Cluster Headache at Peter Soros

Cluster headache (CH) is a rare, primary headache disorder that has a specific constellation of symptoms and a unique time course.

CH is also known as ciliary neuralgia, erythro-melalgia of the head, erythroprosopalgia of Bing, hemicrania angioparalytica, hemicrania neuralgiformis chronica, histaminic cephalalgia, Horton’s headache, Harris-Horton’s disease, migrainous neuralgia (of Harris), petrosal neuralgia (of Gardner).

Symptoms

Cluster headache is characterised by severe attacks of unilateral pain and cranial autonomic dysfunction. The pain is usually felt in and around the orbita or adjacent areas of the head. CH attacks last 15 – 180 min and occur, during a cluster period, from once every other day to several times a day. The symptoms of autonomic dysfunction are always ipsilateral to the pain and can involve the eye (conjunctival injection, lacrimation, miosis, ptosis, eyelid edema), the nose (nasal congestion, rhinorrhea), and the face (sweating). In the image of a patient with CH, the area of most intense pain is marked in red. During the cluster attack, this patient has an ipsilateral ptosis.

Pathophysiology

The exact pathophysiology of CH is, more than 250 years after the first description of episodic CH by the Dutch physician Gerard van Swieten (1700 - 1772, portrait on the left) in 1745, still unresolved. Several convergent lines of evidence suggest that CH is a primary neurovascular disorder. The hypothalamus is supposed to be involved in the pathogenesis of CH and might even be pivotal for the timing of cluster periods and single attacks.

Symptomatic cluster headache

Challenging the notion of an exclusively primary headache disorder, there are numerous reports of patients who developed cluster headache shortly after traumatic brain injury or structural brain disease. In many of these reports, vascular disorders (e.g. arterio-venous malformations, aneurysms, dissections or fistulas of the cranial vasculature) were associated with the onset of CH. In other patients, intracranial neoplasms (e.g. meningeomas or adenomas) or local inflammatory processes were seen associated with the onset of cluster headache.

In my work, I reported the development of cluster headache shortly after the removal of an eye and extraction of a tooth. For a short update on the current literature about symptomatic cluster headache, have a look at my comment in BMC Neurology (comment #2).

Own work

Sörös P, Vo O, Gerding H, Husstedt IW, Evers S. Enucleation and development of cluster headache: a retrospective study. BMC Neurology 2005;5(1):6. PubMed Fulltext Reprint

Sörös P, Frese A, Husstedt IW, Evers S. Cluster headache after dental extraction: implications for the pathogenesis of cluster headache? Cephalalgia 2001;21(5):619-22. PubMed Reprint

Sörös P, Evers S. Symptomatischer Clusterkopfschmerz – Klinik, Diagnostik und mögliche Pathogenese. Nervenheilkunde. 2001;20(7):370-374.