Recurrent headache mimicking cluster headache: a diagnostic challenge.

SYMPATHETIC NERVE ENTRAPMENT POINT INJECTION AS AN ADJUVANT TREATMENT FOR INTRACTABLE CLUSTER HEADACHE: A CASE REPORT

ObjectiveAlcohol has been recognized as the main trigger for a cluster headache attack, but clinical features to distinguish between cluster headache in drinkers and nondrinkers are unclear. Thus, the present study aimed to investigate the differences in clinical features of cluster headache between drinkers and nondrinkers.MethodsThis retrospective, observational study compared the clinical features of cluster headache between drinkers and nondrinkers among patients who were diagnosed with cluster headache between November 2004 and April 2018 at the Japanese Red Cross Shizuoka Hospital. Demographic and clinical data were collected from medical records and/or by patient interview.ResultsOf 131 patients, 98 (75%) were drinkers, and 33 (25%) were nondrinkers. Compared with nondrinkers, drinkers had significantly more frequent conjunctival injection (43% vs. 21%, p = 0.037) but significantly less frequent nasal congestion (31% vs. 52%, p = 0.0037), vomiting (11% vs. 30%, p = 0.014), and photophobia (29% vs. 45%, p = 0.008).ConclusionAmong individuals with cluster headache, the frequencies of conjunctival injection, nasal congestion, vomiting, and photophobia were different between drinkers and nondrinkers. These results suggested that drinking might influence the responses of the cranial autonomic reflex with respect to conjunctival injection or nasal congestion.

Cluster headache (CH) consists of attacks of severe, unilateral orbital/supraorbital/temporal pain, lasting for 15–180 min, occurring once or more times a day, and associated with ipsilateral conjunctival injection, lacrimation and other symptoms. Cataract is clouding of the lens of the eye causing a progressive and painless loss of vision. We describe the cases of two men (not relative, but with the same last name, which originates from north-eastern Italy) that in young adult age, after years of suffering from chronic CH, developed cataract on the same side of the pain attacks. Patient 1 was diagnosed as having cataract 18 years after the onset of episodic (and subsequently chronic) CH. Patient 2 began suffering from chronic CH at the age of 44 years and after 8 years he developed cataract. This is the first report of cataract in patient suffering of CH and occurring in the eye affected by the pain attack.

SUNCT syndrome in a child: a rare cause of paroxysmal headache

This Editorial introduces the 2015 Open Thematic Series dedicated to Cluster Headache (CH) and other Rare Headaches. For too long researchers have focused their attention on other most popular, sometimes clumsy or foggy forms of headache, overlooking this important and quasi-rare headache disorder [1]. Apparently CH represents the most peculiar form among the Trigeminal Autonomic Cephalalgias (TACs). The unbearable periorbital side-locked pain is coupled by ipsilateral cranial autonomic symptoms (conjunctival injection, lacrimation and rhinorrhea) and causes a vast disability although the overall burden of this disease has not yet found a complete systematization. Especially its chronic form, characterized by more days with attacks than not in the year span, represents a strict minority (10 % of CH, 0,01 % overall) and hits 70.000 on a sample of 100 millions of people. Even if these numbers are relatively small the impact of this disease is destructive, thus CH represents a niche worth to be re-focused. Recently Chronic CH (CCH) refractory to whichever medical therapy (rCCH) has been systematized from a clinical point of view [2]. The personal burden caused by CH, meant as loss of employment, homebound days and disability is higher in women than in men [3]. CH lifetime prevalence is 1/1000 and over the past decades a progressive reduction of the male/female ratio has been reported, being now 2.1:1 [4]. Even if CH is easy to diagnose on the basis of its peculiar features, the timing over the day of the attacks (up to 8), a temporally defined active cluster period (60–90 days) and its seasonal recurrence, only 1/3 of these patients is rightly diagnosed, with an unacceptable delay of 5.3 years and consequently more than 2/3 of them never receive a correct treatment [1, 5, 6]. Actually the current pathophysiological theory of CH is oriented towards a posterior hypothalamic dysfunction [7] but further pharmacological studies shall selectively focus this area. Recently the allele G of the G1246A HCRTR2 polymorphism was been found to be associated with CH, indicating that hypocretin/orexin system’s peptides may be involved in the transmission of pain, in autonomic and neuroendocrine functions, and in the pathogenesis of CH [8, 9]. For too many years sumatriptan, verapamil and corticosteroids have been the cornerstones of CH treatment [1]; we hope that the starting era of monoclonal antibodies against Calcitonin Gene-Related Peptide might include CH as therapeutic target. An ongoing phase 3 RCT on the use of LY2951742 in episodic CH through its preventative subcutaneous administration every 30 days could change the future management of CH [10]. Other approaches to chronic cluster headache are the new mini-invasive and non-invasive neuromodulation techniques. The European Headache Federation recommends caution in using these techniques [11] because only few controlled studies have been carried out yet. Among these new approaches, Vagal Nerve Stimuation (VNS) and sphenopalatine ganglion stimulation (SPG) seem promising but still classified at Class IV evidence [12, 13]; SPG shows contrasting evidence [14]. All these approaches need to be further confirmed and validated by ad hoc RCTs. Lastly, patients’ education is another important topic to be re-considered: sleep pattern changes, alcoholic beverages, NO-derived cardiovascular drugs and phosphodiesterase inhibitors widely self-prescribed for erectile dysfunction trigger additional bouts during CH active phases [1]. Furthermore, a more diffuse physicians education on CH management should lead to an earlier diagnosis and a more adequate treatment of this headache disorder [15]. Thus we trust that this Open Thematic Series dedicated to Cluster Headache could thicken the attention on a famed and quasi-rare headache disorder.

Female cluster headache in the United States of America: What are the gender differences?: Results from the United States Cluster Headache Survey

Cluster headache (CH) is estimated to be the most common primary trigeminal autonomic headache, although it is a rare disabling medical condition. Dominant symptoms of CH include severe unilateral orbital, supraorbital, and/or temporal pain, lasting from 15 to 180 minutes if untreated, associated with at least one of various autonomic symptoms during the headache, such as conjunctival injection, lacrimation, nasal congestion and rhinorrhea, facial sweating, miosis, ptosis, and eyelid edema. Headache is not frequently a symptom of multiple sclerosis (MS). The most commonly reported primary headaches are migraine without aura and a tension-type headache. Several described cases involved complicated migraine, ophthalmoplegic migraine-like headache, and finally cluster-like headache. We present a case of a 45-year-old male patient who had typical CH attacks as the initial and only clinical manifestation of MS, which was diagnosed after cerebrospinal fluid (CSF) isoelectric focusing and brain magnetic resonance imaging (MRI) investigation. He presented as a typical cluster-like headache patient since in the background of the CH symptoms and signs, were MS demyelinating lesions. In a patient with CH symptoms one should always think about the possibility of cluster-like-headache, which presents the CH patient with different underlying diseases, so we proposed a protocol to evaluate such patients and exclude diseases that could be in the background of CH symptoms.

To present results from the United States (US) Cluster Headache Survey including data on cluster headache demographics, clinical characteristics, suicidality, diagnostic delay, triggers, and personal burden. There are few large-scale studies looking at cluster headache patients and none from the USA. This manuscript will present data from The US Cluster Headache Survey, the largest survey ever completed of cluster headache patients living in the USA. The total survey was composed of 187 multiple-choice questions that dealt with issues related to cluster headache including demographics, clinical characteristics, comorbid medical conditions, family history, triggers, smoking history, and personal burden. The survey was placed on a Web site from October through December 2008. A total of 1134 individuals completed the survey (816 male, 318 female). Some key highlights from the survey include the following: (1) diagnostic delay: there remains a significant diagnostic delay for cluster headache patients on average 5+ years with only 21% receiving a correct diagnosis at time of initial presentation. (2) Suicidality: suicidal ideations are substantial, occurring in 55%. (3) Eye color: the predominant eye color in cluster headache patients is brown and blue, not hazel as suggested in previous descriptions. (4) Laterality: cluster headache has a right-sided predominance. (5) Attack profile: in US cluster headache sufferers, most attacks occur between early evening and early morning hours with peak time of headache onset between midnight and 3 am; the circadian periodicity for cluster headache is present but is not as predominant in the population as previously thought. (6) Triggers: beer is the most common type of alcohol trigger in US cluster headache patients; noted migraine triggers such as weather changes and smells are also very common cluster headache triggers. (7) Medical comorbidities: peptic ulcer disease does not have a high prevalence in US cluster headache patients as suggested by previous literature; cluster headache is associated with a low prevalence of cardiac disease as well as cerebrovascular disease even though the majority of patients are chronic heavy smokers. In US cluster headache sufferers, there appears to be comorbidity with restless leg syndrome, and this has not been demonstrated in non-US cluster headache populations. (8) Personal burden: cluster headache is disabling to the individual as almost 20% of cluster headache patients have lost a job secondary to cluster headache, while another 8% are out of work or on disability secondary to their headaches. Some findings from the US Cluster Headache Survey expound on what is currently known about cluster headache, while some of the results contradict what has been previously written, while other information is completely new about this fascinating headache disorder.

Purpose: Secondary trigeminal autonomic cephalalgias (TACs) are typically associated with posterior fossa abnormalities, such as tumors and vascular malformations. However, TACs following brainstem infarctions are rarely reported. This study aimed to characterize the clinical and anatomical features of TACs after unilateral dorsolateral medullary infarction.Methods: We analyzed four patients with dorsolateral medullary infarction who developed secondary TACs, diagnosed using the International Classification of Headache Disorders, third edition criteria. All patients underwent detailed neurological examinations and neuroimaging, including diffusion-weighted magnetic resonance imaging and magnetic resonance angiography. Additionally, five published cases were identified through a literature review and analyzed in conjunction with our cohort.Results: All patients exhibited stabbing or electric shock-like pain in the ipsilateral periorbital, hemifacial, and temporal regions. Headaches developed weeks to months post-stroke with brief attacks (1–2 minutes) occurring 1–5 times daily. Lacrimation and conjunctival injection were common. Three patients were diagnosed with short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), while a fourth had short-lasting unilateral neuralgiform with cranial autonomic symptoms (SUNA). Each patient, as well as four of the five from the literature, exhibited ipsilateral facial sensory loss, suggesting involvement of the trigeminal spinal tract and nucleus. Delayed headache onset was more frequent in persistent cases.Conclusion: Headache characteristics were more consistent with SUNCT/SUNA than with typical cluster headaches. Careful neurological examination is essential to detect focal signs and guide neuroimaging for identifying secondary causes. Clinicians should consider secondary TACs in patients with new-onset SUNCT/SUNA and focal brainstem signs.

Cluster headache belongs to the trigeminal autonomic cephalgias. Clinically, it is characterized by attacks of severe pain localized orbitally, supraorbitally or temporally, lasting for 15–180 min and occuring from once every other day to eight times a day. The attacks are associated with one or more of the following: conjunctival injection, lacrimation, nasal congestion, rhinorrhoea, forehead and facial sweating, miosis, ptosis and eyelid oedema (1). Attacks occur in series (so-called cluster periods or bouts) that are usually separated by remission periods lasting for months or years. In chronic cluster headache (CCH) substantial remission periods are lacking. Mean age at onset is 20–40 years and, for unknown reasons, 80% of patients are male. Treatment usually consists of drug therapy. Agents used for acute therapy are inhalation of high-flow oxygen, sumatriptan subcutaneous injection or nasal spray and zolmitriptan nasal spray. For transitional or short-term prophylaxis, corticosteroids and ergotamine derivatives are used. The cornerstone of maintenance prophylaxis is verapamil, but lithium and methysergide may also be used. Some patients respond to melatonin or topiramate (2). Nevertheless, there are a significant percentage of patients with CCH that do not experience satisfactory pain relief with drug therapy alone. In the last years new invasive techniques have emerged, and operational criteria have been proposed to define pharmacologically intractable headache (3). Case report

The SUNCT syndrome refers to Short-lasting Unilateral Neuralgiform headache with Conjunctival injection and Tearing. It is characterized by brief attacks of severe unilateral pain in the orbitotemporal region, associated with ipsilateral cranial autonomic disturbances. All SUNCT patients experience ipsilateral conjunctival injection and lacrimation. Mean age of onset is 50 years with a male predominance. The syndrome is often misdiagnosed as trigeminal neuralgia or cluster headache. Primary and secondary forms exist, the secondary form is most commonly associated with lesions of the posterior fossa or pituitary adenoma. The SUNCT syndrome is refractory to most commonly employed therapies. Lamotrigine has recently been reported as an effective first line therapy.

The trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders characterised by lateralized symptoms: prominent headache and ipsilateral cranial autonomic features, such as conjunctival injection, lacrimation and rhinorrhea. The TACs are: cluster headache (CH), paroxysmal hemicrania (PH), short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)/short-lasting neuralgiform headache attacks with cranial autonomic features (SUNA) and hemicrania continua (HC). Their diagnostic criteria are outlined in the International Classification of Headache Disorders, third edition-beta (ICHD-IIIb). These conditions are distinguished by their attack duration and frequency, as well as response to treatment. HC is continuous and by definition responsive to indomethacin. The main differential when considering this headache is chronic migraine. Other TACs are remarkable for their short duration and must be distinguished from other short-lasting painful conditions, such as trigeminal neuralgia and primary stabbing headache. Cluster headache is characterised by exquisitely painful attacks that occur in discrete episodes lasting 15-180 min a few times a day. In comparison, PH occurs more frequently and is of shorter duration, and like HC is responsive to indomethacin. SUNCT/SUNA is the shortest duration and highest frequency TAC; attacks can occur over a hundred times every day.

Chronic paroxysmal hemicrania (CPH) is a rare, unilateral primary headache syndrome. Recent studies suggest hypothalamic dysfunction as the likely cause of CPH. Therapeutic response to deep brain stimulation of the hypothalamus has been observed in the treatment of related trigeminal autonomic cephalgias. We explored the therapeutic effectiveness of posterior hypothalamic stimulation for the treatment of CPH in a patient intolerant of medical management. A 43-year-old woman with CPH reported acute onset of lancinating, unilateral headache pain focused about the right orbit. These debilitating headaches were accompanied by ipsilateral nasal congestion, conjunctival injection, tearing, and ptosis lasting minutes before resolving spontaneously. The patient exhausted attempts at medical management. A deep brain stimulator microelectrode was placed under stereotactic guidance. The posterior hypothalamic target was 3 mm posterior, 5 mm inferior, and 2 mm ipsilateral to the midcommissural point. The electrode was connected to a standard pulse generator and set to final amplitude of 1.5 V, a pulse width of 60 microseconds, and a frequency of 185 Hz. The patient's headache symptoms were durably alleviated with intraoperative activation. No complications were observed. This preliminary success suggests a role for posterior hypothalamic stimulation as a safe and effective treatment in patients with medically refractory CPH. As a therapeutic incremental innovation, this off-label use of technology for symptomatic therapy contributes to results of studies that support a central pathophysiological role for hypothalamic dysfunction in headaches classified among the trigeminal autonomic cephalgias.

Cluster headache (CH) is characterized by recurrent attacks of short-lasting excruciating pain accompanied by signs of autonomic dysfunction. Although neuroimaging results are usually normal in primary headaches, rarely structural lesions may be associated with CH like presentation. Most symptomatic CH is due to pituitary tumors and less commonly due to arteriovenous malformations (AVM) and aneurysms. Here we describe a case of 44-year-old male who presented to us with new onset episodic CH with headache features typical. Initially, headache responded to treatment, but later the headache became continuous, severe in intensity and refractory to treatment. Since magnetic resonance imaging was normal, he was subjected to digital substraction angiography (DSA) to rule out aneurysm or AVM. DSA revealed indirect carotid-cavernous fistula (CCF). Once the fistula was obliterated with onyx embolization, headache subsided completely. MRI brain is often routinely performed in CH to rule out secondary causes. When MRI brain is normal, detailed vascular imaging with DSA must be performed in patients with CH with especially in those with atypical features. Like continuous pain refractory to treatment, sudden increase in severity in the first episode, minimal or absent autonomic features and abnormal physical findings like persistent ptosis in the headache-free period. Although many cases of symptomatic CH have been reported in literature we report the first case of CCF presenting as CH. Early identification is essential as it is completely curable with endovascular treatment.

This article reports a case of secondary short-lasting unilateral neuralgiform headache with conjunctival injection and tearing following head and neck trauma due to a violent assault. Following the incident, the patient began experiencing 4 to 30 shooting/sharp pain attacks per day in the left anterior temporal and supraorbital areas, with an intensity of 10/10 on a numeric rating scale. Each attack lasted between 10 and 60 seconds. These attacks were accompanied by ipsilateral conjunctival injection, tearing, ptosis of the left eye, blurry vision, and occasional rhinorrhea. Significant improvements in sleep, autonomic symptoms, and pain were observed with a combination of melatonin 10 mg per day, gabapentin 300 mg twice daily, physical therapy, and psychotherapy. This case highlights the relevance of a multidisciplinary approach in the treatment of challenging cases when there is evidence of more than one contributing factor, with the aim of reducing pain and improving the patient's quality of life.