Headaches: Clinical characteristics and Treatment of Different Kinds

Cephalalgia is a very common complaint among the general population, and patients complaining about headache can appear in any situation: the office, urgent care, ER or the hospital. This article is a quick guide about the most prevalent kinds of headaches, general clinical characteristics and treatment. To see the specific diagnostic criteria of each headache, you can check this different article.

GENERAL DEFINITIONS:

Primary headache: A headache that does not have an identifiable secondary cause (e.g. migraine, tension-type headache, trigeminal autonomic cephalalgias –TACs, among others).
Secondary headache: A headache due to a secondary condition (tumor, trauma, infection, inflammatory, autoimmune).

Regardless of the type, headaches may be sporadic or chronic. Chronic headaches can be defined as below:

Chronic daily headaches (CDH): Syndrome that encompasses primary and secondary headaches. The term primary CDH is applied to headaches that occur for at least 15 days a month for more than 3 months. Chronic daily headaches may be divided in two different groups.
Chronic daily headaches (CDH) with symptoms that last > 4 hours

Chronic migraine headache
Chronic tension-type headache
Medication overuse headache
Hemicrania continua
New daily persistent headache

Chronic daily headaches (CDH) with symptoms that last < 4 hours

Chronic cluster headache (must persist for at least one year without remission)
Chronic paroxysmal hemicrania (must persist for at least one year without remission)
Hypnic headache
Short-lasting unilateral, neuralgiform headache attacks
Primary stabbing headache

HEADACHE CHARACTERISTICS THAT SHOULD ELICIT FURTHER INVESTIGATION (RED FLAGS):

Red flags are clinical details that, when present in any kind of headache, should increase the suspicion for a secondary cause (SNOOP acronym):

Systemic symptoms, illness, or condition (e.g., fever, weight loss, cancer, pregnancy, immunocompromised state including HIV)
Neurological symptoms or abnormal signs (e.g., confusion, impaired alertness or consciousness, papilledema, focal neurological symptoms or signs, meningeal signs, or seizures)
Onset is new (particularly for age >40 years) or sudden (e.g., “thunderclap”)
Other associated conditions or features (e.g., head trauma, illicit drug use, or toxic exposure; headache awakens from sleep, is worse with Valsalva maneuvers, or is precipitated by cough, exertion, or sexual activity)
Previous headache history with headache progression or change in attack frequency, severity, or clinical features

SPECIFIC HEADACHES, CLINICAL CHARACTERISTICS AND TREATMENTS:

Below you will find information regarding clinical the manifestations and treatment for the following headaches (click on the name to go straight to the topic):

Migraines
Tension type headache
Cluster headache
Hemicrania continua
Paroxysmal hemicrania
Short-lasting unilateral neuralgiform headache attacks (with conjunctival injection and tearing – SUNCT or with cranial autonomic symptoms – SUNA)
Sinus headache
Primary stabbing headache
Idiopathic Intracranial Hypertension
Trigeminal neuralgia
Hypnic headache
Nummular headache
Temporomandibular Joint Related Pain
Medication overuse headache
Giant Cell Arteritis (Temporal Arteritis)

Migraines

Epidemiology: Global prevalence is around 15%, with chronic migraine affecting around 2% of the population. It is three times more common in women than men. Most often affects those aged between 35-45 years (but can occur in any stage of life). Most cases (75%) are without aura. A genetic component may exist.

Causes: Currently it is believed that neuronal dysfunction is the main causative agent of migraine (in opposition to the previous theory that believed that migraine was caused by vasodilation). Neuronal activation causes waves that activate nociceptive areas in the meninges and the brain.

Location: Often unilateral (but may change during episodes, hence the name).

Duration: The painful episodes may last anywhere from 4 to 72 hours.

Characteristics of the pain: An attack can have the following stages: prodrome -> aura -> headache -> postdrome. The headache itself has throbbing (pulsatile) quality with variable intensity, starting as a mild to moderate pain and increasing as time passes. Photophobia, phonophobia and nausea are common, and the patient may experience relief by staying in a quiet, dark room. Physical activity can make the symptoms worse. Precipitating or exacerbating factors include emotional stress, hormonal variations (menses), fasting, weather, sleep disorders, odors, and alcohol, among others.

Associated symptoms: In addition to photophobia, phonophobia and nausea, aura can be present. Aura is a neurological dysfunction that can present in different ways, such as visual (bright lines, shapes, objects, visual loss), auditory (tinnitus, noises), somatosensory (burning, pain, paresthesia, tingling, numbness), motor (repetitive movements, weakness, hemiplegia), and language (dysphasia). In some cases a patient can have aura without pain.
The prodrome (euphoria, depression, irritability, food cravings) may occur 24-48 hours before the pain. After the pain (postdrome) patients may feel tired.

Physical exam and diagnosis: Physical exam may be normal or show signs compatible with the aura that the patient is experiencing. Regardless of the presence or absence of aura, a throbbing/pulsating headache with nausea and photophobia is characteristic of migraine.

Acute treatment: In the emergency setting, the patient may receive triptans (sumatriptan 6mg sc), antiemetics (metroclopramide 10mg-20mg IV or prochlorperazine 10mg IV), ergots combined with antiemetics (dihydroergotamine – DHE45 – 1mg IV with metoclopramide 10mg IV) or ketorolac (30mg IV or 60mg IM). When using antiemetics, the use of diphenhydramine (12.5-25mg IV) may be helpful to prevent dystonic reactions. The addition of dexamethasone (10-25mg IV or IM) may help to reduce recurrence. Patients that do not respond to any other intervention may respond to haloperidol.
Outpatient acute treatment for mild to moderate attacks can be done with oral analgesics (acetaminophen or NSAIDs) with antiemetics. Moderate and severe attacks may need triptans (oral, nasal or subcutaneous), analgesics and antiemetics. Triptan example: sumatriptan (50-100mg oral, may be repeated after 2 hours; nasal solution 10mg, may be repeated after 2 hours, transdermal 6.5m patch). If the symptoms do not improve or if the patient does not have the medications, he may go to an urgent care center or emergency department (in which case parenteral medications can be provided as said above).

Chronic treatment: Preventive migraine treatment is useful for patients that have frequent, long lasting attacks or attacks that cause disability. In addition to the avoidance of triggers, medications can be used. First line medications for preventive treatment include beta blockers (propranolol 20-80mg BID; metoprolol 25-100mg BID; atenolol 25-100mg qDay), antidepressants (amitryptiline 10-50mg at bedtime; venlafaxine 37.5-150mg qDay), or anticonvulsivants (or topiramate 25mg to 100mg BID or valproate). These medications should be started at lower doses that should be increased slowly. Calcium channel blockers are also an alternative. Patients that do not respond to one preventive medication may be switched to a different class of medication. Treatments with sub-optimal efficacy compared to medication include Botox, acupuncture, butterbur (Petasites hybridus), coenzyme Q10, among others. Other second and third-line treatments include riboflavin, candesartan, tizanidine, memantine, and zonisamide.

Tension-type headache (TTH)

Epidemiology: TTH is the most common type of headache in the general population (one year prevalence ~40-80%). It may be classified as infrequent episodic TTH (episodes that last less than one day, less than one day a month), frequent episodic TTH (episodes for 1 to 14 days a month) or chronic TTH (>15 days a month).

Causes: Mechanism is uncertain, but it is believed that peripheral activation of nociceptive receptors on the muscles play a role.

Location: Bilateral pain in most cases.

Duration: Episodes may last from 30 minutes to 7 days, and many episodes can occur in the same day.

Characteristics of the pain: Mild to moderate pressure pain (dull, like a tight cap or heavy weight on head). It is non-pulsating. Psychological stress may trigger painful episodes.
Associated symptoms: Pericranial muscle tenderness is usually present. Nausea, vomiting and autonomic symptoms are usually absent.

Physical exam and diagnosis: Nothing major is detected during physical examination (except maybe the muscle tenderness). Diagnosis is clinical.

Acute treatment: NSAIDs or aspirin (500mg or 1g) may be used. The combination of caffeine with the analgesics is more effective than the analgesics alone. Patients that do not improve with these interventions may receive parenteral metoclopramide, chlorpromazine or intramuscular ketorolac.

Chronic treatment: Indicated for frequent, long lasting or significantly disabling attacks. Tricyclic antidepressants are probably the most useful agents (amitriptyline 12.5-100mg at night). Other alternatives include SSRIs (venlafaxine) and anticonvulsants (gabapentin up to 2400mg in divided doses (x3) or topiramate 25-100mg daily). Nonpharmacological strategies include relaxation, cognitive-behavioral therapy, electromyography (EMG) biofeedback, and/or acupuncture.

Cluster headache

Epidemiology: More common in males than females (4:1). The prevalence is <1%.

Causes: It is a member of the group called trigeminal autonomic cephalalgias (TACs). The pain is believed to happen due to hypothalamic activation with secondary activation of the trigeminal-autonomic reflex.

Location: Orbital, supraorbital or temporal. During a single attack the symptoms stay in one side, but in a different attack the symptoms may occur on the other side.

Duration: The painful attacks last between 15-180 minutes and occur in numbers from one to 8 times a day. It may occur daily for some weeks (6-12) with periods of remission after (up to 12 months), or chronically without periods of remission.

Characteristics of the pain: Rapid onset with severe bouts of excruciating pain (burning, stabbing, boring or squeezing). Attacks may occur commonly at night.

Associated symptoms: Agitation, restlessness and autonomic symptoms (lacrimation, conjunctival injection, miosis, nasal congestion, ptosis).

Physical exam and diagnosis: Autonomic signs may be observed during an attack. History is usually sufficient for the diagnosis. MRI may be useful to rule out secondary causes of pain.

Acute treatment: Oxygen 100% is usually effective. If oxygen is not available or sufficient, sumatriptan (6mg SC or 20mg intranasal contralateral to the side of the headache) may be used. For patients that do not have access to or cannot tolerate either, intranasal lidocaine (same side of the pain) or ergots may be used. The preventive therapy should also be started (see chronic treatment below).

Chronic treatment: Preventive therapy includes the use of verapamil (240-480mg daily in three divided doses) preferably in addition to steroids in the first weeks (prednisone 60-100mg/day for at least 5 days with reduction of 10mg/day after) since the verapamil itself takes time to reduce the episodes of pain. Other medications that may be beneficial include topiramate and lithium. After reaching pain control, the dose of the medications should be reduced to the smallest dose capable to keep the patient pain free. Another treatments that may be tried but are not necessarily backed up by substantial evidence yet include noninvasive vagal stimulation and deep brain stimulation.

Hemicrania continua

Epidemiology: More common during the 3rd decade of life and among women.

Causes: It is believed that the activation of the posterior hypothalamus and dorsal rostral pons leads to secondary disinhibition of the trigeminal-autonomic reflex, causing the symptoms.

Location: Orbital, temporal and frontal regions may have pain, as well as the neck. The pain usually occurs always on the same side.

Duration: Usually continuous pain that fluctuates in severity (exacerbations last from minutes to days).

Characteristics of the pain: Mild to severe pain is present (burning, stabbing, boring or squeezing).

Associated symptoms: Autonomic symptoms are often present, such as lacrimation, nasal congestion, conjunctival injection, ptosis, flushing, sweating, and eyelid edema.

Physical exam and diagnosis: The autonomic symptoms can be observed during an acute attack. The response to indomethacin is substantial and for that reason the drug, in association with history, can be used to confirm the diagnosis. MRI of the brain is useful to exclude secondary causes of pain.

Acute treatment: Both to confirm the diagnosis and to relief the pain, indomethacin should be used (25-50mg q8hr).

Chronic treatment: Indomethacin may be continued at the lowest effective and tolerable dose.

Paroxysmal hemicrania

Epidemiology: Rare condition (prevalence is estimated to be around 1/25000). It affects all races in any age group and the mean age of onset is 34-41 years.

Causes: It is a member of the group called trigeminal autonomic cephalalgias (TACs).

Location: Unilateral headache that almost always affects the same side in every episode. Pain occurs on the trigeminal distribution V1 (frontal, orbital, temporal and nasal areas) more often. It may radiate to the shoulder or arm.

Duration: It has intermittent attack duration and frequency. The attacks may occur multiple times a day (>20), and each attack usually lasts between 2-30 minutes (but can last up to 2 hours). The episodes of pain may occur daily during anywhere between 2 weeks to 4 months, followed by remission periods that can last between 1 month and 3 years. A chronic form (no episode free interval) may also be present.

Characteristics of the pain: Severe sharp, burning, throbbing or stabbing pain.

Associated symptoms: Autonomic features often occur, such as lacrimation, conjunctival injection, nasal congestion, and facial flushing, sweating or eyelid edema.

Physical exam and diagnosis: The autonomic features may be observed during an acute attack. Diagnosis is confirmed by history and the response to treatment (indomethacin). MRI is useful to exclude structural brain lesions, since paroxysmal hemicrania may be secondary to other processes.

Acute treatment: The disease responds dramatically to indomethacin (it can even be used to confirm the diagnosis). The initial dose is 25mg PO TID that can be increased to up to 50mg TID after 10 days and to 75mg TID after another 10 days if needed. After reaching control, the lowest effective dose should be continued chronically.

Chronic treatment: Indomethacin should be maintained in the best tolerable dose that provides relief without significant side effects or complication. In patients that cannot tolerate indomethacin, another NSAID or verapamil can be used.

Short-lasting unilateral neuralgiform headache attacks (with conjunctival injection and tearing – SUNCT or with cranial autonomic symptoms – SUNA)

Epidemiology: SUNCT and SUNA are rare and have uncertain prevalence. Onset is in adulthood (35-65 years) and it is more predominant in men.

Causes: It is a member of the trigeminal autonomic cephalalgias (TACs) group. It is caused by activation of the autonomic nervous system of the trigeminal nerve in the face due to hypothalamic influence.

Location: Unilateral, orbital, supra-orbital, retro-orbital or temporal.

Duration: Brief attacks (mean duration ~60 seconds but may range from second to 10 minutes) that may occur from 3 to 200 times a day (mean 60 attacks a day) for many days alternating with remission periods, or it may be chronic.

Characteristics of the pain: Severe stabbing (single stabs, groups of stabs or saw-tooth pattern), burning or electrical pain that may occur spontaneously or may be triggered by touching of the face, shaving, chewing, coughing, talking, blowing the nose or showering.
Associated symptoms: In SUNCT you have unilateral autonomic symptoms (rhinorrhea, miosis, ptosis, congestion, sweating) with conjunctival injection and lacrimation. In SUNA, you may have conjunctival injection OR lacrimation, but not both.

Physical exam and diagnosis: Physical exam may show signs of autonomic activity during an attack. A trial of indomethacin may be useful to exclude paroxysmal hemicrania and hemicrania continua (SUNCT and SUNA have poor response to indomethacin). A trial of oxygen may be useful to exclude cluster headache (SUNCT and SUNA have poor response to oxygen). MRI may be useful to exclude secondary causes.

Acute treatment: Lidocaine IV (loading dose of 1mg/kg over 15 min followed by1.5-3.5mg/kg/hour) is useful and it may be used for up to 7 days. It is important to do continuous ECG monitoring during infusion and observe the patient for cardiac arrhythmias, cognitive impairment and other adverse effects.

Chronic treatment: Lamotrigine (start with 25mg/day and increase by 25mg/day every two weeks until reaching 200-400mg/day divided in two doses) can be used as a preventive medication. Alternative medications include topiramate and gabapentin. For patients that do not respond to medication, other procedures have been proposed such as greater occipital nerve blockade, stimulation, and deep brain stimulation, although evidence for these treatments is still scarce.

Sinus headache

Epidemiology: Around 15% of the population will have an episode of sinusitis each year. It is more common in women than men, and ages 45-65 years.

Causes: Rhinosinusitis can be acute (< 4 weeks), subacute (4-12 weeks), chronic (> 12 weeks) or recurrent acute (four or more episodes in a year). The etiology may be viral (rhinovirus, influenza virus, parainfluenza virus) or bacterial (Streptococcus pneumoniae, Haemophilus influenzae, Moraxella catarrhalis). Fungal rhinosinusitis, allergic diseases and autoimmune diseases (Wegeners) are less common causes.

Location: Frontal and maxillary pain.

Duration: May be acute or chronic depending on the causative agent.

Characteristics of the pain: Mild to moderate pressure pain on the face that can get worse when the patient leans forward or touches the area.

Associated symptoms: Fever, postnasal drip, nasal obstruction, cough, malaise, hyposmia or anosmia, ear pain. Complications include orbital cellulitis, meningitis, intracranial or epidural abscesses, venous sinus thrombosis and osteomyelitis of the sinus bones.

Physical exam and diagnosis: Systemic signs (fever), visible postnasal drip, pain on palpation of facial sinuses and cheeks may be observed. Diagnosis is based on clinical signs, symptoms and imaging (CT scan) if needed.

Acute treatment: Viral sinusitis (98% of the cases) should be treated with supportive care (analgesics, saline irrigation and maybe intranasal steroids) and most patients resolve within 10 days. Patients with more than 7-10 days of symptoms may have bacterial sinusitis and antibiotics should be used (amoxicillin-clavulonate 1g/125mg ER tablets PO BID OR Doxycicline 100mg BID). Other options for allergic patients include levofloxacin 500mg qDay or moxifloxacin 400mg qDay. The antibiotics can be given for 5-7days. If the first antibiotic does not work, a second one can be started. Patients that do not respond to two different antibiotic trials should have imaging (CT scan) to look for anatomic blockage.

Chronic treatment: Patients with chronic rhinosinusitis (>12 weeks) should be evaluated with imaging (CT scan) and for secondary causes and confirmation of diagnosis. Referral to ENT for acquisition of sinus material for culture is relevant. Topical saline may relief the symptoms, and steroids may help reducing secretions and inflammation. Antileukotrienes may also be useful. Some patients may need oral steroids (20mg BID for 5 days followed by 20mg daily for 5 days) and antibiotics (3 to 4 weeks). Functional endoscopic sinus surgery may be helpful, particularly in patients with fungal sinusitis.

Primary stabbing headache

Epidemiology: Prevalence is not really clear, but it may be responsible for around 13% of the headaches in headache clinics.

Causes: Not clear. Most patients have also another type of primary headache (so the stabbing headache may be caused by the activation of random neurons that are often activated by the other headache).

Location: It can occur anywhere in the head.

Duration: Transient, short duration attacks at irregular intervals.

Characteristics of the pain: Sharp, stabbing or jabbing pain that may occur isolated or in in groups. It has mild intensity.

Associated symptoms: Not associated with autonomic symptoms.

Physical exam and diagnosis: Physical exam is normal and diagnosis is made by history. MRI may be useful in excluding secondary causes.

Treatment: Melatonin (3-12mg/day), indomethacin (75-150mg/day) or rofecoxib may improve the pain.

Idiopathic Intracranial Hypertension (Pseudotumor cerebri)

Epidemiology: The annual incidence of IIH is 1-2/100.000. It is more common among obese women aged 15-44 years.

Causes: The etiology is not completely known, although some mechanisms are proposed, such as cerebral venous outflow abnormalities, increased CSF outflow resistance and obesity-related increase in intracranial venous pressure.Some medications such as growth hormone, tetracyclines and vitamin A may be related to IIH.

Location: Lateral, retrobulbar or retrorbital. Sometimes the pain may follow the trigeminal nerve.

Duration: The headache may be intermittent or persistent.

Characteristics of the pain: Throbbing or pulsatile in character that sometime may be exacerbated by postural changes. Pain during eye movements is also common. The pain can be disabling.

Associated symptoms: Transient visual obscurations, pulsatile tinnitus, photopsia (perceived flashes of light), back pain, retrobulbar pain, diplopia and visual loss may be present. Nausea and vomiting may also be present during pain episodes.

Physical exam and diagnosis: Papilledema on funduscopic examination is characteristic in patients with IIH. Visual field loss and sixth nerve palsy may also be present, as well as other cranial nerves (I, III, IV, V, VII, VIII). The diagnosis should be suspected in any patient with headache and papilledema. Neuroimaging (e.g. MRI, preferably with venography) should be used to exclude secondary causes. Lumbar puncture will shall elevated opening pressure (higher than 250mmH2O) and normal CSF composition.

Acute treatment: Agents that may worsen the headache should be discontinued. Acetazolamide 500mg BID can be used, as well as short term high dose prednisone (60-100mg/day). This treatment can be done outpatient as long as the patient is not experiencing ongoing visual field loss. If the visual field or acuity worsening does not improve after 24-48h of medication, emergency CSF shunting should be done (other indications for shunting include intractable headache, hypotension risk and noncompliance).

Chronic treatment: Weight loss is important. Acetazolamide should be continued as tolerated and furosemide may be added for additional benefit. Other medications such as valproate, topiramate, propranolol and tricyclic antidepressants can be used for headache prophylaxis. Patients that do not respond to therapy may benefit from CSF shunting or optic nerve sheath fenestration (ONSF).

Trigeminal neuralgia

Epidemiology: Rare (although one of the most common neuralgias) and more common in women than men. The majority of cases occur after 50 years of age.

Causes: The compression of the trigeminal nerve root by a blood vessel is probably the most common mechanism. Other mechanisms include infections (herpes), demyelinating or inflammatory conditions (multiple sclerosis) or tumor compression (cist, schwannoma, among others).

Location: Unilateral, occurring in one or more divisions of the trigeminal nerve (V1-ophthalmic, V2-maxillary or V3-mandibular). V2 and V3 are most commonly affected.

Duration: Paroxysmal, recurrent episodes with abrupt onset and termination. They may last from few to many seconds and may have a refractory period of many minutes.

Characteristics of the pain: Intense, superficial, shock-like pain (sharp or stabbing).

Associated symptoms: Facial muscle spasms may be present (tic doulourex). Autonomic symptoms may be present when V1 is affected.

Physical exam and diagnosis: Physical exam may show tic dolorourex or autonomic symptoms during an attack. The pain may also be triggered by pressure in the nerve areas or chewing, talking, brushing teeth and smiling. Diagnosis is based on clinical suspicion, and the exclusion of secondary causes is often important. In such cases, MRI and magnetic resonance angiography (MRA) may be used.

Acute treatment: The short nature of the pain episodes makes acute treatment often unnecessary. On cases in which a medication is needed, some believe that phenytoin infusion can be used to relief the pain acutely, although this conduct is not backed up by good evidence.

Chronic treatment: The chronic treatment should start as soon as the disease is identified. Carbamazepine (100-200mg BID that may be increased slowly until 1200mg/day) may be used. Alternatives include oxcarbazepine (600-1800mg/day), baclofen (40-80mg daily), lamotrigine (400mg daily), topical lidocaine, among others. Refractory pain may benefit from Botox injections and surgical therapy or procedures (microvascular decompression, radiosurgery, peripheral neurectomy or rhizotomy).

Hypnic headache

Epidemiology: Rare, with unknown incidence and prevalence (probably less than 0.5%), it most often occurs after 50 years of age and is more common among females (2:1 female to male).

Causes: Not really understood, it may be associated with hypothalamic dysfunction and/or a REM sleep disorder.

Location: It may be bilateral or unilateral.

Duration: After waking the patient up the pain may persist for at least 15 minutes, usually lasting no more than 3 hours. It happens frequently (> 10 days a month).

Characteristics of the pain: Dull or throbbing pain that wakes the patient up from sleep.

Associated symptoms: Autonomic symptoms are usually not present.

Physical exam and diagnosis: Physical exam is normal. Diagnosis is made by the characteristics of the pain (that occurs only at night during sleep). The exclusion of secondary causes is important (e.g. MRI).

Acute treatment: Caffeine (40-60mg tablet or beverage) or triptans (sumatriptan 40mg) may be used for acute treatment.

Chronic treatment: Some drugs may have a prophylactic effect, such as lithium. However, lithium has a narrow therapeutic window (therapeutic plasma levels 0.5 to 1.0mmol/L) and the side effects can be significant, so it is usually not a first line treatment. Caffeine may be used before bedtime as a prophylactic treatment, as well as indomethacin (25 to 150mg/day) and topiramate (25mg per day at night).

Nummular headache

Epidemiology: Rare condition that may be primary or secondary to another process.

Causes: Not clear. It may be related to autoimmune conditions.

Location: Parietal region of the scalp is affected most often in a focal pattern (one circular area of pain).

Duration: Continuous pain, although remission periods may exist.

Characteristics of the pain: Mild to moderate coin-shaped headache – small, circumscribed round or elliptical (2-6cm diameter) areas of pain – with a pressure or stabbing character. Worsening periods may occur with lancinating exacerbations.

Associated symptoms: No autonomic symptoms associated. The area of pain may have allodynia, paresthesia or hyperesthesia during pain-free intervals.

Physical exam and diagnosis: Nothing major can be detected. History is what determines the diagnosis. MRI may be useful to exclude secondary causes.

Acute treatment: Indomethacin (25mg BID) may be useful.

Chronic treatment: Gabapentin (900-1800mg daily) can be effective, although nummular headaches may become refractory to treatment.

Temporomandibular Joint Related Pain

Epidemiology: Prevalence is higher between 18 and 44 years, among Caucasians, and ~ 1.5 times higher in women than men.

Causes: Anything that can affect the TMJ position or function (trauma, head and cervical posture, environment, psychiatric disorders).

Location: Unilateral pain in the jaw area that may radiate to the ear, temple or neck.

Duration: Pain occurs on and off and duration varies widely.

Characteristics of the pain: Dull, constant pain with alternating increases and decreases in intensity, worsened by jaw motion and in the morning.

Associated symptoms: Tinnitus, dizziness, sensation of neck pain or sinus pain.

Physical exam and diagnosis: Tenderness of the TMJ may be present as well as a decreased range of motion, neck and facial muscle stiffness, postural asymmetry, and abnormal mandibular movements. Imaging may be useful (panoramic radiography of the jaws, followed by CT or MRI of TMJ if necessary).

Acute treatment: In addition to behavioral orientation, NSAIDs may be used (e.g. naproxen 250-500mg BID) for 10-14 days. Muscle relaxants (e.g. cyclobenzaprine 10mg at night) may also be used with the NSAIDs. Benzodiazepines (e.g. diazepam 2.5-5mg at night for 2-3 weeks) may be used as well.

Chronic treatment: Patient education (avoid triggering behaviors), physical therapy if needed, dentist evaluation is important to assess dental anatomy (and conduct treatment as needed) and to provide a splint if necessary. The use of tricyclic antidepressants (e.g. amitriptyline 25mg qDay for 14 days-4 months) may be useful in patients that do not respond to NSAIDs and muscle relaxants. Cases that do not respond to any of the above interventions may need intra-articular injections (steroids or hyaluronic acid), Botox injections or even surgery.

Medication Overuse Headache

Epidemiology: Estimated prevalence of 1-1.5% in the general population.

Causes: Usually a complication of the treatment of another type of headache (usually migraine or tension-type headache) due to overuse of medication to treat the primary pain (which causes rebound headache).

Location: Depends on the baseline headache that was being treated.

Duration: It occurs on 15 or more days per month.

Characteristics of the pain: Depends on the baseline headache that was being treated.

Associated symptoms: Depends on the baseline headache that was being treated.

Physical exam and diagnosis: Diagnosis is based on history (pain > 15 days and frequent use of medications that should be used only to treat acute episodes, multiple times a day during more than 15 days).

Acute treatment: Suspension of the medication being overused along with a bridge therapy (prednisone or long acting NSAID orally or dihydroergotamine or metoclopramide IV) and the beginning of a preventive medication for the baseline headache if it exists.

Chronic treatment: Patient education and use of alternative strategies and medicines for the chronic treatment of the primary headache.

Giant Cell Arteritis (Temporal Arteritis)

Epidemiology: The most common systemic vasculitis, affecting 1% of the women and 0.5% of the men and occurring after 50 years of age (peak around 70`s). Northern European and Scandinavian people seem to be affected more often, suggesting a genetic factor.

Causes: Giant cell arteritis is a large and medium size vessel vasculitis that commonly affects the branches of the aortic arch. The etiology is not completely known, but it is believed to occur due to activation of immune cells on the vessel wall, causing inflammatory reaction and the formation of giant cells. It is frequently associated with polymyalgia rheumatica (that can cause pain and stiffness on the shoulders, neck, torso and hip, worse in the morning).

Location: Pain may occur in any affected vessel, but most prominently occurs in the temporal region.

Duration: Variable, on and off.

Characteristics of the pain: Tenderness of the scalp (temporal, frontal or occipital), with that may or may not be pulsating.

Associated symptoms: Loss of vision, jaw claudication, fever, weight loss, polymyalgia rheumatica symptoms, extremity claudication.

Physical exam and diagnosis: The temporal artery may be palpable and tender. The patient may have systemic symptoms and the visual acuity tests may be abnormal. The diagnosis of temporal arteritis should be considered in any patient over 50 with: new headache, scalp tenderness, jaw and tongue pain, visual disturbances, systemic symptoms (fever, weight loss), polymyalgia rheumatica and elevated inflammatory markers. The diagnosis can be confirmed by a biopsy of an affected artery (usually temporal). Blood tests (ESR and CRP in particular may be elevated) may be helpful. MRI/MRA and PET scan may also indicate vasculitis.

Acute treatment: Because there is a risk of blindness the acute treatment should not wait for the confirmatory diagnosis (biopsy). Steroids should be initiated promptly – methylprednisolone 1000mg/day IV for 3 days followed by 40-60mg/day of oral prednisone. Pains usually improve very well after 24-48 hours, but the steroids should continue for between 2-4weeks and with tapering after that.

Chronic treatment: Aspirin (81-100mg) should be given to all patients to reduce the risk of visual loss, TIA and stroke. After 2-4weeks of high dose prednisone the tapering should start (reduce 60->50mg after two weeks, reduce 50->40mg after other two weeks, reduce 10% of the dose every two weeks after that until reaching 10mg/day. After 10mg/day is achieved, reduce 1mg/month before stopping). During the tapering, the challenge is monitoring disease activity and flares. Symptoms, ESR and CRP are useful for such purpose. Patients that cannot tolerate prednisone may benefit from methotrexate, tocilizumab or cyclophosphamide. In rare situations, revascularization may be needed to reduce claudication symptoms.

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