Among the 363 main headache diagnoses that are classified today, in addition to the common diseases migraine and tension-type headache, there are a number of very rare headache disorders. These include, in particular, episodic and chronic cluster headache (CK), episodic and chronic paroxysmal hemicrania (CPH), SUNCT syndrome (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing - see adjacent video, description below) and other types of headache . The one-year prevalence of individual forms of these so-called trigemino-autonomous cephalgias (TACs) is well below 0.01%. The diseases are clinically characterized by extremely severe unilateral headache attacks in conjunction with ipsilateral autonomic symptoms. What these headaches have in common is the devastating severity of the pain, which very often leads to suicide if diagnosis and treatment are ineffective. The correct and rapid diagnosis of TACs is essential because their treatment is fundamentally different from the treatment of other forms of headache.

However, targeted, adequate diagnoses are often made very late or not at all, and effective therapies are therefore not available or are only initiated after many agonizing years of a pain odyssey, doctor-hopping, doctor-shopping, use of unconventional methods and serious complications. Additionally, largely unexplored, undiagnosed and mistreated or untreated, TACs are among the most malignant and at the same time most disabling pain diseases in humans. Social isolation, personality changes, fear, depression, despondency, anger, sadness, despair and giving up the will to live are the various companions. Relatives usually suffer, frightened and desperate. Today, TACs can usually be diagnosed quickly and precisely with specialized knowledge. There are very effective treatment options that, if known and applied appropriately, usually provide effective and quick help

The project aims to spare patients a treatment odyssey, diagnostic delays and ineffective therapies. Severe and rare headache disorders such as TACs in particular manifest themselves with complex phenomenology, have multi-causal mechanisms and present with a wide range of physical and psychological effects. As a rule, they cannot be treated in a comprehensive, specialized and up-to-date manner. Without knowledge of contemporary diagnostic criteria, bundling of experience through specialization and implementation of current scientifically proven treatment paths, misdiagnoses and ineffective treatment courses arise.

On average, it takes eight to twelve years until those affected receive an adequate diagnosis and specific treatment. Rare headache disorders are an area of ​​medicine that is very difficult to access randomized controlled trials due to the rarity of the diseases. Evidence-based guidelines, training, further education and training measures for doctors as well as research efforts by industry have so far been missing.

The aim of the diagnosis, treatment and research network is therefore to treat patients with rare headache disorders in a coordinated, interdisciplinary manner and at the latest international scientific level. The project is a special area of ​​responsibility within the framework of the nationwide headache treatment network, a coordinated care network for the specialized treatment of headaches in cooperation between the Kiel Pain Clinic, the Techniker Krankenkasse, cooperating health insurance companies and self-help associations. Research projects are also intended to develop and evaluate new therapy options and more effective treatment paths. Public relations should provide those affected and their relatives with up-to-date information.

In collaboration with the Kiel Pain Clinic, the Federal Association of Cluster Headache Self-Help Groups (CSG eV) and the Techniker Krankenkasse, the idea of ​​competence centers for cluster headaches (CCC) was developed to overcome the supply deficits and was implemented for the first time in 2007. In cooperation with the Techniker Krankenkasse, a nationwide integrated care network for the treatment of headache disorders was developed, into which the competence centers were integrated. The goal was to enable nationwide coordinated and interdisciplinary treatment paths for effective, coordinated, rapid and barrier-free diagnostics and therapy. On the one hand, this supply network should enable local access to specialized practices. Relevant pain therapy regional centers can be found nationwide via the Internet. At the same time, the supply channels were coordinated and optimized in terms of efficiency through treatment paths.

The video shows SUNCT syndrome. The abbreviation SUNCT syndrome stands for “shortlasting unilateral neuralgiform headache attacks with conjunctival injection, tearing, sweating and rhinorrhea”.

  • With this syndrome, a clinical picture very similar to that of paroxysmal hemicrania has been described in individual patients.
  • However, in contrast to paroxysmal hemicrania, the pain is characterized by very short episodes that last between 15 and 60 seconds and can occur with a high attack frequency of 5 to 30 attacks per hour.
  • The pain is localized around one eye and is associated with the typical accompanying disorders of paroxysmal hemicrania.
  • The attacks can be triggered by chewing maneuvers, but do not respond to indomethacin or carbamazepine.
  • SUNCT syndrome must be carefully differentiated from trigeminal neuralgia in the differential diagnosis.
  • The prevalence is unknown. It is probably less common than paroxysmal hemicrania. The male:female ratio is approximately 1.5:1.
  • SUNCT syndrome is characterized by short-lasting unilateral pain attacks that are significantly shorter than other trigeminal-autonomic headache disorders. As a rule, significant lacrimation (tearing of the eyes) and conjunctival injection (redness of the eyes) occur on the painful side.