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The trigeminal autonomic cephalalgias are a group of primary headache disorders characterized by unilateral trigeminal distribution of pain that occurs in association with ipsilateral cranial autonomic features. The most prominent one is cluster headache, a dreadful disease with excrutiating pain attacks. These attacks last no longer than two hours but may occur several times per day. It is mandatory to find an efficient therapy for these patients, but some are unresponsive to all treatments. In these intractable cases invasive procedures are introduced, but the available evidence (while conflicting) illustrates that trigeminal denervation may not be effective in preventing the headache attacks or autonomic symptoms of chronic cluster headache. Modern neurostimulating approaches, such as stimulation of the greater occipital nerve and hypothalamic deep brain stimulation, supersede neurodestructive procedures. Both stimulation methods are exquisite and potentially life-saving treatment options in otherwise intractable patients, but they need to be better characterized and further long-term data are needed.

Neuromodulatory Approaches to the Management of Medically Refractory Cluster Headache