Complementary and Integrative Medicine for the Treatment of Trigeminal Neuralgia and Trigeminal Autonomic Cephalalgia.

PURPOSE OF REVIEW: Trigeminal neuralgia (TN) and trigeminal autonomic cephalalgias (TACs) are both painful diseases which directly impact the branches of the trigeminal nerve, which supply the face. Patients who have experienced adverse effects, have not responded to mainstream treatments, or have a personal preference for nonmedication options, often turn to complementary and integrative medicine (CIM). The aim of this review is to discuss the efficacy and safety of CIM therapies available for the treatment of TN and TACs. RECENT FINDINGS: Not only are there limited therapeutic options for TN and TAC patients, but also is there a proportion of patients who are intolerant to standard medical treatments. Recent findings have illustrated that 86% of patients with headache disorders utilize CIM modalities in combination with mainstream medical therapy. CIM modalities can be helpful for these diseases and have primarily been studied in combination with standard medical therapy. There is limited evidence for CIM and behavioral therapies in managing these conditions, and more research is needed to confirm which therapies are safe and effective.

Paroxysmal hemicrania and hemicrania continua: Review on pathophysiology, clinical features and treatment.

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.

[Trigeminal autonomic cephalgias: features of diagnosis and treatment]. / Trigeminal'nye avtonomnye tsefalgii: osobennosti diagnostiki i lecheniya.

Trigeminal autonomic cephalgias is a group of primary headaches, including cluster headache, paroxysmal hemicrania and hemicrania continua, as well as two forms of short- lasting unilateral neuralgiform headache attacks, the complexity of diagnosis of which is determined by the low prevalence and some similarity of clinical manifestations both among themselves and with other diseases in particular with migraine and trigeminal neuralgia. Despite the rather short duration of headache attacks, the intensity of the pain syndrome reaches a severe and very severe degree, and the high frequency of paroxysms per day significantly complicates abortion treatment and leads to a pronounced professional and social maladjustment. At the same time, the possibility of using effective specific prophylactic therapy determines the importance of accurate diagnosis and, therefore, the knowledge of neurologists on this issue.

Indomethacin-Induced Headache: Causing the Problem You Are Trying to Solve.

PURPOSE OF REVIEW: Indomethacin is an important medication in the headache medicine toolbox given its utility for both of the diagnosis and treatment of several primary headache disorders. Despite its prevalence in earlier rheumatologic studies, the possibility of drug-induced headache is a not commonly discussed in headache literature. RECENT FINDINGS: Herein, we describe a case of drug-induced headache after indomethacin trial for the treatment of an undifferentiated trigeminal autonomic cephalgia. Recognition of indomethacin-induced headache has important implications for patient education and interpreting the response to indomethacin when used both as a therapeutic and as a diagnostic tool.

Beyond chronic migraine: a systematic review and expert opinion on the off-label use of botulinum neurotoxin type-A in other primary headache disorders.

Introduction: Botulinum neurotoxin type-A (BoNTA) is licensed for the treatment of chronic migraine (CM), but it has been tested off-label as a therapeutic choice in other primary headaches (PHs). We aimed to provide a systematic review and expert opinion on BoNTA use in PHs, beyond CM.Areas covered: After providing an overview on PHs and mechanism of BoNTA action, we report the results of a systematic review, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) recommendations, of BoNTA therapeutic trials in PHs beyond CM. Studies and results were reviewed and discussed, and levels of evidence were graded. We also collected data on relevant ongoing trials.Expert opinion: Although there are contradictory findings on PHs other than CM, BoNTA may represent a therapeutic option for patients who do not respond to conventional prophylactic treatments. Based on limited available evidence, BoNTA may be considered in refractory tension-type headache, trigeminal autonomic cephalalgias, primary stabbing headache, nummular headache, hypnic headache, and new daily persistent headache, after the primary nature of cephalalgia has been documented and other drugs have failed. Experienced physicians in BoNTA treatment are required to guide the therapeutic protocol for each patient to optimize good and safe outcomes.

Management of cluster headache and other trigeminal autonomic cephalalgias in pregnancy and breastfeeding.

Many clinicians lack experience in managing trigeminal autonomic cephalalgias (TACs) in pregnancy and lactation. In addition to cluster headache, TACs include hemicrania continua, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing/autonomic symptoms (SUNCT/SUNA). Treating these rare, severe headache conditions often requires off-label drugs that have uncertain teratogenic potential. In the last few years, several new treatment options and safety documentation have emerged, but clinical guidelines are lacking. This narrative review aimed to provide an updated clinical guide and good clinical practice recommendations for the management of these debilitating headache disorders in pregnancy and lactation.

Trigeminal Autonomic Cephalalgias.

The trigeminal autonomic cephalalgias (TACs) are a group of primary headache syndromes all marked by unilateral headache and ipsilateral cranial autonomic features. The TACs include cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing, and hemicrania continua. Pathophysiology includes the trigeminal pain system, autonomic system, hypothalamus, and more recently an identified role for the vagus nerve. Diagnosis is made after looking at headache frequency, duration, and accompanying symptoms. Each TAC has its own unique treatment, which is discussed in depth.

The Exacerbation of Hemicrania Continua Mimics Trigeminal Neuralgia.

We report the case of a 46-year-old man with hemicrania continua presenting as exacerbations mimicking trigeminal neuralgia. The patient was tentatively diagnosed with trigeminal neuralgia, and treatment with various combinations of drugs was performed after the onset of pain. However, when the condition of the patient did not improve, we suspected hemicrania continua, and treatment with indomethacin was initiated. There was a marked alleviation of his pain within 24 hours. Thus, clinicians should be aware that the duration and frequency of exacerbations of hemicrania continua are variable.

Hemicrania Continua: a Clinical Perspective on Diagnosis and Management.

PURPOSE OF REVIEW: Hemicrania Continua (HC) is a daily and persistent form of headache that is characterized by side-locked pain which is continuous, varies in severity and can be associated with conjunctival injection, lacrimation, nasal congestion, rhinorrhea, eyelid edema, forehead or facial sweating and miosis and/or ptosis. RECENT FINDINGS: Functional imaging studies have shown activation of subcortical structures such as the posterior hypothalamus and dorsal rostral pons, which are known to disinhibit the trigeminal autonomic reflex, a reflex responsible for autonomic outflow through trigeminal efferents. A similar pathway activation is seen in other Trigeminal autonomic cephalalgias (TAC) which solidifies HC as a TAC. While we also discuss promising treatments in our review, more evidence is needed before making them a standard of therapy for HC. This article aims to review the recent research on the diagnosis and clinical management of this potentially underdiagnosed primary headache disorder.

Treatment of disabling headache with greater occipital nerve injections in a large population of childhood and adolescent patients: a service evaluation.

BACKGROUND: Pediatric headache disorders can be extremely disabling, with marked reduction in the quality of life of children and their carers. Evidenced-based options for the treatment of primary headache disorders with preventive medication is limited and clinical outcomes are often unsatisfactory. Greater occipital nerve injections represent a rapid and well-tolerated therapeutic option, which is widely used in clinical practice in adults, and has previously shown a good outcome in a pediatric population. METHODS: This service evaluation reviewed greater occipital nerve injections performed unilaterally with 30 mg 1% lidocaine and 40 mg methylprednisolone, to treat disabling headache disorders in children and adolescents. RESULTS: We analyzed a total of 159 patients who received 380 injections. Of the population, 79% had chronic migraine, 14% new daily persistent headache, 4% a trigeminal autonomic cephalalgia, 3% secondary headache and one patient had chronic tension-type headache. An improvement after injection was seen in 66% (n = 105) of subjects, lasting on average 9 ± 4 weeks. Improvement was seen in 68% of patients with chronic migraine, 67% with a trigeminal autonomic cephalalgia and 59% with new daily persistent headache. Side effects were reported in 8% and were mild and transient. Older age, female gender, chronic migraine, increased number of past preventive use, medication overuse and developing side effects were all associated with an increased likelihood of positive treatment outcome. CONCLUSIONS: This large single centre service evaluation confirms that unilateral injection of the greater occipital nerve is a safe, rapid-onset and effective treatment strategy in disabling headache disorders in children, with a range of diagnoses and severity of the condition, and with minimal side effects.

Therapeutical approaches to paroxysmal hemicrania, hemicrania continua and short lasting unilateral neuralgiform headache attacks: a critical appraisal.

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.

LASH: A Review of the Current Literature.

PURPOSE OF REVIEW: The purpose of this review is to evaluate and explain our current understanding of a very rare disorder, long-lasting autonomic symptoms with associated hemicranias (LASH). RECENT FINDINGS: At present, there are four known cases in the literature of LASH. Its characteristics and reported response to indomethacin link it most closely to the trigeminal autonomic cephalalgias (TACs). Its pathophysiology and epidemiology remain unclear. Variance in the pain and autonomic symptom relationship in the existing TAC literature along with the reports of TAC sine headache suggests that LASH may represent a far end of the spectrum of TACs, with most similarities to paroxysmal hemicrania (PH) and hemicrania continua (HC).

[Headache News]. / Neues bei Kopfschmerzen.

A review of the latest and most relevant information on different disorders of head and facial pain is presented. News from epidemiologic studies regarding the relationship between migraine and patent foramen ovale, the cardiovascular risk in migraine, and migraine behavior during menopause, and the development of white matter lesions or migraine genetics are presented. Regarding pathophysiology there are very recent insights regarding the role of the hypothalamus during prodromal phase and the interplay of brain-stem and hypothalamus during the attack. In the last year studies and metaanalysis generated new knowledge for the use of triptans in general as in menstrual related migraine and hemiplegic variants. Furthermore, new hope rises for the CGRP (calcitonin-gene related peptide)-antagonists, as the data for ubrogepant do not suggest hepatotoxicity but efficacy. In prophylactic migraine treatment the news are manly on how the new therapeutic approach with monoclonal antibodies against CGRP or its receptor is moving on. Additional newly generated data for already known prophylactic agents as for new approaches are compactly discussed. Although main developments in headache focus on migraine new data on trigemino-autonomic headache trigeminal neuralgia and new daily persistant headache became available.

Two Cases of Hemicrania Continua-Trigeminal Neuralgia Syndrome: Expanding the Spectrum of Trigeminal Autonomic Cephalalgia-Tic (TAC-TIC) Syndrome.

BACKGROUND: Trigeminal neuralgia (TN) has been described in association with various primary headache disorders. So far, no case of TN has been reported in association with hemicrania continua (HC). CASE REPORT: Here, we report two patients of hemicrania continua associated with TN (HC-tic syndrome). These patients had both headaches concurrently. Both patients responded to a combination of carbamazepine and indomethacin. The skipping or tapering of carbamazepine led to the recurrence of the neuralgic pain of TN. In the same way, the skipping of indomethacin resulted in the relapse of the pain, typical of HC. CONCLUSION: With these two cases of HC-tic syndrome, we suggest that TN has a special predilection for all types of TACs. Various speculations suggest that such associations are more than a simple coincidence, and both diseases may be causally interrelated. The identification of this association is important as both disorders may need separate drugs.

Trigeminal autonomic cephalalgia as a presenting feature of Neuromyelitis Optica: "A rare combination of two uncommon disorders".

Neuromyelitis Optica (NMO) can have atypical presentations like hiccups, vomiting, etc. which is classically described as the area postrema syndrome. Here we report a case of a 39 year old male patient who presented with features of Trigeminal Autonomic Cephalalgia (TAC). MRI spine showed long segment myelitis. Diagnosis of NMO was confirmed by a positive Anti aquaporin 4 antibody assay. TACs are a rare group of headache disorders characterized by severe unilateral headache in the V1 distribution of the trigeminal nerve and autonomic symptoms. This presentation in NMO is hitherto unreported in literature.

Trigeminal autonomic cephalalgias.

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

The Neuropharmacology of Cluster Headache and other Trigeminal Autonomic Cephalalgias.

Trigeminal autonomic cephalalgias (TACs) are a group of primary headaches including cluster headache (CH), paroxysmal hemicrania (PH) and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT). Another form, hemicrania continua (HC), is also included this group due to its clinical and pathophysiological similarities. CH is the most common of these syndromes, the others being infrequent in the general population. The pathophysiology of the TACs has been partly elucidated by a number of recent neuroimaging studies, which implicate brain regions associated with nociception (pain matrix). In addition, the hypothalamic activation observed in the course of TAC attacks and the observed efficacy of hypothalamic neurostimulation in CH patients suggest that the hypothalamus is another key structure. Hypothalamic activation may indeed be involved in attack initiation, but it may also lead to a condition of central facilitation underlying the recurrence of pain episodes. The TACs share many pathophysiological features, but are characterised by differences in attack duration and frequency, and to some extent treatment response. Although alternative strategies for the TACs, especially CH, are now emerging (such as neurostimulation techniques), this review focuses on the available pharmacological treatments complying with the most recent guidelines. We discuss the clinical efficacy and tolerability of the currently used drugs. Due to the low frequency of most TACs, few randomised controlled trials have been conducted. The therapies of choice in CH continue to be the triptans and oxygen for acute treatment, and verapamil and lithium for prevention, but promising results have recently been obtained with novel modes of administration of the triptans and other agents, and several other treatments are currently under study. Indomethacin is extremely effective in PH and HC, while antiepileptic drugs (especially lamotrigine) appear to be increasingly useful in SUNCT. We highlight the need for appropriate studies investigating treatments for these rare, but lifelong and disabling conditions.

Hemicrania continua with contralateral cranial autonomic features: a case report.

BACKGROUND: Hemicrania continua is characterized by continuous strictly unilateral head pain with episodic exacerbations. Episodic exacerbations are associated with ipsilateral cranial autonomic features. CASE DESCRIPTION: We report a 24-year female with a 2-year history of continuous right-sided headache with superimposed exacerbations. Episodic exacerbations were associated with marked agitation and contralateral cranial autonomic features. The patient showed a complete response to indomethacin within 8 hours. DISCUSSION: The dichotomy of pain and autonomic features is in accordance with the concept about the possibility of two separate pathways for pain and autonomic features in trigeminal autonomic cephalalgias.