User:D23sunn/Project Proposal II: Electric Boogaloo

Chronic paroxysmal hemicrania (CPH), also known as Sjaastad syndrome, is a severe debilitating unilateral headache, usually affecting the area around the eye. Unlike a migraine it has no neurological symptoms associated with it. It normally consists of multiple severe yet short headache attacks affecting only one side of the cranium, hence the term hemicrania. It is more common in females than males. CPH headaches are treated with non-steroidal anti-inflammatory drugs, in particular indomethacin, which is usually totally effective in eliminating the symptoms.

There are two types of paraxysmal hemicrania, episodic and chronic. The episodic type takes place at least twice a year that last anywhere from seven days to a year with pain free periods of 1 month or longer separating the attacks. However, chronic attacks occur for longer than a year without remission or remissions lasting less than a month.

History
Chronic Paroxysmal Hemicrania, also known as Sjaastad Syndrome, was first discovered by Ottar Sjaastad and Inge Dale in 1974. The term “Chronic Paroxysmal Hemicrania” (CPH) was first used in 1976 by Sjaastad to describe the condition of of two of their patients who were experiencing repeated attacks daily. They were solitary and limited attacks (paroxysmal). The sensation they were feeling was a unilateral headache that did not shift sides of the cranium (hemicrania).

Chronic Paroxysmal Hemicrania has been in the International Headache Society (IHS) classification system since 1988.

Diagnosis
Chronic Paroxysmal Hemicrania takes is a long-term disease with symptoms lasting for longer than a year without remission or remissions that last less than a month. In order to be diagnosed with DPH, 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 headache needs to take place with one of the following: ipsilateral conjunctival injection and/or lacrimation ipsilateral nasal congestion and/or rhinorrhoea ipsilateral eyelid oedema ipsilateral forehead and facial sweating ipsilateral miosis 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 Attacks are prevented completely by therapeutic doses of indomethacin Finally, the symptoms cannot be attributed to another disorder.

Epidemiology
While CPH is often compared to cluster headaches, CPH is much less prevalent. The number of people who have CPH is only 1-3% of that who have cluster headaches. Chronic Paroxysmal Hemicrania occurs in 1 in 50,000 people. However, it could be even rarer. Cluster headaches are comparatively more common and are found in 1 in 1000 people. Interestingly, while Cluster headaches primarily exist in men, CPH is more common in women than men, despite CPH in men being on the rise. The ratio of female to men can range anywhere from 1.6:1 to 2.36:1. CPH has been found in several countries. It can begin at any age, but onset usually takes place in adulthood with a mean starting ages in the thirties.

Symptoms
Individuals with CPH suffer multiple severe short-lived headaches a day (often more than 5), with most lasting between 5 and 30 minutes each. Each headache is centered around the eye, temple and forehead 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 or vomiting.

Treatments
A 10 patient study conducted by Pareja JA, Caminero AB, Franco E, Casado JL, Pascual J and Sanchez del Rio M. Dose, found that all patients were responsive to indometacin with complete control of 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. Almost all cases respond positively and effectively to indometacin but as much as 25 percent of patients discontinue use of the drug due to adverse effects, mostly complications in the gastrointestinal tract. According to a case study by Aysel Milanlioglu, Temel Tombul, and Refah Sayin, 100mg of lamotrigine, an antiepileptic drug, administered twice daily cured all painful symptoms. After two months of this treatment, no side-effects were noted. This dose of lamotrigine was decreased to 50mg a day after the first two months, and no symptoms or side-effects were recorded after a three month followup. Use of topiramate has also been found as an effective treatment.