• Ipsilateral conjunctival injection and/or lacrimation
• Ipsilateral nasal congestion and/or rhinorrhea
• Ipsilateral forehead and facial sweating or flushing
• Ipsilateral eyelid edema
• Ptosis and/or miosis (less common)
The TAC disorders include (1) cluster headache, (2) paroxysmal hemicrania, (3) short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), and (4) short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). Although sharing several features, the TACs differ in attack duration and frequency, as well as in their therapeutic response (see Plate 13-7). Cluster headache (CH) has the longest attack length and a relatively low-attack frequency. Paroxysmal hemicrania (PH) has an intermediate attack length and an intermediate attack frequency. SUNCT/SUNA headache attacks have the shortest attack length and the highest attack frequency. Most importantly, underlying structural brain lesions can mimic these disorders. Therefore brain magnetic resonance imaging/magnetic resonance angiography (MRI/MRA) is indicated when a TAC diagnosis is considered.
CLUSTER HEADACHE
Although rare, CH is nevertheless the most common of the TAC disorders with a prevalence of less than 1% and a male-to-female ratio of 4 : 1 to 5 : 1. In addition to the cranial autonomic symptoms, several clinical features help characterize cluster headache. The pain is usually piercing, boring, or stabbing; it usually begins precipitously without premonitory symptoms, rapidly reaching crescendo and becoming excruciatingly severe. The pain may begin in the temporal, lower facial, or occipital region, remains unilateral, and is typically maximal behind and around the eye. The headache usually lasts 60 to 90 minutes, with a range of 15 to 180 minutes, and occurs from every other day to eight times per day; often at the same time each day or night. Photophobia and phonophobia occur in up to 50% of CH individuals, typically ipsilateral to the pain. In contrast to migraine, where activity typically aggravates the pain, more than 90% of patients with cluster headache report restlessness and agitation and avoid remaining recumbent.
The term “cluster” headache was coined because, in its most prototypical form, it is episodic and usually occurs at least once every 24 hours for weeks at a time, often for 8 to 12 weeks, that is, in clusters. During an active cluster period, attacks can usually be precipitated by ingestion of alcohol. A frequent pattern, especially in the first few years, is for cluster periods to occur seasonally, often in the spring or fall. This periodicity often decreases after a few years as periods of cluster activity become less predictable, occurring any time of the year. Approximately 10% of sufferers develop chronic CH characterized by the absence of prolonged remissions.
Mechanisms of Cluster Headache. Pathophysiology of cluster headache is not well understood. The recurrence of attacks at similar times of day during cluster bouts is one of this syndrome’s most striking characteristics and suggests possible hypothalamic involvement. Positron emission tomography (PET) studies support this, demonstrating activation in the posterior hypothalamus during nitroglycerin-induced cluster headaches. Once the hypothalamus is activated, it may activate the trigeminal-autonomic reflex, leading to unilateral pain mainly within the ophthalmic division of the trigeminal nerve as well as the ipsilateral autonomic features, including tearing, rhinorrhea, partial Horner syndrome, and orbital vasodilation. Oculosympathetic paresis in some patients during cluster headache attacks implicates involvement of pericarotid sympathetic fibers. The cavernous sinus is suggested as another important source for cluster headache pathogenesis because this location allows convergence of trigeminal, sympathetic, and parasympathetic fibers.
Cluster Headache Management. This is divided into treatment of acute cluster attacks as well as therapeutic options to transition out of a cluster period or prophylactic therapy preventing future attacks. Options for the acute treatment of CH include inhalation of 100% oxygen, subcutaneous sumatriptan or nasal sumatriptan, oral or nasal zolmitriptan, octreotide, nasal lidocaine, and subcutaneous dihydroergotamine.
Transitional prophylaxis may be used for a few weeks to quickly end or markedly reduce the frequency of attacks. A 2- to 3-week course of corticosteroids often leads to a substantial reduction of attacks. Greater occipital nerve blockade with a local anesthetic and a corticosteroid may significantly reduce attacks and sometimes leads to a remission. For longer-term prophylaxis, verapamil is usually the drug of choice because of its efficacy and side-effect profile. Lithium carbonate can also be efficacious but is usually reserved for chronic intractable cluster headache. The use of other agents, such as topiramate, divalproex sodium, and pizotifen, may occasionally be useful. For medically intractable chronic CH, wherein the patient’s activities of daily living are totally incapacitated by the pain., occipital nerve stimulation and deep brain stimulation appear useful as rescue options.
SUNCT/SUNA
This is a very rare TAC characterized by extremely brief episodic, unilateral severe head pain in the trigeminal nerve’s first division, most commonly in orbital, periorbital, or temporal regions. Some patients have pain in other cephalic locations. Pronounced cranial autonomic features accompany the pain.
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