The term paroxysmal hemicrania describes a specific form of headache disorder. It is characterized by seizure-like, hemiparesis, very severe attacks of pain accompanied by redness on the affected side of the face. The duration of the attacks is from a few minutes to about 45 minutes in rare cases.
What is paroxysmal hemicrania?
Infographic on the causes and symptoms of migraine and headache. Click image to enlarge. Paroxysmal hemicrania translates as seizure-like, hemifacial headache, which already expresses the most important features: because in this form of headache disorder, the affection of one side is just as characteristic as the relatively short duration of the pain attacks. The daily frequency ranges from 5 times to 40 times per day. Sufferers describe the sudden, seizure-like pain as extremely severe, stabbing, drilling or pulsating. Typically, the region of the orbits and the forehead and temple are mainly involved. Paroxysmal hemicrania also has compelling associated symptoms in the form of redness and swelling of the eye and conjunctiva, including increased lacrimation and rhinitis-like symptoms. The rare disease usually appears for the first time between the ages of 30 and 40. Initially, the condition appeared to be more common in women, but recent research shows that there are no gender differences in the risk of developing paroxysmal hemicrania.
Causes
There is no firm evidence regarding the reproducible causes of paroxysmal hemicrania. This is partly because this type of headache has only been known as a distinct symptom and disorder for a few decades and has been observed more closely as such. In fact, the seizure-like hemiparesis headache is characterized by a combination of very specific symptoms that only a few headache sufferers report. In contrast, sufferers often report observable triggering moments that precede an attack of pain. These include physical exertion, stress, alcohol consumption, various foods (e.g., coffee, cheese, or chocolate), as well as certain movements in the head region or even temperature changes. Recent research has found evidence linking paroxysmal hemicrania to a pituitary adenoma and is also investigating the role of arterio-venous malformations as a possible origin of the disease. Since the occurrence of strictly unilateral pain is observed in paroxysmal hemicrania, but the vegetative symptoms (including swelling, redness, lacrimation, etc.) may be bilateral, an injury to the central nervous system, specifically in the midline region, is suspected. A familial cluster has also been observed.
Symptoms, complaints, and signs
Paroxysmal hemicrania involves seizure-like headaches that are similar in symptomatology to cluster headaches. The stabbing, probing, and pulsating headaches occur unilaterally. They are localized mainly in the area of the eyes, forehead, or temples. The patient experiences at least five headache attacks a day. On average, there are ten attacks. In extreme cases, up to 40 attacks per day are also observed. Each pain attack lasts between 2 and 45 minutes. In contrast to cluster headaches, the pain attacks in paroxysmal hemicrania are shorter. In addition, the number of attacks is greater. During the attack, the patient’s need for rest increases, whereas in cluster headache, unusual restlessness is evident. The efficacy of the drug indomethacin is also crucial in differentiating paroxysmal hemicrania from cluster headache. The headache attacks are typically accompanied by other symptoms. These symptoms include conjunctivitis, eye tearing, runny nose, swelling of the nasal mucous membranes, 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. Usually it is the chronic paroxysmal hemicrania. Here, the symptoms occur every day. However, there is also episodic paroxysmal hemicrania. In this form of the disease, pain-free intervals occur over several weeks and months.
Diagnosis and course
The diagnosis of paroxysmal hemicrania requires an extremely precise history because of its specifically pronounced symptomatology. The clearly unilateral attacks of pain are characteristic, as is the need for rest during the attacks. Not only this last factor, but also the sudden occurrence of shorter (between 2 and about 45 minutes) and several times daily attacks distinguishes paroxysmal hemicrania from the similar cluster headache. Furthermore, paroxysmal hemicrania is always accompanied by typical vegetative symptoms: These include, with emphasis on the affected side, swelling of the eyelids, redness of the face and conjunctiva, increased lacrimation, nasal mucosal swelling, and even drooping of the upper eyelid. Different courses are also described: Chronic paroxysmal hemicrania with mostly daily attacks represents the more common form. In rarer cases, the affected patients are symptom-free for weeks, even months – in this episodic paroxysmal hemicrania, long headache-free intervals are observed. The decisive diagnostic criterion is in any case the therapeutic response to the administration of indomethacin – without its efficacy the diagnosis “paroxysmal hemicrania” must not be made! In the course of careful neurological examination, magnetic resonance imaging is also performed, especially with regard to the pituitary region.
Complications
As a rule, those affected by this disease suffer from very severe headaches. These occur mainly in episodes and can also spread to other regions of the body. In many cases, earaches or toothaches also occur. The affected half of the face is usually also completely affected by the pain. During the pain, redness or swelling may also occur in the eye, so that patients may briefly suffer from visual problems. 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 lacrimation. The treatment of this disease depends on the underlying disease. As a rule, however, this is carried out with the help of medication. Complications do not occur, but a positive course 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?
When the characteristic drilling headache or any other sign of paroxysmal hemicrania occurs, the doctor should be consulted. The pain attacks increase in intensity and duration relatively quickly and therefore require prompt evaluation. If accompanying symptoms such as swelling of the mucous membranes or skin changes occur, a visit to the doctor is recommended. Women are particularly frequently affected by paroxysmal hemicrania. The causes may be psychological, although physical triggers such as chronic pain disorders are also possible. Anyone who is affected by these risk factors should see a doctor if the symptoms described occur. Paroxysmal hemicrania is diagnosed and treated by the family doctor or a neurologist. If the symptoms are severe, inpatient treatment may also be appropriate. Initially, sufferers should contact emergency medical services if a recurrence of the headache interval occurs. Since the disease occurs in phases, causal research can be carried out during the pain-free phases. Close consultation with the responsible physician is necessary in this regard.
Treatment and therapy
The first goal in the treatment of paroxysmal hemicrania is to achieve freedom from pain. To achieve this, therapy must be tailored precisely to the particular form of the disease and its course. The drug of choice for paroxysmal hemicrania is indomethacin. To relieve patients of 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 a continuous therapy, and if pain relief is successful, the daily dose can be gradually decreased to find the minimum required maintenance dose.A side effect of treatment with indomethacin is inhibition of prostaglandin synthesis; this makes effective protection of the gastric mucosa necessary, e.g., by means of a proton pump inhibitor. If the administration of (a maximum of about 300 mg/day) indomethacin does not sufficiently relieve the severe symptoms, local anesthesia is also a therapeutic option. Alternatively, non-steroidal anti-inflammatory drugs, so-called NSAIDs, can be administered, e.g. in the form of diclofenac, naproxen or flurbiprofen. In the chronic course of paroxysmal hemicrania, psychotherapy is always recommended. If triggering factors (eg, high stress or certain foods) can be observed, avoidance of these is also a goal to strive for, as this may be the only causative therapeutic option for affected individuals.
Outlook and prognosis
Those suffering from paroxysmal hemicrania must live with multiple daily headache attacks. The pain always occurs on one side. It is severe and stabbing, and may also pulsate. Paroxysmal hemicrania is present when the affected person experiences at least five headache attacks a day. In addition, certain accompanying symptoms must be present. The prognosis for such attacks depends in part on careful diagnostic differentiation of the disorder from similar seizure-like headaches. In addition, it must be elicited whether the condition is chronic or periodic paroxysmal hemicrania. Once the diagnosis of paroxysmal hemicrania is unequivocal, the prognosis is usually not very good. In extreme cases, sufferers experience up to 40 seizures per day. They are in need of rest afterwards. A working life is therefore 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 can also last for three quarters of an hour. A more favorable prognosis can only be given if the rarely occurring periodic course 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 is relatively rare.
Prevention
Since the exact causes and the mechanisms of development of paroxysmal hemicrania have not been clearly understood and known to date, hardly any preventive measures can be recommended. A lifestyle that is as stress-free and recreational as possible, with a good work-life balance, may be a preventive measure. However, no confirmed preventive behaviors can currently be mentioned for this rare condition, as the etiology is still largely unclear.
Follow-up
In paroxysmal hemicrania, sufferers must take special care to refrain from all external influences that may lead to headache. Affected individuals should avoid stress and have adequate and well-groomed sleep. During phases of strong emotional stress, various therapies should be used to provide cognitive relief. Examples include yoga or meditation. This can help reduce and alleviate triggers for stress. Any conflict situations should be avoided by sufferers. A great help for aftercare is to increase the quality of life and strengthen the joy of living. Relaxing leisure activities should be planned and undertaken. Help from relatives should be sought for important errands. Help is also needed with everyday tasks. The disease limits those affected, so social contacts become more important. Above all, relationships with family and relatives should be maintained so that help can be requested at any time. The symptoms usually occur more frequently when sufferers are under mental stress. Therefore, a healthy lifestyle should be the first priority. This means avoiding any stress, maintaining a healthy diet, avoiding excess weight and also refraining from consuming nicotine and alcohol.
This is what you can do yourself
In everyday life, minimize all influences that could lead to the triggering of a headache. Optimal sleep hygiene and sufficient sleep are just as necessary 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 alleviate as well as reduce stressors. Conflict situations should be avoided and permanently resolved 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 living conditions. In addition, leisure activities that provide relief should be planned. Since the disease is a great hindrance in coping with everyday duties, important errands should be rescheduled in good time. With a stable social environment, the assistance of relatives or friends can be utilized. Therefore, social contacts should be maintained. In the phases of freedom from symptoms, the affected person should carefully examine for himself which life decisions should be reconsidered. In many patients, the symptoms occur more frequently when they are exposed to strong emotional stresses and compromises. Overall, the affected person should lead a healthy lifestyle. This includes a balanced diet, avoidance of excess weight, and adequate exercise. The consumption of nicotine and alcohol should be refrained from.
