Anna S. Cohen

Posterior hypothalamic and brainstem activation in hemicrania continua

Objective.—To determine the brain structures involved in mediating the pain of hemicrania continua using positron emission tomography.

High-flow oxygen for treatment of cluster headache: a randomized trial.

CONTEXT Cluster headache is an excruciatingly painful primary headache syndrome, with attacks of unilateral pain and cranial autonomic symptoms. The current licensed treatment for acute attacks is subcutaneous sumatriptan. OBJECTIVE To ascertain whether high-flow inhaled oxygen was superior to placebo in the acute treatment of cluster headache. DESIGN, SETTING, AND PATIENTS A double-blind, randomized, placebo-controlled crossover trial of 109 adults (aged 18-70 years) with cluster headache as defined by the International Headache Society. Patients treated 4 headache episodes with high-flow inhaled oxygen or placebo, alternately. Patients were randomized to the order in which they received the active treatment or placebo. Patients were recruited and followed up between 2002 and 2007 at the National Hospital for Neurology and Neurosurgery, London, England. INTERVENTION Inhaled oxygen at 100%, 12 L/min, delivered by face mask, for 15 minutes at the start of an attack of cluster headache or high-flow air placebo delivered alternately for 4 attacks. MAIN OUTCOME MEASURES The primary end point was to render the patient pain free, or in the absence of a diary to have adequate relief, at 15 minutes. Secondary end points included rendering the patient pain free at 30 minutes, reduction in pain up to 60 minutes, need for rescue medication 15 minutes after treatment, overall response to the treatment and overall functional disability, and effect on associated symptoms. RESULTS Fifty-seven patients with episodic cluster headache and 19 with chronic cluster headache were available for the analysis. For the primary end point the difference between oxygen, 78% (95% confidence interval, 71%-85% for 150 attacks) and air, 20% (95% confidence interval, 14%-26%; for 148 attacks) was significant (Wald test, chi(5)(2) = 66.7, P < .001). There were no important adverse events. CONCLUSION Treatment of patients with cluster headache at symptom onset using inhaled high-flow oxygen compared with placebo was more likely to result in being pain-free at 15 minutes. TRIAL REGISTRATION isrctn.org Identifier: ISRCTN94092997.

Posterior hypothalamic activation in paroxysmal hemicrania.

Paroxysmal hemicrania (PH) is a severe, strictly unilateral headache that lasts 2 to 30 minutes, occurs more than five times daily, is associated with trigeminal autonomic symptoms, and is exquisitely responsive to indomethacin. The purpose of the study was to determine the brain structures active in PH.

SUNCT syndrome responsive to intravenous lidocaine

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a primary headache syndrome that has been reported to be resistant to treatment with intravenous lidocaine. We report four cases of SUNCT in whom intravenous lidocaine (1.3-3.3 mg kg−1 h−1) completely suppressed the headaches for the duration of the infusion. The headache returned after cessation of treatment. Two patients went on to have their symptoms controlled on topiramate (50-300 mg daily). One patient had typical migrainous aura in association with some of the attacks of pain but never migrainous headaches. These cases suggest that treatment with lidocaine can be considered when acute intervention is required to suppress a severe exacerbation of SUNCT, and further broaden the therapeutic and clinical background of this syndrome.

Electrocardiographic abnormalities in patients with cluster headache on verapamil therapy

Background: High dose verapamil is an increasingly common preventive treatment in cluster headache (CH). Side effects include atrioventricular block and bradycardia, although their incidence in this population is not clear. Method: This audit study assessed the incidence of arrhythmias on high dose verapamil in patients with cluster headache. Results: Of three hundred sixty-nine patients with cluster headache, 217 outpatients (175 men) received verapamil, starting at 240 mg daily and increasing by 80 mg every 2 weeks with a check electrocardiogram (EKG), until the CH was suppressed, side effects intervened, or to a maximum daily dose of 960 mg. One patient had 1,200 mg/day. Eighty-nine patients (41%) had no EKGs. One hundred eight had EKGs in the hospital notes, and a further 20 had EKGs done elsewhere. Twenty-one of 108 patients (19%) had arrhythmias. Thirteen (12%) had first-degree heart block (PR > 0.2 s), at 240 to 960 mg/day, with one requiring a permanent pacemaker. Four patients had junctional rhythm, and one had second-degree heart block. Four patients had right bundle branch block. There was bradycardia (HR < 60 bpm) in 39 patients (36%), but verapamil was stopped in only 4 patients. In eight patients the PR interval was lengthened, but not to >0.2 s. The incidence of arrhythmias on verapamil in this patient group is 19%, and bradycardia 36%. Conclusion: We therefore strongly recommend EKG monitoring in all patients with cluster headache on verapamil, to observe for the potential development of atrioventricular block and symptomatic bradycardia.

Unilateral photophobia or phonophobia in migraine compared with trigeminal autonomic cephalalgias

Our objective was to compare the presence of self-reported unilateral photophobia or phonophobia, or both, during headache attacks comparing patients with trigeminal autonomic cephalalgias (TACs)—including cluster headache, shortlasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and paroxysmal hemicrania—or hemicrania continua, and other headache types. We conducted a prospective study in patients attending a referral out-patient clinic over 5 months and those admitted for an intramuscular indomethacin test. Two hundred and six patients were included. In episodic migraine patients, two of 54 (4%) reported unilateral photophobia or phonophobia, or both. In chronic migraine patients, six of 48 (13%) complained of unilateral photophobia or phonophobia, or both, whereas none of the 24 patients with medication-overuse headache reported these unilateral symptoms, although these patients all had clinical symptoms suggesting the diagnosis of migraine. Only three of 22 patients (14%) suffering from new daily persistent headache (NDPH) experienced unilateral photophobia or phonophobia. In chronic cluster headache 10 of 21 patients (48%) had unilateral photophobia or phonophobia, or both, and this symptom appeared in four of five patients (80%) with episodic cluster headache. Unilateral photophobia or phonophobia, or both, were reported by six of 11 patients (55%) with hemicrania continua, five of nine (56%) with SUNCT, and four of six (67%) with chronic paroxysmal hemicrania. Unilateral phonophobia or photophobia, or both, are more frequent in TACs and hemicrania continua than in migraine and NDPH. The presence of these unilateral symptoms may be clinically useful in the differential diagnosis of primary headaches.

Trigeminal Autonomic Cephalalgias: Current and Future Treatments

The trigeminal autonomic cephalgias include cluster headache, paroxysmal hemicrania, and short‐lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). The evidence for the current treatment options for each of these syndromes is considered, including oxygen, sumatriptan, and verapamil in cluster headache, indomethacin in paroxysmal hemicrania, and intravenous lidocaine and lamotrigine in SUNCT. Some treatments such as topiramate have an effect in all of these, as well as in migraine and other pain syndromes. The involvement of the hypothalamus in functional imaging studies implies that this may be a substrate for targeting treatment options in the future.

Trigeminal Autonomic Cephalalgias: Paroxysmal Hemicrania, SUNCT/SUNA, and Hemicrania Continua

The trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders that include cluster headache (CH), paroxysmal hemicrania (PH), and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing/cranial autonomic features (SUNCT/SUNA). Hemicrania continua (HC) is often included with this group, although the second edition of The International Classification of Headache Disorders did not link the entities. Trigeminal autonomic cephalalgias are generally characterized by relatively short-lasting attacks of severe pain and lateralized associated features including the pain, cranial autonomic symptoms, and where present, migrainous symptoms, such as photophobia. Paroxysmal hemicrania has intermediate duration and intermediate attack frequency. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing has the shortest attack duration and the highest attack frequency. Hemicrania continua has a continuous pain with exacerbations that can include cranial autonomic symptoms as part of the phenotype. The syndromes share much in their pathophysiology and investigation paths; however, their treatment is distinct, so that the accurate differentiation is important for optimal management.

Trigeminal autonomic cephalalgias: diagnostic and therapeutic developments.

Purpose of reviewThe review was designed to summarize recent research relevant to the trigeminal autonomic cephalalgias, which include cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing/cranial autonomic features (SUNCT/SUNA). Hemicrania continua is included for completeness. Recent findingsCluster headache has the longest attack duration and relatively low attack frequency. Paroxysmal hemicrania has intermediate duration and intermediate attack frequency. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing have the shortest attack duration and the highest attack frequency. Hemicrania continua has a continuous pain with exacerbations that can include cranial autonomic symptoms as part of the phenotype. The importance of diagnosing these syndromes resides in their excellent but highly selective response to treatment. SummaryConsiderable advances have been made in the diagnostic and therapeutic approaches to trigeminal autonomic cephalalgias and these are important for neurologists to consider.

SUNCT Syndrome in the Elderly

SUNCT syndrome (Short-lasting unilateral Neuralgiform headache attacks with Conjunctival Injection and Tearing) is a rare form of strictly unilateral headache with prominent autonomic symptoms (1). Diagnostic criteria are now established which require at least 20 attacks of unilateral, moderately severe, orbital or temporal, stabbing of throbbing pain, lasting for 5–240 s and associated with ipsilateral conjunctival injection and lacrimation (2). The syndrome is slightly predominant in males, with a mean age of onset at around 50 (3). Most primary headache syndromes present in early adulthood to middle age, yet there are case reports of SUNCT or SUNCT–like syndrome with onset in later life. Five patients with onset after the age of 70 have been reported; the reported ages of onset are 71 (4), 73 (5), 74 (6, 7), and 77 (8). We report a case of an elderly woman whose SUNCT syndrome started at the age of 75, and who presented to our clinic at the age of 85. Her misdiagnosis as trigeminal neuralgia illustrates the importance of recognizing SUNCT in this age group.