Rare Types of Chronic Headache and Migraine

From TACs to hemicranias, these are the headaches you may not be aware of

There are an abundance of headache disorders, all of which present in unique ways. It’s important to be evaluated by a headache specialist if you suspect you may have one of the rare headache conditions detailed below, such as cluster headaches, paroxysmal hemicrania, or cervicogenic headache. These headaches have a low prevalence among the global population, but can be quite severe, differing from the more common tension headache (which affects up to 12% of the US population) and other primary headaches. A primary headache is caused by overactivity of or problems with the pain-sensitive structures in your head while secondary headaches are caused by another underlying condition. Getting an early diagnosis can make it easier to track your attacks and manage them appropriately.

Trigeminal Autonomic Cephalalgias (TACs)

Trigeminal autonomic cephalalgias, or TACs, are a group of primary headaches characterized by pain that occurs on one side of the head or face. TACS are often accompanied by a small number of symptoms that may last for a short period of time on the same side of the head, such as teary eyes, nasal congestion, and, in some cases, disrupted nerve pathways such as facial tics or paralysis. These types of headaches are often challenging to diagnose and treat due to their specific nature.

Among the plethora of headache disorders, these rare kinds may be affecting you. (Source: 123RF)


Types of TACs

Cluster Headache

While often confused for a migraine, cluster headaches can present different symptoms. Cluster headache (CH) is a primary headache disorder that consists of severe headaches occurring on one side of the head that may be associated with red or teary eyes, runny or stuffy nose, flushing or sweating of the face, and/or a sense of restlessness and agitation, according to the American Migraine Foundation. The term “cluster” refers to the recurrence of headache attacks in a series (cluster periods) lasting for weeks or months, separated by periods of remission lasting for months or years. Extremely rare, cluster headache affects about 1 in 1,000 adults, according to the World Health Organization, occurring mostly in men of middle age (cluster headache affects six men to every woman). They are most likely caused by abnormalities in the region of the brain called the hypothalamus and can occur without a trigger.

For acute cluster headache pain, your doctor may prescribe oxygen therapy, triptans (selective serotonin receptor agonists that stimulate serotonin in the brain to reduce inflammation and constrict blood vessels to relieve pain), ergots (medications that constrict and tighten blood vessels to relieve pain) such as dihydroergotamine, or lidocaine nasal spray. Preventive treatments may include verapamil, corticosteroids, lithium, and anti-seizure drugs such as topiramate and valproate, among others. Surgical options and neurostimulation have also shown promise as potential alternative and complementary treatments, according to the American Headache Society.

Hemicrania Continua

Hemicrania continua is a primary chronic daily headache marked by continuous, fluctuating pain that varies from mild to moderate severity (with occasional attacks of severe pain), always occurring unilaterally (on one side of the head/face), and superimposed with additional debilitating symptoms, such as watery or red eyes, blocked nasal passages, and runny nose, according to the National Institute of Neurological Disorders and Stroke and the Migraine Trust. However, some individuals with hemicrania continua report bilateral pain (that is, pain on both sides of the head/face). Patients have also reported “ice pick” pain (see below) with hemicrania continua, according to the American Migraine Foundation.

According to the Migraine Trust, the incidence and prevalence of hemicrania continua (along with paroxysmal hemicrania, detailed below) remains unknown. Researchers have found that hemicrania continua is more common in women, and, although the condition usually starts in adulthood, the range of onset has been reported from 5 to 67 years of age.

Hemicrania continua headaches are typically diagnosed when a patient has had a one-sided daily or continuous headache of moderate intensity with occasional short, piercing head pain for more than 3 months without shifting sides or pain-free periods. The headache must also be completely responsive to the treatment of a non-steroidal anti-inflammatory drug (NSAID) called indomethacin, according to the National Institute of Neurological Disorders and Stroke.

Indomethacin (brand name: Indocin) is a medication that fights inflammation, and is the only currently available medication that functions as a key to stop hemicrania continua headaches, according to the American Headache Society. Starting at a low dose (25 mg) three times a day, the dose is increased until the head pain is relieved. Therefore, doses can sometimes reach as high as 75 mg at three times a day or more before the pain is fully blocked, notes the AHS. When taking this medication, stomach protection against ulcers and bleeding is generally required. Thus, proton pump inhibitors (PPIs) such as omeprazole or lansoprazole, or H2 receptor antagonists such as ranitidine or famotidine, are usually prescribed by a doctor.

If you suspect you are experiencing hemicrania continua headaches, ask your doctor to be assessed. An evaluation is necessary to rule out other types of chronic headaches and chronic migraine.

Paroxysmal Hemicrania

This rare form of headache occurs primarily in adults with patients reporting severe throbbing, claw-like, or boring pain, usually on one side of the face; in, around, or behind the eye; and occasionally reaching the back of the neck, according to the National Institute of Neurological Disorders and Stroke. Episodes of paroxysmal hemicrania typically occur from 5 to 40 times per day and last 2 to 30 minutes. Along with attacks, the headache may cause episodes of redness or tearing of the eye, runny or stuffy nose, and sweating or flushing of the face on the same side of the headache pain (called unilateral cranial autonomic symptoms), according to the American Migraine Foundation. Certain movements of the head or neck, or external pressure to the neck, may trigger these headaches in some patients.

Brain imaging, usually with magnetic resonance imaging (MRI), is often necessary for diagnosis, especially if a patient does not respond well to treatment, according to the American Migraine Foundation. Much like hemicrania continua, the responsiveness to indomethacin as a treatment is an indicator for the headache. If patients cannot take indomethacin, verapamil, or pericranial, your doctor may recommend nerve blocks.

Primary headache disorders that might mimic paroxysmal hemicrania include: primary stabbing headache, cluster headache (see above), short lasting unilateral neuralgiform headaches with conjunctival injection and tearing (known as SUNCT and described below) and trigeminal neuralgia, according to the American Migraine Foundation.

SUNCT Headaches

Short-Lasting Unilateral Neuralgiform Headache with Conjunctival Injection and Tearing (SUNCT)

SUNCT syndrome headaches stand for Short-lasting Unilateral Neuralgiform headache with Conjunctival injection and Tearing, a rare disorder that primarily affects men over the age of 50. This condition is marked by bursts of moderate to severe burning, piercing, or throbbing pain, usually on one side of the head and around the eye or temple. The pain usually peaks within seconds of onset and may follow a pattern of increasing and decreasing intensity, according to the National Institute of Neurological Disorders and Stroke. Attacks typically occur in daytime hours and last from 5 seconds to 4 minutes per episode, with five to six attacks per hour.

SUNCT headaches occur in approximately 6 out of every 100,000 individuals and represent a major subset of SUNA syndrome, which stands for Short-lasting Unilateral Neuralgiform headache attacks with cranial Autonomic symptoms.

Typical nervous system responses include watery eyes, reddish or bloodshot eyes caused by dilation of blood vessels (conjunctival injection), nasal congestion, runny nose, sweating, swelling of the eyelids, and increased pressure within the eye on the affected side of the head. Blood pressure may also rise during the attacks and movement of the neck may trigger these headaches.

Prescribed corticosteroids and the anti-epileptic drugs gabapentin, lamotrigine, and carbamazepine may help to relieve some symptoms of these headaches in some patients, but SUNCT headaches are normally unresponsive to usual treatments and may require broader interventions.

Cervicogenic Headache

A cervicogenic headache occurs as a symptom of a bone, disc, and/or soft tissue disorder of the cervical spine. These secondary headaches are essentially “referred pain,” meaning that while the pain is perceived to be coming from the head, it is actually coming from a source in the neck, according to the American Migraine Foundation.

To differentiate this type from other likely headaches, there must be evidence—typically found through imaging such as MRI—of a disorder or lesion within the cervical spine or the soft tissues of the neck, such as a:

  • tumor
  • fracture
  • infection
  • or rheumatoid arthritis of the upper cervical spine.

According to the American Academy of Physical Medicine and Rehabilitation, the prevalence of cervicogenic headache is estimated to be up to 4.1% in the general population. However, its prevalence can be as high as 17.5% among patients with severe headaches, and 53% in patients who suffer headache after whiplash. The mean age of patients with cervicogenic headaches is 42.9 years, and the disorder is four times more prevalent in women.

Those suffering from cervicogenic headache often report tenderness or a reduced range of motion in their neck, or a worsening headache associated with neck movement (however, the headache itself may or may not be associated with neck pain). The headaches typically occur on one side of the head, and the pain may radiate from the neck or back of the head up to the front of the head or behind the eye. There is also the possibility that cervical spondylosis, or age-related wear and tear affecting the spinal discs in the neck, may cause this type of headache.

Treatment for cervicogenic headache aims to target the cause of the pain in the neck and varies from patient to patient. Nerve blocks can be used to relieve pain and can also serve as a diagnostic tool. For instance, if numbing the cervical area gets rid of the headache, a doctor may be able to confirm the presence of cervicogenic headache, according to the American Migraine Foundation. Other treatment options include over-the-counter NSAIDs (prescription strength if needed) and physical therapy, with a continuous exercise regimen. Pain specialists, neurosurgeons, and/or orthopedic surgeons may be involved as part of your treatment team.

Primary Stabbing/Ice Pick Headache

Primary stabbing headache, commonly called “ice pick” headache, are short, stabbing, and extremely intense headaches that generally last for only seconds and are common in patients with migraine, according to John Hopkins Medicine. While ice pick headaches occur in about 2% of the general population, prevalence has been reported as high as 35% in some cases, according to the National Headache Foundation. Other common names for stabbing headache include ice-pick pains; jabs and jolts; needle-in-the-eye syndrome; ophthalmodynia periodica; and sharp short-lived head pain, according to the American Migraine Foundation.

Symptoms of primary stabbing headache include single or multiple stabs of pain that occur seemingly out of nowhere. These stabs can last seconds (many lasting under three seconds), and the stabs can move from one area to another in either the same side of the head or the opposite side. If the stabs occur only in one place, it is important to be evaluated by your doctor to exclude any structural changes or injury to a nerve at that site. At most, primary stabbing headaches occur only a few times a day, but may occur more frequently in rare cases. If you have frequent episodes of this type of headache, anticonvulsants or antidepressants may be helpful as preventive treatment. However, many patients do not require treatment.

If you suspect you may have a stabbing headache, request a formal evaluation with your doctor to rule out any underlying causes and to differentiate the headache from other common mimicking disorders, recommends the American Migraine Foundation. If you experience other symptoms with the stabbing pains, such as watery/red eyes, runny or stuffy nose, or swelling and flushing of the face, you may be showing signs of SUNCT headaches (see above). Other similar headaches that need to be ruled out include occipital or other cranial neuralgias, trigeminal neuralgia, and nummular headache.

Talk to Your Doctor

When you get a bad headache, your first instinct may be to think of it as a migraine. But if your headaches become more frequent and varied in their symptoms, talk to your healthcare provider about ruling out one or more of these rare types of chronic headache.

Updated on: 09/30/20
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