Headache
Neuromodulatory Approaches to the Management of Medically Refractory Cluster Headache
Arne May, MD1 and Peter J Goadsby, MBBS2
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 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.
Keywords Cluster headache, deep brain stimulation (DBS), occipital nerve stimulation (ONS), hypothalamus
Disclosure: Arne May, MD, has no conflicts of interest to declare. Peter J Goadsby, MBBS, has consulted or performed research for Autonomic Technologies, Boston Scientific and Medtronic. Received: May 9, 2010 Accepted: June 10, 2010 Citation: US Neurology, 2010;6(2):125–8 Correspondence: Peter J Goadsby, MBBS, Headache Group, Department of Neurology, University of California, San Francisco, 1701 Divisadero St, Suite 480, San Francisco, CA 94115. 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
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).
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 also be considered for these newer approaches.
This condition can be divided into episodic CH (ECH) and chronic CH (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 for one month or longer, while a diagnosis of CCH requires attacks to occur for more than one year without remission or with remission lasting for less than one month. CCH affects about 10% of CH patients. General aspects of therapy of the disorder are covered elsewhere.4,5
and as they can © TOUCH BRIEFINGS 2010
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 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 to respond to 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 to fundamentally be a brain condition.9
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