TITLE: Headache
and Facial Pain
SOURCE: Grand Rounds Presentation, UTMB, Dept. of Otolaryngology
DATE: January 31, 2001
RESIDENT PHYSICIAN: Robert H. Stroud, M.D.
FACULTY PHYSICIAN: Byron J. Bailey, M.D.
SERIES EDITOR: Francis B. Quinn, Jr., M.D.
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"This
material was prepared by resident physicians in partial fulfillment of
educational requirements established for the Postgraduate Training Program of
the UTMB Department of Otolaryngology/Head and Neck Surgery and was not
intended for clinical use in its present form.
It was prepared for the purpose of stimulating group discussion in a
conference setting. No warranties,
either express or implied, are made with respect to its accuracy, completeness,
or timeliness. The material does not
necessarily reflect the current or past opinions of members of the UTMB faculty
and should not be used for purposes of diagnosis or treatment without
consulting appropriate literature sources and informed professional
opinion."
Each
of us is likely to have experienced headache either sporadically or
chronically. Indeed, it is estimated
that 40% of the worldwide population suffers with severe, disabling headache at
least annually. It is well that such a
common ailment usually has a benign course, but headache may be the presenting
symptom of life-threatening disease.
Given this, and the frequency that the complaint is encountered in ENT
practice, we as otolaryngologists should be comfortable with the evaluation,
diagnosis and treatment of headache and facial pain.
Brain
parenchyma itself is not pain sensitive, but the meninges and supporting
structures are heavily innervated. Pain
may be elicited when these structures are inflamed or distorted. In the case of migraine, it is believed that
activation of trigeminovascular structures is the source of the headache. Processes inside the cranial vault may
produce pain referred to areas of the jaw, neck, face and scalp. Pathology within the anterior or middle
cranial fossae may elicit pain that is referred to the scalp or face anterior
to the coronal suture. Lesions in the
posterior fossa cause pain in the more posterior portions of the head and upper
neck. Pain arising from processes in
the sphenoid or sella is commonly referred to the vertex. Simultaneous anterior and posterior headache
may arise from lesions of the incisura, but are more commonly seen with
generalized intracranial disease such as meningitis or subarachnoid hemorrhage.
Much
attention has been to given to the sinuses and nose as a source of acute and
chronic head and face pain. It is
therefore worth a brief review of sinonasal innervation. The general sensory innervation of the
mucosa of the nose and paranasal sinuses is from the ophthalmic and maxillary
branches of the trigeminal nerve, with minor contributions from the greater
superficial petrosal branch of the 7th nerve. Pain arising from processes within the
sinuses is therefore frequently referred to the corresponding cutaneous
dermatome innervated by the 5th nerve, or to the auricular or
periauricular region due to the contributions of the 7th nerve. The mucosa of the sinuses is rather
insensitive to pain, and the turbinates are more sensitive than the nasal
septum. The region of the ostia of the
sinuses is the most sensitive of any of the nasal structures.
Frontal
sinus innervation is derived from the ophthalmic branch of the trigeminal
nerve. Irritation of the mucosa of the
frontal sinus therefore produces pain in the forehead or anterior cranial fossa
related to the dural and cutaneous distribution of the nerve. The ophthalmic division of the 5th
cranial nerve also innervates the anterior ethmoid air cells via the anterior
ethmoidal nerve, a branch of the nasociliary.
The anterior ethmoids may also receive some innervation from the small
supraorbital branch that supplies the frontal sinus. The anterior ethmoid nerve has the largest territory of
innervation within the nasal cavity and supplies the anterior septum and
lateral nasal wall, including the superior, middle and inferior turbinates and
middle meatus.
The
posterior ethmoid air cells and sphenoid sinus derive most of their sensory
innervation from the maxillary division of the 5th cranial nerve via
the inconsistently present posterior ethmoid nerve. The maxillary division supplies the posterior septum and a large
portion of the superior and middle turbinates as well. Some of the innervation of the territory of
the posterior ethmoid and sphenoid sinuses is derived from branches of the
greater superficial petrosal branch of the 7th cranial nerve, and
the ophthalmic branch of the trigeminal nerve.
The maxillary sinuses are innervated by the posterior superior alveolar,
infraorbital and anterior superior alveolar nerves, all of which are branches
of the maxillary division of the trigeminal nerve.
Otalgia
is a frequent complaint among patients with head and face pain. While ear pain may be due to processes
arising within the ear, it may be referred from a distant process and manifest
as ear pain. This is a well-known
association, but it should be kept in mind when evaluating patients with
headache or facial pain. A brief review
of the innervation of the ear is warranted.
Sensory innervation of the ear is derived from five sources – cranial
nerves V, VII, IX and X as well as the cervical plexus. Pain arising from the distributions of any
of these nerves may be referred to the ear.
Pathology of the anterior tongue or oral cavity (including the
temporomandibular joint) may cause otalgia via the third division and
auriculotemporal branch of the trigeminal nerve. Lesions of the tongue base and tonsillar fossa can cause ear pain
through the petrosal ganglion and Jacobson’s branch of the glossopharyngeal
nerve. Lesions of the hypopharynx and
supraglottic larynx may cause ear pain by stimulation of fibers of the jugular
ganglion and Arnold’s branch of the vagus nerve.
The
most important information in the accurate diagnosis of headache and facial
pain comes from the patient’s history.
This may require a redirected or more targeted history after more common
causes are excluded. Indeed, the
otolaryngologist frequently must first reverse a self-diagnosis of “sinus headache”
reached by the patient before a more thorough history can be attained and more
accurate diagnosis reached. As Acquadro
and Montgomery point out, the evaluation of headaches can be quite
time-intensive, requiring diagnostic and treatment modalities foreign to most
otolaryngologists. In fact, there is a
growing number of physicians who focus their practice on the diagnosis and
treatment of headache. For many
diagnoses however, the otolaryngologist may elect to treat the patient himself,
remain a consultant with recommendations to the primary care physician or refer
the patient to a headache specialist.
Regardless of who eventually leads the patient’s healthcare team, there
is an increasing emphasis on a multidisciplinary approach. The more experience a clinician gains in
obtaining a history for headache, focusing on pertinent symptoms, the more
skilled the clinician will become in diagnosis.
A
complete history begins with determining the age when the patient first
experienced the headache. The events
occurring to the present should be recorded as well. The rate of onset and offset, intensity, quality, location,
duration and response to treatments should be recorded. The frequency and timing of attacks may hold
important information. The patient’s
own description of the quality of the pain may aid determining its source. Pressure-like pain is common in viscous or
chamber-derived pain, while sharp and shooting pains are more characteristic of
neuritic pain. Throbbing pain is
typical in vascular headaches. Burning
and aching suggests muscular pain.
Any
associated symptoms – nausea and vomiting, fever, diplopia, syncope,
hiccoughing, lacrimation, nasal congestion, photophobia, phonophobia, diarrhea,
polyuria – should be noted. Does the
patient experience any warning or aura prior to the attack? Any precipitating factors such as head
movements, eating, relation to sleep, foods, stress, menses, weather,
medications or alcohol should be recorded.
Factors providing relief may not only guide diagnosis, but treatment as
well.
A
thorough past medical history should include inquiries as to prior head
injuries, intracranial infections or processes and past surgeries. Any past or present medical and psychiatric
illnesses should be documented. Inquiry
should be made as to current medications including the type and frequency of
use of over-the-counter analgesics, oral contraceptives, herbal medicines and
topical agents used on the head and face.
Special attention should be paid to antihypertensives and vasodilators. A complete family and social history with
amount and duration of tobacco, alcohol and recreational drug use should be
noted. Life stressors should be
assessed. A complete review of systems
is mandatory in the complete evaluation of head and face pain.
The
physical examination in patients with headache or facial pain is guided by the
history. A complete head and neck and
neurological examinations are mandatory.
Special attention should be placed on the evaluation of the cranial
nerves, with an evaluation of the eyes including fundoscopy. Limited or asymmetric jaw opening should be
noted, as should any crepitus in the temporomandibular joint (TMJ) with opening
and closing of the mouth. The teeth are
carefully inspected for abrasions and the tongue for scalloped edges indicative
of bruxism or tongue compression against the dentition at night. The muscles of mastication should be
carefully palpated for tenderness. The
temporal arteries should be palpated for tenderness or nodules. Trigger points within the trapezius or
posterior cervical triangle should be sought.
The patient’s posture and range of motion of the neck are
evaluated. The occipital notch and scalp
are palpated and percussed for differential tenderness indicative of occipital
neuralgia or underlying subdural process.
A positive Romberg test is a relatively common finding in this
population. In the event of a positive
test (instability on standing with the eyes closed), the test is repeated after
asking the patient to try to recognize a number traced on the forehead with the
eyes closed. Improved performance may
indicate that the positive test is of “psychogenic” rather than neurologic
origin.
After
the complete history and physical examination, laboratory tests may be ordered. The necessary and sufficient evaluation of
the headache and facial pain patient is the matter of some debate. In general, the tests requested should be
guided by the history and physical examination findings. In the patient with recent onset headache of
moderate to severe intensity and findings on the neurologic examination, EEG
may be the test of first choice. EEG is
also helpful in the patient with muscle contractions in whom epilepsy is
suspected. Radiographic studies (CT and
MRI) are useful to evaluate new onset moderate to severe headaches, or to rule
out intracranial pathology in patients with long-standing, chronic headache or
facial pain. Lab tests directed to rule
out autoimmune disease or other systemic disease should be ordered if the history
suggests a generalized process. EMG may
be useful if a diagnosis of primary muscle disease or neuropathy is
suspected. Tenderness of the TMJ may be
further evaluated by radiographic imaging of the joint. Postural disorders with muscle pain and trigger
points in the neck and shoulder should be evaluated with cervical spine
radiographs.
Psychometric
testing may be of benefit in the evaluation and treatment of the patient with
headache and facial pain. Many tests
have been applied, but probably the most widely used is the Minnesota Multiple
Personality Inventory (MMPI). While
especially useful in the evaluation of the chronic headache and facial pain
patient, a thorough discussion of psychometric testing is beyond the scope of
this discussion and mentioned here only for completeness.
Tension-Type Headache
Tension-type headache (TTH) is the most common type of headache. It occurs in 69% of men and 88% of women over a lifetime and the annual prevalence is 63% in men and 88% in women. TTH can be further distinguished as “episodic” TTH (ETTH) or “chronic” TTH (CTTH). The distinction is made largely on frequency of occurrence – less than 15 days a month for ETTH and greater than 15 days a month for CTTH. The diagnostic criteria that distinguish TTH from other headache syndromes are largely related to the quality, intensity, location and duration of the pain. Headaches last from 30 minutes to 7 days. They are often described as pressing or tightening (non-pulsating) in quality. The intensity is mild to moderate and may limit but not prohibit activities. Its location may be bilateral or variable. There is no aggravation with physical activity, nausea and vomiting is rare, and photophobia or phonophobia may occur though not simultaneously.
Patients who acknowledge the role of stress in the
etiology of their headaches, especially those with ETTH, are frequently well
managed by biofeedback and stress reduction techniques. Posture correction and physical exercises
should be prescribed as indicated.
Patients with bruxism may benefit from a dental splint. For patients with ETTH, medications may be
avoided, but when needed, the patient may do well with low dose benzodiazepines
or amitriptyline once daily in a short course spanning several weeks. Pharmaceuticals are more likely to be
necessary in the patient with CTTH.
Abortive medications include aspirin, acetaminophen,
aspirin-caffeine-butalbital or phenacetin combinations or short half-life
non-steroidal anti-inflammatory medications (NSAIDs). Preventive medications include daily antidepressants, muscle
relaxants and long half-life NSAIDs.
Opiates and benzodiazepines may be effective but prolonged use is contraindicated. Daily NSAID use should be limited to less
than one week. The treatment regimen
employed must be individualized based upon the triggering factors elucidated in
the history and physical exam findings.
Migraine
Migraines are perhaps the most studied of the
headache syndromes. This is due in part
to the high incidence and significant loss of productivity and limitation on
quality of life suffered by those with the syndrome. It is estimated that 17% of females and 6% of males have migraine
headaches. Onset is usually in the
second or third decade. Migraines are
characterized by headaches of moderate to severe intensity located unilaterally
with a pulsating quality. Attacks last
from 4-72 hours (2-48 hours in children less than 15 years old) and are
aggravated by routine physical activities.
In order to meet diagnostic criteria, there must be nausea, vomiting,
photophobia or phonophobia. Migraines
may occur with or without aura.
Migraine with aura is less common.
Vision complaints are the most common manifestation of aura, but
patients may experience paresthesia, aphasia, nausea and vomiting prior to the
onset of headache. These findings are
completely reversible and precede the headache by no more than 60 minutes.
Migraines seem to have a triggering event that
precipitates a sterile inflammatory response around intracranial vessels that
is mediated by the trigeminovascular system.
Triggering factors may include stress, menses, pregnancy and oral
contraceptive pills, infection in the head and neck, trauma or surgery, red wine,
aged cheeses, vasodilating medications, strong odors, irregular diet or sleep
and bright sunlight or flickering lights.
Recent studies have discovered serotonin receptor subtypes in the
central nervous system that play significant roles in the neurologic changes
and intracranial blood vessel change.
Newly available treatments such as sumatriptan target these
receptors. Several other neuropeptides
have been identified as pro-inflammatory and are believed to play a significant
role in migraine development. Further
investigation is hoped to provide treatment alternatives with fewer side
effects.
The treatment of migraine headaches may be
approached using several strategies:
aborting the attacks at their onset, controlling the pain once is fully
evolved and reducing the frequency of attacks.
Therapies aimed at aborting an attack should be started as soon as the
premonitory or warning signs are noted.
Abortive therapy has been revolutionized with the introduction of
5-hydroxytryptamine (5-HT) receptor agonists.
These include sumatriptan (Imitrex) available in oral,
subcutaneous injection or nasal spray forms, naratriptan (Amerge),
rizatriptan (Maxalt) and zolmitriptan (Zomig) all available in
oral preparations. These medications
have allowed the migraine sufferer to quickly and effectively treat attacks
several times a month with minimal side effects. Other medications used to abort headaches include ergotamine
tartrate administered sublingually, or in combination with caffeine by mouth. Dihydroergotamine 45 is administered in a
nasal spray. Butorphanol is a mixed
narcotic agonist/antagonist available by nasal spray. It does have potential for abuse and chronic use is
contraindicated. Midrin is an
orally administered compound of acetaminophen, isometheptene mucate, a
sympathomimetic amine and dichloralphenazone, a mild sedative. It has a low side effect profile and may be
used until relief is attained. Many
NSAIDs have been shown to be effective in migraine headaches. The short-rise time, short-acting
medications such as naproxen, ketorolac, ibuprofen and choline magnesium
trisalicylate have the greatest usefulness.
Lidocaine administered intranasally in 4% spray, either singly or in
combination with nasal decongestants, has been shown to be effective, although
of short duration. Intravenous
lidocaine with diphenhydramine may also be effective.
If abortive therapy fails, management should be
aimed at reducing the intensity of the pain and controlling associated symptoms
such as nausea and vomiting. It is
desirable to avoid opiates for the treatment of migraine. Finkel et. al. recommend several
treatment regimens: (1)
prochlorperazine (Compazine) IV push that may be repeated in 20 minutes
if no effect, (2) dihydroergotamine IV push followed by IV prochlorperazine,
(3) chlorpromazine (Thorazine) IV push, may repeat in 20 minutes if
needed and (4) haloperidol IV push followed by lorazepam IV push. Options (1) and (3) should not be combined,
but may be followed by (2) or (4) if necessary.
Patients experiencing 2 or more attacks per month
should be started on a prophylactic regimen.
Appropriate first steps are to limit the activities or factors that
trigger the headaches. This may be
effective by itself, but medical prophylaxis is often needed as well. Multiple antidepressant medications have
been shown to be effective in the prevention of migraine headache. These include amitriptyline, nortriptyline,
doxepin, trazodone, imipramine and desipramine. The newer selective serotonin re-uptake inhibitors (SSRI)
including Prozac and Zoloft, have not been shown to be effective
in migraine therapy. Bellergal,
a low dose, sustained relief ergotamine may be useful in preventing
attacks. NSAIDs have some usefulness in
the prevention of attacks as well as the treatment of the acute headache. b-blockers, specifically
propranolol, nadolol, atenolol, timolol and pindolol have been used with some
success but are contraindicated in patients with depression, asthma or
diabetes. Calcium channel blockers
(verapamil, nifedipine, nimodipine) have shown some effectiveness preventing
migraine attacks as well.
Binder et al. found 51% of migraine sufferers
obtained complete prophylaxis for an average of 4.1 months duration after the
injection of botulinum toxin type A (BOTOX) into the facial and scalp musculature. An additional 38% obtained a partial
response for an average duration of 2.7 months. The investigators also reported a 70% complete response rate
among patients treated acutely for migraine headache within 1-2 hours
post-treatment. These results hold
promise for a novel treatment modality for the migraine sufferer.
Needless to say, the treatment of migraine can be a
time-consuming and frustrating proposition.
Lifestyle changes with the avoidance of the triggering event must be
stressed to the patient. Medication
changes should be adequately evaluated before dismissed as ineffective, and all
medications should be started one at a time at the lowest dose. It is often necessary to combine medications
for acute pain or abortive therapy with those used for prophylaxis, however
some interactions do occur and this should be done with caution. The reader is referred to the manufacturers’
data regarding recommended dosages, contraindications and complete list of
interactions and side effects for all the medications listed.
Cluster headache (CH) is characterized by intensely
severe pain (sometimes termed suicide headache) with boring or burning
qualities located unilaterally in the orbit, supraorbital or temporal
area. Attacks last from 15 to 180
minutes. The headache is associated
with at least one symptom of autonomic hyperactivity: conjunctival injection, lacrimation, nasal congestion,
rhinorrhea, forehead and facial sweating, miosis, ptosis or eyelid edema. Attacks occur between one every two days to
eight per day. At least 5 such attacks
must occur to meet the diagnostic criteria.
Nausea and vomiting is uncommon and there is no aura. Onset is usually in the second to fifth
decades. Cluster headache is the only
headache syndrome with a male preponderance.
It is associated with alcohol use and intolerance, and during an active
phase or “cluster”, alcohol may precipitate an attack.
There are both episodic and chronic types. Episodic CH has periods of activity
alternating with periods of inactivity.
Active periods vary in frequency from two or more per year, to one every
two or more years and tend to occur in regular intervals. The duration of active periods ranges from
seven days to a year. In chronic forms,
the remission phases last less than 14 days while the prolonged ones are absent
for at least one year.
Treatment is aimed at preventing an attack during a
cluster. Once an effective therapy is
discovered, it is continued for 6 to 8 weeks and then gradually tapered. Options for treatment include calcium
channel blockers (nifedipine, nimodipine, verapamil), low dose daily ergotamine
(Bellergal) and lithium carbonate (especially in chronic forms of
CH). Methysergide has been found to be
effective but use is limited to 4 months as prolonged continuous use may cause
retroperitoneal fibrosis. Trials with
valproic acid are ongoing. Some have
used antihistamines, both H1 and H2 blockers with limited
success. The role of steroids is
controversial, but they are frequently used in prophylaxis during an active
period.
Some treatments have been found to be effective in
the acute treatment of an attack.
Oxygen inhalation, 6-10 liters per minute administered by face mask
seems to be particularly effective in young patients with attacks primarily at
night. 5-HT receptor agonists are
effective in shortening an attack if given at the first indication of
pain. Intranasal lidocaine administered
either 4% topical or 2% viscous at the posterior aspect of the inferior
turbinate affecting a sphenopalatine block may be effective in terminating an
acute attack.
Temporal arteritis is characterized by daily
headaches of moderate to severe intensity, scalp sensitivity, fatigue and
various non-specific complaints with a general sense of illness. 95% are over 60 years old. The pain is usually unilateral, although
some cases of bilateral or occipital pain do occur. The pain is a continuous ache with superimposed sharp, shooting
head pains. The pain is similar to and
may be confused with that of CH, but CH tends to occur in younger
patients. The two may also be
distinguished on physical exam when dilated and tortuous scalp arteries are
noted. The erythrocyte sedimentation
rate (ESR) is markedly elevated in temporal arteritis as well.
Definitive diagnosis is made by artery biopsy from
the region of the pain, although negative biopsy may be due to the spotty
nature of the disease and does rule out the diagnosis. High dose steroid therapy usually precipitates
a dramatic decrease in head pain.
Failure to respond to steroid therapy with a negative biopsy should call
the diagnosis into question. If the
diagnosis seems likely based on history and physical examination, steroids
should be started immediately to avoid vision loss, the most common
complication of the disorder occurring in 30% of untreated cases. The biopsy remains positive for 7-10 days
from starting steroid therapy. Steroids
may be tapered to an every other day maintenance schedule when the pain resolves
and ESR normalizes. The disease is
usually active for 1-2 years, during which time steroids should be continued to
prevent vision loss.
Chronic Daily Headache
Chronic daily headache (CDH) is described as
headache occurring at least 6 days a week for a period of at least 6
months. The pain is usually present
throughout the day with little time spent pain-free. The head pain is typically bilateral, frontal or occipital, non-throbbing
and moderately severe. The syndrome is
associated with the overuse and abuse of many common over-the-counter pain
medications (aspirin, acetaminophen, ibuprofen, etc.), barbiturates and opioid
analgesics. A carefully taken history
will reveal an increasing need for medications and the emergence of a chronic
headache that is qualitatively distinct from the headache for which is was
originally taken. This led to the idea
of CDH being a “transformed migraine”.
Trigeminal neuralgia (formerly also known as tic
doloureux) is characterized by paroxysmal pain attacks lasting from a few
seconds to less than two minutes. The
pain is severe and distributed along one or more of the branches of the
trigeminal nerve with a sudden, sharp, intense stabbing or burning
quality. Between attacks the patient is
completely asymptomatic without neurological defects (no facial numbness, loss
of corneal reflex or change in taste or smell). The pain may be precipitated from trigger areas or with certain
daily activities such as eating, talking, washing the face or brushing the
teeth. Attacks are the same in an
individual patient. Structural causes
of facial pain should be excluded. The
syndrome is most common in patients over 50.
The course may fluctuate over many years and remissions of months or years
are not uncommon.
Medical treatment of the disorder includes
carbamazepine, gabapentin, baclofen, phenytoin, or sodium valproate. Tricyclic antidepressants (TCA) and NSAIDs
may be used as adjuvant therapy.
Opiates are usually ineffective.
Surgical treatment is occasionally necessary when medical therapy fails
to control the pain attacks.
Glossopharyngeal neuralgia is characterized by pain
attacks similar to those in trigeminal neuralgia, but located unilaterally in the
distribution of the glossopharyngeal nerve.
Pain is most common in the posterior pharynx, soft palate, base of
tongue, ear, mastoid or side of the head.
Swallowing, yawning, coughing or phonation may trigger the pain. Management is similar to that for trigeminal
neuralgia.
Atypical Facial Pain
Traumatic injuries may induce a pain syndrome due to
the development of neuroma. This occurs
most often on the occipital and parietal regions of the scalp. The diagnosis of post-traumatic neuralgia is
made based upon the history of trauma temporally correlated with the
development of the characteristic neuritic pain. Poor wound closure, infections, foreign material in the wound,
hematoma, skull fracture, diabetes mellitus or peripheral neuropathy elsewhere
in the body predispose to neuroma development.
Pains commonly begin 2 to 6 months after the injury. Medical therapy is similar to that used in
trigeminal neuralgia. Bupivicaine
injection of trigger areas may be effective, relieving pain for months. A well-defined trigger point may be amenable
to surgical excision.
Herpetic skin eruption is caused by the reactivation
of latent varicella-zoster virus from the sensory nerve ganglia. The reactivated virus is carried via the
axons distally to the skin where it produces a painful rash with crusting
vesicles in a dermatomal distribution.
The trigeminal nerve is the second most commonly affected after nerves
in the thoracic region. Steroids are
often used for the acute eruption in otherwise healthy individuals, while
antivirals, NSAIDs and opiates are often used in immunocompromised
patients.
Pain that persists 2 or more months after the acute
eruption is known as post-herpetic neuralgia.
NSAIDs and opiates are of little use in the treatment of the
neuralgia. Anticonvulsants in
conjunction with TCA or baclofen are most useful for the control of shooting
neuritic pains.
Temporomandibular Disorders
Temporomandibular disorders (TMD) include a
heterogeneous group of processes all with a similar clinical presentation. Common symptoms of TMD include temporal
headache, earache, facial pain, limited jaw opening or joint noise. The majority of TMD originate spontaneously
with only 40% able to recall a specific event, usually trauma, preceding the
onset of pain. This suggests that there
is a significant role of emotional and psychological factors in the etiology of
spontaneously occurring TMD. As a
result of relatively recent advances in the understanding of the pathogenesis of
TMD, they may be further classified as internal derangements, degenerative
joint disease (DJD) and myofascial pain.
Pain that truly originates in the TMJ is rare and
characterized by tenderness to palpation of the condyle and pain with joint
movement. Internal derangements are
characterized by anterior and medial displacement of the articular disk. This produces a “click” as the disk with
reduced with mouth opening. This
clicking may be benign, with minimal clinical symptoms or discomfort. When symptomatic, pain occurs just before or
during the click and the joint is mildly tender to palpation. Patients may have limited mouth opening as a
result of attempts to avoid pain. DJD
has a similar presentation, with pain at joint movement and crepitus over the
joint. The painful stage usually lasts
less than a year. Long-standing DJD
causes flattening of the condyle and osteophyte formation making it easily
recognizable radiographically. The vast
majority of patients, 60%-70% have combined muscle and joint pain with muscle
pain dominating the clinical picture.
These patients usually have tenderness to palpation of the muscles of
mastication.
NSAIDs and physical therapy are the mainstays of
treatment for TMD. Similar to tension
headache, biofeedback and trigger point injection may be beneficial. Benzodiazepines are useful for muscle pain,
but chronic use may lead to dependence and tolerance. Muscle relaxants are of little benefit. In chronic muscle pain, antidepressants may be more useful that
analgesics or anxiolytics. TCA are
useful in those patients with sleep disturbance, or SSRIs may be used for
patients intolerant of TCA.
Pseudotumor Cerebri (Benign Intracranial
Hypertension)
Pseudotumor cerebri presents with intermittent
headache of variable intensity. The
patient has a normal neurological examination, although a sixth nerve palsy is
rarely found. There is papilledema with
no evidence of hydrocephalus or mass on CT scan. Cerebrospinal fluid (CSF) pressures are greater than 200 mm H2O
with normal CSF chemistries and cultures.
The history may also be characterized by one or several of the
following: (1) mastoid or ear
infection, (2) menstrual irregularity or other endocrine disorder, (3) recent
weight gain of greater than 10% over baseline weight over 6 months, (4)
exposure to steroids (especially withdrawal), vitamin A, tetracycline or
nalidixic acid, (5) retro-orbital or vertex headache, especially with empty
sella syndrome (6) fluctuations in vision, (7) recurrent unilateral or
bilateral tinnitus, (8) constriction of visual fields lasting longer than
several months. Medical therapy is
aimed at reducing CSF production with acetazolamide and furosemide. Weight loss and low-salt diet are also
beneficial. In chronic cases with loss
of visual fields that do not respond to weight loss and medical therapy,
lumboperitoneal or ventricular shunting with incision of the optic nerve sheath
may be necessary.
Primary or metastatic tumors of the brain produce
headache 30% of the time. 15% have
headache as the presenting symptom. The
pain is described as dull or aching, lateralized and relatively mild
initially. The pain may crescendo with
increasing intensity and frequency with progression of the disease. Initial response to non-narcotic analgesics
is common, becoming resistant as the pain intensifies. Early morning headache is present only in
10% but is strongly suggestive when present, especially when accompanied by
vomiting without significant nausea.
The pain may increase with coughing, Valsalva or changes in body
position. Focal neurological signs may
be a late finding despite significant head pain.
Subdural hematoma (SDH) presents with a fluctuating
level of consciousness with moderate to severe headache after trauma. The pain is usually lateralized to the side
of the hematoma with tenderness to percussion over the hematoma. The patient may also have Battle’s sign
(ecchymosis over the mastoid) and hemotympanum. In chronic SDH, the traumatic occurrence may be remote or not
remembered at all.
Subarachnoid hemorrhage (SAH) presents with the
sudden onset of severe, generalized headache (“the worst headache of my
life”). Early in the process the pain
may be localized. Nausea, vomiting and
stiff neck are common, progressing to back pain as blood circulates into the
spinal subarachnoid space. Imaging may
not show the blood collection or a mass effect, in which case a lumbar puncture
should be performed to establish the diagnosis.
There is little diagnostic dilemma when headache is
associated with fever and stiff neck.
However, many inflammatory conditions of the CNS may produce headache
with a paucity of associated symptoms.
These include epidural abscess, fungal, tuberculous or luetic
meningitis, acquired immunodeficiency syndrome (AIDS) of the CNS, and meningeal
sarcoidosis. Diagnosis is dependent on
history and physical with lumbar puncture for CSF studies after CT or MRI has
ruled out intracranial mass.
Chronic, untreated hypertension is an occasional
cause of headache. It is most likely to
occur in patients with diastolic pressures over 115 mm Hg. The pain is often described as throbbing and
may be associated with nausea. Acute
headache associated with rapid rises in blood pressure may be found in
pheochromocytoma, renal artery stenosis or hyperadrenalism.
Headache resulting from the
removal of CSF with subsequent decreases in intracranial pressure (ICP) is a
well-known phenomenon. This most
commonly occurs after lumbar puncture (LP), but may occur spontaneously or as a
result of trauma. The headache is
usually worsened by sitting or standing upright and improves or disappears with
lying flat. Pain is steady, often
described as pulling, and occurs most commonly in the vertex or occipital
regions. Nausea is common and transient
3rd or 6th nerve palsies have been reported. Nearly all of these headaches will resolve
spontaneously over several days. Bed
rest and fluids are recommended but have not been conclusively shown to speed
recovery. In cases occurring after LP
that do not resolve with conservative management, autologous blood patching in
the epidural space is indicated.
While
acute sinusitis is widely accepted and recognized as a cause of headache with
pain referred to the skin or intracranial structures also innervated by the
nerve branches providing the sinuses, chronic sinusitis or sinonasal
abnormalities as a cause of headache has been more controversial. According to the International Headache
Society Manual for Headache Diagnosis published in 1988: “Other conditions which may cause headache
such as nasal passage abnormality due to septal deflection, hypertrophic
turbinate and atrophic sinus membranes are not sufficiently validated as causes
of headache. Chronic sinusitis is not
validated as a cause of headache unless relapsing into an acute phase. Migraine and tension-type headache are often
confused with true sinus headache because of similarity in location.” Much investigation as to the treatment of
headache and facial pain presumed to be of sinonasal origin has been performed,
with multiple authors demonstrating good outcomes in carefully selected cases.
The
pain associated with acute sinusitis is commonly described as constant, dull
and aching. Occasionally the pain is
sharp and may be worsened by jarring of the head, bending forward or
stooping. Although the discomfort is
most often located over the acutely inflamed sinuses, the pain may be referred
to other areas of the head or face based upon the anatomic structures sharing
innervation with affected sinuses as described previously. In most instances, the pain will be
accompanied by such symptoms as purulent nasal discharge, malaise and
congestion.
In
1988, Stammberger and Wolf described their experience with headaches and sinus
disease. They proposed classifying
headaches into 3 groups for diagnostic purposes: (1) those with headaches clearly attributable to sinus disease
(i.e. acute sinusitis), (2) those with headaches clearly due to non-sinus
causes such as migraine, neuralgias, etc, and (3) those with complex problems
and in whom there seems to be no overt indication of sinus disease. It is this last group that presents the biggest
diagnostic and treatment dilemma. With
the use of nasal endoscopy and CT scanning in the coronal plane, a thorough
evaluation of the sinus anatomy allows the identification of mucosal contact
points that are hypothesized to produce head pain even in the absence of other
sinonasal pathology.
Multiple
neuropeptides have been found in the nasal mucosa that function as
neurotransmitters. One of these is
Substance P (SP), which functions to mediate pain impulses to the cerebral
cortex. Receptors within the sinonasal
mucosa are stimulated by chemical and caloric irritants, as well as by
mechanical irritants such as pressure.
These receptors are one component of local reflexes mediated by SP, and
when stimulated produce vasodilation, plasma extravasation (neurogenic edema)
and hypersecretion. Pressure exerted on
the nasal mucosa by polyps or mucosal swelling can be enough to trigger an
SP-mediated pain sensation. Due to the
axonal reflex, relatively small lesions may lead to a vicious cycle and
significant symptoms. Certain anatomic
conditions predispose to the process.
Not all of these conditions are disease states per se, but they are
factors that may reduce the already narrow spaces of the anterior ethmoid and
middle meatus, thereby giving rise to areas of mucosal contact, secretion retention
and malventilation of the sinuses promoting polypoid degeneration of opposing
mucosal surfaces. All of these
conditions, even when small and well circumscribed, may have one dominating
clinical symptom – headache.
Agger
nasi cells lie anterior and superior to the insertion of the middle turbinate
on the lateral nasal wall. Even in the
absence of disease, these cells may narrow the frontal recess. When diseased, the agger nasi cells may
completely obstruct the frontal recess.
The
uncinate process may present with varied deflections. The posterior margin may be deflected anteriorly and displaced
into the middle meatus to such an extent that it contacts the lateral aspect of
the middle turbinate. This contact is
even more prone to occur if the middle turbinate has a paradoxical curve or is
pneumatized (concha bullosa). The
uncinate may be curved laterally and posteriorly as well, narrowing the hiatus
semilunaris and ethmoid infundibulum and impeding drainage of the anterior
ethmoid cells. Pneumatization of the
uncinate itself has been described and may further narrow the middle meatus.
Already
mentioned, the middle turbinate may possess a laterally oriented convexity, or
paradoxically bent middle turbinate.
When extreme, the mucosa of the turbinate may contact the lateral nasal
wall and obstruct the middle meatus.
Pneumatization of the middle turbinate, also known as concha bullosa,
may occur to such an extreme as to contact the septum and the lateral nasal
wall thereby obliterating the middle meatus.
Variations
of the ethmoid bulla may serve to obstruct the middle meatus. It may be so extensively pneumatized that
the middle meatus is filled, with mucosa from the bulla contacting the middle
turbinate. Stammberger and Wolf note
that this is a key finding in patients with headache. Haller’s cells, or anterior ethmoid cells located on the floor of
the orbit, may narrow the maxillary sinus ostium, especially when
diseased. This may predispose to
recurrent maxillary sinusitis.
Patients
with headaches of sinus origin do not necessarily present with the typical
sinusitis history. The physical
examination and plain radiographs may be negative as well. When in question, the combination of rigid
nasal endoscopy and CT scanning provide the most complete information regarding
sinus anatomy and disease. Multiple
authors have reported good successes with sinus surgery targeted at these
points of mucosal contact and anatomical variation for the treatment of chronic
headache presumed to arise from the nose and sinuses. Clerico performed directed sinus surgery on 7 of 10 patients
previously diagnosed with headache syndromes with improvement in all. Patients were offered surgery only if they
had a clearly positive response to intranasal decongestion and topical
anesthesia to the area of presumed mucosal contact. Theoretically, if mucosal contact is the cause of the patient’s
symptoms, relief of the contact, whether it is medical or surgical, should
provide headache relief. The surgical
procedure in each case was individualized to each patient’s symptoms and
anatomic findings. Parsons and Batra
noted that 91% of 34 patients experienced a decrease in headache intensity
after rhinologic surgery directed to the relief of contact points, and 85%
reported a decrease in frequency. They
still cautioned against the use of the presence of contact points as the sole
indication for surgery, as the majority of patients in their series also had
chronic sinusitis that served as the primary indication for surgical intervention.
While
the evidence is inconclusive, it seems that there is at least some support for
mucosal contact as a cause of headache.
Any surgical intervention aimed at the relief of headache must be
clearly limited to the area in question.
It stands to reason that if indeed mucosal contact and obstruction are
the cause of the headache, some objective evidence of abnormal sinus function,
whether it is mucosal edema or polypoid degeneration, should exist on
examination or imaging. Until further
evidence is uncovered, using headache as the only indication for surgical
intervention is to be discouraged. When
it is performed, clear demonstration of improvement in the headache should be
obtained with topical decongestants and anesthesia to the proposed site of
origin of the pain.
Headache
and facial pain are common ailments with many varied causes and are commonly
encountered in otolaryngologic practice.
The correct diagnosis can usually be reached by history and physical
alone. A significant behavioral
component is involved in most cases, therefore it is important that the correct
diagnosis be reached quickly and appropriate therapy instituted for the
greatest chance of successful treatment.
Failure to properly identify the cause and treat accordingly may lead to
patient frustration and distrust with a high likelihood of non-compliance and
failure. Effective treatment is
rewarding to the clinician as the patient’s productivity and quality of life are
greatly improved.
A
29 year old white male presents with complaint of severe headache. It is located unilaterally on the right in
the peri/retro-orbital region. He
describes the pain as excruciating.
There is a constant boring pain that is punctuated by lancinating pain radiating
posteriorly to the mastoid area and occasionally to the upper neck. He notices that it is difficult to keep his
eyes open when having the pain. He
denies any nausea or vomiting. He has
had similar headaches everyday for the past three weeks. They tend to occur during the night,
awakening him from sleep, or in the afternoons. Each headache lasts from 30 minutes to 2 hours without
treatment. In the past he has used NSAIDs
with some success, but now they have no effect. In addition, inhalational oxygen has been beneficial, but now
provides little relief.
He is otherwise healthy and is not on any medications when not having the headaches. He has no known drug allergies. Past surgical history is remarkable only for hernia repair. There is no significant history of head trauma. He is a non-smoker and drinks alcohol sporadically. He does not use recreational drugs. When he has the headaches, he avoids alcohol as he has noticed that alcoholic beverages may precipitate an attack.
On
further questioning, he has had these headaches several times before. The last was 3 years ago in the spring. The time prior to that was 5 years ago from
the current episode. The first time he
experienced the headache was at age 19 when he was awakened from sleep by a
severe headache. It was not followed by
any further headaches until several years later. These had a marginal response to NSAIDs. Each of these episodes lasted approximately
one month. With the last episode, he
was treated with Verapamil and a Prednisone taper with cessation of the
headaches.
Examination
is performed while he is not experiencing an attack and is normal.
The
patient is diagnosed as having cluster headaches of the episodic type. He is begun on Prednisone taper and
Verapamil. For abortive therapy, he is
given a prescription for sumatriptan nasal inhalers and subcutaneous injection.
He
returns one month later. The headaches
have now resolved. They decreased in
intensity and frequency shortly after starting the steroids and Verapamil. The sumatriptan provided relief almost
instantaneously at first, then with slower onset as the frequency of use
increased. It continued to provide
relief however.
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MA, Montgomery WW. Treatment of chronic paranasal sinus pain with minimal sinus
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WJ, Brin MF, Blitzer A, et al. Botulinum toxin type A (BOTOX) for
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DM. Sinus headaches reconsidered: referred cephalgia of rhinologic origin
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AG, Mann D, Lundeen TF. Headache and facial pain. In: Bailey BJ, ed. Head
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HL. Otorhinolaryngologic causes of headache. Medical Clin North Am
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Posted 6/15/2001