ABSTRACT
A. International Headache Society (IHS) Criteria 1. IHS has developed criteria to divide headaches into
primary and secondary types 2. Primary type: headaches without specific cause
(migraine, tension, cluster, miscellaneous) 3. Secondary type: headaches with underlying structural
or metabolic cause
B. Headache History and Physical Examination 1. Headache history
a. Aimed at distinguishing between primary and secondary types of headache
b. Typical questions to consider 1) Prodrome or aura 2) Onset and course 3) Duration and frequency 4) Pain quality and severity 5) Associated features 6) Precipitating factors 7) Ameliorating factors 8) Family and social history
c. Worrisome features in history for secondary type 1) Thunderclap (acute) onset 2) Late-onset headache with no previous headache
history 3) Systemic disease (infectious, metabolic, toxic) 4) Patient with history of cancer 5) Patient with history of immunosuppressed state 6) Headache worsens with cough or strain, especially
worrisome if associated with transient visual obscurations
2. Physical examination a. Aimed at distinguishing between primary and secondary
types of headache b. Worrisome features in physical examination suggesting a
secondary type
1) Focal neurologic deficits 2) Papilledema
A. Clinical Presentation 1. Determine onset and course
a. Did the headache reach maximum severity in seconds, minutes, hours, or days-thunderclap presentation (maximum severity within seconds) is particularly worrisome
b. Is the headache improving, stable, worsening 2. Determine associated symptoms
a. Nausea or vomiting b. Photophobia c. Visual phenomena d. Focal limb deficits (sensory or motor): can be suggestive
of intracranial lesion e. Visual obscurations: may be suggestive of increased
intracranial pressure 3. Determine if there were or are any provoking factors
a. Postural: upright vs. laying down (if headache worse in upright position, may suggest low-pressure headache syndrome)
b. Coughing, sneezing, or straining: may suggest increased intracranial pressure
c. Exertion
B. Differential Diagnosis of Acute Thunderclap Headache (Table 18-1)
C. Diagnostic Approach 1. Subarachnoid hemorrhage must be ruled out in all
patients presenting with a thunderclap headache 2. Sensitivity of computed tomography (CT) of the head
for subarachnoid hemorrhage a. CT without contrast is 92% sensitive in detecting
subarachnoid hemorrhage if performed within 24 hours b. Sensitivity falls to approximately 50% by end of day 5
after symptom onset c. If CT is negative and clinical suspicion high, lumbar
puncture is necessary 3. Cerebrospinal fluid (CSF) examination
a. Best to perform at least 6 hours after symptom onset, but no more than 2 to 3 weeks after symptom onset
b. Variables evaluated: opening pressure, cell counts in tube 1 and tube 4, protein, glucose, Gram stain, xanthochromia
c. Interpretation of CSF findings (see Chapter 10) 4. Magnetic resonance imaging (MRI) and magnetic reso-
nance (MR) venography: may be performed to exclude venous sinus thrombosis when CSF is normal or shows increased opening pressures and is otherwise normal, thus excluding subarachnoid hemorrhage and meningitis
5. Consideration of additional tests a. MR or conventional angiography
1) If suspicion for subarachnoid hemorrhage or sentinel leak of aneurysm is high
2) Conventional angiography has greater sensitivity for small aneurysms than MR angiography
b. MRI head: if increased intracranial pressure, hypertensive crisis, or intracranial hemorrhage suspected
D. Treatment: specific to the cause
A. Epidemiology 1. Lifetime prevalence: estimated to be approximately
20% to 25% for women, 8% to 10% for men 2. Begins in males earlier than females 3. Positive family history for migraine is common
B. Pathophysiology 1. Etiology: may have genetic component, for example, P
type calcium channels may be implicated in cause of migraine headaches (involved with 5-hydroxytryptamine [5HT] release)
2. Migraine aura a. Hypoperfusion of blood flow is seen often before
headache begins and in association with aura b. This wave of reduced cerebral blood flow travels across
cerebral cortex 2 to 3 mm/min 3. Headache
a. Intracranial blood vessels innervated by pain-sensitive fibers of trigeminal nerve (supratentorial area) and cervical nerves (posterior fossa)
b. One theory: antidromic stimulation of trigeminal nerve releases substance P, calcitonin gene-related peptide (CGRP), and neurokinin A, producing neurogenic inflammation
c. Neurogenic inflammation results in vessel dilatation and plasma protein extravasation
d. Central processing of pain 1) Afferent projections to trigeminal spinal nucleus 2) Synaptic relay from trigeminal spinal nucleus is in
thalamus: ventral posteromedial (VPM), intralaminar, and posterior nuclei
3) Thalamic projections to cerebral cortex 4) Influence from the dorsal raphe nucleus and locus
ceruleus e. 5HT1b receptor: responsible for cranial vasoconstriction
1) Three receptor types: G protein-coupled receptors, ligand-gated ion channels, and transporters
2) Seven classes of receptors (1-7) 3) 5HT1: five receptor subtypes (Table 18-2)
f. Triptan medications are 5HT1b/1d agonists 1) Inhibit release of vasoactive peptides 2) Promote vasoconstriction 3) Affect modulating pain pathways in brainstem
C. Clinical Presentation 1. Prodrome
a. Hours to days before headache b. Occurs in more than 50% of patients with migraine
Table 18-1. Differential Diagnosis of Acute Thunderclap Headache
c. Can consist of mental state changes, poor concentration, food cravings, gastrointestinal and urinary symptoms
2. Aura a. Focal neurologic symptoms preceding headache typically
lasting less than 1 hour b. Most commonly consist of visual disturbances but also
can include sensory (second-most common), motor, language dysfunction
c. May occur alone or associated with headache 3. Location
a. Unilateral more often than bilateral b. Often frontal
4. Quality: throbbing, pounding (not always) 5. Associated symptoms
a. Nausea and/or vomiting
b. Photophobia and/or phonophobia c. Anorexia d. Worse with exertion, better in dark room
6. Duration: hours to days 7. Precipitating factors
a. Variable b. May include
1) Food and/or drink (red wine, monosodium glutamate)
2) Stress 3) Certain odors 4) Minor trauma 5) Menses
D. Differential Diagnosis 1. Structural lesions (secondary headaches) 2. Metabolic disorders (secondary headache syndromes),
including mitochondrial myopathy, encephalopathy, lactic acidosis, stroke (MELAS) syndrome
3. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
4. Tension headaches
E. Diagnostic Approach 1. IHS criteria for migraine are listed in Table 18-3 2. Diagnosis is based on clinical symptomatology 3. Neurologic examination and head neuroimaging are
usually negative
F. Treatment 1. Acute treatment
a. Early treatment: should be given soon after symptom onset
b. Options for acute treatment 1) General analgesics 2) Migraine-specific remedies (serotonergic [“triptan”]
medications and dihydroergotamine) c. Management of associated symptoms (e.g., nausea and
vomiting) d. Treatments available for acute migraine headache are
reviewed in Table 18-4 e. Current triptan medications are reviewed in Table 18-5
2. Prophylaxis a. Should be considered for patients whose headache
frequency is functionally disabling b. Prophylactic options are listed in Table 18-6
3. Nonmedication therapy a. Biofeedback b. Physical therapy c. Acupuncture
Table 18-2. 5-Hydroxytryptamine (5HT) Subclass 1 Receptors
Subtype of 5HT1 receptor Agonist Location Function
Table 18-3. International Headache Society Criteria for Migraine Headaches
Table 18-3. continued
Table 18-4. Treatments for Acute Migraine Headache
Medication Side effects Contraindications (relative and absolute)
G. Status Migraine 1. Definition: severe persistent migraine headache often
with intractable nausea and vomiting 2. Management
a. Rule out secondary causes of headache b. Hydrate patient with intravenous fluids c. Specific treatment (administered individually)
1) Dihydroergotamine 2) Valproate intravenous 3) Chlorpromazine or prochlorperazine intravenous 4) Ketorolac intravenous 5) Metoclopramide 6) Corticosteroids 7) Narcotic medication
H. Transformed Migraine (see section VI)
I. Familial Hemiplegic Migraine (FHM) 1. Definition: diagnostic criteria listed in Table 18-3 2. Clinical description
a. Typically begins in childhood b. Hemiplegia may precede or occur concomitantly with
headache or headache may be absent c. Hemiplegia may last hours to days
3. Genetics and pathophysiology a. Hemiplegic migraine may be sporadic or familial b. Autosomal dominant with variable penetrance c. Three types have been identified
1) FHM1, linked to chromosome 19p13 in majority of
families (CACNA1A gene): results in defect in P/Q calcium channel subunit
2) FHM2, linked to chromosome 1q23 (ATP1A2 gene): results in defect in A1A2 sodium-potassium ATPase channel
3) FHM3, linked to chromosome 2q24 (SCN1A gene): results in cortically expressed presynaptic and postsynaptic voltage-gated sodium channel
J. Migrainous Cerebral Infarction (see section IX)
A. Epidemiology 1. Up to 80% of people experience one tension headache
in their lifetime 2. More common in women than men 3. Peak prevalence: between age 20 and 50 years
B. Clinical Presentation 1. Location: majority are bilateral occipital, frontal, or
fronto-occipital, but may occur in any location 2. Quality: dull ache or pressure 3. Severity: often mild to moderate 4. Associated symptoms
a. Tenderness to palpation b. Absence of nausea, vomiting, photophobia c. Not aggravated by physical activity
5. Precipitating factors include sleep deprivation
C. Diagnostic Approach 1. Diagnosis is based on clinical criteria and evaluating
for secondary causes 2. IHS criteria are noted in Table 18-7
D. Chronic Tension Headache (see section VI)
E. Treatment 1. Pharmacologic
a. Abortive 1) Nonsteroidal anti-inflammatory drugs 2) Aspirin 3) Acetaminophen
b. Prophylactic 1) Tricyclic antidepressants 2) Antiepileptic medications (most commonly, topiramate,
valproic acid, gabapentin) 3) Selective serotonin reuptake inhibitors 4) β-Blockers 5) Calcium channel blockers
Table 18-5. Comparison of Triptan Agents (Selective Serotonin Agents)
Onset of Duration Triptan Dosage forms action (half-life)
Table 18-6. Migraine Prophylactic Regimens*
Medication Contraindications Recommended for patient Examples
2. Nonpharmacologic: behavioral treatment, biofeedback, treatment of concurrent depression
A. Epidemiology 1. Incidence: 15.6/100,000 person-years for men;
4.0/100,000 person-years for women 2. Mean age at onset: 20s to 30s
B. Pathophysiology 1. Unknown 2. Because of episodic clustering of headaches and cyclical
bouts on yearly basis, hypothalamus (suprachiasmatic nucleus) may be involved
3. Retro-orbital pain suggests involvement of the trigeminal nerve (ophthalmic division)
4. Sympathetic dysfunction and parasympathetic overactivity suggest role of autonomic system
C. Clinical Presentation 1. Timing
a. Cluster periods may last weeks to months b. Cluster periods often separated by remission (if not, may
represent chronic cluster)
c. Attacks within a cluster period may be daily to several times daily, each lasting 15 to 180 minutes
2. Location: unilateral, orbital, or temporal pain 3. Quality: often severe 4. Precipitating factors: alcohol and other solvents,
nitrates 5. Patients tend to pace, rather than lay down (as with
patients with migraine headaches) 6. Associated symptoms
a. Conjunctival injection b. Lacrimation c. Nasal congestion d. Rhinorrhea e. Forehead and facial sweating f. Miosis g. Ptosis h. Eyelid edema
D. Diagnostic Approach and Differential Diagnosis 1. Diagnosis is generally a clinical one 2. Important distinction is eliminating potential
secondary causes 3. Differential diagnosis: trigeminal autonomic cephalgias,
cavernous sinus disease, sinusitis, vertebral arterial dissection, Raeder’s paratrigeminal syndrome, temporal arteritis, Tolosa-Hunt syndrome
E. Treatment 1. Abortive (of individual attacks)
a. Oxygen 1) Use oxygen by face mask at flow rate of 7 to 10
L/minute for 15 minutes 2) May help in up to 60% to 70% of patients
b. Sumatriptan 1) 6 mg subcutaneously has rapid onset and is effective
in most patients within 15 minutes 2) Contraindications (Table 18-4)
c. Lidocaine: intranasal lidocaine drops may be used d. Dihydroergotamine (DHE): 1 mg intramuscularly or
intravenously is effective rapidly 2. Prophylaxis (prevention of cluster periods and reduce
length of present attacks) a. Consider if
1) Frequent attacks would require overusing certain abortive agents
2) Attacks are too short lived for abortive treatment 3) Frequent cluster periods
b. Treat throughout cluster period and 2 weeks after last headache
c. Options are noted in Table 18-8
Table 18-7. International Headache Society Criteria for Tension-Type Headaches
d. Choice of medication may depend on comorbid conditions
e. Avoidance of known triggers
A. Overview 1. A group of headache disorders that occur most days per
month lasting 4 hours or more 2. May include both primary and secondary headache types
a. Primary: transformed migraine, chronic tension, new daily persistent headache (NDPH), and hemicrania continua
b. Secondary: cerebral venous thrombosis, increased or decreased intracranial hypertension, lesion in cervical or trigeminal distribution, space-occupying mass, central nervous system (CNS) or sinus infection, cervical spine disorders, post-traumatic, Arnold-Chiari malformation, sleep apnea
3. Primary types may be seen with or without medication overuse (rebound)
Table 18-8. Prophylaxis for Cluster Headaches*
Medication Starting dose Typical maintenance dose Side effects
B. Transformed Migraine 1. Definition
a. Patients have previous episodes of migraine headache that increase in frequency to daily or near daily
2. Common features a. Patients have history of episodic migraine that increases
in frequency over time b. Associated symptoms such as nausea, photophobia, and
phonophobia decrease but headache becomes constant, occurring more than 15 days per month
c. Headache may take on a chronic tension-type quality d. Increased frequency commonly occurs in setting of over-
use of analgesics e. Depression is common
3. Differential diagnosis: includes other chronic daily headaches (primary and secondary)
4. Treatment a. If medication overuse, withdrawal of (taper) or limiting
offending agent is important b. Preventive medications include those commonly used for
migraine prophylaxis
C. Chronic Tension Headache 1. Clinical features
a. Patients may have had a previous history of episodic tension headaches that increased in frequency to occur 15 or more days per month
b. Headache characteristics similar to episodic tension headache but may include nausea
c. Headache duration: generally more than 4 hours d. Criteria require these characteristics to be present for at
least 6 months e. Transformation to chronic daily headache may or may
not be associated with increased analgesic use 2. Differential diagnosis
a. Includes other chronic daily headaches (primary and secondary)
b. IHS criteria are listed in Table 18-7 3. Treatment: prophylactic agents and nonpharmacologic
treatment similar to that of tension headaches
D. New Daily Persistent Headache (NDPH) 1. Clinical features
a. Headache typically develops within 1 to 3 days and persists b. Headache characteristics similar to those of tension
headache c. May or may not be associated with medication overuse
2. Differential diagnosis a. Includes other chronic daily headaches (primary and
secondary)
b. Criteria for NDPH are listed in Table 18-9 3. Treatment: strategies similar to those for transformed
migraine and chronic tension headache
E. Hemicrania Continua (see section VII)
F. Medication Overuse Headache (rebound headache) 1. Epidemiology
a. Offending agents: many types, including narcotics, barbiturates or barbiturate-containing combination medications, general analgesics, triptans, ergotamine, caffeine
b. Common presentation in headache clinics 2. Pathophysiology
a. Can occur in patients taking offending agents as few as 2 times weekly to several times daily
b. Some believe this represents a withdrawal syndrome of offending agent
3. Clinical features a. Headache is generally described as a constant, diffuse,
dull headache b. Anxiety and depression are common
4. Treatment a. Prevention is important in patients being prescribed any
analgesic, including triptans b. Refractory to prophylactic medications until offending
analgesic is tapered and limited or withdrawn c. Offending agent should be tapered by 10% to 25% per
week d. Prophylactic agents used for other chronic daily
headaches can be considered
Table 18-9. International Headache Society Criteria for New Daily Persistent Headache
A. Trigeminal Autonomic Cephalgia: definitions 1. Unifying features include pain in distribution of
trigeminal nerve and autonomic signs reflecting activation of cranial parasympathetic systems (superior salivatory nucleus innervation of lacrimal glands and nasal mucosa via sphenopalatine and otic ganglia)
2. Includes cluster, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome
3. Hemicrania continua was thought a trigeminal autonomic cephalgia, but recently was designated as “other primary headache”
4. Because many of these syndromes are unilateral and associated with autonomic symptoms, neuroimaging is often necessary to rule out structural causes
5. Differential diagnosis is listed in Table 18-10
B. Paroxysmal Hemicrania 1. Epidemiology
a. Female:male is 2:1 b. Onset: generally in 30s, but range can be wide c. Two types: episodic and chronic
2. Clinical presentation a. Headache
1) Unilateral, temporal, or orbital 2) Severe, boring, throbbing, stabbing pain 3) Short lasting (average, 30 minutes)
4) Multiple attacks daily common (often 10-20 daily) 5) Episodes can last weeks to months, with remissions in
between 6) Can occur at night
b. Associated features 1) Lacrimation 2) Nasal congestion 3) Conjunctival injection 4) Rhinorrhea
c. Episodic and chronic types have similar clinical features, but episodic paroxysmal hemicrania has clear pain-free periods between attacks, typically lasting months to years
3. Diagnosis a. Diagnostic criteria are listed in Table 18-11 b. Neuroimaging, complete blood count, erythrocyte sedi-
mentation rate are important to rule out secondary causes
4. Treatment a. Indomethacin b. Other potential treatments: alternative nonsteroidal
anti-inflammatory drugs, verapamil or other calcium channel blockers
C. Hemicrania Continua 1. Epidemiology
a. More common in females than males b. Presentation age variable, but mean age in the 30s
2. Clinical presentation a. Headache
1) Unilateral 2) Moderate to severe throbbing pain 3) Often continuous pain, but may occur in episodes
lasting weeks to months with pain-free periods b. Associated symptoms
1) Autonomic features similar to paroxysmal hemicranias and SUNCT
2) Nausea, vomiting 3) Photophobia, phonophobia, osmophobia
3. Diagnosis: depends on ruling out secondary causes (Table 18-12)
4. Treatment a. Response to indomethacin is diagnostic b. May also respond to alternative nonsteroidal anti-inflam-
matory drugs, corticosteroids, dihydroergotamine, gabapentin, lithium, methysergide, and lamotrigine
D. SUNCT Syndrome 1. Characterized by brief, unilateral attacks of stabbing
pain with ipsilateral autonomic manifestations 2. Epidemiology
Table 18-10. Differential Diagnosis of Trigeminal Autonomic Cephalgias (Secondary Causes)
Chronic paroxysmal hemicrania SUNCT syndrome
a. More common in males than females b. Clinical presentation: mean age, 45 years
3. Clinical presentation a. Headache
1) Strictly unilateral 2) Localized to orbit or frontal region 3) Characterized by stabbing or burning pain 4) Paroxysms of pain can occur 5 times per hour with
each paroxysm lasting 15 to 120 seconds b. Associated symptoms
1) Autonomic symptoms similar to paroxysmal hemicranias, with conjunctival injection and tearing being common
4. Diagnosis (Table 18-13): Neuroimaging may be needed to exclude structural causes found in secondary cases
5. Treatment a. Difficult to treat; indomethacin is not useful b. Medications tried with variable efficacy: sumatriptan,
dihydroergotamine, prednisone, verapamil, valproate, carbamazepine, lithium
A. Trigeminal Neuralgia (tic douloureux) 1. Epidemiology
a. Male:female is 2:3 b. Idiopathic trigeminal neuralgia typically presents in older
patients c. Trigeminal neuralgia presenting in young patients should
raise concern for potential structural lesion 2. Pathophysiology
a. Irritation of trigeminal nerve b. Usually idiopathic but may be related to structural caus-
es: multiple sclerosis, aneurysm, arterial compression, tumor, dental problem
c. Vascular compression may cause demyelination of trigeminal nerve, producing pain
Table 18-11. International Headache Society Criteria for Paroxysmal Hemicrania
Table 18-12. International Headache Society Criteria for Hemicrania Continua
Table 18-13. International Headache Society Criteria for SUNCT Syndrome
3. Clinical presentation a. Lancinating (electric shocklike) pain within trigeminal
nerve distribution b. Most commonly unilateral, rarely bilateral c. May be provoked by wind, touch, talking, brushing teeth d. Episodes typically occur several times daily and last
seconds to less than 2 minutes e. Idiopathic trigeminal neuralgia: physical examination
is generally normal; concern for structural cause if trigeminal sensory loss or motor dysfunction
4. Diagnostic approach a. Diagnosis can be made by clinical history (IHS criteria
are listed in Table 18-14) b. MRI of brain: rule out structural, compressive cause
(Fig. 18-1) 5. Treatment
a. Factors affecting treatment decisions 1) Age of patient: younger age, may favor surgical inter-
vention if intolerant or not controlled with pharmacologic treatment; older age, may favor pharmacologic management or ablative procedures over surgery
2) Etiology (e.g., if multiple sclerosis is the cause, the headache does not respond well to microvascular decompression)
3) Atypical face pain does not respond well to ablative procedures or surgery
b. Pharmacologic treatment 1) Carbamazepine (first-line treatment) 2) Phenytoin 3) Gabapentin 4) Baclofen 5) More recently, lamotrigine, oxcarbazepine, and
topiramate 6) Narcotics, nonsteroidal anti-inflammatory drugs not
generally useful c. Ablative procedures
1) Procedure choices include a) Alcohol block b) Glycerol block c) Balloon compression d) Radiofrequency ablation e) Gamma knife radiosurgery
2) These procedures are compared in Table 18-15 d. Surgery-microvascular decompression
1) Efficacious, with immediate pain relief reported in as many as 90% of patients
2) Recurrence rate of pain: 3.5% annually 3) Less efficacious for trigeminal pain that is atypical or
due to multiple sclerosis 4) Advantages: preservation of facial sensation, highly
efficacious 5) Disadvantages
a) Complications can include cranial nerve deficits, CSF leak, hemorrhage
b) Anesthesia dolorosa (continuous burning pain) can develop in approximately 5% of patients
B. Geniculate Neuralgia 1. Pathophysiology
a. Irritation of facial nerve b. May be idiopathic or related to structural irritation
(tumor, aneurysm, trauma)
Table 18-14. International Headache Society Criteria for Idiopathic Trigeminal Neuralgia
2. Clinical presentation a. May present as unilateral lancinating pain within the ear,
episodes last seconds to minutes and may occur several times daily
b. Can also develop into chronic, dull pain within the ear 3. Diagnostic approach
a. Clinical diagnosis b. Rule out glossopharyngeal neuralgia c. Rule out herpes zoster oticus (Ramsay Hunt syndrome)
1) Reactivation and migration of varicella-zoster virus latent in geniculate, auditory, and vestibular ganglia
2) Usual manifestations: pain and small vesicles in external ear, with or without facial paralysis, and inner ear symptoms
d. Appropriate neuroimaging (head MRI) to rule out structural cause
4. Treatment a. Pharmacologic: similar to that for trigeminal neuralgia b. Surgical: reserved for intractable pain despite pharmaco-
logic treatment (several options) 1) Surgical excision of nervus intermedius 2) Microvascular decompression 3) Geniculate ganglionectomy
C. Glossopharyngeal Neuralgia 1. Pathophysiology
a. Irritation of glossopharyngeal nerve, thought to be due to blood vessel compression in idiopathic cases
Table 18-15. Comparison of Ablative Therapies for Trigeminal Neuralgia
Procedure Description Efficacy Side effects
b. Structural causes: tumors, aneurysms, trauma 2. Clinical presentation
a. Unilateral lancinating pain in throat, which may radiate to ear
b. Episodes last seconds to minutes, can occur multiple times daily
c. Triggers: chewing, swallowing, coughing, yawning d. Rarely, syncope may result from hypersensitivity of
baroreceptor reflex 3. Diagnostic approach
a. Clinical diagnosis b. Rule out structural lesion with neuroimaging (may need
to image head and neck) c. Application of local anesthetic to throat can be diagnostic
4. Treatment a. Pharmacologic: similar to that for trigeminal neuralgia b. Surgical: section glossopharyngeal nerve and portion of
vagus nerve at the level of jugular foramen
D. Occipital Neuralgia 1. Unilateral or bilateral occipital pain that may or may
not radiate to vertex 2. Continuous dull and/or throbbing pain often superim-
posed by sharp, sometimes lancinating, pain 3. Pain may be reproduced or exacerbated by applying
pressure (or percussion) over occipital nerves 4. Pathophysiology may be related to
a. Chronic, excessive contraction of neck and scalp muscles b. Trauma or compression c. Entrapment of the greater and/or lesser occipital nerves
5. Treatment a. Local massage and rest b. Anticonvulsants c. Tricyclic antidepressants d. Local nerve blocks and injection of corticosteroids: both
diagnostic and therapeutic measures
E. Hypnic Headache 1. Mean age at onset: about 63 years 2. Clinical presentation (IHS criteria are listed in Table
18-16) a. Also called “alarm clock” headache because of nocturnal
nature of headache b. Moderate to severe pain variably described as dull, throb-
bing, or sharp that typically occurs after falling asleep (most commonly during REM sleep)
c. May be unilateral or bilateral d. Associated with nausea (20% of patients) e. Can occur several times per night, typically lasting
1 hour per episode (some longer)
3. Pharmacologic treatment a. Sumatriptan and oxygen have been tried with little
benefit b. Aspirin may provide some benefit c. Lithium may be helpful prophylaxis
F. Exertion-Induced Headaches 1. Epidemiology
a. More common in men than women b. Typically occurs in middle age c. Overall prevalence: about 1%
2. Clinical presentation (Table 18-17) a. Classified by type of exertion that precipitates headache b. Benign cough headache
1) Precipitated by cough (also any Valsalva maneuverrelated activity)
2) Sudden, severe bilateral headache within seconds after Valsalva maneuver
3) Rare to have nausea or vomiting in benign types 4) Differential diagnosis of cough headache: mass in
posterior fossa (cyst or tumor), increased intracranial pressure, Chiari malformation
c. Benign exertional headache 1) Precipitated by physical exercise 2) Pain onset with exertion lasting minutes to hours 3) Differential diagnosis of exertional headache
a) Mass lesion, sinus disease b) If sudden, severe onset, consider differential diag-
nosis of a sudden, acute headache noted in Table 18-1
c) Other possibilities: pheochromocytoma, migraine, increased intracranial pressure
d. Headache associated with sexual activity (Table 18-17) 1) Headache is often bioccipital or holocephalic; may
last minutes to 24 hours (mean duration of severe
Table 18-16. International Headache Society Criteria for Hypnic Headache
pain, 4 hours), often followed by a longer-lasting (up to 72 hours) pain of milder severity
2) Precipitated by sexual activity 3) Three types
a) Type 1 (dull type): starts early as mild headache and slowly intensifies as sexual excitement increases, onset before orgasm (may be tension type headache with pathophysiology related to excessive muscle contractions during early phase of coitus)
b) Type 2 (explosive type): sudden onset of severe throbbing pain with orgasm (pathophysiology may be related to Valsalva maneuver during coitus)
c) Type 3: postural headache occurring after orgasm (debatable entity)
4) Associated with migraine headache disorders 5) Differential diagnosis of the explosive type includes
entities listed in Table 18-1 3. Diagnostic approach: exclusion of structural causes
with neuroimaging and/or lumbar puncture if subarachnoid hemorrhage is a consideration
4. Treatment a. Trial of prophylactic indomethacin if headaches are
frequent; may require concurrent gastritis prophylaxis b. Alternative: naproxen, other nonsteroidal anti-inflam-
matory drugs
G. Idiopathic Stabbing Headache (ice-pick headache) 1. Associated with migraine, cluster, and other trigeminal
autonomic cephalgia headaches
2. Clinical presentation a. Characterized by stabs of sudden, severe pain located
commonly in orbit, temple, or parietal region b. Pain episodes generally less than 1 second c. Pain episodes can occur 1 to 50 times daily, episodes can
occur in clusters throughout the year 3. Treatment
a. Treatment requires prophylaxis because of brevity of pain episode
b. May respond to indomethacin
H. Cold-Stimulus Headache (ice cream headache) 1. Induced by cold stimulus (e.g., ice cream) touching
trigeminal-innervated pharynx and mouth 2. Most often mid-frontal localization 3. Occurs more often in migraineurs than general
population
A. Neoplasm 1. Epidemiology
a. Headache is present in up to 50% of patients with brain tumors
b. Depends on location and type of tumor and associated mass effect and/or hydrocephalus 1) Posterior fossa tumors commonly associated with
headache 2) Pituitary tumor, craniopharyngioma, and cerebello-
pontine angle infrequently produce headache c. More common in children with brain tumor than adults
(likely because of prevalence of posterior fossa tumors in children)
2. Pathophysiology: may be related to increased intracranial pressure, impingement of dural structures, hydrocephalus
3. Clinical presentation a. Headache may be unilateral, bilateral, or generalized b. Often of moderate severity and constant c. May be present upon awakening d. May have nausea and vomiting e. Some worsen with bending or straining f. Examination may show papilledema and/or focal neuro-
logic symptoms based on tumor location 4. Treatment: diagnosis and treatment of tumor based on
type and location (see Chapter 16)
B. Infection 1. Overview
Table 18-17. International Headache Society Criteria for Benign Exertional Headaches
a. Wide variety of intracranial and extracranial infections and systemic infection can lead to headache
b. Intracranial: meningitis, encephalitis, abscesses with mass effect, empyema
c. Extracranial: sinusitis, tooth abscess, orbital cellulitis 2. Pathophysiology
a. Febrile illnesses may produce interferons that have a role in generating headache
b. Infections may irritate meninges, producing headache c. Some infections such as abscesses may be associated with
mass effect and increased intracranial pressure 3. Clinical presentation
a. Acute meningitis 1) Rapidly progressive severe generalized headache with
radiation to neck 2) Pain may worsen with eye movement 3) Nausea and vomiting are common 4) Photophobia may occur 5) Fever 6) Alteration of consciousness may occur 7) Cranial nerve palsies may occur 8) Examination
a) May confirm above b) Kernig’s sign and Brudzinski’s sign may be present
b. Chronic meningitis 1) Subacute, progressive generalized headache may be
only sign 2) Intermittent fever and neurologic focal signs may also
be present 4. Diagnostic approach and treatment (see Chapter 15 on
management of infectious conditions)
C. Chiari Malformation 1. Definition
a. Chiari I malformation: tonsillar herniation below the level of foramen magnum
b. May be associated with syringomyelia (50%-70% of patients) and hydrocephalus
2. Pathophysiology of headaches associated with Chiari I malformation: compression of neural tissues and alteration of CSF dynamics
3. Clinical presentation a. May present in adulthood or childhood b. Headaches located typically in occipital region, often pre-
cipitated by cough c. Many patients may have posterior fossa symptoms from
brainstem compression: vertigo, ataxia, hoarseness, dysphagia, nystagmus, hearing loss, spasticity
d. Patients with syrinx may have weakness, sensory loss, and atrophy at segmental level
4. Diagnostic approach: MRI of brain (sagittal) can help detect Chiari malformations a. Definition of Chiari I is age-dependent because cerebel-
lar tonsils ascend with age b. Degree of herniation of cerebellar tonsils below foramen
magnum considered to be abnormal, by age 1) First decade: 6 mm 2) Second and third decades: 5 mm 3) Fourth-eighth decades: 4 mm 4) Ninth decade: 3 mm
5. Treatment a. Observation if asymptomatic b. Surgical: suboccipital craniectomy considered for defi-
nitely symptomatic patients, especially those with brainstem signs or symptoms
D. Stroke and Migraine 1. Introduction
a. Case control studies have suggested patients younger than 45 years with history of migraine have increased risk of ischemic stroke compared with controls (especially for women)
b. Ischemic stroke may be related to migraine because of 1) Regional blood flow changes related to migraine
pathophysiology 2) Alteration in platelet function 3) Possible association with diseases that have concomi-
tant migraine and stroke, such as CADASIL, MELAS, antiphospholipid antibody syndrome
2. Classification a. Coexisting stroke and migraine: patient has stroke and
history of migraine, but migraines are remote from time of stroke
b. Stroke with clinical features of migraine 1) “Symptomatic migraine”: patients with structural
vascular lesion that presents with features of migraine 2) “Migraine mimic”: acute stroke accompanied by
headache that acts similar to migraine with aura c. Migraine-induced stroke (migrainous cerebral infarction)
1) Patient must have previous history of migraine with aura
2) Cerebral infarction occurs during course of typical migraine with aura but aura not reversible, resulting in infarction
3) Other causes of ischemic infarction are ruled out with appropriate clinical studies
d. Uncertain: patient has migraine and stroke, but causal relationship cannot be established
3. Evaluation includes clinical testing for causes of ischemic stroke (see Chapter 11)
E. Sinus Disease 1. Clinical presentation
a. Facial tenderness and pain 1) Maxillary: localized to upper jaw teeth and cheek 2) Frontal: localized to forehead 3) Sphenoid: generalized or posteriorly localized pain 4) Ethmoid: localized between orbits
b. Nasal congestion (with ethmoid and maxillary involvement)
c. Purulent nasal discharge d. Anosmia e. Fever (50%-60% of patients)
2. Diagnostic approach: pain associated with purulent discharge and abnormal sinus imaging findings suggests the diagnosis
3. Treatment a. Treat bacterial infection b. Decongestants
F. Obstructive Sleep Apnea (Table 18-18) 1. Epidemiology: headache may present in up to 50% of
patients with obstructive sleep apnea 2. Pathophysiology: hypotheses on relation of headache
to obstructive sleep apnea a. Fluctuation of oxygen saturation during night, with
hypercapnia, vasodilatation, and increased in intracranial pressure
b. Degree of oxygen desaturation may be risk factor (debated)
c. Obstructive sleep apnea may precipitate idiopathic migraine headaches
3. Clinical presentation a. Headaches commonly occur in morning (upon awaken-
ing) and last several hours
b. May be unilateral or bilateral, variable location c. Typical quality of pain is described as tight or squeezing
pain of mild to moderate severity 4. Treatment: primarily treatment of underlying disorder
G. Post-traumatic 1. Clinical presentation
a. Headache present within 14 days after injury b. Trauma may be minor (without loss of consciousness) or
major (with loss of consciousness) c. Headaches often have features of either migraine or
tension headache d. Associated symptoms: dizziness, blurred vision, memory
and other cognitive complaints, alteration of taste and smell, depression, sleep disturbances
e. Specific syndromes associated with trauma 1) Low-pressure headaches (see below) 2) Temporomandibular joint pain 3) Carotidynia due to arterial dissection 4) Increased intracranial pressure due to cerebral edema
2. Diagnostic approach a. Appropriate neuroimaging and spine films in acute
setting are important b. IHS criteria (Table 18-19)
3. Treatment a. Appropriate management of fractures, intracranial
pressure, arterial dissection b. Tailored to headache characteristics c. If cervical component, some patients may benefit from
physical therapy
H. Intracranial Hypertension 1. Definition
a. May be idiopathic or secondary cause b. Secondary (structural or nonstructural): intracranial
mass, infection, hypertensive encephalopathy, trauma, hydrocephalus
c. Causes of increased intracranial pressure without structural abnormalities seen on neuroimaging are listed in Table 18-20
d. Idiopathic intracranial hypertension (pseudotumor cerebri): may or may not be associated with papilledema (rest of discussion below is on pseudotumor cerebri)
2. Epidemiology of pseudotumor cerebri a. More common in women than men b. Often occurs in young women in 20s c. Obesity is risk factor
3. Pathophysiology of pseudotumor cerebri (several hypotheses) a. Increased CSF formation
Table 18-18. International Headache Society Criteria for Headache Associated With Sleep Apnea
b. Decreased CSF absorption c. Increased venous pressure (congenital stenoses of venous
sinuses) 4. Clinical presentation of pseudotumor cerebri
a. Clinical symptoms 1) Majority, but not all, patients have headache
a) Unilateral or bilateral b) Frontal or occipital c) Quality similar to tension headache d) May be worse in morning
2) Associated symptoms may include a) Transient (seconds) visual obscurations: temporary
graying of vision, especially with straining b) Bilateral pulsatile tinnitus c) Diplopia (abducens palsy) d) Visual loss e) Nausea and vomiting
b. Physical examination 1) Papilledema 2) Abducens palsy 3) Visual loss (enlarging blind spot)
c. Subgroup of idiopathic intracranial hypertension without papilledema 1) Similar symptomatology except no visual loss or
papilledema 2) Elevated opening pressure diagnosed with lumbar
puncture 5. Diagnostic approach
a. Clinical symptomatology b. Visual field examination c. Neuroimaging to rule out secondary causes of increased
intracranial pressure (including veno-occlusive disease) d. Laboratory studies to rule out secondary causes of
increased intracranial pressure e. CSF examination to measure opening pressure and rule
out secondary causes (meningitis) 1) Normal CSF pressure: 40 to 200 mm H2O 2) Abnormal CSF pressure in nonobese patients: >200
mm H2O 3) Abnormal CSF pressure in obese patients: >250 mm
H2O 6. Treatment of pseudotumor cerebri
Table 18-19. International Headache Society Criteria for Post-traumatic Headache
a. Nonpharmacologic: weight loss b. Pharmacologic
1) If no evidence of visual loss, consider treatment of headache alone; if evidence of visual loss, consider carbonic anhydrase inhibitor
2) Carbonic anhydrase inhibitor (e.g., acetazolamide) a) Common side effects: weight loss, diarrhea,
nausea and/or vomiting, altered taste sensation, paresthesias, confusion, polyuria
b) Serious side effects: metabolic acidosis, tinnitus, sulfonamide adverse reaction
c) Contraindications: adrenal gland failure, angleclosure glaucoma, cirrhosis, hyponatremia and/or hypokalemia, hyperchloremic acidosis, severe hepatic or renal disease, sensitivity to sulfonamides
3) Topiramate a) Common side effects: nausea, confusion, dizziness,
memory impairment, nystagmus, paresthesias, somnolence, motor retardation, tremor, poor concentration, anxiety, angle-closure glaucoma, fatigue
b) Serious side effects: metabolic acidosis, pancreatitis, nephrolithiasis, renal tubular acidosis, hyperchloremia, hyperammonemia, hepatitis, liver failure, anemia, leukopenia
c) Contraindications: sensitivity to topiramate d) Use with precaution in behavioral disorders or cog-
nitive deficits, medical conditions or therapies that
predispose to acidosis, patients with inborn errors of metabolism, renal or hepatic impairment
4) Short course of high-dose prednisone (controversial) c. Procedures (serial high-volume lumbar punctures) d. Surgery
1) Reserved for failure of conservative treatment and progressive clinical symptoms or signs
2) Lumboperitoneal shunt or ventriculoperitoneal shunt (used to reduce severe headaches not amenable to conservative treatment or progressive visual loss despite conservative treatment)
3) Optic nerve fenestration: incision of dural covering of intraorbital optic nerve, used to prevent progressive visual loss
7. Outcome of pseudotumor cerebri a. Early treatment prevents visual loss b. Blindness may occur in up to 10% of patients c. Can spontaneously remit
I. Low CSF Pressure Headaches 1. Pathophysiology
a. Etiology: hypovolemic state, CSF shunt overdrainage, CSF leak (may be iatrogenic or spontaneous)
b. Iatrogenic CSF leaks occur with trauma, lumbar puncture, epidural catheterization, spinal or cranial neurosurgical procedures
c. Spontaneous CSF leaks may occur in setting of dural tear due to spondylosis or dural weakness due to meningeal diverticuli
d. Spontaneous CSF leaks most often occur at level of thoracic spine
e. Low CSF pressure results in traction on supporting structures (including pain-sensitive meninges and venous sinuses) resulting in pain, occasionally subdural hematoma (stretching of bridging veins), and rarely cerebral venous thrombosis
2. Clinical presentation a. Headache occurs with upright posture and is relieved
with supine posture 1) Typically bilateral (not always) 2) May be bifrontal, bioccipital, fronto-occipital, or
holocephalic 3) Over time, chronic daily headache may also evolve and
not worsen with positional changes or to a lesser extent b. Associated symptoms may include
1) Stiff neck (sometimes orthostatic) 2) Nausea and vomiting (often orthostatic) 3) Horizontal diplopia (unilateral or bilateral abducens
palsy) 4) Distortion of hearing
Table 18-20. Differential Diagnosis of Increased Intracranial Pressure in Absence of a Structural Lesion
5) Visual blurring 6) Photophobia 7) Visual field defect (superior binasal) 8) Dizziness 9) Back pain, radicular pain
10) Rare complications: subdural hematoma, coma, encephalopathy, dementia, parkinsonism
3. Diagnosis a. Clinical symptomatology b. Diagnostic criteria (Table 18-21) c. MRI of head with gadolinium may show findings
suggestive of low-pressure syndrome (Fig. 18-2) 1) Dural (pachymeningeal) enhancement 2) Downward displacement of brain: downward
displacement of cerebellar tonsils, caudal displacement of pons, effacement of pontine cistern, caudal displacement of optic chiasm and hypothalamus
3) Pituitary enlargement 4) Ventricular size may be decreased 5) Engorgement of venous sinuses 6) Subdural fluid collections
d. CSF 1) Opening pressure is typically very low and rarely normal
2) CSF protein may be normal or high (up to 100 mg/dL)
3) CSF leukocyte count may be normal or mildly elevated
e. Radioisotope cisternography 1) Indium-111 is placed into the spinal subarachnoid
space and movement followed with sequential scans for up to 24 to 48 hours
2) In normal patients, indium-111 can be detected over the cerebral convexities by 24 hours
3) In presence of CSF leak, indium-111 does not reach the convexities by 24 to 48 hours and appears early in kidneys and bladder
f. CT myelography 1) May detect region of spinal leak showing extradural
egress of contrast or meningeal diverticulum 2) Most common site of leak is thoracic spine
4. Treatment a. First-line symptomatic measures
1) Bed rest with hydration 2) Caffeine: effectiveness is questionable and short-lasting
Table 18-21. International Headache Society Criteria for Headache Attributed to Spontaneous Low CSF Pressure
3) Corticosteroids: anecdotal evidence only; partial, temporary improvement only
4) Intrathecal or epidural fluid infusions of crystalloids may be considered (potential for infectious complications)
b. Epidural blood patch (second-line therapy): may need more than one attempt
c. Surgery 1) Reserved for patients in whom conservative treatment
failed and area of spinal leak has been identified 2) Repair of dural defect or packing the defect with
fibrin glue or an absorbable gelatin sponge (Gelfoam) has been performed
1. A 38-year-old woman who takes oral contraceptives presented with a 2-week history of progressive dull headache, then sudden severe worsening. This is the worst headache she has experienced. The neurologic examination is unremarkable. Computed tomography (CT) of the head is unremarkable. Cerebrospinal fluid examination shows the following: leukocyte and no erythrocytes per high-power field; glucose, 50 mg/dL; protein, 40 mg/dL; and no evidence of xanthochromia. Opening pressure was 30 cm H2O. What is the next best management strategy? a. Obtain magnetic resonance (MR) venogram b.Treat patient with sumatriptan and discharge c. Admit patient for observation d.Obtain conventional angiogram to rule out cerebral
aneurysm
2. A female patient presents with headache that immediately worsens in the upright position and improves in the supine position. Also, she has neck pain, nausea, and vomiting when upright. Which of the following radiographic findings might you see on magnetic resonance imaging (MRI) of the brain? a. Pachymeningeal enhancement b.Engorgement of venous sinuses c. Cerebellar tonsillar ectopia d.All the above
3. Which of the following headache types is commonly associated with autonomic features? a. Exertional headache b.Hypnic headache c. SUNCT (short-lasting unilateral neuralgia form
headache with conjunctival injection and tearing) headache
d.Trigeminal neuralgia
4. A 60-year-old man with a history of coronary artery disease and hypetension has developed severe unilateral right retro-orbital pain that lasts 30 minutes at a time and occurs several times a day, but mainly at night. He has tearing of his right eye and nasal congestion. Secondary causes were ruled out. The best abortive treatment to try first in this patient is: a. Sumatriptan subcutaneous at onset of headache b.Oxygen 2 L/min by nasal cannula at onset of headache c. Dihydroergotamine injection at onset of headache d.Lithium carbonate 300 mg orally
5. A 25-year-old man presents with jabbing pain in the throat often triggered by chewing or yawning. In addition, he has had one episode of unexplained loss of consciousness. The headache syndrome described here is: a. Trigeminal neuralgia b.Geniculate neuralgia c. Glossopharyngeal neuralgia d.Occipital neuralgia
6. A serious, preventable complication of untreated pseudotumor cerebri is: a. Chronic daily headache b.Cerebral hemorrhage c. Venous thrombosis d.Visual loss
7. For a patient with rapidly escalating migraine headaches, the quickest acting oral medication may be: a. Rizatriptan b.Frovatriptan c. Propoxyphene d.Almotriptan
8. What is a common complication of nonoperative procedures for trigeminal neualgia (such as balloon compression and radiofrequency ablation)? a. Infection b.Facial weakness c. Anesthesia dolorosa d.Numbness in distribution of treated trigeminal
subdivision
9. What primary headache type is characterized by moderate bilateral head pain that occurs at night, resulting in awakening, and has no associated autonomic symptoms? a. Idiopathic stabbing headache b.Hypnic headache c. Cluster headache d.Exertional headache
10.The designation “post-traumatic headache” requires that the person suffered major head trauma with loss of consciousness. a. True b.False
1. Answer: a. Venous sinus thrombosis can present with acute headache, but it often has a subacute, progressive course. CT and neurologic examination findings can be normal. The high opening pressure in this situation should make you consider venous sinus thrombosis. MR venography is an appropriate next step.
