) is fairly distinct from that of other arte-ritides.11 GCA is an inammatory vasculitis that involves the temporal artery as well as others and may rapidly lead to blindness secondary to granulomatous occlusion of the carotid artery vasculature. GCA should be suspected and ruled out in all individuals, especially women over the age of 50 years who present with a new headache and associated visual changes and/or jaw claudication. Patients may complain of pain on combing their hair as a consequence of allodynia of the scalp. Patients with GCA may have polymyalgia rheumatica that causes pain, stiffness, and weakness in the neck, shoulders, and proximal arms and legs. On examination, they may have an enlarged, ten-der temporal artery. Laboratory investigations should include an ESR and CRP test. Temporal artery biopsy may often be required for diag-nosing GCA, but high doses of corticosteroids should be initiated as soon as possible, even prior to the biopsy, to avoid permanent loss of vision.11 An adequate biopsy sample is neces-sary for diagnosis due to the histopathologic occurrence of skip lesions, where pathologic changes may be interspersed with segments that appear normal.11 GCA can usually be dis-tinguished from temporomandibular disorders by palpation of enlarged, tender temporal ar-teries with reduced pulsatility, an abnormal fundoscopic examination, and an elevated ESR and CRP. The level of ESR and CRP values should always be interpreted in the context of the practitioner’s pretest probability, as GCA may even occur in the presence of normal sys-temic inammatory markers.11 Headache Attributed to Cranial or Cervical Vascular Disorder 68Vascular and Nonvascular Intracranial Causes of Orofacial Pain4Cervical carotid or vertebral artery disorder (ICHD 6.5)Carotid (ICD-10 I77.71) or vertebral artery dis-section (ICD-10 I77.74) presents with ipsilateral focal pain in the neck, face, or head, frequently associated with neurologic symptoms includ-ing transient ischemic attacks or stroke.6 The pain may be associated with pulsatile tinnitus or Horner syndrome because of the intimate association of sympathetic bers with the in-ternal carotid artery.12,13 This is an important cause of stroke in younger patients but can happen at any age. Patients may be able to re-call a specic activity that may have provoked a traumatic dissection like blunt trauma to the neck, riding a roller coaster, surng, chiroprac-tic manipulation, dancing, or anything that twists their neck. However, there is often no history of injury, suggesting a spontaneous dissection that could be associated with pre-ceding arterial damage, such as bromuscular dysplasia or even just a history of migraine. Blood dissects between the medial and subin-timal layers of an artery, which may then ll to form a pseudoaneurysm and cause ischemia by either partial occlusion of the lumen or em-bolic debris.Besides checking for new pupillary asym-metry, audible bruits should be sought in the neck or over the orbit or temporal bone, which may indicate the presence of turbulent blood ow. If dissection is suspected, patients should immediately be referred to either a neurologist or a neurosurgeon, who will likely investigate this further with extracranial duplex ultrasound vascular scanning, MRI, and MRA or CTA of the cervical vessels.5,6 Arterial dissection is easily missed unless the clinician is thinking of it. Clinical trial data indicates no difference in outcomes among patients treated with either antiplatelet or anticoagulation therapy.14 Idiopathic carotidynia is usually a nonworri-some cause of focal arterial tenderness. In re-cent years, this diagnosis has fallen out of favor due to a lack of specicity, and a critical review of the literature revealed that it is not a valid single pathologic entity.6 Carotidynia may have a viral etiology. Other causes include but are not limited to carotid artery dissection, post-carotid endarterectomy, aneurysm, GCA, and bromuscular dysplasia.6 Therefore, manage-ment should include referral to a neurologist. Cerebral venous thrombosis (ICHD 6.6)Venous thrombosis (ICHD 6.6; ICD-10 I63.6) may present with acute severe pain or sub-acute to chronic pain.6 Patients may com-plain of a severe new headache associated with visual disturbances, signs of increased intracranial pressure (eg, nausea, vomiting, or papilledema), seizures, or frank neurologic decits. Headache may also be the sole mani-festation.15 Venous congestion can produce ischemic or hemorrhagic cerebral infarction. Predisposing factors include dehydration, oral contraceptive use, postpartum state, pro-thrombotic blood dyscrasia, neoplastic con-ditions, mild to moderate head trauma, and local infection.16 Patients suspected of having a venous thrombosis should be immediately referred to a neurologist or a neurosurgeon, who will likely investigate further by obtaining an MRI scan with MR venography or CT scan with CT venography. Treatment may consist of modifying the predisposing factors and at least 6 months of anticoagulation therapy.16 Other intracranial arterial disorder (ICHD 6.7)Reversible cerebral vasoconstriction syndrome (RCVS) (ICHD 6.7.3) has a pathognomonic pre-sentation of recurrent thunderclap headache.5 The syndrome is frequently precipitated by substance or medication exposures, most often selective serotonin reuptake inhibitors, stimulants, or marijuana. RCVS may occur postpartum as well. The syndrome may be complicated by stroke or hemorrhage, in-cluding cortical SAH, all leading to signicant 69morbidity. RCVS may be diagnosed with an MRA, CTA, or conventional angiography, and it is treated with calcium channel blockers to reverse arterial vasospasm. The offending agent should be discontinued promptly. CSF evaluation is often pursued to exclude angiitis (vasculitis), which may have similar ndings on vascular imaging of arterial beading.Genetic vasculopathy (ICHD 6.8)Cerebral autosomal dominant arteriopathy with subcortical infarcts (CADASIL) (ICHD 6.8.1) is a rare genetic cause of stroke and dementia in young adults.17 There is a strong association with migraine with aura, which may be a pre-senting early feature. Neuroimaging with MRI will demonstrate characteristic white matter hyperintensities involving the anterior tempo-ral poles and external capsule. The diagnosis can be conrmed with electron microscopy of a skin biopsy and/or genetic testing for the Notch3 mutations.Mitochondrial encephalopathy, lactic acido-sis, and stroke-like episodes (MELAS) (ICHD 6.8.2) is a rare heterogenous disorder that may present with migraine, seizures, vomiting, and/or focal cortical decits (eg, visual eld loss). As implied by the name, lactic acidosis may be seen in the blood or elevated in the brain as measured using magnetic resonance spec-troscopy. The diagnosis may be conrmed with muscle biopsy or genetic testing. As with other mitochondrial disorders, the transmis-sion is maternal in origin.Pituitary apoplexy (ICHD 6.9, ICD-10 E23.6)Pituitary apoplexy refers to a stroke of the pi-tuitary gland, often with a preexisting pituitary tumor. The disorder is almost always accompa-nied by acute headache and is frequently as-sociated with visual eld decits and diplo pia. The diagnosis can be conrmed with a CT or MRI scan. Patients may require surgery or may be managed conservatively. Systemic compli-cations like hypotension can occur as a result of associated endocrine deciencies requiring hormone replacement. Because of the high mortality rate, prompt recognition and treat-ment are critical.18Headache Attributed to Nonvascular Intracranial Disorders (ICHD 7)Increased CSF pressure (ICHD 7.1)Increased intracranial pressure (ICHD 7.1; ICD-10 G93.2) may yield a nonspecic headache involving any region of the head.19 Patients may have a mass that exerts pressure or have a process that impairs the normal circulation and egress of CSF, such as venous thrombosis or meningitis. This headache typically worsens with a Valsalva maneuver or recumbence and may be associated with nausea/vomiting, vi-sual problems, and neurologic decits.19 Exam-iners should look for extraocular movement ab-normalities, especially diplopia, and evidence of early papilledema. Idiopathic intracranial hypertension (ICD-10 G93.2) (which used to be called pseudotumor cerebri) occurs most often in young, obese females.19 Oral contra-ceptive pills and certain other medications like tetracycline may also be risk factors. Typi-cally, this condition produces a holocephalic daily headache with intermittent visual distur-bances and pulsatile tinnitus. Neuroimaging (ie, MRI) should be unremarkable except for slit-like ventricles and sometimes stenosis of both transverse sinuses, especially apparent on MR venography. Examination may reveal early or frank papilledema and/or bilateral ab-ducens (sixth) nerve palsy. Increased open-ing pressure on lumbar puncture obtained in the lateral decubitus position is usually higher than 250 mm H2O, and this nding along with a normal MRI scan of the head conrms the diagnosis. Blindness may ensue if this syn-drome is left untreated; it should therefore Headache Attributed to Nonvascular Intracranial Disorders 70Vascular and Nonvascular Intracranial Causes of Orofacial Pain4be managed along with an ophthalmologist to monitor vision.19 Fortunately, the increased intracranial pressure can remit with as little as 5% weight loss. Treatment also includes the use of acetazolamide, which decreases pro-duction of CSF.20,21 Some patients may require multiple lumbar punctures to lower CSF pres-sure, optic nerve sheath fenestration for relief of papilledema, or ventriculoperitoneal shunt-ing of CSF to reduce headaches and prevent further visual loss.19Low CSF pressure (ICHD 7.2)Headaches due to low CSF pressure (ICHD 7.2; ICD-10 G97.1) are classically worsened within a few minutes of standing and relieved by re-cumbence.21 They may be accompanied by neck stiffness or pain, tinnitus, hypacusia, par-esthesia of the neck and arms, photophobia, or nausea. Headaches due to low CSF pres-sure may be iatrogenic after dural or lumbar punctures (ICHD 7.2.1; ICD-10 G97.0), postop-erative, or spontaneous (ICHD 7.2.3; ICD-10 G97.1). Spontaneous CSF volume depletion may be associated with meningeal diverticula, weakened dura mater, or connective tissue diseases such as Marfan syndrome.21 The positional component usually becomes less evident over time. This form of headache is produced by traction placed on the dura mater and its pain-sensitive vasculature by the sag-ging brain when standing. An MRI study with intravenous gadolinium is required to reveal the classic nding of pachymeningeal en-hancement of the dura and perhaps descent of the cerebellar tonsils, with attening of the prepontine cistern and distortion of the brain-stem. The headaches may resolve spontane-ously or within 48 to 72 hours of treatment. Treatment may involve prolonged, complete bedrest without head elevation for 2 to 3 days, mild analgesics, caffeine or theophylline, or an epidural blood patch.21 The blood patch has a pain relief success rate of over 90% in iatro-genic cases.Noninfectious inammatory diseases (ICHD 7.3)Headaches with inammatory conditions in-clude neurosarcoidosis (ICHD 7.3.1; ICD-10 D86.8) and aseptic meningitis (ICHD 7.3.2; ICD-10 A87.9).22 These entities cause vari-ous nonspecic symptoms whose recogni-tion comes only with vigilant observation and detection of red ags. The only red ag that might be obvious for aseptic meningitis is a se-vere headache occurring during or after a viral infection. Some drugs, classically NSAIDs and intravenous immunoglobulin, can also cause aseptic meningitis. Investigations necessary for diagnosis often include an MRI scan of the head, CSF examination, and blood studies.Intracranial neoplasia (ICHD 7.4)Intracranial neoplasms are accompanied by headaches in approximately 50% of patients during the course of the illness and are one of the primary presenting symptoms in ap-proximately 20% of these cases.23 Patients presenting with headache frequently worry about having a brain tumor, but headache is very rarely the sole symptom (one case se-ries reported 2%).23 More often, the patient presents with focal neurologic symptoms or a seizure. Headaches may be attributed to in-creased intracranial pressure or hydrocepha-lus caused by the neoplasm (ICHD 7.4.1), to the pressure from the neoplasm itself (ICHD 7.4.2), or to carcinomatous meningitis (ICHD 7.4.3; ICD-10 C79.32).23 The earlier concept that headaches present upon awakening were often caused by a brain tumor has been largely abandoned. The time course is most typically subacute to chronic, and there will usually be other symptoms that make the diagnosis suspect: weight loss, personality changes, seizures, and/or neurologic decits such as focal weakness or numbness, trouble walking, or visual disturbances. Head pain brought on acutely by coughing or a Valsalva maneuver is 71usually benign but can, in rare cases, be due to a tumor causing increased intracranial pres-sure. Occasionally, neoplastic processes may intermittently occlude normal CSF ow, pro-ducing a ball-valve mechanism that manifests as posture-dependent symptoms. If a patient presents with a new headache with some of these symptoms, a neurologic consultation and a contrast-enhanced MRI study of the head are warranted. There is an association between headache and pituitary tumors where the headache most often resembles migraine, but it can also present with more unusual phe-notypes, such as primary stabbing headache, cluster headache, or short-lasting unilateral neuralgiform headache attacks with conjuncti-val injection and tearing (SUNCT).24 Chiari malformation type I (ICHD 7.7; ICD-10 Q07.0)Herniation of the cerebellar tonsils 3 to 5 mm below the foramen magnum or caudally to the C2 level represents a structural malforma-tion of the brainstem and dura known as the Arnold-Chiari malformation.25 In a patient pre-senting with headache, it should be noted that this radiographic appearance can be mimicked by both intracranial hypertension and hypoten-sion, where a descent of the base of the brain may occur.21 This syndrome is commonly de-lineated by the type of abnormality and may include hydrocephalus, myelomeningocele, sy-ringomyelia, other spinal cord cavitations (ie, syrinx), and distortions of the components of the middle and posterior fossa.25 There may be attening of the pons and effacement of the prepontine cistern with kinking of the brain-stem. The syndrome can be associated with headaches, hemifacial spasm, coughing with or without symptoms of central sleep apnea, the inability to speak, dysphagia, and nystag-mus.25 Arnold-Chiari malformation may cause traction or compression of one or more cranial nerves, which requires a denitive neurologic assessment as well as an MRI study. More serious complications occur if the medulla or cervical spinal cord is compressed, which could lead to weakness of the limbs and hy-perreexia with extensor plantar responses (positive Babinski reex). Milder cases without neurologic symptoms should be followed con-servatively. Headache also is not an indication for surgery; surgical decompression is most often required if there are neurologic signs. This abnormality can exist in children as well as in adults.25Headache Attributed to Infection (ICHD 9)Headaches may be attributed to intracranial infections, including bacterial meningitis (infec-tion of the meninges) (ICHD 9.1.1; ICD-10 G00.9), lymphocytic meningitis (ICHD 9.1.2; ICD-10 G03.9), encephalitis (infection of the brain parenchyma) (ICHD 9.1.3; ICD-10 G04.90), brain abscess (localized walled-off infection of the brain substance) (ICHD 9.1.4; ICD-10 G06.0), and subdural empyema (infection local-ized to the subdural space) (ICHD 9.1.5; ICD-10 G06.2).22 The headache is most commonly holocephalic in association with fever, arthral-gia, stiff neck, photophobia, nausea and/or vomiting, altered consciousness, and confu-sion.22 These symptoms may develop over minutes to hours, and the condition of the pa-tient may deteriorate rapidly. Patients with en-cephalitis are often drowsy, confused, and disoriented on examination. Those with menin-gitis have stiff necks that cannot be exed and occasionally positive Kernig and Brudzinski signs.26 Progression of symptoms over hours may be the most important clue because fever or meningismus may be absent, especially in the very young, old, or immunocompromised. Patients may die within hours if not properly treated. Interestingly, many brain abscesses can present as a space-occupying mass with neurologic ndings related to the mass but without any meningeal irritation, abnormalities Headache Attributed to Infection 72Vascular and Nonvascular Intracranial Causes of Orofacial Pain4on CSF testing, or fever. Scans show only an avascular mass, often with a ring around its edge. Meningococcal meningitis is deadly if not treated within hours. Any patients with a rash, fever, headache, stiff neck, and vomiting should have an emergency diagnostic lumbar puncture after a CT or MRI scan and be treated pending the CSF results.Herpes simplex is the most common cause of nonepidemic viral encephalitis and typically presents with acute headache, fever, cognitive changes, drowsiness, and focal ndings such as hemiparesis and seizures. Early antiviral therapy greatly reduces permanent neurologic sequelae. Patients with an abscess or subdural empyema present similarly to those with se-vere meningitis, with headache, fever, malaise, and possibly signs of increased intracranial pressure. Certain fungal meningitides can be difcult to diagnose because they present with no fever and spinal uid appearing normal, re-quiring special CSF antigen testing, staining, and cultures. Sometimes a history of the in-dividual’s geographic location and whom they had been in contact with can be revealing.The diagnostic procedures include blood cultures and complete examination of CSF, both with cultures and gram staining, poly-merase chain reaction, and imaging tech-niques including CT and MRI scans. The clini-cian should be aware that patients with AIDS may suffer head, neck, and orofacial pain from numerous infectious etiologies, including any of those discussed previously. In rare cases, dental and surgical procedures can be compli-cated by central nervous system infection.References1. Headache Classication Committee of the International Headache. The international classication of headache disorders, 3rd edition (beta version). Cephalalgia 2013; 33:629–808.2. Locker TE, Thompson C, Rylance J, Mason SM. The util-ity of clinical features in patients presenting with non-traumatic headache: An investigation of adult patients attending an emergency department. Headache 2006; 46:954–961.3. Sheeler RD, Garza I, Vargas BB, O’Neil AE. Chronic daily headache: Ten steps for primary care providers to re-gain control. Headache 2016;56:1675–1684.4. Ray BS, Wolff HG. Experimental studies on headache: Pain-sensitive structures of the head and their signi-cance in headache. Arch Surg 1940;41:813–856.5. Schwedt TJ, Matharu MS, Dodick DW. Thunderclap headache. Lancet Neurol 2006;5:621–631.6. Kapoor S. Headache attributed to cranial or cervical vas-cular disorders. Curr Pain Headache Rep 2013;17:334.7. Verdelho A, Ferro JM, Melo T, Canhão P, Falcão F. Head-ache in acute stroke. A prospective study in the rst 8 days. Cephalalgia 2008;28:346–354.8. Linn FH, Rinkel GJ, Algra A, van Gijn J. Headache char-acteristics in subarachnoid haemorrhage and benign thunderclap headache. J Neurol Neurosurg Psychiatry 1998;65:791–793.9. Thompson BG, Brown RD Jr, Amin-Hanjani S, et al. 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Antiplatelet treatment compared with anticoagulation treatment for cervical artery dissection (CADISS): A randomised trial. Lancet Neurol 2015;14:361–367.15. Biousse V, Ameri A, Bousser MG. Isolated intracranial hypertension as the only sign of cerebral venous throm-bosis. Neurology 1999;53:1537–1542.16. Ferro JM, Canhão P. Acute treatment of cerebral ve-nous and dural sinus thrombosis. Current Treat Options Neurol 2008;10:126–137.17. Sabbadini G, Francia A, Calandriello L, et al. Cerebral autosomal dominant arteriopathy with subcortical in-farcts and leucoencephalopathy (CADASIL). Clinical, neuroimaging, pathological and genetic study of a large Italian family. Brain 1995;118(Pt 1):207–215.18. Singh TD, Valizadeh N, Meyer FB, Atkinson JL, Erickson D, Rabinstein AA. Management and outcomes of pitu-itary apoplexy. J Neurosurg 2015;122:1450–1457.19. Wakerley BR, Tan MH, Ting EY. Idiopathic intracranial hypertension. Cephalalgia 2015;35:248–261.20. 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