Do paroxysmal hemicrania and hemicrania continua represent different headaches? A retrospective study.

OBJECTIVE: Hemicrania continua and paroxysmal hemicrania are considered different headaches belonging to a group of trigeminal autonomic cephalalgias. However, they share many clinical features. Both headaches also show complete response to indomethacin, which is a mandatory criterion for their diagnosis. Are they really different headaches? To answer this question, we compared the pain characteristics and autonomic features between two headaches. We also determined whether paroxysmal hemicrania transforms into hemicrania continua or vice versa in their natural history. METHODS: The patients with hemicrania continua and paroxysmal hemicrania were compared for severity, location, character, and mean effective indomethacin dose. The number of autonomic features and their severity was also compared. The natural history of headache was looked into to see the evolution of hemicrania continua and paroxysmal hemicrania from episodic and chronic pains, respectively. RESULTS: We included 35 patients with hemicrania continua and 27 patients with paroxysmal hemicrania from July 2015 to March 2017. The mean age of patients with paroxysmal hemicrania was 34.42 years, and hemicrania continua was 37 years. Both groups were similar for majority of pain characteristics and number/severity of autonomic features. However, paroxysmal hemicrania had higher pain severity. Five patients transformed from paroxysmal hemicrania to hemicrania continua, and 3 patients transformed from hemicrania continua to paroxysmal hemicrania. CONCLUSION: Paroxysmal hemicrania and hemicrania continua were similar on majority of pain characteristics and autonomic features. The paroxysmal hemicrania and hemicrania continua are not exclusive headaches and can transform into each other.

Primary Headache Disorders Part I- Migraine and the Trigeminal Autonomic Cephalalgias.

In Primary Headache Disorders, Part 1, we discuss three of the primary headache disorders using the headache definitions from ICHD-III (Beta): Migraine, with and without aura; its pathophysiology and treatment are discussed. We then discuss the Trigeminal Autonomic Cephalalgias (TACs), including Cluster Headache and Hemicrania Continua, two more primary headache disorders, as well as the other TAC Headaches. We discuss pathophysiology as well as diagnosis, treatment, and pharmacotherapeutic management of these headache diatheses.

[Trigemino-autonomous headaches (TAH)--conservative and interventional options]. / Trigeminoautonome Kopfschmerzen (TAK)-- konservative und operative Therapieverfahren.

Trigemino-autonomous headaches (TAH) are relatively rare, thus physicians are not familiar with the diagnostic and therapeutic procedures. In terms of their intensity and duration, they differ distinctly from other primary headaches such as migraine or tension-type headache. Nevertheless, patients experience a significant psychosocial burden and therefore economic consequences, i.e. direct and indirect costs. Fast diagnosis and optimised treatment should lead to significant improvements. This article aims to offer advice by detailing appropriate classification systems and guideline-based treatment strategies.

[Therapy of trigeminal autonomic headaches]. / Therapie des trigeminoautonomen Kopfschmerzes.

Trigeminal autonomic cephalgias (TAC) are characterized by severe and strictly unilateral headaches with a frontotemporal and periorbital preponderance in combination with ipsilateral cranial autonomic symptoms, such as lacrimation, conjunctival injection, rhinorrhea, nasal congestion, and restlessness or agitation. One main differentiating factor is the duration of painful attacks. While attacks typically last 5 s to 10 min in SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing), paroxysmal hemicrania lasts 2-30 min and cluster headaches 15-180 min. Hemicrania continua represents a continuous TAC variant. From a therapeutic view, TACs differ substantially. Lamotrigine is used as first-choice prevention in SUNCT syndrome and indometacin in paroxysmal hemicrania. For cluster headaches, acute therapy with inhaled pure oxygen and fast-acting triptans (sumatriptan s.c. and intranasal zolmitriptan) is equally important to short-term preventive therapy with methysergide and cortisone and long-term prophylactic treatment comprising verapamil as drug of first choice and lithium carbonate and topiramate as drugs of second choice. In refractory cases of chronic cluster headache, neuromodulatory approaches such as occipital nerve stimulation and sphenopalatine ganglion stimulation are increasingly applied.

Strictly unilateral headaches: considerations of a clinician.

The aim of the lecture is to draw attention to the role that clinical practice and clinical observation have had in stimulating research on the pathophysiology of cluster headache (CH) and other trigeminal autonomic cephalalgias (TACs). The symptoms of cluster headache-in particular the typical circadian periodicity of the headaches and the seasonal recurrence of cluster periods-were fundamental in shifting attention away from peripheral pathogenetic hypotheses to the idea that cluster headache could have a central origin. Initially, solid neuroendocrinological data pointed to hypothalamic involvement. For example, CH patients were shown to have alterations in biorhythms. Subsequently, modern functional neuroimaging techniques were able to demonstrate that the homolateral posterior hypothalamus is activated during TAC headaches, so implicating this region in TAC pathogenesis. It is known that the hypothalamus has a modulatory effect on nociceptive and autonomic pathways, particularly on the nociceptive trigeminovascular system. Future research should clarify whether the hypothalamus is the generator of TAC headaches, or whether it is activated in response to an alteration of the homeostatic equilibrium between limbic emotional-affective components and autonomic-nociceptive components modulated by the hypothalamus.

Evaluation and management of "sinus headache" in the otolaryngology practice.

Patients, primary care doctors, neurologists and otolaryngologists often have differing views on what is truly causing headache in the sinonasal region. This review discusses common primary headache diagnoses that can masquerade as "sinus headache" or "rhinogenic headache," such as migraine, trigeminal neuralgia, tension-type headache, temporomandibular joint dysfunction, giant cell arteritis (also known as temporal arteritis) and medication overuse headache, as well as the trigeminal autonomic cephalalgias, including cluster headache, paroxysmal hemicrania, and hemicrania continua. Diagnostic criteria are discussed and evidence outlined that allows physicians to make better clinical diagnoses and point patients toward better treatment options.

Concomitant occurrence of different trigeminal autonomic cephalalgias: a case series and review of the literature.

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.

[Recent pathophysiology of cluster headache (trigeminal autonomic cephalalgias; TACs)].

BACKGROUND: Cluster headache (CH), known as one of the trigeminal autonomic cephalalgias, is a stereotyped primary pain syndrome characterized by unilateral severe pain, the pathophysiology of which are not well understood. PATHOPHYSIOLOGY: The underlying pathophysiology of CH is incompletely understood. The periodicity of the attacks suggests the involvement of a biologic clock within the hypothalamus which controls circadian rhythms, with central disinhibition of the nociceptive and autonomic pathways, the trigeminal nociceptive pathways. Positron emission tomography and voxel-based morphometry have identified the posterior hypothalamic gray matter as the key area for the basic defect in CH. Functional hypothalamic dysfunction has been confirmed by abnormal metabolism based on the N-acetylaspartate neuronal marker in magnetic resonance spectroscopy. A recent case study demonstrated the release of both trigeminal and parasympathetic neuropeptides during a bout of pain in the same pattern previously described in CH. It is hypothesis that trigeminal activation leads to reflex autonomic activation. At a clinical level, there should be a pain threshold above which autonomic symptoms occur, modified by the highly somato- topic and functionally organized central connections of the trigeminovascular system.

[Update on trigeminal autonomic cephalalgia]. / Neues zu trigeminoautonomen Kopfschmerzen.

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.

Sistema trigémino vascular y cefalea / Trigeminovascular System and Primary Headache

La fisiopatología de las cefaleas primarias es compleja e incluye un sinnúmerode interacciones que regulan el proceso nociceptivo. Dentro de los principalesresponsables de generar el dolor se encuentra el sistema trigémino vascular, que esun conjunto de estructuras que integran vías tanto centrales corticosubcorticales comoperiféricas, que desempeñan un papel activo no solo en la génesis del dolor, sino enlas manifestaciones autonómicas y visuales que acompañan la cefalea. Así mismo, estesistema es el responsable de los mecanismos de sensibilización central característicosdel dolor. En el artículo se desarrollan brevemente las principales estructuras queparticipan en la génesis de las cefaleas primarias y sus interacciones en las diferentespartes del sistema nervioso...

The pathophysiology of primary headache iscomplex and it includes several interactionsthat regulate the nociceptive process. The trigeminal-vascular system is perhaps one of theprincipal structures that generate pain due tothe integration of several pathways both centraland peripheral. In addition to this, the trigeminalvascular system also plays a central role inthe autonomic and visual symptoms that affectindividuals with headache and in the centralsensitization process. In this article we brieflydiscuss the main structures that participate in thepathophysiology of primary headaches and theirinteractions in the different levels of the centralnervous system...

[Trigeminal autonomic cephalgias - new aspects and concepts]. / Trigemino-autonome Kopfschmerzen - neue Aspekte und Konzepte.

Cluster headache, paroxysmal hemicrania and SUNCT syndrome are a group of primary headaches classified as "trigeminal autonomic cephalgias" (TACs) and characterised by relatively short attacks of unilateral head pain associated with ipsilateral craniofacial autonomic manifestations. Duration of attacks is the main feature to distinguish between the three forms of TACs. Modern functional neuroimaging indicates that the hypothalamus may play a crucial role in the pathophysiology of these headaches. Therapeutic regimes consist of acute treatment as well as application of prophylactic medication. After unsuccessful application of conservative treatment options, invasive neuromodulative procedures are finally justified. These treatments should be rather of neurostimulative than of neurodestructive intentions.

Trigeminal autonomic cephalalgias.

PURPOSE OF REVIEW: This article covers the clinical manifestations and differential diagnosis of the trigeminal autonomic cephalalgias (TACs). RECENT FINDINGS: TACs comprise a subgroup of primary headache disorders presenting with lateralized, often severe, pain accompanied by cranial autonomic features. The key syndromes are cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)/short-lasting unilateral neuralgiform headache attacks with cranial autonomic features (SUNA), and hemicrania continua. Lateralization of symptoms and signs is the key feature differentiating the TACs and migraine. When diagnosing a TAC, it is appropriate to consider underlying pituitary or pituitary region pathology. Cluster headache responds to oxygen and parenteral triptans, with verapamil having the most success for prevention. Paroxysmal hemicrania responds to indomethacin. SUNCT/SUNA responds to lamotrigine and topiramate. Hemicrania continua responds to indomethacin. SUMMARY: TACs are a unique group of primary headache syndromes with individual features and specific responses to treatment that make their identification crucial for optimum management.

Headache.

Headache is one of the most common complaints among patients presenting to an outpatient neurology practice. The evaluation, diagnosis, and treatment of headache can be rather cumbersome and at times quite challenging for even the most seasoned neurologist. Many complex issues that although not causative, can play an exacerbating role in the genesis of headaches. In this article, the authors review some of the essential elements that are part of headache evaluation including headache-specific history, physical examination, warning signs of secondary headache disorders, and when to consider further studies. They then provide a brief review on the diagnosis of primary headache disorders according to the International Headache Society's International Classification of Headache Disorders, 2nd Edition (ICHD-2), and treatment strategies of the more common primary headache disorders with a focus on migraine, trigeminal autonomic cephalalgias, tension-type headache, and chronic daily headache.

Characteristics of the first 1000 headaches in an outpatient headache clinic registry.

OBJECTIVE: To analyze the incidence and characteristics of the first 1000 headaches in an outpatient clinic. BACKGROUND: Headache is a common cause of medical consultation, both in primary care and in specialist neurology outpatient clinics. The International Classification of Headache Disorders, 2nd Edition (ICHD-II), enables headaches to be classified in a precise and reproducible manner. METHODS: In January 2008, an outpatient headache clinic was set up in Hospital Clínico Universitario, a tertiary hospital in Valladolid, Spain. Headaches were classified prospectively in accordance with ICHD-II criteria. In each case we recorded age and sex, duration of headache, ancillary tests required, and previous symptomatic or prophylactic therapies. RESULTS: In January 2010, the registry included 1000 headaches in 682 patients. The women/men ratio was 2.46/1 and the mean age of the patients was 43.19 ± 17.1 years (range: 14-94 years). Patients were referred from primary care (53.4%), general neurology clinics (36.6%), and other specialist clinics (9%). The headaches were grouped (ICHD-II classification) as follows: group 1 (Migraine), 51.4%; group 2 (Tension-type headache), 16%; group 3 (Trigeminal autonomic cephalalgias), 2.6%; group 4 (Other primary headaches) and group 13 (Cranial neuralgias), 3.4%. The diagnostic criteria of chronic migraine were satisfied in 8.5% of migraines. Regarding secondary headaches, 1.1% of all cases were included in group 5 (Headaches attributed to trauma) and 8.3% in group 8 (Headaches attributed to a substance or its withdrawal). Only 3.4% of headaches were classified in group 14 (Unspecified or not elsewhere classified), and 5.2% were included in the groups listed in the ICHD-II research appendix. CONCLUSION: This registry outlines the characteristics of patients seen in an outpatient headache clinic in a tertiary hospital; our results are similar to those previously reported for this type of outpatient clinic. Migraine was the most common diagnosis. Most headaches can be classified using ICHD-II criteria.

Cefaléias inusitadas primárias reconhecidas pela sociedade internacional de cefaléias: revisão breve / Primary unsual headaches recognized by the international headaches society: brief review

As cefaléias são dores que assumem características clínicas, genéticas, fisiopatológicas e terapêuticas que as distinguem fundamentalmente das demais dores do ser humano. Dividem-se entre dores primárias ? as mais freqüentes ? e secundárias, de acordo com a presença ou não de desordens causadoras subjacentes. Nesta revisão salientamos os principais aspectos das cefaléias raras listadas pela Classificação Internacional das Cefaléias e que incluem: a hemicrania paroxística, SUNCT/SUNA, cefaléia primária em facadas, cefaléia primária da tosse, cefaléia primária do exercício, cefaléia primária associada à atividade sexual, cefaléia hípnica, cefaléia em trovoada, hemicrania contínua e a cefaléia nova diária e persistente. O diagnóstico depende da colheita de uma história cuidadosa e atenta, posto que reside no quadro clínico a chave para o diagnóstico diferencial.

Headaches are conditions fundamentally distinct from pain elsewhere due to clinical, genetic, pathophysiological and therapeutic reasons. They are first and foremost divided into primary - much more common - and secondary diseases according to the presence of underlying causes. In this short review, we highlight the most characteristic of the relatively rare primary headaches listed in the HIS Cassification of Headache Disorders, including proxysmal hemicrania, SUNCT/SUNA, pimary stabbing headache, pimary cough headache, pimary exertional headache, pimary sexual headache, hpnic headache, rimary thunderclap headache, hemicrania continua and new daily persistent headache. Diagnosis depends on a comprehensive case history, as the clue for differentiation among such conditions reside on clinical grounds.

Is hemicrania continua a single entity or the association of two headache forms? Considerations from a case report.

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.

Sluder's neuralgia: a trigeminal autonomic cephalalgia?

The objective was to formulate distinctive criteria to substantiate our opinion that Sluder's neuralgia and cluster headache are two different clinical entities. A systematic review was carried out of all available, original literature on Sluder's neuralgia. Pain characteristics, periodicity and associated signs and symptoms were studied and listed according to frequency of appearance. Eleven articles on Sluder's neuralgia were evaluated. Several differences between Sluder's neuralgia and cluster headache became evident. Based on described symptoms, new criteria for Sluder's neuralgia could be formulated. Sluder's neuralgia and cluster headache could possibly be regarded as two different headache syndromes, and Sluder's neuralgia could be a trigeminal autonomic cephalalgia.

Symptomatic trigeminal autonomic cephalalgias.

BACKGROUND: The trigeminal autonomic cephalalgias (TACs) are a group of primary headache syndromes characterized by strictly unilateral head pain that occurs in association with ipsilateral cranial autonomic features. The group includes cluster headache, paroxysmal hemicrania, and short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. These syndromes differ in attack duration and frequency as well as the response to therapy. Most of the cases of these syndromes are primary headaches, though numerous symptomatic cases have been described albeit that it is difficult to establish a causal relationship with the underlying pathology in most cases. REVIEW SUMMARY: We reviewed the literature to identify the cases of symptomatic TACs that were likely to be secondary to the reported underlying lesion. We also attempted to identify any clinical features that may be pointers for distinguishing these cases from primary cases and thereby inform the diagnostic workup of these disorders. CONCLUSION: Forty cases of symptomatic TACs were identified. These symptomatic headaches were associated with atypical phenotypes, abnormal examination, and poor treatment response though a significant minority had a typical presentation. A relatively high proportion of all TACs were secondary to pituitary tumors. It is difficult to draw up guidelines for the diagnostic workup required on the basis of this small retrospective case series. It remains unclear whether every TAC patient requires neuroimaging, though if it is considered then magnetic resonance imaging is the preferred modality. In addition, we suggest that all TAC patients should be carefully assessed for pituitary disease related symptoms but further investigations with magnetic resonance imaging of the pituitary gland and pituitary hormonal profile should only be undertaken in patients with atypical features, abnormal examination, or those resistant to the appropriate medical treatment.

[Trigeminal autonomic cephalgias. After initial diagnosis, consider secondary causes]. / Trigeminoautonome Zephalgien. Bei Erstdiagnose auf sekundäre Formen achten.

Trigeminal autonomic cephalgias (TAC) are classified as primary headache syndromes. The use of instrumental procedures including neuroimaging in the diagnostic workup of the TACs is controversially discussed in the literature. Several case reports have been previously published, reporting trigeminal autonomic cephalgias related to structural lesions. We contribute two of our own cases of symptomatic TACs and demonstrate that a "classic" clinical presentation does not preclude a symptomatic etiology. Thus, we advocate a systematic diagnostic evaluation including neuroimaging in every patient presenting with symptoms indicative of TAC for the first time.