Disease: Cluster Headaches

    What are cluster headaches?

    Cluster headache is pain that occurs along one side of the head. It's frequently described as pain that occurs around, behind, or above the eye and along the temple in cyclic patterns or clusters. The pain of a cluster headache is very severe. Many patients describe a “drilling” type of sensation. For classification as a true cluster headache, associated autonomic features such as tearing/watering of the eye, redness of the conjunctiva, rhinorrhea or nasal stuffiness, eyelid drooping, sweating on one side of the face, or changes in pupil size (with the pupil on the affected side becoming notably smaller) are usually present. The headache lasts from 15 minutes to a maximum duration of about 3 hours. However, the headache can recur up to eight times daily. Cluster headache was originally described in the 17th century, but it wasn't until the mid- 20th century that it became known by this name.

    Who gets cluster headaches?

    Males are two to four times more likely to develop cluster headache than females; however, the overall frequency is quite low, with a prevalence rate of about 1 per 1,000. Because of the rarity of the condition, limited information is available.

    Although the vast majority of patients are adults, cluster headache has been reported in children as young as 6 years of age.

    What are the symptoms and signs of cluster headaches?

    Cluster headache is always unilateral, or one-sided. However, some patients may experience some variability of the side on which their headache occurs. Most patients describe their pain as occurring around or behind the eye. Pain is also described as radiating along the forehead, into the jaw or along the gum line and into the teeth, or across the cheek of the affected side. Infrequently, pain may extend into the ear, neck, or shoulder. Although watering (tearing) of the eye is frequently identified, some patients may only experience some redness of the conjunctiva. Eyelid drooping or swelling and a runny nose (rhinorrhea) are often associated with the pain of a cluster headache. Symptoms more commonly identified with migraine headaches, including sensitivity to light, sounds, or odors may occur. However, unlike migraine headache, movement does not worsen the pain of a cluster headache. In fact, many patients describe a sense of restlessness during their pain.

    The headaches associated with cluster occur in groups. While the headaches themselves may be brief (as short as 15 minutes), the headaches can recur up to eight times in 24 hours. Headaches may last as long as 3 hours. Cluster cycles may last for only a single day, or may linger for many weeks.

    What causes cluster headaches?

    The specific cause and anatomic origination of cluster headaches isn't known. MRI studies suggest dilation of the ophthalmic artery during an acute cluster headache, while PET scans reveal activity within the cavernous sinus. However, many patients with other headache types also have revealed abnormalities in similar regions, so these tests aren't definitive. There is some evidence that the hypothalamus may be involved in the recurrence cycle of cluster headaches. Activation of the trigeminal ganglion can cause many changes associated with cluster headache, but the trigger for activation of this region hasn't been identified.

    What triggers cluster headaches?

    Many patients report their headaches begin while sleeping. Additionally, alcohol can trigger cluster headaches in patients who are in the midst of a cycle. Histamines and nitroglycerin can trigger cluster headaches in patients. Seasonal variation has been described, although this is inconsistent for many patients. Some patients have clusters precipitated by environmental changes or changes in stress or activity levels. Hormonal factors, or menstruation, do not seem to trigger cluster headache. Other risk factors include smoking and a family history of the problem.

    How are cluster headaches diagnosed?

    The diagnosis of cluster headache is typically made after the history of headaches has been explored and a physical examination is completed. Cluster headaches are unique in their presentation, and often the history is sufficient to make the diagnosis. While no imaging study or specific blood test can confirm the diagnosis of cluster headache, an MRI or CT scan of the brain may be ordered to confirm that there are no other contributing factors that may mimic cluster headache symptoms. In some cases, ophthalmologic evaluation is needed to exclude problems within the eye itself that may be causing symptoms.

    What is the treatment for cluster headaches?

    The treatment of cluster headache can be divided into two distinct categories -- relief of the acute headache and prevention of future headaches. Oxygen delivered by face mask has been shown to help a majority of patients within a short period of time. However, this can be unwieldy, and most patients are unable or unwilling to transport oxygen canisters if they need to travel. Injectable sumatriptan has been shown to be beneficial in many of patients with cluster headache. This treatment is contraindicated in patients with cardiac disease or untreated hypertension. Nasal spray or oral versions of this medication have been less effective than the injectable. Dihydroergotamine, given intravenously, can be extremely effective in treating a cluster headache, but can be difficult to administer acutely and cannot be used if a patient has used sumatriptan in the preceding 24 hours. Intranasal lidocaine has been suggested as a treatment option, but must be administered in a specific manner and is ineffective if not given correctly.

    Steroids can be extremely effective to decrease a headache cycle; these can be used infrequently and are for short-term use only as long-term use can lead to significant complications. Verapamil, lithium, valproic acid, topiramate, and melatonin can all be of benefit in reducing the frequency and severity of cluster cycles. In intractable cases, surgery has been suggested. Radiofrequency lesioning of the trigeminal ganglion can decrease cluster headache frequency, but is associated with significant side effects and nerve loss; gamma knife lesioning and deep brain stimulation are being studied as possible options with less risk of permanent nerve change.

    What causes cluster headaches?

    The specific cause and anatomic origination of cluster headaches isn't known. MRI studies suggest dilation of the ophthalmic artery during an acute cluster headache, while PET scans reveal activity within the cavernous sinus. However, many patients with other headache types also have revealed abnormalities in similar regions, so these tests aren't definitive. There is some evidence that the hypothalamus may be involved in the recurrence cycle of cluster headaches. Activation of the trigeminal ganglion can cause many changes associated with cluster headache, but the trigger for activation of this region hasn't been identified.

    What triggers cluster headaches?

    Many patients report their headaches begin while sleeping. Additionally, alcohol can trigger cluster headaches in patients who are in the midst of a cycle. Histamines and nitroglycerin can trigger cluster headaches in patients. Seasonal variation has been described, although this is inconsistent for many patients. Some patients have clusters precipitated by environmental changes or changes in stress or activity levels. Hormonal factors, or menstruation, do not seem to trigger cluster headache. Other risk factors include smoking and a family history of the problem.

    How are cluster headaches diagnosed?

    The diagnosis of cluster headache is typically made after the history of headaches has been explored and a physical examination is completed. Cluster headaches are unique in their presentation, and often the history is sufficient to make the diagnosis. While no imaging study or specific blood test can confirm the diagnosis of cluster headache, an MRI or CT scan of the brain may be ordered to confirm that there are no other contributing factors that may mimic cluster headache symptoms. In some cases, ophthalmologic evaluation is needed to exclude problems within the eye itself that may be causing symptoms.

    What is the treatment for cluster headaches?

    The treatment of cluster headache can be divided into two distinct categories -- relief of the acute headache and prevention of future headaches. Oxygen delivered by face mask has been shown to help a majority of patients within a short period of time. However, this can be unwieldy, and most patients are unable or unwilling to transport oxygen canisters if they need to travel. Injectable sumatriptan has been shown to be beneficial in many of patients with cluster headache. This treatment is contraindicated in patients with cardiac disease or untreated hypertension. Nasal spray or oral versions of this medication have been less effective than the injectable. Dihydroergotamine, given intravenously, can be extremely effective in treating a cluster headache, but can be difficult to administer acutely and cannot be used if a patient has used sumatriptan in the preceding 24 hours. Intranasal lidocaine has been suggested as a treatment option, but must be administered in a specific manner and is ineffective if not given correctly.

    Steroids can be extremely effective to decrease a headache cycle; these can be used infrequently and are for short-term use only as long-term use can lead to significant complications. Verapamil, lithium, valproic acid, topiramate, and melatonin can all be of benefit in reducing the frequency and severity of cluster cycles. In intractable cases, surgery has been suggested. Radiofrequency lesioning of the trigeminal ganglion can decrease cluster headache frequency, but is associated with significant side effects and nerve loss; gamma knife lesioning and deep brain stimulation are being studied as possible options with less risk of permanent nerve change.

    Source: http://www.rxlist.com

    The treatment of cluster headache can be divided into two distinct categories -- relief of the acute headache and prevention of future headaches. Oxygen delivered by face mask has been shown to help a majority of patients within a short period of time. However, this can be unwieldy, and most patients are unable or unwilling to transport oxygen canisters if they need to travel. Injectable sumatriptan has been shown to be beneficial in many of patients with cluster headache. This treatment is contraindicated in patients with cardiac disease or untreated hypertension. Nasal spray or oral versions of this medication have been less effective than the injectable. Dihydroergotamine, given intravenously, can be extremely effective in treating a cluster headache, but can be difficult to administer acutely and cannot be used if a patient has used sumatriptan in the preceding 24 hours. Intranasal lidocaine has been suggested as a treatment option, but must be administered in a specific manner and is ineffective if not given correctly.

    Steroids can be extremely effective to decrease a headache cycle; these can be used infrequently and are for short-term use only as long-term use can lead to significant complications. Verapamil, lithium, valproic acid, topiramate, and melatonin can all be of benefit in reducing the frequency and severity of cluster cycles. In intractable cases, surgery has been suggested. Radiofrequency lesioning of the trigeminal ganglion can decrease cluster headache frequency, but is associated with significant side effects and nerve loss; gamma knife lesioning and deep brain stimulation are being studied as possible options with less risk of permanent nerve change.

    Source: http://www.rxlist.com

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