02 Nov Case Studies – Paroxysmal Hemicrania
By George R. Nissan, D.O.
Diamond Headache Clinic, Chicago, Illinois, and Clinical Assistant Professor of Medicine, The Chicago Medical School of Rosalind Franklin University of Medicine and Science
Ellen is a 43-year old female who experiences a right-sided, stabbing and sometimes throbbing headache primarily in the orbital and temporal regions (i.e., eye socket and temple areas), with up to 15 headache attacks per day. Each attack lasts from 5 to 30 minutes. The head pain is associated with tearing of the right eye, nasal congestion of the right nostril, and droopiness of the right eyelid, all of which disappear when the head pain resolves. She prefers to lie down in bed in the fetal position when the attacks occur.
Ellen has no significant family history of either migraine or cluster headaches, nor any significant medical history. She is a non-smoker and exercises regularly.
Ellen’s symptoms do not appear to fit the diagnostic criteria for migraine or tension-type headaches, and while some of the features are similar to cluster headache, the number of headache attacks she has per day is much greater than what a typical cluster headache sufferer experiences (15 per day versus 2 to 5 per day). Also, patients with cluster headache prefer to pace around the room during an attack, while this patient prefers to lie down.
The presentation of uncommon primary headache disorders (i.e., not caused by a secondary, underlying condition) can pose a dilemma for many healthcare providers both in primary care and in general neurology. These disorders can be very disabling and are often undiagnosed or misdiagnosed unless a detailed medical history is obtained.
Ellen has a condition called chronic paroxysmal hemicrania (CPH). CPH was not officially named until 1976. The disorder was originally described as multiple, short-lived, one-sided attacks that occurred on a daily basis without remission. However, it became clear that not all patients suffered an unremitting course. Some patients reported a remitting pattern with discrete periods of headache and then prolonged periods of pain-free remission. This pattern was then later named episodic paroxysmal hemicrania (EPH). The International Headache Society, however, only recognizes the term chronic paroxysmal hemicrania. In clinical practice we prefer the terms CPH, EPH, and CPH that evolved from EPH.
Unlike cluster headache, CPH is predominantly a disorder of females, with a 2:1 female to male ratio. The onset is usually in adulthood, with a mean age of approximately 33 years. Some cases have also been reported in children and teenagers. Most patients have no family history of the disorder though approximately 20% to 25% have a positive family history of migraine.
The pain of CPH is often described as pulsating, throbbing or stabbing, and ranges from moderate to excruciating in severity. Headache attacks can recur from one to forty times daily. The majority of patients report 15 or more attacks a day. Each individual headache can last from 2 minutes to 2 hours, although most commonly last less than 25 minutes. Attacks can occur throughout the day and night, even during REM sleep. Approximately 60% of patients report tearing from one eye on the same side of the headache. Other associated symptoms on the same side as the headache include droopiness of the eyelid, redness and irritation of the eyelid, and nasal congestion.
CPH can occur in conjunction with migraine, cluster or trigeminal neuralgia. It is important to have an imaging study, such as an MRI or CT scan of the brain with contrast, to evaluate for rare secondary causes of paroxysmal hemicrania such as a stroke, collagen vascular disease or a tumor.
Indomethacin, a nonsteroidal anti-inflammatory medication (NSAID), is the treatment of choice for CPH and EPH. Headache resolution is often prompt. In fact, a positive response to indomethacin is often used as a diagnostic indicator for CPH. Dosage adjustments are typically necessary during the initial phase of treatment. Sustained-release indomethacin taken before bed can sometimes help prevent nighttime attacks. When normally therapeutic doses of indomethacin do not provide headache relief, the diagnosis of CPH should be reconsidered. Other medications that may be of some clinical benefit include verapamil, steroids, naproxen and acetylsalicylic acid. It is also very important for patients who are taking indomethacin daily to monitor for gastrointestinal side effects, as is the case with all NSAID medications. To help minimize these side effects, patients may be treated with over-the-counter antacids, histamine receptor antagonists (i.e., Pepcidý, Zantacý, etc.), or omeprazole.
After initial treatment with indomethacin three times a day, Ellen became pain-free within seven days. She has been able to return to work full-time and is on maintenance doses of indomethacin in addition to omeprazole to help minimize gastrointestinal side effects.