Chronic paroxysmal hemicrania (CPH) is a severe debilitating unilateral

headache A headache, also known as cephalalgia, is the symptom of pain in the face, head, or neck. It can occur as a migraine, tension-type headache, or cluster headache. There is an increased risk of Depression (mood), depression in those with severe ...

usually affecting the area around the eye. It normally consists of multiple severe, yet short, headache attacks affecting only one side of the cranium. Retrospective surveys indicated that paroxysmal hemicrania was more common in women. However, subsequent prospective research showed an equal prevalence between females and males, with a ratio close to 1:1. Unlike in

migraine Migraine (, ) is a complex neurological disorder characterized by episodes of moderate-to-severe headache, most often unilateral and generally associated with nausea, and light and sound sensitivity. Other characterizing symptoms may includ ...

, it has no neurological symptoms associated with it. CPH headaches are treated through the use of

non-steroidal anti-inflammatory drugs A nonsteroidal compound is a drug that is not a steroid nor a steroid derivative. Nonsteroidal anti-inflammatory drugs (NSAIDs) are distinguished from corticosteroids as a class of anti-inflammatory agents. List of nonsteroidal steroid receptor m ...

, with

indomethacin Indometacin, also known as indomethacin, is a nonsteroidal anti-inflammatory drug (NSAID) commonly used as a prescription medication to reduce fever, pain, stiffness, and swelling from inflammation. It works by inhibiting the production of pr ...

found to be especially effective in eliminating symptoms. Paroxysmal hemicrania is classified by the characteristic (high) frequency and (short) duration of attacks experienced by patients that is somewhat similar to

cluster headache Cluster headache is a neurological disorder characterized by recurrent severe headaches on one side of the head, typically around the eye, eye(s). There is often accompanying eye watering, nasal congestion, or swelling around the eye on the aff ...

s, despite some important differences explained below. Episodic paroxysmal hemicrania attacks occur at least twice a year and last anywhere from seven days to a year with pain free periods of a month or longer separating them. Chronic paroxysmal hemicrania attacks occur over the course of more than a year without remission or with remissions lasting less than a month.Goadsby, Peter J., Stephen D. Silberstein, and David Dodick. "Chronic Paroxysmal Hemicrania". ''Chronic Daily Headache for Clinicians''. Hamilton, Ont.: BC Decker, 2005. N. pag. Print.

Signs and symptoms

Individuals with CPH suffer multiple short, severe headaches a day, often more than five, with most lasting between 5 and 30 minutes each. When compared to cluster headaches, CPH attacks are typically shorter. Each headache is centered around the eye, temple and forehead or the back of the head and is localized to one side of the head. While redness and watering of the eye are associated with CPH, patients typically do not experience

nausea Nausea is a diffuse sensation of unease and discomfort, sometimes perceived as an urge to vomit. It can be a debilitating symptom if prolonged and has been described as placing discomfort on the chest, abdomen, or back of the throat. Over 30 d ...

vomiting Vomiting (also known as emesis, puking and throwing up) is the forceful expulsion of the contents of one's stomach through the mouth and sometimes the nose. Vomiting can be the result of ailments like food poisoning, gastroenteritis, pre ...

. Although less common, CPH may also present as severe unilateral ear pain accompanied by autonomic symptoms. Autonomic symptoms may include the presence of red ear syndrome. Attacks hit the patient many times a day, from 5 times a day up to 40 times a day with an average of 11 a day. Mild background pain can persist between attacks. They come in bouts that last from 7 days to 1 year separated by remission periods that can last more than 3 months in episodic patients, or less than 3 months in chronic patients. Onset is in adulthood and the disorder may last indefinitely or spontaneously go into remission. Circadian mechanisms are likely involved in paroxysmal hemicrania due to its highly cyclic nature. No particular circannual recurrence characterizes symptomatic periods, although some patients can experience a seasonal preponderance.

Causes

The causes of paroxysmal hemicrania are ultimately still unknown. Sympathetic symptoms such as miosis and ptosis might be linked with a generalized sympathetic dysfunction. Neuropathic mechanisms may be involved, since attacks can be triggered by mechanical stimulation. Perivascular neurogenic inflammatory processes can worsen symptoms or increase pain. Dilated blood vessels may contribute in stimulating trigeminal nociceptors directly, although they cannot be the origin of pain, since even suppression of vasodilation does not stop it once it’s started. Many secondary conditions have been reported to be possible causes of CPH, according to Mehta et al., most of which are arterial abrasions or tumors. These include

aneurysms An aneurysm is an outward bulging, likened to a bubble or balloon, caused by a localized, abnormal, weak spot on a blood vessel wall. Aneurysms may be a result of a hereditary condition or an acquired disease. Aneurysms can also be a nidus ( ...

in the

circle of Willis The circle of Willis (also called Willis' circle, loop of Willis, cerebral arterial circle, and Willis polygon) is a circulatory anastomosis that supplies blood to the brain and surrounding structures in reptiles, birds and mammals, including huma ...

, middle cerebral artery

infarction Infarction is tissue death (necrosis) due to Ischemia, inadequate blood supply to the affected area. It may be caused by Thrombosis, artery blockages, rupture, mechanical compression, or vasoconstriction. The resulting lesion is referred to as a ...

, parietal

arteriovenous malformation An arteriovenous malformation (AVM) is an abnormal connection between arteries and veins, bypassing the capillary system. Usually congenital, this vascular anomaly is widely known because of its occurrence in the central nervous system (usually ...

, cavernous sinus and petrous ridge meningiomas,

pituitary adenoma Pituitary adenomas are tumors that occur in the pituitary gland. Most pituitary tumors are benign, approximately 35% are invasive and just 0.1% to 0.2% are carcinomas.Pancoast tumor A Pancoast tumor is a tumor of the apex of the lung. It is a type of lung cancer defined primarily by its location situated at the top end of either the right or left lung. It typically spreads to nearby tissues such as the ribs and vertebrae. Most ...

gangliocytoma A ganglioglioma is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently occurs in the temporal lobes of children and young adults. They are mixed cell tumors containing both neural ganglionic cells and neural gli ...

of the

sella turcica The sella turcica (Latin for 'Turkish saddle') is a saddle-shaped depression in the body of the sphenoid bone of the human skull and of the skulls of other hominids including chimpanzees, gorillas and orangutans. It serves as a cephalometric la ...

, and malignant frontal

tumors A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

.Mehta, Noshir R., George E. Maloney, Dhirendra S. Bana, and Steven J. Scrivani. "Head, Face, and Neck Pain: Science, Evaluation, and Management: An Interdisciplinary Approach". Hoboken, NJ: Wiley, 2009. Print. This accentuates the urgency for those diagnosed with CPH to receive an

MRI Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and rad ...

head scan.

Diagnosis

CPH is a long-term disease with symptoms lasting for longer than a year, either without remission or with remissions that last less than a month. In order to be diagnosed with CPH, a patient needs to have had at least 20 attacks filling the following criteria: *Attacks of severe unilateral orbital, supraorbital, or temporal pain lasting between 2 and 30 minutes. *The

needs to take place with one of the following: **

Ipsilateral Standard anatomical terms of location are used to describe unambiguously the anatomy of humans and other animals. The terms, typically derived from Latin or Greek roots, describe something in its standard anatomical position. This position prov ...

conjunctiva In the anatomy of the eye, the conjunctiva (: conjunctivae) is a thin mucous membrane that lines the inside of the eyelids and covers the sclera (the white of the eye). It is composed of non-keratinized, stratified squamous epithelium with gobl ...

l injection and/or

lacrimation Tears are a clear liquid secreted by the lacrimal glands (tear gland) found in the eyes of all land mammals. Tears are made up of water, electrolytes, proteins, lipids, and mucins that form layers on the surface of eyes. The different types of ...

**Ipsilateral nasal congestion and/or

rhinorrhoea Rhinorrhea (American English), also spelled rhinorrhoea or rhinorrhœa (British English), or informally runny nose is the free discharge of a thin mucus fluid from the nose; it is an extremely common condition. It is a common symptom of allergie ...

**Ipsilateral eyelid

oedema Edema (American English), also spelled oedema (British English), and also known as fluid retention, swelling, dropsy and hydropsy, is the build-up of fluid in the body's tissue. Most commonly, the legs or arms are affected. Symptoms may inclu ...

**Ipsilateral forehead and facial sweating **Ipsilateral

miosis Miosis, or myosis (), is excessive constriction of the pupil.Farlex medical dictionary
citing: ...

and/or ptosis *Attacks need to occur more than five times a day for more than half of the time, although periods of lower frequency can occur. *The symptoms cannot be attributed to another disorder. *Attacks can be prevented completely by therapeutic doses of

. In addition, diagnosis of CPH requires that

neuropathy Peripheral neuropathy, often shortened to neuropathy, refers to damage or disease affecting the nerves. Damage to nerves may impair sensation, movement, gland function, and/or organ function depending on which nerve fibers are affected. Neuropa ...

of the supraorbital area in the temporal branch of the

facial nerve The facial nerve, also known as the seventh cranial nerve, cranial nerve VII, or simply CN VII, is a cranial nerve that emerges from the pons of the brainstem, controls the muscles of facial expression, and functions in the conveyance of ta ...

be ruled out.Jay, Gary W. The Headache Handbook: Diagnosis and Treatment. Boca Raton: CRC, 1998. Print.

Comparison to cluster headaches

Though outwardly similar to cluster headaches, chronic paroxysmal hemicrania is rather different, and the two headaches are not a subset of one or the other. Key differences include: *CPH attacks occur more frequently, but are shorter. *Individuals with CPH are far more responsive to indomethacin than individuals with cluster headaches. *CPH attacks can be provoked by neck movement. *In a study conducted by Sjaastad, heating a patient's body will cause the painful side of the forehead to sweat more in CPH patients, while there will be less sweating on that side for those suffering from cluster headaches.

Treatments

A ten-patient study conducted by Pareja et al. found that all patients diagnosed with CPH were responsive to indomethacin and were able to completely control their symptoms. Doses of the drug ranged from 25 mg per day to 150 mg per day with a median dose of 75 mg per 24-hour period.Pareja, Ja, Ab Caminero, E. Franco, Jl Casado, J. Pascual, and M. Sanchez Del Ri'o. "Dose, Efficacy and Tolerability of Long-term Indomethacin Treatment of Chronic Paroxysmal Hemicrania and Hemicrania Continua". ''Cephalalgia''. 21.9 (2001): 906-10. Print. Almost all cases of CPH respond positively and effectively to indometacin, but as much as 25 percent of patients discontinued use of the drug due to adverse side effects, namely complications in the

gastrointestinal tract The gastrointestinal tract (GI tract, digestive tract, alimentary canal) is the tract or passageway of the Digestion, digestive system that leads from the mouth to the anus. The tract is the largest of the body's systems, after the cardiovascula ...

.Camarda, Cecilia, Rosolino Camarda, and Roberto Monastero. "Chronic Paroxysmal Hemicrania And Hemicrania Continua Responding To Topiramate: Two Case Reports". ''Clinical Neurology & Neurosurgery''. 100.1 (2008): 88-91. Academic Search Complete. Web. 27 Feb. 2014. According to a case study by Milanlioglu et al., 100 mg of

lamotrigine Lamotrigine ( ), sold under the brand name Lamictal among others, is a medication used to treat epilepsy and stabilize mood in bipolar disorder. For epilepsy, this includes focal seizures, tonic-clonic seizures, and seizures in Lennox-Gastau ...

, an

antiepileptic Anticonvulsants (also known as antiepileptic drugs, antiseizure drugs, or anti-seizure medications (ASM)) are a diverse group of pharmacological agents used in the treatment of epileptic seizures. Anticonvulsants are also used in the treatmen ...

drug, administered twice daily alleviated all painful symptoms. No side effects were noted after two months of treatment. Dosage of lamotrigine was decreased to 50 mg a day after the first two months, and no symptoms or side-effects were recorded after a three-month followup.Milanlioglu, A., T. Tombul, and R. Sayin. "Chronic Paroxysmal Hemicrania Responsive to Lamotrigine". ''Pakistan Journal of Medical Sciences''. 27.2 (2011): 456-57. Web. Use of

topiramate Topiramate, sold under the brand name Topamax among others, is a medication used to treat epilepsy and prevent migraines. It has also been used for alcohol dependence and essential tremor. For epilepsy, this includes treatment for generali ...

has also been found to be an effective treatment for CPH, but cluster headache medications have been found to have little effect.

Epidemiology

Although CPH is often compared to

cluster headaches Cluster headache is a neurological disorder characterized by recurrent severe headaches on one side of the head, typically around the eye(s). There is often accompanying eye watering, nasal congestion, or swelling around the eye on the affecte ...

, it is much less prevalent, occurring in only 1–3% of those who experience cluster headaches. CPH occurs roughly in 1 in 50,000 people, while cluster headaches are comparatively more common and are found in 1 in 1000 people. Cluster headaches occur primarily in men, while CPH is more commonly diagnosed in women. The female to male ratio of diagnosed patients can range anywhere from 1.6:1 to 2.36:1. However, more recent prospective research showed an equal prevalence between females and males, with a ratio close to 1:1. Symptoms may begin to appear at any age, but onset usually occurs in adulthood with a mean starting age within the thirties.

History

CPH was discovered by Norwegians Ottar Sjaastad and Inge Dale in 1974. The term ''chronic paroxysmal hemicrania'' was first used in 1976 by Sjaastad to describe a condition seen in two of their patients who were experiencing repeated solitary and limited daily headache attacks on only one side of the cranium. It is possible that chronic paroxysmal hemicrania was first described by Johann Oppermann in 1747 under the term "hemicranias horologica". Oppermann's report included a 35-year-old woman who had hemicranial pain that lasted for 15 minutes and recurred regularly every hour.Eadie, Mervyn J. "Chronic Paroxysmal Hemicrania". ''Headache: Through the Centuries''. New York: Oxford UP, 2012. 222-24. Print. CPH has been included in the International Headache Society's classification system since 1988.

References

External links

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