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Background: The association between paroxysmal hemicrania (PH) and trigeminal neuralgia-the so-called PH-tic syndrome-has rarely been described. However, a correct diagnosis is crucial since both disorders require specific treatments. Little is known about pathophysiological mechanisms, and, to date, there are no electrophysiological studies in patients with PH-tic syndrome., Case: We describe the case of a 52-year-old man with a PH-tic syndrome successfully treated with an association of carbamazepine (1200 mg/day) and indomethacin (150 mg/die). Patient underwent trigeminal reflex testing, including blink and masseter inhibitory reflex, and laser-evoked potential (LEP) recording after supraorbital region stimulation in the affected and unaffected side. Both neurophysiological investigations resulted normal; LEPs failed to detect any latency asymmetry between both sides., Conclusions: Neurophysiological findings demonstrate for the first time the integrity of somatosensory system in a primary PH-tic syndrome case. Central pathophysiological mechanisms and hypothalamic dysregulation may contribute to the development of this rare syndrome., (© 2024. Fondazione Società Italiana di Neurologia.)

Objective: This article describes the clinical features and treatment of the indomethacin-responsive headache disorders paroxysmal hemicrania and hemicrania continua., Latest Developments: Both paroxysmal hemicrania and hemicrania continua are treated with indomethacin at the lowest clinically useful dose. It has recently become clear that some patients with either condition may respond to treatment with noninvasive vagus nerve stimulation, which can be both indomethacin sparing and, in some cases, headache controlling. Given the lifelong nature of both paroxysmal hemicrania and hemicrania continua, brain imaging with MRI is recommended when the conditions are identified, specifically including pituitary views., Essential Points: Paroxysmal hemicrania and hemicrania continua are indomethacin-responsive headache disorders that offer a rewarding and unique opportunity to provide marked clinical improvement when recognized and treated appropriately. These disorders share the final common pathway of the trigeminal-autonomic reflex, with head pain and cranial autonomic features, and are differentiated pathophysiologically by the pattern of brain involvement, which can be seen using functional imaging. They have distinct differential diagnoses to which the clinician needs to remain alert., (Copyright © 2024 American Academy of Neurology.)

Background: There is lack of population-based studies evaluating the prevalence of paroxysmal hemicrania, hemicrania continua and short-lasting unilateral neuralgiform headache attacks., Objectives: The aim of this study was to investigate the gender-specific 1-year prevalence of cluster headache, paroxysmal hemicrania, hemicrania continua, and short-lasting unilateral neuralgiform headache attacks., Methods: A nationwide study was conducted from January 1 2022 and December 31 2022 by linking diagnostic codes from Norwegian Patient Registry and prescription of relevant drugs from Norwegian Prescription Database on an individual basis. The 1-year prevalence with 95% confidence intervals (CI) of cluster headache, paroxysmal hemicrania, hemicrania continua and short-lasting unilateral neuralgiform headache attacks are estimated based on the combination of diagnostic codes, prescription of drugs and corresponding reimbursement codes., Results: Among 4,316,747 individuals aged ≥ 18 years, the 1-year prevalence per 100,000 was 14.6 (95% CI 13.5-15.8) for cluster headache, 2.2 (95% CI 1.8-2.7) for hemicrania continua, 1.4 (95% CI 1.0-1.8) for paroxysmal hemicrania, and 1.2 (95% CI 0.8-1.4) for short-lasting unilateral neuralgiform headache attacks. For all the trigeminal autonomic cephalalgies, cluster headache included, the prevalence was higher for women than men., Conclusions: In this nationwide register-based study, we found a 1-year prevalence per 100,100 of 14.6 for cluster headache, 2.2 for hemicranias continua, 1.4 for paroxysmal hemicranias, and 1.2 for short-lasting unilateral neuralgiform headache attacks. This is the first study reporting higher prevalence of cluster headache for women than men., (© 2024. The Author(s).)

Objective: To assess the prevalence or relative frequency of paroxysmal hemicrania and its clinical features in the adult general population and among adult patients evaluated for headache in tertiary care., Background: Paroxysmal hemicrania is a rare trigeminal autonomic cephalalgia with characteristic attacks of headache, associated cranial autonomic symptoms and signs, and an absolute response to indomethacin. Its epidemiological burden remains unknown in both the adult general population and among adult patients evaluated for headache in a tertiary care setting. Moreover, the frequencies of the clinical features associated with paroxysmal hemicrania have not been well established., Methods: A literature search of PubMed and Embase was conducted from January 1, 1988, to January 20, 2023. Eligible for inclusion were observational studies reporting the point prevalence or relative frequency of paroxysmal hemicrania or its clinical features in the adult general population or among adult patients evaluated for headache in tertiary care. Two independent investigators (M.J.H. and J.G.L.) performed the title, abstract, and full-text article screening. Each included study's risk of bias was critically appraised using the Joanna Briggs Institute Critical Appraisal Checklist for Studies Reporting Prevalence Data. Estimates of prevalence or relative frequency were calculated using a random-effects meta-analysis. The between-study heterogeneity was assessed using the I 2 statistic and further explored with meta-regression. This study was pre-registered on PROSPERO (identifier: CRD42023391127)., Results: A total of 17 clinic-based studies and one population-based study met the eligibility criteria. Importantly, an overall high risk of bias was observed across the eligible studies. The relative frequency of paroxysmal hemicrania was estimated to be 0.3% (95% CI, 0.2%-0.5%) among adult patients evaluated for headache in tertiary care with considerable heterogeneity (I 2  = 76.4%). No cases with paroxysmal hemicrania were identified among 1,838 participants in a population-based sample. Moreover, the most prevalent cranial autonomic symptoms were lacrimation (77.3% [95% Cl, 62.7%-87.3%]), conjunctival injection (75.0% [95% Cl, 60.3%-85.6%]), and nasal congestion (47.7% [95% Cl, 33.6%-62.3%])., Conclusions: Our findings suggest that paroxysmal hemicrania is a rare disorder among adults evaluated for headache in tertiary care, while its prevalence in the general population remains unknown. Further studies focusing on the clinical features of paroxysmal hemicrania are warranted., (© 2023 American Headache Society.)

Trigeminal autonomic cephalgias (TACs) are a well-defined subset of uncommon primary headaches that share comparable onset, pathophysiology and symptom patterns. TACs are characterised by the presentation of one-sided and high-intensity trigeminal pain together with unilateral cranial autonomic signs, which can include lacrimation, rhinorrhea, and miosis. The International Classification of Headache Disorders 3rd Edition recognises four different headache entities in this group, with cluster headache as the most recognised among them. Hemicrania continua (HC) and paroxysmal hemicrania (PH) are both distinctive cephalgias of which the diagnostic criteria include an absolute response to indomethacin. Consequently, for this reason they are often referred to as 'indomethacin-responsive' TACs. The main focus of this review was to discuss the state of knowledge regarding the pathophysiology and key characteristics of PH and HC. Given the limited understanding of these conditions, and their exceptionally uncommon prevalence, a correct diagnosis can pose a clinical challenge and the search for an effective treatment may be prolonged, which frequently has a serious impact upon patients' quality of life. The information provided in this review is meant to help physicians to differentiate indomethacin-sensitive cephalgias from other distinct headache disorders with a relatively similar clinical presentation, such as cluster headache, trigeminal neuralgia, and various migraine conditions.

Background: Paroxysmal hemicrania and hemicrania continua are indometacin-sensitive trigeminal autonomic cephalalgias, a terminology which reflects the predominant distribution of the pain, observable cranial autonomic features and shared pathophysiology. Understanding the latter is limited, both by low prevalence and the intricacies of studying brain function, requiring multimodal techniques to glean insights into such disorders. Similarly obscure is the curious response to indometacin. This review will address what is currently known about pathophysiology, the rationale for the current classification and, features which may confound the diagnosis, such as lack of cranial autonomic symptoms and those which are typically associated with migraine such as nausea, photophobia, phonophobia and aura. Despite these characteristics, a dramatic response to indometacin, which is not seen in migraine nor the other trigeminal autonomic cephalalgias , provides the hallmark of the diagnosis. The main clinical differential for paroxysmal hemicrania is based on temporal pattern and lies between cluster headache and short-lasting-neuralgiform headache attacks with tearing or additional cranial autonomic symptoms. For hemicrania continua it is more challenging as the main differential for which the disorder is often treated is migraine. A prior episodic pattern, often days at a time, and the tendency to exacerbation with analgesics will further deflect from the diagnosis. The relevance of this is that there is little overlap in therapeutics between paroxysmal hemicrania and hemicrania continua and other headache disorders and there are limited effective alternatives to indometacin. The most effective are other non-steroidal anti-inflammatory drugs including the newer COX-II inhibitors. Even though early reports suggest that a higher indometacin dose-requirement may herald a secondary precipitating pathology, this does not seem to be the case, with syndrome and response to treatment being similar with the primary disorder. In this context imaging of new onset paroxysmal hemicrania or hemicrania continua and implication of the results will be discussed as will alternative treatment options.

Importance: Trigeminal autonomic cephalalgias (TACs) comprise a unique collection of primary headache disorders characterized by moderate or severe unilateral pain, localized in in the area of distribution of the first branch of the trigeminal nerve, accompanied by cranial autonomic symptoms and signs. Most TACs are rare diseases, which hampers the possibility of performing randomized clinical trials and large studies. Therefore, knowledge of treatment efficacy must be based only on observational studies, rare disease registries, and case reports, where real-world data and evidence play an important role in health care decisions., Observations: Chronic cluster headache is the most common of these disorders, and the literature offers some evidence from randomized clinical trials to support the use of pharmacologic and neurostimulation treatments. Galcanezumab, a monoclonal antibody targeting the calcitonin gene-related peptide, was not effective at 3 months in a randomized clinical trial but showed efficacy at 12 months in a large case series. For the other TACs (ie, paroxysmal hemicrania, hemicrania continua, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing, and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms), only case reports and case series are available to guide physicians in everyday management., Conclusions and Relevance: The accumulation of epidemiologic, pathophysiologic, natural history knowledge, and data from case series and small controlled trials, especially over the past 20 years from investigators around the world, has added to the previously limited evidence and has helped advance and inform the treatment approach to rare TACs, which can be extremely challenging for clinicians.

Objective: We aimed to report the accessible demographic, clinical, and radiological characteristics of reported pediatric paroxysmal hemicrania (PH)., Introduction: It has been a while since PH in a child was first described. However, it is still unknown whether children's PH follows the same patterns as adults., Methods: This study followed the latest version of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses). PubMed, Web of Science, and Scopus were searched systematically without time limitation. We included all English-language, peer-reviewed articles, including observational or interventional studies reporting PH cases in children or adolescents based on the International Classification of Headache Disorders (ICHD) criteria. Data extracted included PH class; sex; age; age of onset; frequency, duration, site, severity, and quality of pains; triggers; and autonomic and migrainous symptoms, as well as a sense of restlessness/agitation, response to treatment, laboratory investigations, imaging, comorbidity, and family history. For quality assessment, two independent reviewers (MB and VM) assessed the methodological quality of the included studies through the Joanna Briggs Institute's critical appraisal checklist., Results: A total of 182 records were identified and reduced to 116 after removing duplicates. After screening, 22 articles met the inclusion criteria. Overall, the studies represented 35 children or adolescents with PH. We found a boy-to-girl ratio of 1.125:1. Onset occurred at a broad range of 1 to 14 years old. The mean age of onset among reported cases in children and adolescents was 6.5 years, while the mean age of diagnosis was 8.2 years. [Correction added on 22 August 2022, after first online publication: In the preceding sentence, 6.3 and 7.9 years were changed to 6.5 and 8.2 years, respectively.] The attacks' frequency and duration were greatly varied. Left-sided pain occurred twice as often as right-sided pain. The characteristics of the pain were usually severe in intensity. In nearly all of the cases, it was accompanied by ipsilateral cranial autonomic features. While most attacks were spontaneous, there were some common triggers. The physical examination, electroencephalogram, and brain magnetic resonance imaging had normal findings. Almost all patients benefited from indomethacin and showed complete responses to treatment, while some needed combination treatment of indomethacin with other medications., Conclusion: Although pediatric-onset PH has similar features to adult-onset PH, there are some challenges with ICHD criteria for younger children that limit the ability to confidently assign a diagnosis. Moreover, owing to concomitant migrainous features, PH may be confused with migraine in children and adolescents., (© 2022 American Headache Society.)

A 26-year-old woman with a history of migraine reported right-sided, severe stabbing orbital pain with cranial autonomic symptoms (CASs) for approximately 2 years. The attack duration was approximately 30 minutes, with a frequency of twice per day. Taking loxoprofen was ineffective. Six months earlier, moderate pressing continuous interictal pain without CASs had developed. Indomethacin farnesyl completely resolved the attacks but had no effect on the interictal pain. The patient was diagnosed with probable chronic paroxysmal hemicrania in accordance with the International Classification of Headache Disorders (ICHD-3) (third version). Continuous interictal pain gradually disappeared with a combination of indomethacin farnesyl and amitriptyline.

Paroxysmal Hemicrania (PH) is classified under trigeminal autonomic cephalalgia (TAC) as per the International Classification of Headache Disorders (ICHD). Since the first description by 0ttar Sjaastad and Inge Dale in 1974, PH has been reported by many authors. A greater understanding of PH phenotype and pathophysiology has resulted in the evolution of its diagnostic criteria, and management. We tabulated major case series of PH to describe the epidemiology, clinical features and recent updates of PH. PH is a rare headache characterized by daily, multiple paroxysms of unilateral, short-lasting (mean duration <20 minutes), side-locked headache in the distribution of ophthalmic division of trigeminal nerve with associated profound cranial autonomic symptoms. Recent ICHD classification added "restlessness" to the criteria for PH. Pain should completely respond to indomethacin to fulfil the diagnostic criteria of PH. PH should be differentiated from cluster headache, SUNCT/SUNA, and other short-lasting side-locked headaches. Trigeminal afferents possibly produce pain in PH and trigeminal-autonomic reflex explains the occurrence of autonomic features. Recently, a "permissive" central role of the hypothalamus is unveiled based on functional imaging studies. Other Cox-2 inhibitors, topiramate, calcium-channel blockers, epicranial nerve blocks have been shown to improve headache in some patients of PH who cannot tolerate indomethacin. Hypothalamic deep brain stimulation has been used in treatment-refractory cases., Competing Interests: None

Background: Paroxysmal hemicrania has not been associated with ipsilateral weakness, loss of sensation and Horner's syndrome. This report is the first of its kind documented in literature., Case Presentation: This was an elderly, sixty-five-year-old Chinese male who presented with a headache fulfilling criteria of paroxysmal hemicrania and was found to have signs of ipsilateral conjunctival injection, Horner's syndrome, weakness and loss of sensation; with resolution of the patient's physical signs after relief of the headache. Brain magnetic resonance imaging did not show any strokes or other headache mimics. The patient had a marked response to indomethacin and a decrease of headache intensity and frequency with indomethacin prophylaxis., Conclusions: Paroxysmal hemicrania has joined the list of stroke chameleons and that it would be one of the differentials in a patient with hemiplegia, hemisensory loss, autonomic signs and severe headache. It suggests that paroxysmal hemicrania in the elderly present atypically.

Purpose of Review: Primary headaches are less common and differ in presentation in older versus younger individuals. Secondary headaches become more common among older patients., Recent Findings: Diagnosis and management of headaches in those > 65 years are discussed. Migraine and tension-type headaches are rarely new onset in this age group and should be a diagnosis of exclusion. In older individuals, migraine is more likely to be bilateral with less sensory sensitivities. Migraine aura may present without headache; careful assessment is needed to exclude stroke. Other primary headaches discussed include cough, hypnic, and other headaches. Secondary causes discussed include giant cell arteritis, trigeminal post-herpetic neuropathy, sleep apnea, cardiac cephalgia, cervicogenic pain, vascular etiologies, medications, and burning-mouth syndrome. In older individuals, primary headaches are diagnoses of exclusion, and treatment is affected by comorbidities and polypharmacy. Secondary headaches are a major consideration requiring appropriate workup. Many treatments can safely be offered regardless of age.

The primary headaches are composed of multiple entities that cause episodic and chronic head pain in the absence of an underlying pathologic process, disease, or traumatic injury. The most common of these are migraine, tension-type headache, and the trigeminal autonomic cephalalgias. This article reviews the clinical presentation, pathophysiology, and treatment of each to help in differential diagnosis. These headache types share many common signs and symptoms, thus a clear understanding of each helps prevent a delay in diagnosis and inappropriate or ineffective treatment. Many of these patients seek dental care because orofacial pain is a common presenting symptom., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Hemicrania continua (HC), paroxysmal hemicrania (PH) and short lasting neuralgiform headache attacks (SUNCT and SUNA) are rare syndromes with a difficult therapeutic approach. The aim of this review is to summarize all articles dealing with treatments for HC, PH, SUNCT and SUNA, comparing them in terms of effectiveness and safety., Methods: A survey was performed using the pubmed database for documents published from the 1st January 1989 onwards. All types of articles were considered, those ones dealing with symptomatic cases and non-English written ones were excluded., Results: Indomethacin is the best treatment both for HC and PH. For the acute treatment of HC, piroxicam and celecoxib have shown good results, whilst for the prolonged treatment celecoxib, topiramate and gabapentin are good options besides indomethacin. For PH the best drug besides indomethacin is piroxicam, both for acute and prolonged treatment. For SUNCT and SUNA the most effective treatments are intravenous or subcutaneous lidocaine for the acute treatment of active phases and lamotrigine for the their prevention. Other effective therapeutic options are intravenous steroids for acute treatment and topiramate for prolonged treatment. Non-pharmacological techniques have shown good results in SUNCT and SUNA but, since they have been tried on a small number of patients, the reliability of their efficacy is poor and their safety profile mostly unknown., Conclusions: Besides a great number of treatments tried, HC, PH, SUNCT and SUNA management remains difficult, according with their unknown pathogenesis and their rarity, which strongly limits the studies upon these conditions. Further studies are needed to better define the treatment of choice for these conditions.

The association of paroxysmal hemicrania with trigeminal neuralgia (TN) has been described and called paroxysmal hemicrania-tic syndrome (PH-tic). We report the case of a patient diagnosed as having chronic PH-tic (CPH-tic) syndrome as a clinically isolated syndrome of the central nervous system (CNS) (CIS).A forty year old woman was admitted to our hospital suffering from right facial pain for the last 2 years. The attacks were paroxysmal, neuralgiform, consisting of throb-like sensations, which developed spontaneously or were triggered by different stimuli in right facial (maxilar and mandibular) areas. Parallel with those, she felt a throbbing orbital and frontal pain with homolateral autonomic symptoms such as conjunctival injection, lacrimation, and the feeling that the ear on the same side was full. This pain lasted most often between 15 and 20 minutes. Beyond hemifacial hypoesthesia in the region of right maxilar and mandibular nerve, the other neurological finding was normal. Magnetic resonance imaging (MRI) study showed a T2-weighted multiple hyperintense paraventricular lesion and hyperintense lesion in the right trigeminal main sensory nucleus and root inlet, all of them being hypointense on T1-weighted image. All of these lesions were hypointense in gadolinium-enhanced T1-weighted images. Neurophysiological studies of trigeminal nerve (somatosensory evoked potentials and blink reflex) correlated with MRI described lesions. The patient's pain bouts were improved immediately after treatment with indomethacin, and were completely relieved with lamotrigine for a longer period. According to the actual McDonald's criteria, clinical state was defined as CIS which was clinically presented by CPH-tic syndrome.Even though it is a clinical rarity and its etiology is usually idiopathic, CPH-tic syndrome can also be symptomatic. When dealing with symptomatic cases, like the one described here, when causal therapy is not possible due to the nature of the primary pathological process, a therapeutic approach, although symptomatic, can be fully effective in controlling this painful syndrome. The case report could be a contribution to the pathophysiological and clinical understanding of the association of CPH and TN.Key words: Paroxysmal hemicrania, trigeminal neuralgia, clinically isolated syndrome.

The differential diagnosis of strictly unilateral hemicranial pain includes a large number of primary and secondary headaches and cranial neuropathies. It may arise from both intracranial and extracranial structures such as cranium, neck, vessels, eyes, ears, nose, sinuses, teeth, mouth, and the other facial or cervical structure. Available data suggest that about two-third patients with side-locked headache visiting neurology or headache clinics have primary headaches. Other one-third will have either secondary headaches or neuralgias. Many of these hemicranial pain syndromes have overlapping presentations. Primary headache disorders may spread to involve the face and / or neck. Even various intracranial and extracranial pathologies may have similar overlapping presentations. Patients may present to a variety of clinicians, including headache experts, dentists, otolaryngologists, ophthalmologist, psychiatrists, and physiotherapists. Unfortunately, there is not uniform approach for such patients and diagnostic ambiguity is frequently encountered in clinical practice.Herein, we review the differential diagnoses of side-locked headaches and provide an algorithm based approach for patients presenting with side-locked headaches. Side-locked headache is itself a red flag. So, the first priority should be to rule out secondary headaches. A comprehensive history and thorough examinations will help one to formulate an algorithm to rule out or confirm secondary side-locked headaches. The diagnoses of most secondary side-locked headaches are largely investigations dependent. Therefore, each suspected secondary headache should be subjected for appropriate investigations or referral. The diagnostic approach of primary side-locked headache starts once one rule out all the possible secondary headaches. We have discussed an algorithmic approach for both secondary and primary side-locked headaches.

Background: Transient or permanent neurological symptoms occur in 17-40% of patients with aortic dissection. They can distract from or even mask the underlying life-threatening condition., Case Presentation: We present the case of a young Caucasian man who consulted for recurrent episodes of stereotyped right-sided sudden-onset severe headache. Upon questioning, he also reported a dull chest pain. Clinical examination and brain magnetic resonance imaging were unremarkable. The concomitant presence of chest pain made us consider aortic dissection. Contrast-enhanced cervico-thoraco-abdominal computerized tomography revealed type A aortic dissection. The patient underwent surgical replacement of the ascending aorta and reported no further episode of headache thereafter. Differential diagnosis of headache in this case includes paroxysmal hemicrania, cluster headache, migraine, trigeminal neuralgia and short lasting unilateral neuralgiform headache with conjunctival injection and tearing. Failure to match diagnostic criteria for any of these primary headache disorders and the resolution of pain episodes following surgery led us to postulate that these new-onset hemicrania episodes were symptomatic of aortic dissection. We hypothesize that aortic wall ischemia could have activated the trigeminovascular system and thereby caused hemicranial pain. Such an effect might be mediated by two different pathways that can be referred to as anatomical and humoral. The humoral hypothesis would posit that ischemia results in synthesis of pro-inflammatory mediators released from the aortic wall into the blood stream, such that they reach the central nervous system and directly stimulate specific receptors. The anatomical hypothesis would imply that pain signals generated by nociceptors in the aortic wall are transferred to the trigeminal ganglion via the cardiac plexus, the first cervical ganglion and the internal carotid nerve such that pain perception is referred to related cranio-cervical dermatomes., Conclusion: In cases of isolated headache that does not match key diagnostic criteria for a primary headache entity; a thorough review of systems should be performed to look for symptoms that may indicate symptomatic headache from potentially life-threatening conditions. Neurologists should consider aortic dissection in patients presenting with acute headache and chest pain. Further clinical or experimental studies are required to refute or validate the pathophysiological hypothesis discussed here.

The question of whether the trigeminal autonomic cephalalgias (TACs) represent primary diagnoses or points on a continuum has been debatable for a number of years. Patients with TACs may present with similar clinical characteristics, and occasionally, TACS respond to similar treatments. Prima facie, these disorders may seem to be intimately related. However, due to the current evidence, it would be challenging to accurately conclude whether they represent different primary headache diagnoses or the same primary headache disorder represented by different points on the same continuum. Ultimately, the TACs may utilize similar pathways and activate nociceptive responses that result in similar clinical phenotypes but "original and initiating" etiology may differ, and these disorders may not be points on the same continuum. This paper seeks to provide a brief comparison of TACs via diagnostic criteria, secondary causes, brief overview of pathophysiology, and the use of some key treatments and their mechanism of actions to illustrate the TAC similarities and differences.

Paroxysmal hemicrania and hemicrania continua are primary headache disorders characterized by unilateral attacks of severe pain around the orbit with associated autonomic features. They are unique in their absolute response to indomethacin. Diagnosis is made when patients with suspected paroxysmal hemicrania or hemicrania continua have the resolution of headache with therapeutic doses of indomethacin. Once diagnosis is made, limited data exists on the ongoing management of these patients. For patients who do not tolerate indomethacin, or wish to come off medication, there remain few options. This article will discuss the diagnosis of paroxysmal hemicrania and hemicrania continua and the ongoing management of patients on indomethacin, as well as options for patients who do not tolerate or need to come off indomethacin.

Review of literature on the trigeminal autonomic cephalgias are presented. Trigeminal autonomic cephalgias are primary headaches with phenotype consisting of trigeminal pain with autonomic sign including lacrimation, rhinorrhea and miosis. Discussed are issues of classification, pathogenesis, clinical picture, diagnosis, differential diagnosis and treatment of this headache. Special attention is paid to cluster headache, paroxysmal hemicrania, SUNCT syndrome, hemicrania continua.

Introduction: Hemicranias are an uncommon type of headache characterised by strictly unilateral pain, either as a continuous, although fluctuating, headache in hemicrania continua (HC) or in the form of recurring attacks in paroxysmal hemicrania (PH). In both types of headache, an absolute response to indomethacin is reported. AIMS. To analyse the fulfilment of current diagnostic criteria for HC and PH and the recent introduction of HC within the group of trigeminal-autonomic cephalgias., Patients and Methods: The clinical and therapeutic characteristics of patients diagnosed with HC or PH were evaluated retrospectively. Demographic and symptomatological information as well as data regarding the analogical pain scale and response to indomethacin were included., Results: A sample of 12 HC (four males and eight females) was evaluated from a total of 520 cases (2.3%). Mean age at onset: 47.1 ± 16.4 years. Baseline pain intensity: 3.3 ± 1,9. Exacerbations: 9.2 ± 1.1. Eight cases (66.7%) presented autonomic symptoms, four (33.3%) followed a time pattern, and two (16.7%) did not respond to indomethacin. We evaluated a sample of 11 PH (100% females) from 520 cases (2.1%). Mean age at onset: 37.0 ± 13.9 years. Pain intensity: 8.7 ± 2.7. Nine cases (81.8%) presented autonomic symptoms, three (27.3%) followed a time pattern and one (9.1%) did not respond to indomethacin., Conclusions: Hemicranias are not frequently diagnosed in day-to-day clinical practice. Their diagnosis requires the fulfilment of certain criteria that are sometimes not fully satisfied. We believe that the criteria need revising and we also support the recent inclusion of HC within the group of trigeminal-autonomic cephalgias.

Paroxysmal hemicrania (PH) is an underreported and underdiagnosed primary headache disorder. It usually begins in the third or fourth decade of life. The recent observations indicate that it is equally prevalent in both males and females. PH is characterized by severe, strictly unilateral head pain attacks that occur in association with ipsilateral autonomic features. The attacks in PH are shorter and more frequent compared with cluster headache (CH) but otherwise PH and CH have similar clinical features. The hallmark of PH is the absolute cessation of the headache with indomethacin. However, a range of drugs may show partial to complete relief in certain groups of patients. Neuromodulatory procedures, such as greater occipital nerve blockade, blockade of sphenopalatine ganglion and neurostimulation of the posterior hypothalamus, are reserved for refractory PH.

Introduction: The trigeminal autonomic cephalalgias (TACs) subsume four primary headache disorders. Hemicrania continua is increasingly regarded as an additional TAC. In rare cases patients may present with two different TACs or a TAC and hemicrania continua., Cases: We report four patients with two different TACs or one TAC and hemicrania continua. Two patients presented with cluster headache and paroxysmal hemicrania, one patient with cluster headache and hemicrania continua, and one patient suffered from cluster headache and SUNCT., Discussion: While the International Classification of Headache Disorders (ICHD-II) proposes specific diagnostic criteria, the variability of clinical presentation may make clear diagnosis difficult. All patients fulfilled the ICHD-II criteria. The manifestation of two different TACs or hemicrania continua in one patient is uncommon but possible and should be taken into account especially when chronic headache patients present with changing headache symptoms.

Cluster headache, paroxysmal hemicrania and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome) are classified under trigeminal autonomic cephalalgia (TAC). The proposed revision of the international classification of headache disorders (ICDH-3 beta) adds hemicrania continua to this diagnostic group. Moreover, diagnostic criteria of the other TACs were modified and are characterized by persistent headache or headache attacks accompanied by cranial autonomic symptoms. The main difference between the various TACs is the duration of attacks. Differentiation is important because different pharmacological strategies are necessary.

Although severe short-lasting headaches are rare, they can be considered disabling conditions with a major impact on the quality of life of patients. These headaches can divided broadly in to those associated with autonomic symptoms, so called trigeminal autonomic cephalgias (TACs), and those with few or no autonomic symptoms. The TACs include cluster headache, paroxysmal hemicranias, hemicrania continua, and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms as well as short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome. In all of these syndromes, half-sided head pain and ipsilateral cranial autonomic symptoms such as lacrimation or rhinorrhea are prominent. The paroxysmal hemicranias have, unlike cluster headaches, a very robust response to indomethacin, leading to a notion of indomethacin-sensitive headaches. The diagnosis of TACs is exclusively a clinical task. Because of the fact that cluster headache is strictly half-sided, typically involves the region around the eye and temple and often starts in the upper jaw, most patients first consult a dentist or ophthalmologist. No single instrumental examination has yet been able to define, or ensure, the correct diagnosis, or differentiate idiopathic headache syndromes. It is crucial that a trained neurologist sees these patients early so that management can be optimized and unnecessary procedures can be avoided. Although TACS are, in comparison to migraine, quite rare, they are nevertheless clinically very important for the neurologist to consider as they are easy to diagnose and the treatment is very effective in most patients., (© 2013 American Headache Society.)

Introduction: The response to indomethacin is an important feature for the diagnosis of hemicrania continua., Case Description: We report the case of a 34-year-old female patient with a one-year history of strictly unilateral continuous headache with accompanying ipsilateral autonomic symptoms. As diagnostic testing was unremarkable, hemicrania continua was suspected. A dose of 150 mg of indomethacin/day reduced the headache by 80%. Although an increase to 225 mg/day led to a further reduction of headaches, a new onset of moderate to severe pulsating migrainous bilateral headache developed., Discussion: As shown in older studies, indomethacin can induce de novo headaches in a presumably dose-related fashion although the exact mechanism in uncertain. A treatable secondary origin of the indomethacin-induced headaches in patients with hemicrania continua or paroxysmal hemicrania (such as reversible cerebral vasoconstriction syndrome or aseptic meningitis) should be considered and excluded by further diagnostic testing if headaches persist after discontinuation of indomethacin.

Primary headache disorders include migraine, tension-type headaches, and the trigeminal autonomic cephalgias (TACs). "Primary" refers to a lack of clear underlying causative pathology, trauma, or systemic disease. The TACs include cluster headache, paroxysmal hemicrania, and short-lasting neuralgiform headache attacks with conjunctival injection and tearing; hemicrania continua, although classified separately by the International Headache Society, shares many features of both migraine and the TACs. This article describes the features and treatment of these disorders., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Primary headache disorders that are more frequently encountered in the paediatric population include migraine and tension-type headaches. The trigeminal autonomic cephalalgias (TACs), which includes cluster headache (CH), paroxysmal hemicrania (PH) and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), are rarely reported in the paediatric population. The 1-year prevalence of CH seems to be 0.03 %. The clinical features of childhood and adolescence onset CH seem to be similar to those of adult onset. Cranial autonomic features and restlessness seem to less prominent in children than in adults. When restlessness is present, it often manifests as thrashing around in children and can distract attention from the headache, thereby contributing to a delay in diagnosis. The frequency of cluster periods seems to be lower in childhood. Similarly, the duration of the single cluster period seems to be shorter. The temporal pattern shows a trend towards a gradual increase of frequency and duration of symptoms in adult life. In terms of management of paediatric CH, oxygen has been used successfully in several paediatric CH patients, and given its good side-effect profile, it is considered the abortive agent of choice for paediatric CH. Verapamil is the preventative drug of choice in both episodic and chronic CH, though paediatric patients should be started on lower doses and titrated according to age. Paediatric-onset PH and SUNCT are very rarely reported. The clinical phenotype and response to treatment seem to resemble the adult-onset form. Paediatric-onset TACs are poorly recognized and there is often a delay of several months or years before the diagnosis is made. Awareness of typical clinical pictures of these excruciating headaches is essential to allow prompt initiation of the appropriate management.

Background: Paroxysmal hemicrania (PH) is a probably underreported primary headache disorder. It is characterized by repeated attacks of severe, strictly unilateral pain lasting 2 to 30 minutes localized to orbital, supraorbital, and temporal areas accompanied by ipsilateral autonomic features. The hallmark of PH is the absolute cessation of the headache with indomethacin. However, these all features may not be present in all cases and a few cases may remain unclassified according to the 2nd Edition of The International classification of Headache Disorders (ICHD-II) criteria for PH., Methods: Twenty-two patients were included in this retrospective observation., Results: We describe 17 patients, observed over six years, who fulfilled the ICHD-II criteria for PH. In parallel, we identified five more patients in whom one of the features of the diagnostic criteria for PH was missing. Two patients did not show any evidence of cranial autonomic feature during the attacks of headache. Another two patients did not fulfill the criteria for PH as the maximum attack frequency was less than five. One patient had an incomplete response to indomethacin., Conclusion: A subset of patients may not have all the defined features of PH and there is a need for refinement of the existing diagnostic criteria.

Trigeminal autonomic cephalalgias are short-lasting primary headache disorders associated with autonomic symptoms. Paroxysmal hemicrania is a rare headache disorder similar to cluster headache. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) are unusual headache syndromes typified by a high frequency of severe, brief, unilateral attacks that usually occur in the distribution of the trigeminal nerve. SUNCT is a subtype of SUNA in which both conjunctival injection and tearing are present. SUNA differs from SUNCT in that autonomic symptoms are less prominent., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Paroxysmal hemicrania is experienced as headache attacks with pain and accompanying symptoms similar to those of cluster headaches. Attacks are, however of shorter duration, occur more frequently, affect predominantly women and respond reliably to indomethacin. Paroxysmal hemicrania can also occur secondary to an identifiable cause. To exclude symptomatic, paroxysmal hemicrania, especially with an atypical clinical picture and poor response to indomethacin, a careful diagnostic approach is necessary. The SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) is characterized by one-sided pain attacks of short duration, much shorter than other trigeminal autonomic cephalgias. Classically, the pain is accompanied by ipsilateral lacrimation and conjunctival injection. Some patients have been described with both cluster headache and trigeminal neuralgia. These patients should receive both diagnoses. It is important to differentiate these headache entities as specific therapy is needed for each to achieve optimal pain relief.

Background: Hemicrania continua (HC) is a headache syndrome characterized by continuous, unilateral head pain, autonomic features, and a complete therapeutic response to indomethacin. Although HC is classified as a unique entity among primary headache disorders, it clearly shares features with other primary headaches, including trigeminal autonomic cephalalgias, and chronic daily headaches, such as chronic migraine and chronic tension-type headache. In addition, the diagnosis is often delayed secondary to a relatively low incidence and the occurrence of some phenotypic variability as found in previous case series., Case: A 62-year-old woman presented with 5 months of unremitting, bilateral headache with significant autonomic symptoms during exacerbations of pain. Neurological examination and imaging studies were normal. After failure to respond to numerous previous therapeutic medicines and interventions, she experienced complete resolution following administration of indomethacin and eventual remission on sustained treatment., Conclusion: This case demonstrates that hemicrania continua with requisite autonomic features can occur in a purely bilateral form. Although the definitive aspects of HC continue to evolve, a bilateral headache meeting the current criteria warrants a therapeutic trial of indomethacin.

The majority of previous studies on unilateral headaches beyond migraine and cluster headache have focussed on certain disorders such as paroxysmal hemicrania, SUNCT and primary stabbing headache. We assessed headache characteristics, importance of neuroimaging and response to indomethacin in an unselected series of uncommon unilateral headaches. We investigated all consecutive patients presented with unilateral headaches not fulfilling ICHD-II criteria of migraine and cluster headache. Patients underwent cranial magnetic resonance imaging or computed tomography as well as an indo-test, i.e. oral indomethacin 75 mg b.i.d. for 3 days. Among 63 patients we diagnosed primary stabbing headache in 12 patients, (probable) paroxysmal hemicrania in 6 and tension-type headache in 3 patients. One patient each had probable SUNCT, new daily persistent headache and nasociliary neuralgia. Eight patients had a secondary headache and 31 could not be classified according to ICDH-II. Imaging revealed lesions causally related to the headache in 8 patients. Indo-test achieved full remission of headache in 13 of 51 patients. At follow-up 11 ± 3 months after the first visit 29% of the patients were headache-free for ≥3 months. In conclusion, almost half of the patients presented with unilateral headaches beyond migraine and cluster headache cannot be classified according to ICHD-II. Among classifiable headaches primary stabbing headache was the most common. Imaging should be considered to rule out secondary headaches. The course is favourable in one third of the patients.

A woman in her fifties, with a long history of side-locked unilateral headache, was hospitalized for left-sided side-locked paroxysmal headache (attacks with 10-20 min duration). Clinical and neurological examinations, and brain MRI revealed normal findings. She responded well to indomethacin (50 mg three times daily). Due to non-compliance because of dyspepsia, which delayed the final diagnosis of chronic paroxysmal hemicrania (CPH) for 16, months indomethacin was administered both rectally and orally. A retrospective review of her medical history showed 15 years of unsuccessfully treated unilateral headache, until she responded completely to rofecoxib. Ipsilateral cranial autonomic symptoms also supported the diagnosis of hemicrania continua, although these symptoms presented before indomethacin was tried. Diagnostic delay and misdiagnoses of unilateral headaches, as illustrated by this case, shows the clinical controversies and difficulties in diagnosing and treating this condition.

Chronic paroxysmal hemicrania (CPH) is a rare primary headache syndrome, which is classified along with hemicrania continua and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) as trigeminal autonomic cephalalgia (TACs). CPH is characterised by short-lasting (2-30 min), severe and multiple (more than 5/day) pain attacks. Headache is unilateral, and fronto-orbital-temporal pain is combined with cranial autonomic symptoms. According to the International Classification of Headache Disorders, 2nd edition, the attacks are absolutely responsive to indomethacin. CPH has been only rarely and incompletely described in the developmental age. Here, we describe two cases concerning a 7-year-old boy and a 11-year-old boy with short-lasting, recurrent headache combined with cranial autonomic features. Pain was described as excruciating, and was non-responsive to most traditional analgesic drugs. The clinical features of our children's headache and the positive response to indomethacin led us to propose the diagnosis of CPH. Therefore, our children can be included amongst the very few cases of this trigeminal autonomic cephalgia described in the paediatric age., (© The Author(s) 2011. This article is published with open access at Springerlink.com)

We report the case of a woman with a 15-year history of chronic hemicrania continua, which over time evolved to remitting hemicrania continua. This is the second reported case in the literature documenting this transition.

Hemicrania continua (HC) belongs to the group of primary headaches and it is characterized by a strictly unilateral, continuous headache of moderate intensity, with superimposed exacerbations of severe intensity that are accompanied by trigeminal autonomic features. The syndrome is completely responsive to indomethacin. Here we report a case of a 49-year-old man with HC, which may be viewed as a combination of different types of headache, ie, chronic tension-type headache and trigeminal autonomic cephalalgia. The analysis of this case raises interesting issues regarding the proper place of HC among the primary headache forms.

Paroxysmal hemicrania is a trigeminal autonomic cephalalgia first described in 1976, characterized by episodic attacks of excruciating unilateral periorbital and temporal stabbing, pulsatile, craw-like, or boring headaches lasting 2 - 30 minutes, accompanied by autonomic features, and alleviated by indomethacin. Paroxysmal hemicrania is divided into an episodic or chronic form, depending on the duration and frequency of the attacks. We describe a case of paroxysmal hemicrania in a patient with a contralateral anterior clinoid meningioma, which resolved after tumor resection. Most cases of autonomic cephalgias are primary headaches and not caused by underlying intracranial structural lesions. Based on our patient and a literature review of secondary causes of trigeminal autonomic cephalalgias, we recommend that all patients with trigeminal autonomic cephalalgias including paroxysmal hemicrania undergo neuroimaging studies. The preferred neuro-radiologic procedure should be a cranial MRI to exclude underlying structural intracranial lesions, particularly in the sellar and parasellar regions.

Whereas paroxysmal hemicrania (PH) is studied extensively in adults, even case reports of PH in children are rare. We present the first prospective follow-up study on PH in children. Our aim was to investigate whether differences exist between paediatric and adult patients. We assessed all children with chronic headache who were referred to our paediatric out-patient pain clinic within 3 years based on interviews and validated questionnaires. Among 628 patients we found five children with PH (0.8%) and three with probable PH (0.5%), in total 1.3%. Pain characteristics, autonomic symptoms and treatment response to indomethacin were similar to adult PH patients. Our results demonstrate that the International Headache Society classification of PH is also applicable to children. We suspect that PH has been underdiagnosed in children and therefore suboptimally treated thus far.