Headache Syndromes

What are headache syndromes?

The primary headache syndromes are migraine, tension-type, and cluster headaches. Migraine and cluster headaches are episodic and recurring conditions. Tension-type headache is usually episodic but like migraine, it can become chronic, occurring daily or almost daily for more than 15 days a month.

What are the causes?

There are more than two hundred types of headaches. Some are harmless and some are life threatening. The description of the headache and findings on neurological examination, determine whether additional tests are needed and what treatment is best.

Primary vs. secondary headaches

Headaches are broadly classified as “primary” or “secondary”. Primary headaches are benign, recurrent headaches not caused by underlying disease or structural problems. For example, migraine is a type of primary headache. While primary headaches may cause significant daily pain and disability, they are not dangerous. Secondary headaches are caused by an underlying disease, like an infection, head injury, vascular disorders, brain bleed or tumors. Secondary headaches can be harmless or dangerous. Certain “red flags” or warning signs indicate a secondary headache may be dangerous.

Primary headaches

90% of all headaches are primary headaches. Primary headaches usually first start when people are between 20 and 40 years old. The most common types of primary headaches are migraines and tension-type headaches. They have different characteristics. Migraines typically present with pulsing head pain, nausea, photophobia (sensitivity to light) and phonophobia (sensitivity to sound). Tension-type headaches usually present with non-pulsing “bandlike” pressure on both sides of the head, not accompanied by other symptoms. Other very rare types of primary headaches include:

  • Cluster headaches: short episodes (15–180 minutes) of severe pain, usually around one eye, with autonomic symptoms (tearing, red eye, nasal congestion) which occur at the same time every day. Cluster headaches can be treated with triptans and prevented with prednisone, ergotamine or lithium.
  • Trigeminal neuralgia: shooting face pain
  • Hemicrania continua: continuous unilateral pain with episodes of severe pain. Hemicrania continua can be relieved by the medication indomethacin.
  • Primary stabbing headache: recurrent episodes of stabbing “ice pick pain” or “jabs and jolts” for 1 second to several minutes without autonomic symptoms (tearing, red eye, nasal congestion). These headaches can be treated with indomethacin.
  • Primary cough headache: starts suddenly and lasts for several minutes after coughing, sneezing or straining (anything that may increase pressure in the head). Serious causes (see secondary headaches red flag section) must be ruled out before a diagnosis of “benign” primary cough headache can be made.
  • Primary exertional headache: throbbing, pulsatile pain which starts during or after exercising, lasting for 5 minutes to 24 hours. The mechanism behind these headaches is unclear, possibly due to straining causing veins in the head to dilate, causing pain. These headaches can be prevented by not exercising too strenuously and can be treated with medications such as indomethacin.
  • Primary sex headache: dull, bilateral headache that starts during sexual activity and becomes much worse during orgasm. These headaches are thought to be due to lower pressure in the head during sex. It is important to realize that headaches that begin during orgasm may be due to a subarachnoid hemorrhage, so serious causes must be ruled out first. These headaches are treated by advising the person to stop sex if they develop a headache. Medications such as propranolol and diltiazem can also be helpful.
  • Hypnic headache: moderate-severe headache that starts a few hours after falling asleep and lasts 15–30 minutes. The headache may recur several times during night. Hypnic headaches are usually in older women. They may be treated with lithium.

Secondary headaches

Headaches may be caused by problems elsewhere in the head or neck. Some of these are not harmful, such as cervicogenic headache (pain arising from the neck muscles). Medication overuse headache may occur in those using excessive painkillers for headaches, paradoxically causing worsening headaches.

More serious causes of secondary headaches include:

  • Meningitis: inflammation of the meninges which presents with fever and meningismus, or stiff neck
  • Bleeding inside the brain (intracranial hemorrhage)
  • Subarachnoid hemorrhage (acute, severe headache, stiff neck WITHOUT fever)
  • Ruptured aneurysm, arteriovenous malformation, intraparenchymal hemorrhage (headache only)
  • Brain tumor: dull headache, worse with exertion and change in position, accompanied by nausea and vomiting. Often, the person will have nausea and vomiting for weeks before the headache starts.
  • Temporal arteritis: inflammatory disease of arteries common in the elderly (average age 70) with fever, headache, weight loss, jaw claudication, tender vessels by the temples, polymyalgia rheumatica
  • Acute closed angle glaucoma (increased pressure in the eyeball): headache that starts with eye pain, blurry vision, associated with nausea and vomiting. On physical exam, the person will have a red eye and a fixed, mid dilated pupil.
  • Post-ictal headaches: Headaches that happen after a convulsion or other type of seizure, as part of the period after the seizure (the post-ictal state)

What are the symptoms?

Migraine

Most migraine patients do not have an aura; migraine with aura occurs in only 15% to 20% of sufferers. The aura is a well-defined visual or neurologic deficit lasting less than 1 hour and is followed by the headache within 1 hour. Most auras are visual, with photopsia (flashing lights) being most common. The aura is initially small, then enlarges or moves across the visual field. A typical migraine aura can occur without a headache. This phenomenon tends to occur later in life. Occasionally, a neurologic aura occurs, with a tingling or weakness that slowly spreads up or down an extremity.

Many patients with migraine have prodromal symptoms for many hours or even a day or so before the onset of an attack. These prodromal symptoms are generally changes in mood or personality. Fatigue also is common, and occasionally hyperactivity occurs.

The migraine attack lasts 6 to 72 hours. The pounding, throbbing pain of moderate to severe intensity is generally unilateral, but some patients experience bilateral pain. Pain caused by migraine worsens with physical activity. Photophobia and phonophobia are very common, and sensitivity to odors is a little less common. Migraine is a sick headache. Nausea occurs in most patients, and vomiting is very common. Dehydration can occur, which increases the pain and disability. Migraineurs want to be quiet, inactive, and in a darkened area during the attack. Approximately 60% of women experience their worst migraine attacks in conjunction with their menstrual period.

Tension-Type Headache

Tension-type headache is characterized by generalized pressure or a sensation of tightness in the head. The discomfort level is usually mild to moderate and does not worsen with activity. Although nausea and photophobia or phonophobia can occur, they generally are not prominent features. Tension-type headache is classified as episodic (<15 days a month) or chronic (>15 days a month).

Some patients with tension-type headache exhibit evidence of increased muscle tension, with prominent scalp tenderness, muscle tenderness in the temporomandibular joint muscles, or tight, tender cervical and trapezius muscles. Poor posture is often evident, which can play a role in causing tension-type headache. If there is no evidence of increased pericranial or cervical muscle tightness (no tenderness or limitation of motion in the neck) found during clinical examination, the pain likely originates centrally or is due to psychological factors.

Cluster Headache

Cluster headache causes intense pain that is generally steady and boring behind one eye. The pain can spread to the temple, face, and even into the upper neck. It is so intense that most sufferers pace the floor or do vigorous exercises during the attack. The attacks are short (usually less than 3 hours in duration) and often last only 30 to 45 minutes. They occur from one to several times a day for a period of several weeks or months, then remit, leaving the patient pain free for several months or years, only to recur.

During a cluster headache cycle, the attacks of pain often occur at the same time each day, most often waking the patient in the early morning hours. Eighty percent of cluster sufferers experience unilateral tearing, with conjunctival injection and ipsilateral nasal congestion. These symptoms clear as the attack leaves. Alcohol brings on an attack within a few minutes in a patient who is in a cluster headache cycle, but it does not induce an attack when the patient is in remission.

Chronic Daily Headache

Daily headache can occur as a chronic tension-type headache, but it is often a combination of tension-type and migraine (the type seen most often in headache clinics). This type of combination headache is not listed in the official classification, so one should diagnose both chronic tension-type headache and migraine in these patients. Most often, this type of combination or mixed headache develops in a person who initially had typical episodic migraine but in whom, over several years, a chronic daily or almost-daily headache develops. Many times, this daily headache seems to occur because of the frequent use of analgesics, especially opiate compounds and those combined with caffeine or butalbital. A daily or near-daily migraine headache can occur from the frequent use of ergotamine tartrate or any of the triptan drugs. This headache pattern has been called rebound headache or medication overuse headache.

What are the treatments?

Primary headache syndromes have many different possible treatments. In those with chronic headaches the long term use of opioids appears to result in greater harm than benefit.

Migraines

Migraine can be somewhat improved by lifestyle changes, but most people require medicines to control their symptoms. Medications are either to prevent getting migraines, or to reduce symptoms once a migraine starts.

Preventive medications are generally recommended when people have more than four attacks of migraine per month, headaches last longer than 12 hours or the headaches are very disabling. Possible therapies include beta blockers, antidepressants, anticonvulsants and NSAIDs. The type of preventive medicine is usually chosen based on the other symptoms the person has. For example, if the person also has depression, an antidepressant is a good choice.

Abortive therapies for migraines may be oral, if the migraine is mild to moderate, or may require stronger medicine given intravenously or intramuscularly. Mild to moderate headaches should first be treated with NSAIDs, like ibuprofen, and/or acetaminophen. If accompanied by nausea or vomiting, an antiemitic such as metoclopramide (Reglan) can be given orally or rectally. Moderate to severe attacks should be treated first with an oral triptan, a medication that mimics serotonin (an agonist) and causes mild vasoconstriction. If accompanied by nausea and vomiting, parenteral (through a needle in the skin) triptans and antiemetics can be given.

Several complementary and alternative strategies can help with migraines. The American Academy of Neurology guidelines for migraine treatment in 2000 stated relaxation training, electromyographic feedback and cognitive behavioral therapy may be considered for migraine treatment, along with medications.

Tension-type headaches

Tension-type headaches can usually be managed with NSAIDs (ibuprofen, naproxen), acetaminophen or aspirin. Triptans are not helpful in tension-type headaches unless the person also has migraines. For chronic tension type headaches, amitriptyline is the only medication proven to help. Amitriptyline is a medication which treats depression and also independently treats pain. It works by blocking the reuptake of serotonin and norepinephrine, and also reduces muscle tenderness by a separate mechanism. Studies evaluating acupuncture for tension-type headaches have been mixed. Overall, they show that acupuncture is probably not helpful for tension-type headaches.

Cluster headaches

Abortive therapy for cluster headaches includes subcutaneous sumatriptan (injected under the skin) and triptan nasal sprays. High flow oxygen therapy also helps with relief.

For people with extended periods of cluster headaches, preventive therapy can be necessary. Verapamil is recommended as first line treatment. Lithium can also be useful. For people with shorter bouts, a short course of prednisone (10 days) can be helpful. Ergotamine is useful if given 1–2 hours before an attack.

Secondary headaches

Treatment of secondary headaches involves treating the underlying cause. For example, a person with meningitis will require antibiotics. A person with a brain tumor may require surgery, chemotherapy and/or brain radiation.

Neuromodulation
Peripheral neuromodulation has tentative benefits in primary headaches including cluster headaches and chronic migraine. How it may work is still being looked into.

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