Different forms of trigeminal autonomic cephalalgias in the same patient: description of a case

The trigeminal autonomic cephalalgias (TACs), including cluster headache, paroxysmal hemicrania and SUNCT, are characterized by the cardinal combination of short-lasting unilateral pain and autonomic phenomena affecting the head. Hemicrania continua (HC) shares many clinical characteristics with TACs, including unilateral pain and ipsilateral autonomic features. Nevertheless, HC is separately classified in the revised International Classification of Headache Disorders (ICHD-II). Here, we describe the case of a 45-year-old man presenting an unusual concurrence of different forms of primary headaches associated with autonomic signs, including subsequently ipsilateral cluster headache, SUNCT and HC. This report supports the theory that common mechanisms could be involved in pathophysiology of different primary headache syndromes.     

Chronic cluster-like headache secondary to prolactinoma: uncommon cephalalgia in association with brain tumors

Headache is a common and disabling aspect of pituitary disease. Chronic and episodic migraine are the most common clinical syndromes of headaches related to pituitary tumors, although other types of headache, such as trigeminal autonomic cephalalgias (TACs), can also be present. TACs include short-lasting, unilateral neuralgiform headache attacks with conjunctival injection and tearing; paroxysmal hemicrania; and cluster headache. We report on a patient with a chronic cluster-like headache associated with a macroprolactinoma. Although cabergoline, pregabalin, and corticosteroids were not effective as preventive treatments, high-dose verapamil showed good efficacy. Morphine and octreotide were efficacious as abortive treatments for attacks, but pain was only partially responsive to oxygen and refractory to subcutaneous sumatriptan.     

Diagnosis and Clinical Features of Trigemino‐Autonomic Headaches

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.     

Side-shifting hemicrania continua with aura (migraine with aura with autonomic symptoms responsive to indomethacin?)

Atypical features of hemicrania continua (HC), including both visual aura and side shifting, have been reported previously. However, auras and variable unilaterality have never been reported together in HC. We report two patients with side-shifting HC with aura. These patients' symptoms are unilateral headaches, visual aura, autonomic features, throbbing pain, nausea and photo/phonophobia. One could speculate that the unilaterality and/or the autonomic symptom modules are indomethacin responsive. The patients can also be classified as chronic migraine with aura, with autonomic symptoms, responsive to indomethacin. Neither migraine subtype nor side-shifting HC with aura is included in the current International Headache Society (IHS) classification, so these patients are not classifiable. Side-shifting HC with aura implies the need to revisit the traditional IHS categorization of headaches into unique diagnostic groups. The modular headache theory may be a tool for the understanding of these rare and complex cases.     

Dental presentations of cluster headaches

Cluster headache has been defined by the International Headache Society (IHS) as one of the primary headaches. A primary headache is a headache that has no other known cause, such as infection or trauma. Cluster headache is also listed as one of the trigeminal autonomic cephalalgias. These headaches are mediated by the trigeminal nerve with accompanying autonomic symptoms that may range from conjunctival injection, lacrimation, nasal congestion, rhinorrhea, forehead and facial sweating, miosis, and ptosis to eyelid edema. The IHS has described cluster headache as "attacks of severe, strictly unilateral pain that is orbital, supraorbital, temporal or in any combination of these sites, lasting 15 to 180 minutes." In the author’s practice, as a dentist treating orofacial pain, patients with cluster headache have dental or midfacial complaints as a primary presentation. This paper introduces such presentations based on interviews with cluster headache patients, with the main purpose of having midfacial complaints considered as an important presentation to be added to the IHS diagnostic criteria for cluster headache.     

Headache in Sports

Headache, a common and disabling symptom in Behçet’s syndrome, may be associated with a variety of neurologic syndromes and ocular inflammation, or may present as an isolated feature. Our objective is to describe the various neurologic and ocular syndromes of Behçet’s syndrome of which headache is a symptom, and to review the features of isolated headaches in Behçet’s. We also report results of a study of headache in Behçet's syndrome patients who are followed at NYU Hospital for Joint Diseases, the first study of its kind in North American patients, and the first to document prevalence of both episodic and chronic daily headache in Behçet’s.     

Stabbing Headache as the Presenting Manifestation of Intracranial Meningioma: A Report of Two Patients

Stabbing headache may occur as a primary headache disorder, may accompany other primary headaches, or, in rare instances, may be associated with secondary headache syndromes. We report two patients with intracranial meningioma in whom stabbing headache was the heralding symptom. Headache remitted promptly following surgical resection. These cases stress the importance of excluding underlying pathology in patients presenting with new-onset stabbing headache.     

Headache Diagnosis in Children and Adolescents

Purpose of Review

Headache phenotypes can differ between adults and children. While most headaches are due to primary headache disorders, in a small population, they can be an indication of a potentially life-threatening neurologic condition. The challenge lies in identifying warning signs that warrant further workup. This article reviews different types of pediatric headaches and headache evaluation in children and teens, and focuses on the approach for diagnosis of secondary headaches.

Recent Findings

Common thought is that increased frequency and severity of headache may reflect secondary pathology; however, headache phenotype may not be fully developed and can evolve in adolescence or adulthood. Headache location, particularly occipital headache alone, does not necessarily signify secondary intracranial pathology. Certain warning signs warrant neuroimaging, but others only warrant imaging in certain clinical contexts. Brain MRI is the neuroimaging modality of choice, though there is a high rate of incidental findings and often does not change headache management.

Summary

A stepwise approach is essential to avoid missing secondary headaches. There are several differences between adults and children in clinical manifestations of headache. Evaluation and diagnosis of pediatric headache starts with a thorough headache and medical history, family and social history, and identification of risk factors. A thorough physical and neurologic exam is important, with close attention to features that could suggest secondary headache pathology. Neuroimaging and other testing should only be performed if there is concern for secondary headache.

     

Secondary headache in children

Headache is one of the common symptoms of fever and headache without fever is also not rare in general pediatric clinics. The common causes of these headaches involve extracranial infection due to viral illness, migraine and trauma. Headache with vomiting, fever and meningeal signs suggests meningitis. Taking blood pressure is necessary, even though headache caused by hypertension is rare in children. Neuroimaging should be performed with abnormal neurological findings, atypical headache pattern, or significant change of preexisting headache. Serious underlying diseases, such as brain tumor or intracranial hemorrhage, are uncommon, however they should be diagnosed immediately using neuroimaging because of their urgency.     

Therapie und Prophylaxe von Cluster-Kopfschmerzen und anderen trigemino-autonomen Kopfschmerzen

Following the new IHS classification, cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT syndrome) are included in the classification as trigeminal autonomic cephalgias (TAC). The similarities of these syndromes suggest a considerable shared pathophysiology. These syndromes have in common that they involve activation of trigeminovascular nociceptive pathways with reflex cranial autonomic activation. Clinically, this physiology predicts pain with some combination of lacrimation, conjunctival injection, nasal congestion, or eyelid edema. Broadly the management of TAC comprises acute and prophylactic treatment. Some types of trigeminal autonomic headaches such as paroxysmal hemicrania and hemicrania continua have, unlike cluster headaches, a very robust response to indomethacin, leading to a consideration of indomethacin-sensitive headaches. This review covers the clinical picture and therapeutic options. Although studies following the criteria of evidence-based medicine (EBM) are rare, most patients can be treated sufficiently.     

A case of short–lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT). Discussion of clinical features and differential diagnosis

Chronic short–lasting headaches, in which trigeminal autonomic cephalalgias (TACs) are included, are relatively rare syndromes and not always well recognised. We present a case highly suggestive of short–lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and we try to affirm essential points to distinguish the diagnosis from other TACs and chronic short–lasting headaches. We conclude that the qualifying points for differential diagnosis are number and duration of attacks in a day, presence of autonomic features and lack of indomethacin effect.     

Case report on a patient with SUNCT-syndrome

Case report on a patient with SUNCT-syndrome (short lasting, unilateral neuralgiform headache attacks with conjunctival injection, sweating, and rhinorrhoea) who was successfully treated with gabapentin. SUNCT, a still relatively unknown headache syndrome, is characterized by attacks of periorbital pain with accompanying ipsilateral autonomic symptoms. Along with this case report the differences of SUNCT to similar headaches are emphasized. Due to clear diagnostic criteria the inclusion of SUNCT in the IHS classification (International Headache Society) as a separate clinical entity should be favoured.     

Therapy of primary chronic headache: chronic migraine, chronic tension type headache and other forms of daily chronic headache

The criteria of the International Headache Society (IHS) define four different primary headache syndromes with daily chronic headaches: chronic migraine, episodic and chronic tension type headache, hemicrania continua, new daily persisting headache. A further important differential diagnosis is medication overuse headache (previously known as analgesia headache). The German, Austrian, and Swiss headache societies now present the first joint guidelines for therapy of these headache syndromes. The current literature was reviewed and a summary is presented. The therapy recommendations do not only include the scientific evidence but also the practical relevance.     

The features and burden of headaches within a chiropractic clinical population: A cross-sectional analysis

ObjectivesThe aim of this study is to a) investigate the headache features and level of headache severity, chronicity, and disability found within a chiropractic patient population and b) to ascertain if patient satisfaction with headache management by chiropractors is associated with headache group or reason for consulting a chiropractor.Design and settingConsecutive adult patients with a chief complaint of headache participated in an online cross-sectional survey (n = 224). Recruitment was via a randomly selected sample of Australian chiropractors (n = 70). Headache features were assessed using International Classification of Headache Disorders criteria and level of headache disability measured using the Headache Impact Test instrument.ResultsOne in four participants (n = 57; 25.4%) experienced chronic headaches and 42.0% (n = 88) experienced severe headache pain. In terms of headache features, 20.5% (n = 46) and 16.5% (n = 37) of participants had discrete features of migraine and tension-type headache, respectively, while 33.0% (n = 74) had features of more than one headache type. ‘Severe’ levels of headache impact were most often reported in those with features of mixed headache (n = 47; 65.3%) and migraine (n = 29; 61.7%). Patients who were satisfied or very satisfied with headache management by a chiropractor were those who were seeking help with headache-related stress or to be more in control of their headaches.ConclusionMany with headache who consult chiropractors have features of recurrent headaches and experience increased levels of headache disability. These findings may be important to other headache-related healthcare providers and policymakers in their endeavours to provide coordinated, safe and effective care for those with headaches.     

Sinus headache: a neurology, otolaryngology, allergy, and primary care consensus on diagnosis and treatment

Sinus headache is a widely accepted clinical diagnosis, although many medical specialists consider it an uncommon cause of recurrent headaches. The inappropriate diagnosis of sinus headache can lead to unnecessary diagnostic studies, surgical interventions, and medical treatments. Both the International Headache Society and the American Academy of Otolaryngology-Head and Neck Surgery have attempted to define conditions that lead to headaches of rhinogenic origin but have done so from different perspectives and in isolation of each other. An interdisciplinary ad hoc committee convened to discuss the role of sinus disease as a cause of headache and to review recent epidemiological studies that suggest sinus headache (headache of rhinogenic origin) and migraine are frequently confused with one another. This committee reviewed available scientific evidence from multiple disciplines and concluded that considerable research and clinical study are required to further understand and delineate the role of nasal pathology and autonomic activation in migraine and headaches of rhinogenic origin. However, this group agreed that greater diagnostic and therapeutic attention needs to be given to patients with sinus headaches.     

Neuroimaging in cluster headache and other trigeminal autonomic cephalalgias

The central nervous system mechanisms involved in trigeminal autonomic cephalalgias, a group of primary headaches characterized by strictly unilateral head pain that occurs in association with ipsilateral craniofacial autonomic features, are still not comprehensively understood. However, functional imaging methods have revolutionized our understanding of mechanisms involved in these primary headache syndromes. The present review provides a brief overview of the major modern functional neuroimaging techniques used to examine brain structure, biochemistry, metabolic state, and functional capacity. The available functional neuroimaging data in cluster headache and other TACs will thus be summarized. Although the precise brain structures responsible for these primary headache syndromes still remain to be determined, neuroimaging data suggest a major role for posterior hypothalamus activation in initiating and maintaining attacks. Furthermore, pathophysiological involvement of the pain neuromatrix and of the central descending opiatergic pain control system was observed. Given the rapid advances in functional and structural neuroimaging methodologies, it can be expected that these non-invasive techniques will continue to improve our understanding into the nature of the brain dysfunction in cluster headache and other trigeminal autonomic cephalalgias.     

Headache Classification and Factor Analysis with a Pediatric Population

SYNOPSIS
The "Headache Symptom Questionnaire-Revised" was administered to 68 pediatric headache sufferers and their parents. The questionnaire as a brief assessment tool can accurately classify 66.2% of the child headache sufferers. This classification accuracy was found to be equivalent to the 67.2% diagnostic agreement between experienced diagnosticians utilizing clinical interviews. Factor analysis of the questionnaire revealed four factors-one being related to muscle contraction headaches, two factors being of a vascular nature, and the fourth factor related to both migraine and muscle contraction headache. This investigation, which utilized a population whose headaches are thought to be in a more "pure" state, continues to lend support to traditional notions of headache etiology and diagnosis.     

Chronic Daily Headache in Children and Adolescents:Current Status and Recommendations for the Future

The Pediatric Committee of the American Association for the Study of Headache was created in 1994 to develop a plan for comprehensively addressing global issues of headache in childhood. It was the impression of clinicians and researchers with an interest in childhood headaches that a clearer focus was needed to facilitate progress in the study and management of pediatric headache. It was further felt that approaches to treatment and outcomes, as well as assessment and classification schema for pediatric patients needed to be examined separately. The goal of the committee is to integrate anecdotal, clinical, and research expertise into a plan for addressing headaches in the pediatric population in the future. During the last 5 years, substantial attention has been devoted to chronic daily headache, primarily in adult populations. It is the purpose of this paper to review the literature of chronic daily headache in children, and propose areas for further exploration, given the recent emergence of interest in this diagnostic entity.     

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) with preserved refractory period: report of three cases

Background  

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform attacks with cranial autonomic features (SUNA) are rare primary headache syndromes characterized by spontaneous or triggered attacks of unilateral, brief, multiple, orbitofrontal pain associated with ipsilateral autonomic features. SUNCT is considered as a subset of SUNA. In SUNA, there may be cranial autonomic symptoms other than conjunctival injection and lacrimation, or either of two is present. SUNCT/SUNA can be triggered immediately after or at the decrescendo phase of the ongoing attack without any intervening refractory period. Refractory period is usually present in trigeminal neuralgia. Absent refractory period is thought to reliably differentiate SUNCT/SUNA from trigeminal neuralgia and has been proposed for inclusion into the International Classification of Headache Disorders (ICHD) diagnostic criteria for SUNCT.     

Refractory headache: classification and nomenclature

There are a number of reasons to attempt to define and classify refractory headache disorders. Particularly important are the potential benefits in the areas of research, treatment, and medical cost reimbursement. There are challenges in attempting to classify refractory forms of headaches, including the lack of biological or other objective markers and a lack of consensus among practitioners as to what qualifies as refractoriness, or even if a separate category for refractory migraine and other refractory headaches needs to be established. A definition of refractory migraine has been proposed by Schulman et al in this issue ("Defining Refractory Migraine [RM] and Refractory Chronic Migraine [RCM]: Proposed Criteria for the Refractory Headache Special Interests Section of the American Headache Society"), which should be tested for validity and usefulness. It seems reasonable to consider adding this defined syndrome to the International Classification of Headache Disorders, second edition (ICHD-II). In this article, options for adding refractory headache syndromes to the ICHD are discussed with pros and cons for each. Two "best" options for adding the disorder "refractory migraine" to the ICHD are presented along with an illustrative case example.