HEADACHE and FACIAL PAIN

Dr Yacoub Bahou MD
Professor of Neurology at the University
of Jordan
I) Introduction
II) Diagnosis
III) Primary headache disorders : migraine, tension-type
headache,trigeminal-autonomic cephalalgias
IV) Opioids in the treatment of headache
V) Secondary headache disorders: vascular, infectious,neoplastic,
traumatic, intracerebral pressure disorders, medication causes
VI) Head and neck disorders
VII) Trigeminal neuralgia
VIII) Summary
I) Introduction
Headache disorders are among the most prevalent medical problems
worldwide

The World Health Organization estimates that 50% to 75% of all adults
between the ages of 18 and 65 years have headaches

This high prevalence results in significant disability and lost

productivity: headache disorders are the 3rd highest cause of years lost
to disability worldwide
Accordingly, headaches are one of the most common reasons patients
present to physicians in primary care settings, in the emergency
department ( ER), or in neurologists’ offices

For clinicians evaluating a patient with headaches, the 1st responsibility

is to diagnose the type of headache correctly, and then treat
appropriately , because most headache disorders have excellent
treatments available, which can reduce the burden of disability
 Headache disorders fall into 2 categories, primary headache disorders
( those caused by the headache disorder itself, not due to other
causes) and secondary headache disorders, those caused by ( or
“symptomatic of”) another underlying medical problem

The pain can be due to the involvement of pain-sensitive structures in

the head , including cranial nerves, cervical nerve roots, blood vessels,
meninges, scalp, temporomandibular joint ( TMJ), teeth, pericranial
and cervical muscles, and paranasal sinuses
Patients may also have multifactorial headaches, so a detailed history
and examination are necessary to identify the contributing factors

Headache disorders may remain refractory to treatment or have an

insufficient response to treatment if these comorbidities are not
identified and addressed
II) Diagnosis

A detailed history and examination are vital in understanding the

headache’s cause

There are no biomarkers currently available for primary headache

disorders
 Key points from the history
When obtaining the history the following information must be elicited:
- Onset
- Precipitants and triggers
- Duration
- Location( unilateral or bilateral; frontal, lateral, vertex ,or occipital)
- Quality and severity
- Frequency
- Alleviating and exacerbating factors
- Positional influences ( better or worse when supine)
- Waking the patient from sleep, or occurring upon awakening
- Associated with menses
- Associated symptoms
 Additional aspects of the history important in evaluating a patient with
headache are:
- Analgesic use
- Caffeine use
- Medical history
- Current or recent pregnancy
- Medications( including asking specifically about contraceptive use,
over-the –counter treatments, and supplements)
- Social history, including detailed screening for illicit drugs
- Family history
- Sleep, including a history of insomnia and snoring, symptoms
suggestive of obstructive sleep apnea
 The semiology of the headache helps to differentiate a primary from a
secondary headache disorder

The history also allows a clinician to identify red flags that suggest a
secondary headache disorder
 RED FLAGS
- Acute onset or progressive worsening from baseline

- New or different headache

- Systemic symptoms: fever, weight loss

- Risk factors: malignancy, immunosuppression, IV illicit substance use,

hypercoagulability, including pregnancy

- Smoking

- Age more than 50 years, or no prior headache history

- Features of increased ICP: waking patient from sleep, worsening with
Valsalva maneuver, supine worsening of pain

- Focal features: seizures, mental status abnormality, cranial nerve

deficits, weakness, sensory changes( loss of sensation, paresthesias,
location of pattern of spread)

- Precipitants: trauma, newly prescribed medications, infection

Key points for the neurological examination
Patients with primary headache disorders usually have normal general
medical and neurological examinations, although an acutely
symptomatic patient with an autonomic cephalalgia may have signs
suggesting that disorder

Some patients with chronic headaches have findings of

temporomandibular joint (TMJ) tenderness on palpation, evidence of
dental wearing, or pain with palpation of the cervical muscles or the
occipital ridge to suggest comorbid causes of headache such as
cervicalgia
 In the era of the smartphone, patients may bring pictures of
themselves to a clinician for review if they have paroxysmal symptoms
and signs( such as ptosis or lacrimation), which can aid in the diagnosis

Patients should have a general medical and neurological examination to

assess for secondary causes of headache

Attention to vital signs is important: patients with significant

hypertension may be susceptible to developing certain secondary
headaches outlined below
Fever may suggest an underlying infection, including a CNS infection

A cardiovascular exam can evaluate for arrhythmia or carotid stenosis,

which can cause secondary headache syndromes

A detailed head and neck exam includes evaluating for nuchal rigidity,
cervical myofascial pain, occipital Tinel sign( evaluated by eliciting
tenderness or tingling when palpating near the occipital protuberance
along the occipital nerve), and palpation of the TMJ, assessment of
dental wearing or chipping to suggest bruxism, and observing the
oropharynx for narrowing that could suggest obstructive sleep apnea
 A full neurological exam should also be performed, with emphasis on
the funduscopic exam to assess for papilledema

The cortical sensory exam can suggest cortical dysfunction that may
occur with venous sinus thrombosis

Focal neurological deficits, including field cuts, cranial nerve palsies,

weakness, or sensory symptoms, often suggest a secondary headache
III) Primary headache disorders
Primary headache disorders are those not due to another medical
condition
Diagnosis is established by history and exam
Migraine is by far the most prevalent primary headache disorder
The table outlines common primary headache disorders based on key
features of the history
The figure shows the common locations of pain in the primary
headache disorders, compared to that of headaches caused by sinus
disease
* MIGRAINE
A) Migraine without aura
It is estimated that 1 in 7 adults worldwide has migraine

It impacts women more than men in a 2:1 ratio

Migraine may start in childhood and manifest occasionally with

abdominal symptoms( “ abdominal migraine”)

Motion sickness in children is a risk factor for the development of

migraine
Migraine headaches are most likely to develop in adolescence and early
adulthood

They can be episodic or chronic

The disability and lost productivity from migraine are substantial,

because it impacts people in their prime working years
Migraine has numerous identified triggers, including weather changes,
menses and caffeine( both withdrawal and overuse)

Many patients identify foods and drinks such as alcohol( most

commonly red wine), soft cheeses, and nitrite-heavy foods, such as
processed meats, as precipitants, but data are sparse in this area and
many migraine attacks occur without identifiable triggers
To diagnose migraine, a patient must have at least 5 attacks with the
following characteristics:
1. The headache lasts for 4 to 72 hours if untreated
2. It must include at least 2 of the following features:
a. Throbbing
b. Unilateral headaches
c. Worsening with activity, such as walking
d. Moderate to severe pain
3. It must be associated with at least one of the following:
a. Nausea, vomiting, or both
b. Photophobia and phonophobia
B) Migraine with aura
Migraine headaches are often preceded by focal neurological
symptoms known as auras

These are also called classic migraine or complicated migraine

Auras are defined as fully reversible neurologic symptoms with a

gradual onset, usually followed by a headache

The aura usually lasts 5 to 60( often 20) minutes and is typically
unilateral

It usually resolves without lingering neurologic deficits

 Patients are diagnosed with this disorder when they have an aura
followed by a headache that meets the criteria for migraine, as above

Some auras occur without a headache ( “ acephalgic migraine”), but

these symptoms usually require additional investigation for a definite
diagnosis

Visual auras are by far the most common

Some include a” fortification” spectrum( zigzag lines off the central

vision, usually spreading gradually) or a scintillating ( or flickering)
scotoma ( an area of decreased visual acuity surrounded by preserved
vision)
Migraine auras can also involve the sensory symptoms, most commonly
paresthesias ( tingling or pins-and-needles sensation)

The paresthesias often “ march” or spread gradually over the course of

several minutes along a limb or extend from an arm to the leg or face

Migraine auras can also include a gradual onset of weakness, a variant

known as hemiplegic migraine when severe

Hemiplegic migraine may be sporadic but there is also a syndrome of

familial hemiplegic migraine, sometimes associated with well-
characterized genes
Migraine auras are believed to be due to “ cortical spreading
depression” in which there is a spread of hyperpolarization of the
cortex followed by a wave of depolarization

Imaging studies have shown decreased regional cerebral blood flow in

the cortex during migraine aura, but not to the level of worrisome
ischemia
C) Complications associated with migraine
*Status migrainosus
When migraine lasts for more than 72 hours, the condition is known as
status migrainosus

This is often caused by abortive medication overuse ( often referred to

as rebound headache) and frequently requires intravenous ( IV)
treatment or a brief course of oral steroids to break the headache cycle
* Stroke risk associated with migraine

Patients with migraine with aura have an increased cardiovascular risk

when compared to healthy controls

The use of oestogen-based contraceptives is therefore contraindicated

in patients with migraine with aura, as the combination results in a
substantially increased stroke risk
 * Migraine and menses
Women of reproductive age frequently have exacerbation of migraine
during menses, most commonly 1 to 2 days prior to onset of bleeding,
often persisting for up to 3 days into bleeding

This is thought to be due to the withdrawal of estrogen that occurs

with menses

Some women have migraine at the time of menstruation only, a

condition known as pure menstrual migraine
Most , however, have a few episodic headaches at other times of the
month, or menstrually related migraine

It is important to identify the relationship of menses to migraine

because there are specific treatments that may be helpful for patients
with a clear exacerbation around their menses
 * Chronic migraine
Patients who have a headache more than 15 days/month for more than
3 months are diagnosed with chronic migraine

Some patients with chronic migraine do not have typical features of

migraine with all headaches, but they must have at least 8 days of
headache consistent with migraine to be diagnosed with chronic
migraine

If the headaches are not consistent with migraine, other diagnoses

must be considered
 Patients often describe a history of gradually progressive episodic
migraines that increase in frequency to the point of meeting criteria for
chronic migraine

With frequent headaches, many patients with chronic migraine have

some component of medication overuse headache ( MOH)

Importantly, patients with chronic migraine can revert to episodic

migraine after effective treatment
 D) Migraine treatments
1. Abortive treatments
Abortive treatments, also called rescue medications, are
medications used to stop a migraine at the onset

All abortive treatments are most effective if the patient is treated at

the onset of the headache

Delay in treatment results in more prolonged disability, so patients

must be counseled on the appropriate use of abortive treatments
 Nonsteroidal anti-inflammatory drugs ( NSAIDs) and triptans
(serotonin 1b/1d agonists) are the mainstay of abortive treatments

Many patients respond to NSAIDs alone

For some patients, however, they are insufficient; some patients have
contraindications to using NSAIDs. In these cases, triptans can be highly
effective

There are numerous different types of triptans , with different rates of

onset of action and half-lives
 There are 2 long-acting triptans ( naratriptan and frovatriptan) and 5
fast-acting triptans ( almotriptan, eletriptan, sumatriptan,rizatriptan,
and zolmitriptan)

There are also numerous different formulations, including oral pills,

disintegrating tablets, nasal sprays, and injectables

Historically, ergotamines were prescribed as abortive treatments, but

they carry a higher cardiovascular risk and have been replaced by
triptans
 Triptans and NSAIDs can be combined when needed and may have a
synergistic effect in treating migraine pain

Caffeine is also often added to many migraine treatments because it

can help abort the pain; many over-the-counter “ migraine
preparations” contain caffeine

Triptans are currently not known to be safe in pregnancy and have a

cardiovascular risk
 Triptans also interact with selective serotonin reuptake inhibitors and
serotonin-norepinephrine reuptake inhibitors , with a low risk of
serotonin syndrome ( recent medications: “ditans” which are 5HT1F
agonists and “gepants” which are CGRP blockers)

Patients must be counseled on side effects of all treatments

Identifying the right abortive treatment requires careful consideration

of the patient’s headache features , comorbidities, concurrent
medication use, cost, and family planning goals
 Using abortive treatments on a chronic basis more than twice a week
can result in medication overuse headache (MOH), so patients should
be counseled to not use any of these treatments chronically more than
twice a week to prevent this complication
 2. Adjuvant treatments
Because nausea and emesis are frequently associated with migraine,
many patients benefit from antiemetics
Interestingly, prochlorperazine and metoclopramide are more effective
than ondansetron, both in alleviating the nausea and in reducing the
severity of the pain
Antiemetics may also be useful in preventing patients from vomiting
their abortive therapies
They are frequently used in emergency room ( ER) and urgent care
settings for patients with severe or refractory migraine
They are often combined with ketorolac and diphenhydramine for
patients with status migrainosus
3. Preventive treatments
Preventive treatments, also called prophylactic treatments, are used for
patients with chronic migraine or frequent and disabling headaches
that do not respond sufficiently to abortive treatments

Preventive therapy aims to reduce the frequency and severity of

migraine, although patients are unlikely to become completely
headache-free and should be counseled accordingly

All prophylactic treatments take some time to have an effect ;patients

should remain on treatment for at least a month ( barring significant
side effects or other concerns) before assuming that the treatment is
ineffective
There are 3 primary categories of preventive oral medications:
antihypertensives, antiseizure medications , and antidepressants

Within each category, there are specific drugs with the most evidence
of efficacy( table)

In addition to oral therapies, onabotulinum toxin A ( often referred to

simply as Botox) was also approved as migraine prophylaxis for chronic
migraine in 2010
 In 2018, a new class of preventative therapy for chronic migraine ,
Calcitonin Gene –Related Peptide ( CGRP) antagonists was approved by
the FDA

Erenumab is an injectable human monoclonal antibody that

antagonizes CGRP receptor function
 As with abortive treatments, selecting the right prophylactic
medication requires careful consideration of the patient’s
comorbidities , concomitant medications, cost , and family planning
goals

Patients must be counseled about treatment options and side effects,

including teratogenicity and impact on contraceptives

Patients who require preventive therapies also require abortive

treatments

Some abortive treatments interact with prophylactic medications ( such

as antidepressants and triptans) which should be taken in consideration
 4. Lifestyle modifications
Lifestyle factors are important to identify

A comorbid sleep disorder ( insomnia, obstructive sleep apnea …)

makes patients more susceptible to migraine

Skipping meals, insufficient fluid, excessive caffeine intake, and lack of

exercise make susceptible patients more prone to migraine attacks

Patients should be counseled on these factors

 * TENSION-TYPE HEADACHE

Tension-type headache ( often referred to as tension headaches, stress

headaches, or ordinary headaches) are the next most prevalent primary
headache disorders, occurring in 30% to 70% of adults worldwide

Pain is usually bilateral and described as pressure or tightness

It is usually mild to moderate and lasts for under an hour to several days

Unlike migraine, it is not associated with photophobia, phonophobia,

nausea, or vomiting

The examination is generally normal , but some patients have pericranial

tenderness to palpation of the scalp, neck, or shoulder muscles
 Tension-type headaches can be episodic or chronic( occurring more
than 15 days/month)

Interestingly, patients with infrequent tension-type headaches

generally do not seek medical attention, because they do not have
significant disability from their symptoms

Patients with frequent or chronic tension-type headaches benefit from

treatment
 Tension-type headache treatment
1. Abortive treatments
Many patients with tension headaches do not require abortive treatments
because the pain is generally mild and does not interfere with the patient’s
functioning

For those with moderate to severe pain, NSAIDs are the mainstay of
treatment

Aspirin and acetaminophen may also be used, but the latter is often less
effective than NSAIDs

Patients should be counseled about the development of MOH and advised to

not use analgesics more than twice a week for long periods
2. Preventive treatments
Antidepressants are the first-line preventive therapy for chronic tension
headache

The tricyclic amitryptiline is the most studied to date and has good
evidence for efficacy

Other antidepressants , including mirtazapine and venlafaxine are

second-line therapies

Muscle relaxants such as tizanidine are helpful sometimes, particularly

in patients with a cervicogenic component
3. Adjuvant treatments
Tension headaches are often reported to be triggered by stress
(physical or emotional); addressing these triggers, if chronic, is
important

Biofeedback ( a mind-body technique used to teach patients greater

body awareness and how to control some physical reactions to pain
and stress) can be effective

Poor posture and neck muscle spasm are also frequent contributors to
chronic tension-type headaches, and physical therapy can help
 * TRIGEMINAL AUTONOMIC CEPHALALGIAS
Trigeminal autonomic cephalalgias ( TACs) are the 3rd major category of
primary headache disorders

They are characterized by unilateral pain associated with cranial

autonomic symptoms

The diagnosis is made by careful evaluation of the pattern of the pain

and its associated features ( table shown on a previous slide)
A) Cluster headache
Cluster headaches are severe headaches characterized by unilateral
pain involving the orbit, supraorbitally, at the temple, or combination
of these( previous figure)
Cluster periods are bouts of recurrent attacks of pain, generally lasting
weeks to months
These periods are followed by remission lasting anywhere from
months to years
The pain is often excruciating
During an attack, patients are often restless and pacing, unlike in
migraine where activity exacerbates the pain
 The pain must be associated with one of the following cranial
autonomic symptoms:
- Conjunctival injection, lacrimation, or both
- Nasal congestion, rhinorrhea, or both
- Eyelid edema
- Forehead and facial sweating or flushing
- Sensation of fullness in the ear
- Miosis, ptosis, or both
Cluster headaches typically last between 15 and 120 minutes

During a cluster period, headaches can occur several times a day or as

infrequently as every other day

Cluster headaches are relatively uncommon but are 3 times more likely
to occur in men

The age of onset is typically in early to mid-adulthood ( 20-40 years)

The cause is unknown, but activation of the posterior hypothalamic

gray matter has been seen in some patients during attacks
Alcohol, histamines, and nitroglycerine are triggers in susceptible patients

A Horner syndrome caused by carotid dissection may mimic a cluster

headache ( figure) but does not usually have the pain characteristics of this
primary headache disorder

Cluster headaches may be episodic or chronic

Chronic cluster headache is defined as intractable cluster headaches with

less than 1 month of remission before the recurrence of symptoms

Fortunately, less than 15% of cluster patients have chronic cluster

Cluster headache treatments
1. Abortive treatments
A first-line abortive treatment for cluster headaches is 100% oxygen,
delivered at 12 to 15L/min

Patients may receive this treatment in an urgent care or ED setting and

if effective, be prescribed a home oxygen tank

For patients who do not respond , or who do not have access to home
oxygen, triptans are prescribed
Sumatriptan and zolmitriptan are effective as abortive therapies

In the past, dihydroergotamine ( DHE) was prescribed as an abortive

treatment , but triptans are favored over DHE given their safety profile

Patients with cluster headache are very susceptible to MOH and must
be counseled appropriately

Occipital nerve block can also be effective to abort a cluster cycle

2. Preventive treatments
Preventive treatments for cluster headache are similar to those used
for migraine and include antihypertensive,antiseizure, and psychiatric
medications

Verapamil is the first-line therapy for cluster headache prophylaxis

If not tolerated or if there are contraindications, glucocorticoids

( prednisone or dexamethasone) are also effective

Lithium and topiramate are often used as second-line agents or as add-

on therapy when needed
B) Short-lasting unilateral neuralgiform headache attacks
Short-lasting unilateral neuralgiform headaches are unilateral,
moderate to severe headaches

Pain is around the orbit or temple but may also occur in the trigeminal
distribution and therefore be mistaken for trigeminal neuralgia

The headache is a stabbing pain or recurrent stabbing sensation lasting

from 1 second to 10 minutes

Patients with a lesion in the posterior fossa may present with

symptoms suggestive of short-lasting neuralgiform headache so brain
imaging with MRI is important to establish that this is a primary and
not secondary headache disorder
 There are 2 forms, differentiated by the types of associated autonomic
symptoms ( which always occur on the same side as the headache):
- Short-lasting unilateral neuralgiform headache attacks with conjunctival
injection and tearing( SUNCT): Autonomic symptoms include both
conjunctival injection and lacrimation
- Short-lasting unilateral neuralgiform headache attacks with cranial
autonomic symptoms( SUNA):
Autonomic symptoms include at least one of the following:
* forehead or facial sweating or flushing
* Ptosis or pupillary miosis
* Eyelid edema
* Nasal congestion, rhinorrhea, or both
* Ear fullness
* Either conjunctival injection or lacrimation , but not both
Both SUNCT and SUNA can be episodic or chronic

The chronic forms are diagnosed by persistent symptoms lasting more

than a year, or for less than a year but less than 1 month of remission
 1. Abortive treatment
SUNCT and SUNA are challenging disorders to treat, given the brevity
of symptoms
Intravenous lidocaine has helped abort the cycle in some patients

2. Preventive treatment
Antiseizure medications including topiramate, gabapentin, and
lamotrigine are used as preventive therapy in patients with frequent or
recurrent symptoms
Occipital nerve blocks can also be helpful, especially when systemic
medications are contraindicated or not tolerated
C) HEMICRANIA
The final TAC is hemicrania, a unilateral headache , differentiated from
the other TACs both by the duration of symptoms and by its unique
response to indomethacin

Patients present with a unilateral orbital or temporal headache

associated with one or more autonomic symptoms on the same side as
the headache( the symptoms and signs are the same as SUNCT and
SUNA)
There are 3 variants of hemicranias, differentiated by the duration of
symptoms:
- Episodic paroxysmal hemicranias:
* Recurrent attacks separated by at least one pain-free month
* Attacks last between 2 and 30 minutes but can recur within a day
- Chronic paroxysmal hemicranias:
* Recurrent attacks without remission, or less than 1 month of
remission before recurrence
* Attacks last between 2 and 30 minutes but can recur within a day
- Hemicrania continua: Intractable pain and autonomic symptoms
consistent with hemicranias, lasting for more than 3 months
All 3 forms respond to indomethacin, and this response to treatment is
required to make the diagnosis

Hemicrania is more common in women and typically occurs in mid-

adulthood (30-40 years of age)

As with other TACs , MRI to exclude a lesion in the posterior fossa is

also advised to exclude a secondary headache syndrome
 Abortive and preventive treatment
Indomethacin is the definitive treatment for hemicranias

An indomethacin trial is both diagnostic and therapeutic

The dose is titrated gradually over 10 days to a maximum of 225 mg a

day, divided into 3 doses, until the patient has a therapeutic response

If there is no response, the diagnosis is not consistent with hemicranias

and other etiologies must be considered
IV) Opioids in headache treatment
Opioids are not more effective than alternative therapy and are
generally strongly discouraged for use in headache medicine

Most headache conditions are recurrent disorders , so to use opiods in

this setting risks development of a secondary opioid use disorder

Opioids also tend to cause MOH

V) SECONDARY HEADACHE DISORDERS
Secondary headache disorders are headaches caused by a medical
condition or medication
They have a broad differential for causes, ranging from preeclampsia
and pheochromocytoma to fever and medication side effects
Most secondary headache disorders are associated with other features
in the history, examination, or laboratory assessment, which aid in the
diagnosis
Treatment is based on addressing the underlying disorder
There are 6 major categories of secondary headaches that may present
with headache only and must be considered
1. Vascular causes
There are numerous vascular causes of headache

All cerebral hemorrhages can cause headache

This includes subarachnoid hemorrhage( SAH) , intraparenchymal

hemorrhage, and subdural and epidural hematomas

These hemorrhages may be spontaneous ( associated with stroke or

hypertension) or traumatic
 Patients with intracerebral hemorrhages typically present with what is
referred to as thunderclap headache; the onset is abrupt and severe

Emergency imaging , usually with a noncontrast CT Brain scan, is

needed to evaluate abrupt-onset headache( figure)

Cerebral vessel imaging is also warranted if a SAH is identified , to

assess for an aneurysm
Ischemic strokes are often associated with headaches

Their semiology is nonspecific, but typically abrupt in onset

Patients may have focal neurologic deficits which aid in the diagnosis

Cerebral thrombosis, either arterial or venous, can also cause headache

Patients with venous sinus thrombosis often have headaches with features of
increased intracranial pressure( ICP)

Thrombosis should be considered particularly in patients with

hypercoagulability states, including pregnancy

The diagnosis is made on imaging, including that of cerebral vessels (figure)

Cerebral vasculitis frequently causes a nonspecific headache

When part of a systemic vasculitis, it is considered a secondary angiitis

If the vasculitis occurs in the cerebral vessels alone, it is referred to as

primary CNS angiitis

In addition to headache, patients often have paroxysmal focal

neurological deficits

Cerebral arterial vessel imaging and lumbar puncture ( LP) are often
required to make this diagnosis
 Giant cell arteritis ( GCA) , also called temporal arteritis, is a peripheral
cranial arterial vasculitis that often presents with unilateral headache

Patients are generally above the age of 50 and report additional

symptoms including vision changes( amaurosis fugax), jaw claudication,
fever, and scalp tenderness

Involvement of the branches of the external carotid artery, and

occasionally the ophthalmic artery, can result in blindness if not readily
identified and treated promptly
 Patients usually have elevated inflammatory markers( erythrocyte
sedimentation rate and C-reactive protein)

Empiric steroids should be started in any patient with a high clinical

concern

Temporal artery biopsy is the gold standard, but GCA can cause “ skip
lesions” and may require serial biopsies to identify the pathology
2. Infectious or inflammatory causes
Intracranial infections , such as encephalitis and meningitis, usually
present with headache and often fever

They may also have nuchal rigidity and altered mental status

As the infection progresses , seizures and focal neurological deficits

may occur

Infections can be bacterial, viral , fungal, or parasitic; the headache

semiology does not help to differentiate the underlying cause
 LP is the crucial diagnostic test and necessary in any patient for whom
there is concern for a CNS infection

There are often other signs of infection, and the headache has a
temporal correlation to the infection

CNS inflammatory and autoimmune conditions such as sarcoidosis and

lupus frequently present with headache
3. Neoplastic causes

Intracranial neoplasms may present with headaches, especially when

there is significant mass effect

The headache semiology may be nonspecific but may have features of

intracranial hypertension, including wakening the patient from sleep ,
being worse when supine, and worse with Valsalva maneuver

The headache may occur early or late with neoplasms and with any
type of primary cancer
4. Traumatic causes

Head and neck injuries often result in headache

To be attributed to trauma, the headache must develop with a

temporal association with the injury

The severity of the injury does not necessarily correlate with the
severity of the headache; even minor head injuries or whiplash may
cause headaches
There is no specific headache semiology that helps with the diagnosis

In patients with a history of significant head or neck injury, it is

important to assess for intracranial hemorrhage or dissection of
cervical vessels- which may require additional treatment
5. Intracerebral pressure disorders
Intracranial hypertension and hypotension can cause headaches, but
with markedly different semiologies
A) Intracranial hypertension
Intracranial hypertension may be “ idiopathic”( most common in obese
young woman) or due to medications or systemic disorders

The headache is often described as worse when supine or sleeping

( awakening the patient from sleep) , or with Valsalva maneuver

Patients often have associated features , including papilledema,

pulsatile tinnitus, or visual symptoms
 Patients should have imaging to exclude a mass lesion or venous sinus
thrombosis

If the imaging is unrevealing , the diagnosis is made with an LP when

the patient is in the lateral decubitus position with legs extended

Intracerebral pressure ( ICP) is elevated above 200 mm CSF

Acetazolamide is the first-line treatment for idiopathic intracranial

hypertension ( IIH)

Patients require monitoring of their visual fields, and treatment for

obesity is warranted when present
B) Intracranial hypotension
Intracranial hypotension may be spontaneous or traumatic
The headache improves when the patient is supine but worsens with
standing
The pain is often most severe at the vertex and can be associated with
neck pain or tinnitus
It is frequently traumatic- occurring after an attempted epidural
puncture or LP
Generally, the dural leak causing the headache heals gradually, without
intervention,but when symptoms persist or when the headache
etiology is unclear, brain MRI may be helpful ;it may show evidence of
sagging( figure)
The definitive diagnosis is made with an LP showing an opening
pressure below 60 mm CSF

If a patient remains symptomatic from intracranial hypotension, a

blood patch may be attempted to cover the dural leak( if it can be
found)
6. Medication causes
Medications associated with headaches are numerous

They range from hormonal therapies, including contraceptives, to nitric

oxide

Withdrawal of a medication or other treatment may also cause a

headache; the most common example is a caffeine withdrawal
headache

Headache semiology is nonspecific, but the temporal association to

medication change helps establish the diagnosis
Medication overuse headache ( MOH), also referred to as rebound
headache or drug-induced headache, is a chronic headache occurring
in patients with a primary headache disorder

The baseline headache disorder is typically markedly exacerbated i.e.,

more frequent, severe, or both) when the medication is overused

MOH is diagnosed when patients use an abortive therapy 10 or more

times per month for more than 3 months with an increase in
headaches
VI) Head and neck disorders
There are many different structural disorders that can cause headache

Treatments are based on the specific causes identified, and these disorders
may occur simultaneously with other primary or secondary headache
disorders

Sinusitis is commonly associated with a headache

It may be bifrontal or unilateral

Acute sinusitis is often associated with other symptoms of a respiratory

tract infection
Temporomandibular joint disorder ( TMD) is another common cause of
headache and may be unilateral or bilateral

On exam, there may be evidence of dental wearing( chipped and

flattened teeth) and discomfort on palpation of the joint

Cervicogenic headaches are also common and may be identified by

palpation of myofascial trigger points in the neck

Cervical range of motion is often reduced

VII) Trigeminal neuralgia
The pain of trigeminal neuralgia is shock-like, occurring in one or all branches
of the trigeminal nerve

The pain is usually paroxysmal and recurrent

It may be triggered by common activities such as brushing hair or teeth

It may be idiopathic or due to structural causes such as a mass or vascular

lesion, or a demyelinating lesion of multiple sclerosis

Carbamazepine is a common first-line treatment

VIII) SUMMARY
Migraines, tension headaches, and TACs are the 3 most common types
of primary headache disorders

The International Headache Society maintains an evidence-based

categorization of primary and secondary headache disorders

If a headache history and pattern is not consistent with a primary

headache disorder, the clinician should consider a secondary headache
disorder, review the rare types of primary headache disorders, or
consider that the headache may be a combination of more than one
headache disorder