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1.  The Neuropsychology of Cluster Headache: Cognition, Mood, Disability, and Quality of Life of Patients With Chronic and Episodic Cluster Headache 
Headache  2015;55(2):287-300.
Cluster headache (CH) is commonly regarded as one of the most disabling headache conditions, and referred to as one of the most painful conditions known to humankind. Although there has been some research indicating the severe impact of CH, there is little comprehensive evidence of its impact on quality of life, disability, mood, and cognitive function in both its episodic (ECH) and chronic (CCH) variants.
This cross-sectional study investigates various aspects of cognitive function including intelligence, executive function, and memory, and mood, disability, and quality of life in 22 patients with ECH and CCH compared with age-matched healthy controls.
The results showed that intelligence and executive functions are intact in patients with CH, but that patients with CH perform significantly worse than healthy controls on tests of working memory and (all P < .05) report greater cognitive failures (P < .05). Around one third of both the ECH and CCH groups achieved “caseness” for depression, while self-reported anxiety was higher in those with CCH than the ECH patients, with 75% of the former compared with 38% of the latter groups achieving “caseness” on the measure of anxiety. Patients with CH reported high levels of disability, which was not significantly different between the 2 groups (P > .05). The patients with CH reported poor quality of life compared with healthy controls; however, this difference was not statistically significant.
Patients with CH show worse working memory, disturbance of mood, and poorer quality of life compared with healthy controls. The differences between patients with ECH and CCH, and the implications of these findings for the management of CH are discussed.
PMCID: PMC4354253  PMID: 25688646
chronic cluster headache; episodic cluster headache; cognition; mood; disability; quality of life
2.  SUNA and red ear syndrome: a new association and pathophysiological considerations 
Red ear syndrome (RES) is characterised by attacks of unilateral or bilateral burning ear pain associated with erythema. Primary and secondary forms have been described. Primary RES appears to have a frequent association with primary headaches especially migraine. Here, we describe the case of a woman with short-lasting unilateral neuralgiform attacks with cranial autonomic symptoms (SUNA) and recurrent episodes of ipsilateral red ear triggerable by cutaneous stimulation. Lamotrigine was beneficial for her SUNA but not for the RES. Both these disorders are extremely rare therefore their coexistence in the same individual may suggest similar pathophysiological mechanisms rather than a chance association.
PMCID: PMC3631130  PMID: 23565730
SUNA; SUNCT; Red ear syndrome; Trigeminal autonomic cephalalgias; Cranial autonomic symptoms
3.  A six year retrospective review of occipital nerve stimulation practice - controversies and challenges of an emerging technique for treating refractory headache syndromes 
A retrospective review of patients treated with Occipital Nerve Stimulation (ONS) at two large tertiary referral centres has been audited in order to optimise future treatment pathways.
Patient’s medical records were retrospectively reviewed, and each patient was contacted by a trained headache expert to confirm clinical diagnosis and system efficacy. Results were compared to reported outcomes in current literature on ONS for primary headaches.
Twenty-five patients underwent a trial of ONS between January 2007 and December 2012, and 23 patients went on to have permanent implantation of ONS. All 23 patients reached one-year follow/up, and 14 of them (61%) exceeded two years of follow-up. Seventeen of the 23 had refractory chronic migraine (rCM), and 3 refractory occipital neuralgia (ON). 11 of the 19 rCM patients had been referred with an incorrect headache diagnosis. Nine of the rCM patients (53%) reported 50% or more reduction in headache pain intensity and or frequency at long term follow-up (11–77 months). All 3 ON patients reported more than 50% reduction in pain intensity and/or frequency at 28–31 months. Ten (43%) subjects underwent surgical revision after an average of 11 ± 7 months from permanent implantation - in 90% of cases due to lead problems. Seven patients attended a specifically designed, multi-disciplinary, two-week pre-implant programme and showed improved scores across all measured psychological and functional parameters independent of response to subsequent ONS.
Our retrospective review: 1) confirms the long-term ONS success rate in refractory chronic headaches, consistent with previously published studies; 2) suggests that some headaches types may respond better to ONS than others (ON vs CM); 3) calls into question the role of trial stimulation in ONS; 4) confirms the high rate of complications related to the equipment not originally designed for ONS; 5) emphasises the need for specialist multidisciplinary care in these patients.
PMCID: PMC3751236  PMID: 23919570
Headache; Chronic migraine; Occipital neuralgia; Neuromodulation; Occipital nerve stimulation
4.  The red ear syndrome 
Red Ear Syndrome (RES) is a very rare disorder, with approximately 100 published cases in the medical literature. Red ear (RE) episodes are characterised by unilateral or bilateral attacks of paroxysmal burning sensations and reddening of the external ear. The duration of these episodes ranges from a few seconds to several hours. The attacks occur with a frequency ranging from several a day to a few per year. Episodes can occur spontaneously or be triggered, most frequently by rubbing or touching the ear, heat or cold, chewing, brushing of the hair, neck movements or exertion. Early-onset idiopathic RES seems to be associated with migraine, whereas late-onset idiopathic forms have been reported in association with trigeminal autonomic cephalalgias (TACs). Secondary forms of RES occur with upper cervical spine disorders or temporo-mandibular joint dysfunction. RES is regarded refractory to medical treatments, although some migraine preventative treatments have shown moderate benefit mainly in patients with migraine-related attacks. The pathophysiology of RES is still unclear but several hypotheses involving peripheral or central nervous system mechanisms have been proposed.
PMCID: PMC3850925  PMID: 24093332
Red ear syndrome; Migraine; Trigemino-autonomic reflex; Trigeminal autonomic cephalalgias; Parasympathetic system; Erythromelalgia
5.  Trigeminal autonomic cephalalgias: A review of recent diagnostic, therapeutic and pathophysiological developments 
Annals of Indian Academy of Neurology  2012;15(Suppl 1):S51-S61.
The trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders that are characterized by strictly unilateral trigeminal distribution pain occurring in association with ipsilateral cranial autonomic symptoms. This group includes cluster headache, paroxysmal hemicrania and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. These disorders are very painful, often considered to be some of the most painful conditions known to mankind, and consequently are highly disabling. They are distinguished by the frequency of attacks of pain, the length of the attacks and very characteristic responses to medical therapy, such that the diagnosis can usually be made clinically, which is important because it dictates therapy. The management of TACs can be very rewarding for physicians and highly beneficial to patients.
PMCID: PMC3444219  PMID: 23024564
Cluster headache; paroxysmal hemicrania; SUNA; SUNCT; trigeminal autonomic cephalalgias
6.  Occipital nerve stimulation in primary headache syndromes 
Chronic daily headache is a major worldwide health problem that affects 3–5% of the population and results in substantial disability. Advances in the management of headache disorders have meant that a substantial proportion of patients can be effectively treated with medical treatments. However, a significant minority of these patients are intractable to conventional medical treatments. Occipital nerve stimulation (ONS) is emerging as a promising treatment for patients with medically intractable, highly disabling chronic headache disorders, including migraine, cluster headache and other less common headache syndromes. Open-label studies have suggested that this treatment modality is effective and recent controlled trial data are also encouraging. The procedure is performed using several technical variations that have been reviewed along with the complications, which are usually minor and tolerable. The mechanism of action is poorly understood, though recent data suggest that ONS could restore the balance within the impaired central pain system through slow neuromodulatory processes in the pain neuromatrix. While the available data are very encouraging, the ultimate confirmation of the utility of a new therapeutic modality should come from controlled trials before widespread use can be advocated; more controlled data are still needed to properly assess the role of ONS in the management of medically intractable headache disorders. Future studies also need to address the variables that are predictors of response, including clinical phenotypes, surgical techniques and stimulation parameters.
PMCID: PMC3251898  PMID: 22276076
Neuromodulation; headache; occipital nerve stimulation; migraine; cluster headache; SUNCT; SUNA; hemicrania continua
7.  Cardiac cephalgia 
The purpose of this review was to provide a critical evaluation of medical literature on so-called “cardiac cephalgia” or “cardiac cephalalgia”. The 2004 International Classification of Headache Disorders codes cardiac cephalgia to 10.6 in the group of secondary headaches attributed to disorder of homoeostasis. This headache is hardly recognizable and is associated to an ischaemic cardiovascular event, of which it may be the only manifestation in 27% of cases. It usually occurs after exertion. Sometimes routine examinations, cardiac enzymes, ECG and even exercise stress test prove negative. In such cases, only a coronary angiogram can provide sufficient evidence for diagnosis. Cardiac cephalgia manifests itself without a specific pattern of clinical features: indeed, in this headache subtype there is a high variability of clinical manifestations between different patients and also within the same patient. It “mimics” sometimes a form of migraine either accompanied or not by autonomic symptoms, sometimes a form of tension-type headache; on other occasions, it exhibits characteristics that can hardly be interpreted as typical of primary headache. Pain location is highly variable. When the headache occurs as the only manifestation of an acute coronary event, the clues for suspicion are a) older age at onset, b) no past medical history of headache, c) presence of risk factors for vascular disorders and d) onset of headache under stress. Knowledge of cardiac cephalgia is scarce, due to its rare clinical occurrence and to the scant importance given to headache as a symptom concomitantly with an ischaemic cardiac event.
PMCID: PMC3451760  PMID: 19139804
Cardiac cephalgia; Exertional headache; Secondary headache; Headache attributed to disorder of homoeostasis; Acute myocardial ischemia

Results 1-7 (7)