Among the 363 primary headache diagnoses currently classified, in addition to common conditions like migraine and tension-type headache, 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 accompanying video, description below), and other headache forms. The one-year prevalence of individual forms of these so-called trigeminal autonomic cephalalgias (TACs) is significantly less than 0.01%. These disorders are clinically characterized by extremely severe unilateral headache attacks in conjunction with ipsilateral autonomic symptoms. A common feature of these headaches is the excruciating severity of the pain, which, if left undiagnosed and untreated, very often leads to suicide. The correct and rapid diagnosis of TACs is essential, as their treatment differs fundamentally from the therapy of other forms of headache.

However, accurate diagnoses are often made very late or not at all, meaning effective therapies are either not available or are only initiated after many agonizing years of a painful odyssey, doctor-hopping, the use of unconventional methods, and serious complications. Furthermore, largely unexplored, undiagnosed, and mistreated or left untreated, TACs are among the most malignant and debilitating pain disorders in humans. Social isolation, personality changes, anxiety, depression, despondency, anger, grief, despair, and a loss of the will to live are among their many accompanying symptoms. Family members usually suffer alongside the patients, often in fear and despair. Yet, with specialized knowledge, TACs can generally be diagnosed quickly and accurately today. Highly effective treatment options exist, which, when understood and applied appropriately, usually provide effective and rapid relief

The project aims to spare patients a lengthy and arduous treatment process, diagnostic delays, and ineffective therapies. Severe and rare headache disorders, such as TACs, manifest with complex phenomenology, have multi-causal mechanisms, and present with diverse physical and psychological effects. Therefore, they generally cannot be treated comprehensively and according to current best practices. Without knowledge of up-to-date diagnostic criteria, the pooling of expertise through specialization, and the implementation of current, scientifically validated treatment pathways, misdiagnoses and ineffective treatment courses result.

On average, it takes eight to twelve years for those affected to receive an adequate diagnosis and specific treatment. Rare headache disorders are an area of ​​medicine that is very difficult to address in randomized controlled trials due to the rarity of the diseases. Evidence-based guidelines, training and continuing education programs for physicians, and industry research efforts have been lacking until now.

The goal of the diagnostic, treatment, and research network is therefore to provide patients with rare headache disorders with coordinated, interdisciplinary, and state-of-the-art international scientific treatment. This project is a specialized area within the nationwide headache treatment network, a coordinated care network for the specialized treatment of headaches, a collaboration between the Kiel Pain Clinic, the Techniker Krankenkasse (a German health insurance company), cooperating health insurance companies, and patient support groups. Research projects will also be conducted to develop and evaluate new treatment options and more effective treatment pathways. Public relations efforts will provide patients and their families with up-to-date information.

In collaboration with the Kiel Pain Clinic, the German Federal Association of Cluster Headache Self-Help Groups (CSG eV), and the Techniker Krankenkasse (a German health insurance company), the concept of Cluster Headache Competence Centers (CCCs) was developed to address gaps in care and was first implemented in 2007. In cooperation with the Techniker Krankenkasse, a nationwide integrated care network for the treatment of headache disorders was also developed, into which the competence centers were integrated. The goal was to enable nationally coordinated and interdisciplinary treatment pathways for effective, coordinated, rapid, and accessible diagnosis and therapy. This care network aims to provide local access to specialized practices. Relevant regional pain therapy centers can be located nationwide via the internet. Simultaneously, care pathways were coordinated and optimized for efficiency through these pathways.

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

  • This syndrome presents with a clinical picture very similar to that of paroxysmal hemicrania in individual patients.
  • However, unlike paroxysmal hemicrania, the pain is characterized by very short episodes lasting 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 associated with the typical accompanying symptoms of paroxysmal hemicrania.
  • The attacks can be triggered by chewing maneuvers, but do not respond to indomethacin or carbamazepine.
  • The 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-to-female ratio is approximately 1.5:1.
  • SUNCT syndrome is characterized by short-lived, unilateral pain attacks that are significantly shorter than those seen in other trigeminal autonomic cephalalgias. Typically, marked lacrimation (tearing of the eyes) and conjunctival injection (redness of the eye) occur on the side of the pain.