The term paroxysmal hemicrania describes a special form of the headache disease. It is characterized by paroxysmal, one-sided, very severe pain attacks that are accompanied by redness on the affected half of the face. Seizures last from a few minutes to, in rare cases, about 45 minutes.
What is Paroxysmal Hemicrania?
Translated, paroxysmal hemicrania means attack-like, one-sided headache, which already expresses the most important features: Because with this form of headache, the affection of one side is just as characteristic as the relatively short duration of the pain attacks. See ezhoushan for What does MDS Stand for.
The daily frequency ranges from 5 to 40 times a day. Those affected describe the sudden and paroxysmal pain as extremely severe, stabbing, boring or strongly pulsating. Typically, the region of the eye sockets and the forehead and temples are mainly involved. Paroxysmal hemicrania also has compelling accompanying symptoms in the form of reddening and swelling of the eye and conjunctiva, including increased lacrimation and runny nose-like symptoms.
The rare disease usually occurs for the first time between the ages of 30 and 40. Initially, the clinical picture was apparently observed more frequently in women, but recent research results show that there are no gender-specific differences in terms of the risk of developing paroxysmal hemicrania.
There is no reliable knowledge about the reproducible causes of paroxysmal hemicrania. This is partly because this type of headache has only been known as an independent symptom and disease for a few decades and has been observed more closely as such. The paroxysmal hemiplegic headache is characterized by a combination of very specific symptoms that only a few headache patients have.
In contrast, those affected often report observed triggering moments that precede an attack of pain. These include physical exertion, stress, alcohol consumption, different foods (e.g. coffee, cheese or chocolate) as well as certain movements in the head area or temperature changes. Recent research has found evidence of an association between paroxysmal hemicrania and pituitary adenoma and is also investigating the role of arteriovenous malformations as a possible origin of the disease.
Since the occurrence of a strictly unilateral pain is observed in paroxysmal hemicrania, but the vegetative symptoms (including swelling, redness, lacrimation, etc.) can occur on both sides, an injury to the central nervous system, namely in the area of the midline, is suspected. Familial accumulation has also been observed.
Symptoms, Ailments & Signs
Paroxysmal hemicrania is a paroxysmal headache with symptoms similar to cluster headaches. The stabbing, boring and pulsating headache occurs on one side. They are mainly localized in the area of the eyes, forehead or temples. The patient suffers at least five headache attacks a day. On average, there are ten seizures.
In extreme cases, up to 40 attacks per day are observed. Each pain attack lasts between 2 and 45 minutes. In contrast to cluster headaches, attacks of pain in paroxysmal hemicrania are shorter. In addition, the number of seizures is greater. Patients’ need for rest increases during the attack, while cluster headaches show an unusual restlessness.
The effectiveness of the drug indomethacin is also decisive for the differentiation of paroxysmal hemicrania from cluster headaches. The headache attacks are typically accompanied by other symptoms. These symptoms include but are not limited to conjunctivitis, watering of the eyes, runny nose, swelling of the nasal mucosa, and swelling and drooping of the eyelids.
Overall, women are affected three times more often than men. Two forms of paroxysmal hemicrania can also be distinguished. It is usually chronic paroxysmal hemicrania. The complaints occur here every day. However, there is still an episodic paroxysmal hemicrania. In this form of the disease, there are pain-free intervals over several weeks and months.
Diagnosis & History
The diagnosis of paroxysmal hemicrania requires an extremely precise anamnesis due to its particularly pronounced symptoms. The clearly unilateral pain attacks are just as characteristic as the need for rest during the attacks.
Not only this latter factor, but also the sudden occurrence of shorter (between 2 and about 45 minutes) and several daily attacks distinguishes paroxysmal hemicrania from the similar-looking cluster headache. In addition, typical accompanying vegetative symptoms always occur with paroxysmal hemicrania: These include swelling of the eyelids, with a focus on the affected side, reddening of the face and conjunctiva, increased tear flow, swelling of the nasal mucosa and even drooping of the upper lid.
Various forms of progression are also described: The more common form is chronic paroxysmal hemicrania with mostly daily attacks. In rarer cases, the affected patients are symptom-free for weeks or even months – long headache-free intervals can be observed in this episodic paroxysmal hemicrania.
In any case, the decisive diagnostic criterion is the therapeutic response to the administration of indomethacin – without its effectiveness the diagnosis “paroxysmal hemicrania” must not be made! In the course of the careful neurological examination, magnetic resonance imaging is also performed, especially with regard to the pituitary region.
As a rule, those affected by this disease suffer from very severe headaches. These mainly occur in flare-ups and can also spread to other regions of the body. In many cases, this also leads to earache or toothache. The affected half of the face is usually completely affected by the pain.
The eye can also become red or swollen during the pain, so that the patient can suffer from visual problems for a short time. However, the further course of this disease depends very much on the exact cause. For this reason, a general prediction is usually not possible. However, if the disease is not treated, it can also lead to inflammation of the conjunctiva and significantly increased tear flow.
The treatment of this disease depends on the underlying disease. However, this is usually done with the help of medication. Although there are no complications, a positive outcome cannot always be guaranteed. A healthy and stress-free lifestyle has a very positive effect on the disease.
When should you go to the doctor?
If the characteristic nagging headache or other sign of paroxysmal hemicrania occurs, the physician should be consulted. The pain attacks increase in intensity and duration relatively quickly and therefore require rapid clarification. If accompanying symptoms such as swelling of the mucous membranes or skin changes occur, a doctor’s visit is recommended. Paroxysmal hemicrania is particularly common in women. The causes can be of a psychological nature, although physical triggers such as chronic pain disorders are also possible.
To whom these risk factors apply, you should consult a doctor if the symptoms described appear. Paroxysmal hemicrania is diagnosed and treated by the general practitioner or a neurologist. In the case of severe symptoms, inpatient treatment can also be useful. First of all, the sick person should contact the medical emergency service if there is a renewed headache interval. Since the disease occurs in phases, the causes can be investigated in the pain-free phases. This requires close consultation with the responsible doctor.
Treatment & Therapy
The first goal in the treatment of paroxysmal hemicrania is to achieve freedom from pain. In order to achieve this, the therapy must be tailored precisely to the respective form of the disease and its progression. The drug of choice for paroxysmal hemicrania is indomethacin. In order to relieve the patients of the severe pain symptoms, the mean dosage of indomethacin is about 150 mg/day, ranging from 30 to 300 mg per day.
Indomethacin is administered as long-term therapy, whereby if the pain is successfully eliminated, the daily dose can be gradually reduced in order to find the minimum maintenance dose required. A side effect of treatment with indomethacin is the inhibition of prostaglandin synthesis; this makes effective protection of the gastric mucosa necessary, for example by means of a proton pump inhibitor. If the administration of (maximum about 300 mg/day) indomethacin does not sufficiently alleviate the severe symptoms, local anesthesia is also a therapeutic option.
Alternatively, non-steroidal anti- inflammatory drugs, so-called NSAIDs, can also be administered, for example in the form of diclofenac, naproxen or flurbiprofen. In the chronic course of paroxysmal hemicrania, accompanying psychotherapy is recommended in any case. If triggering factors (e.g. high levels of stress or certain foods) can be observed, avoiding them is also a goal to strive for, as this may be the only causal therapy option for those affected.
Outlook & Forecast
Anyone suffering from paroxysmal hemicrania has to live with headache attacks that occur several times a day. The pain is always one-sided. It is strong and stinging, it can also pulsate. Paroxysmal hemicrania is present when the sufferer experiences at least five headache attacks a day. In addition, certain side effects must be present.
The prognosis for such attacks depends in part on careful diagnostic differentiation of the condition from similar paroxysmal headaches. In addition, it must be determined whether it is chronic or periodically occurring paroxysmal hemicrania. If the diagnosis of paroxysmal hemicrania is unequivocal, the prognosis is usually not very good.
In extreme cases, those affected experience up to 40 seizures per day. After that they need rest. A working life is no longer possible. In addition, the condition is chronic in the vast majority of cases. The length of the seizures varies. They can be very short, but they can also last three quarters of an hour.
A more favorable prognosis can only be given if the rare periodic form of paroxysmal hemicrania is present. Here there can be longer pain-free periods without headache attacks. Ideally, an affected person can be pain-free for several weeks or even months. It is still unclear why paroxysmal hemicrania occurs. It’s relatively rare.
Since the exact causes and mechanisms of development of paroxysmal hemicrania are still not clearly understood and known, hardly any preventive measures can be recommended. A lifestyle that is as stress-free and relaxing as possible with a good work-life balance may be a preventive measure. However, no reliable preventive behavior can be given for this rare disease at the moment, since the etiology is still largely unclear.
In the case of paroxysmal hemicrania, sufferers must take particular care to refrain from all external influences that can lead to headaches. Those affected should avoid stress and have sufficient and well-groomed sleep. In phases of strong emotional stress, various therapies can be used to create cognitive relief. Examples of this are yoga or meditation.
This can help reduce and relieve triggers for stress. Any conflict situations should be avoided by the sick. A great help for the aftercare is to increase the quality of life and to increase the joy of life. Relaxing leisure activities should be planned and undertaken. For important errands, the help of relatives should be taken up. Help is also needed with everyday tasks.
The disease restricts those affected, so that social contacts become more important. Above all, relationships with family and relatives should be cultivated so that help can be requested at any time. The symptoms usually occur more frequently when those affected are suffering from mental stress. Therefore, a healthy lifestyle should come first. This means avoiding any kind of stress, maintaining a healthy diet, avoiding being overweight and also refraining from consuming nicotine and alcohol.
You can do that yourself
In everyday life, all influences that could trigger a headache should be minimized. Optimal sleep hygiene and sufficient sleep is just as important as avoiding stress. In phases of emotional stress, various techniques should be used to provide cognitive relief. Methods of yoga or meditation can help to relieve and reduce stressors. Conflict situations are to be avoided and should be clarified permanently as quickly as possible.
It is helpful to strengthen the zest for life and improve the quality of life. For many patients, this requires a restructuring of their living conditions. In addition, leisure activities should be planned that provide relief. Since the disease is extremely difficult to cope with everyday duties, important errands must be rescheduled in good time. With a stable social environment, the help of relatives or friends can be used. Therefore, social contacts should be maintained.
In the phases of being free of symptoms, the person concerned should carefully examine which life decisions should be reconsidered. In many patients, the symptoms occur more often when they expose themselves to severe mental stress and compromises. Overall, the sufferer should lead a healthy lifestyle. This includes a balanced diet, avoiding obesity and adequate exercise. The consumption of nicotine and alcohol is to be avoided.