Headache


Neuromodulatory Approaches to the Management of Medically Refractory Cluster Headache


Arne May1 and Peter J Goadsby2


1. Professor of Neurology, Department of Systems Neuroscience, University of Hamburg; 2. Professor of Neurology, Headache Group, Department of Neurology, University of California, San Francisco


Abstract


The trigeminal autonomic cephalalgias are a group of primary headache disorders characterised 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 characterised and further long-term data are needed.


Keywords Cluster headache, deep brain stimulation (DBS), occipital nerve stimulation (ONS), hypothalamus


Disclosure: Arne May has no conflicts of interest to declare. Peter J Goadsby has consulted or performed research for Autonomic Technologies, Boston Scientific and Medtronic. Received: 9 May 2010 Accepted: 10 June 2010 Citation: European Neurological Review, 2010;5(1):97–9 Correspondence: Peter J Goadsby, Headache Group, Department of Neurology, University of California, San Francisco, 1701 Divisadero St, Suite 480, San Francisco, CA 94115, US. E: pgoadsby@headache.ucsf.edu


Cluster headache (CH) is, by neurological standards, a relatively common condition that affects about one in 1,000 people,1


although


compared with other more common primary headaches such as migraine2


it remains rare in practice. CH has been defined in the second edition of the International Classification of Headache Disorders3


as


While it is not directly germane to neuromodulatiuon approaches, an understanding of the broad issues in medical treatment serves as a useful backdrop against which to discuss newer approaches. CH is one of the trigeminal autonomic cephalalgias,6 their therapies are usefully considered to be background7


and as they also


can be considered for these newer approaches. Who Is Suitable for


Neuromodulation Approaches?


It seems reasonable to suggest that neuromodulation approaches to the management of CH be employed in patients with medically intractable forms of the condition. While this is currently true, it reflects the relatively primitive state of current interventions. One should observe


© TOUCH BRIEFINGS 2010


involving recurrent attacks of severe pain on one side of the head for between 15 and 180 minutes, associated with cranial autonomic features such as lacrimation, conjunctival injection, nasal congestion or rhinnorhoea (see Table 1). This condition can be divided into episodic (ECH) and chronic (CCH) forms. A diagnosis of ECH requires at least two cluster periods lasting from seven days to one year separated by pain- free periods lasting one month or longer, while a diagnosis of CCH requires attacks to occur for more than one year without remission or with remission lasting less than one month. CCH affects about 10% of CH patients. General aspects of therapy of the disorder are covered elsewhere.4,5


that as devices become less invasive, the threshold for their use will become lower. For the moment there is a proposed working definition of medically intractable CH.8


The essential components are disabling


headache that fails at least four preventative drugs, including two from the first three of verapamil, lithium, methysergide, melatonin, topiramate and gabapentin (see Table 2). These considerations are particular to CH and, naturally, generic considerations related to requirements for the devices – such as fitness for anesthesia – are part of the overall assessment of patient suitability.


What Approaches Have Been Tried? In essence, two classes of neuromodulation have been explored in CH: peripheral and central. Prior to moving to stimulation approaches, the dreadful pain and disability of medically intractable CH lead to a number of destructive procedures. In principle, these seem unlikely to work if one considers CH as fundamentally being a brain condition.9 Moreover, they may cause both mortality and significant morbidity, and can induce further pain problems such as anaesthesia dolorosa. Previously used approaches have included trigeminal ganglion glycerol injections,10,11 ganglion12


radiofrequency rhizotomy of the Gasserian


microvascular decompression15 N. petrosus superficialis16 ganglion.17–19


or gamma knife aimed at the trigeminal nerve,13,14 and esection or blockade of the or pterygopalatine (sphenopalatine)


There are case series of trigeminal nerve root section20,21 that illustrate all the issues, including inducing further pain, vision impairment or indeed death. Moreover, there are also case reports of the complete inefficacy of surgical treatment in CH.21,22


It must also be remembered that annually about 10% of patients with CCH will revert 97


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