Herpes Zoster Ophthalmicus Initially Diagnosed As Cluster Headache, Complicated by Delayed Eruption.

Herpes zoster ophthalmicus (HZO) manifests as a consequence of the reactivation of the Varicella-zoster virus (VZV) and primarily affects the ophthalmic division of the trigeminal nerve. Identification of the vesicular eruption is central to the diagnostic process; however, the delayed manifestation of this cutaneous phenomenon poses a challenge to timely and accurate diagnosis. This report elucidates the case of a 61-year-old Japanese male with painful trigeminal neuropathy attributed to VZV that was initially diagnosed as cluster headache, mainly due to the delayed cutaneous eruption. Contrary to the expected pattern of cluster headache presentations, there was no discernible fluctuation in headache severity. The transient improvement of symptoms following interventions tailored for cluster headache management, including pure oxygen inhalation and subcutaneous sumatriptan injection, inadvertently contributed to a delay in accurate diagnosis. The importance of distinguishing HZO from cluster headache is emphasized, particularly in cases involving elderly patients or those with persistent cephalo-ophthalmalgia without the characteristic fluctuation of symptoms. In cases where clinical suspicion of HZO is raised, cerebrospinal fluid analysis should be performed. This approach is consistent with the overall goal of facilitating a prompt and accurate diagnosis.

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.

Cigarette smoking history (personal and secondary childhood exposure) in non-cluster headache trigeminal autonomic cephalalgias: A clinic based study.

OBJECTIVE: To look at cigarette smoking history (personal and secondary exposure as a child) in non-cluster headache trigeminal autonomic cephalalgias seen at a headache clinic and to determine smoking exposure prevalence utilizing previously published data. METHODS: Retrospective chart review and PubMed/Google Scholar search. RESULTS: Forty-eight clinic patients met ICHD-3 criteria for non-cluster headache trigeminal autonomic cephalalgias. Four had paroxysmal hemicrania, 75% were smokers and secondary exposure was noted in all. 16 patients had short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or short lasting unilateral neuralgiform headache attacks with autonomic symptoms (SUNA), 12.5% were smokers and secondary exposure was noted in 91%. Twenty-eight patients had hemicrania continua, 21% were smokers and secondary exposure was found in 62.5%.Since 1974 there have been 88 paroxysmal hemicrania, 50 SUNCT or SUNA and 89 hemicrania continua patients with a documented smoking exposure history. From current data and previous studies, a smoking history was noted in 60% paroxysmal hemicrania, 18% SUNCT and SUNA and 21% hemicrania continua patients. CONCLUSION: A cigarette smoking history appears to be connected to paroxysmal hemicrania (personal and secondary exposure) and possibly to SUNCT/SUNA (secondary) and hemicrania continua (secondary).

Hemicrania Continua: An Update.

PURPOSE OF REVIEW: Hemicrania Continua (HC) is a rare and disabling primary headache disorder that is characterized by persistent, unilateral headache with ipsilateral, cranial autonomic symptoms and restlessness or agitation. The diagnosis requires patients to experience an absolute response to therapeutic doses of indomethacin. RECENT FINDINGS: HC is diagnosed in in about 1.8% of adult patients who were evaluated for headache in tertiary care services, albeit this estimate should be interpreted with caution. The most prevalent accompanying symptoms appear to be lacrimation, conjunctival injection and restlessness or agitation. However, the available literature is limited by methodologic issues, and the current diagnostic criteria lack clarity on what defines absolute response to indomethacin. More rigorous studies are thus needed to improve our understanding of HC which, in turn, will facilitate better disease management in clinical practice. Here, we provide a comprehensive overview of HC, including its epidemiology, clinical presentation, diagnostic evaluation, and management.

The spectrum of indomethacin-responsive headaches in children and adolescents.

BACKGROUND: Headaches with marked, specific response to indomethacin occur in children, but the phenotypic spectrum of this phenomenon has not been well-studied. METHODS: We reviewed pediatric patients with headache showing ≥80% improvement with indomethacin, from seven academic medical centers. RESULTS: We included 32 pediatric patients (16 females). Mean headache onset age was 10.9 y (range 2-16 y). Headache syndromes included hemicrania continua (n = 13), paroxysmal hemicrania (n = 10), primary stabbing headache (n = 2), short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (n = 1), primary exercise headache (n = 1) and primary cough headache (n = 1). Adverse events were reported in 13, most commonly gastrointestinal symptoms, which often improved with co-administration of gastro-protective agents. CONCLUSION: Indomethacin-responsive headaches occur in children and adolescents, and include headache syndromes, such as primary cough headache, previously thought to present only in adulthood. The incidence of adverse events is high, and patients must be co-treated with a gastroprotective agent.

Co-morbidity between trigeminal autonomic cephalalgias and complex regional pain syndrome: Two case reports.

BACKGROUND: Trigeminal autonomic cephalalgias and complex regional pain syndrome are rare conditions, and their co-occurrence has not been reported previously.Clinical findings: In two patients, ipsilateral trigeminal autonomic cephalalgias developed after the onset of upper limb complex regional pain syndrome. Hyperalgesia to thermal and mechanical stimuli extended beyond the affected limb to encompass the ipsilateral forehead, and was accompanied by ipsilateral hyperacusis and photophobia. In addition, examination of the painful limb and bright light appeared to aggravate symptoms of trigeminal autonomic cephalalgias. Detailed examination of the association between facial and upper limb pain indicated that both sources of pain cycled together. Furthermore, in one case, stellate ganglion blockade inhibited pain for an extended period not only in the affected limb but also the face. CONCLUSIONS: These findings suggest some overlap in the pathophysiology of complex regional pain syndrome and trigeminal autonomic cephalalgias. Specifically, central sensitization and/or disruption of inhibitory pain modulation on the affected side of the body in complex regional pain syndrome might trigger ipsilateral cranial symptoms and increase vulnerability to trigeminal autonomic cephalalgias.

Indomethacin has no effect on trigeminally provoked parasympathetic output.

BACKGROUND: Unlike other non-steroidal anti-inflammatory drugs, indomethacin has been shown to be highly effective in two forms of trigeminal autonomic cephalalgias, hemicrania continua and paroxysmal hemicrania and in some forms of idiopathic stabbing headaches. This specificity is unique in the headache field. Previous findings suggest the involvement of the trigeminal autonomic reflex to play an important role in the pathophysiology of these diseases. METHODS: 22 healthy participants were enrolled in a double-blind, three-day within-subject design. The participants received indomethacin, ibuprofen or placebo in a randomized order. After an incubation period of 65 min the baseline lacrimation and the lacrimation during intranasal stimulation evoked by kinetic oscillation stimulation were assessed using Schirmer II lacrimation tests. The lacrimation difference in mm was calculated and compared in a repeated measures ANOVA. RESULTS: No significant differences were found between the three conditions. CONCLUSION: In our study, neither indomethacin nor ibuprofen had an inhibitory effect on the trigeminal autonomic reflex. We suggest that blocking this reflex may not be the treatment mechanism of indomethacin.

Hemicrania continua secondary to neurogenic paravertebral tumor- a case report.

OBJECTIVES: Hemicrania continua (HC) is one of the trigeminal autonomic cephalalgias (TAC), where sympathetic dysfunction and autonomic dysfunction resulting in parasympathetic over activation with some evidence of sympathetic inhibition have been suggested as probable causes. However, cases of hemicrania continua secondary to sympathetic dysfunction due to neurogenic paravertebral tumor impinging on the sympathetic chain has not been previously reported. In this case, the probability of the sympathetic dysfunction was more likely based on the clinical features and management. CASE PRESENTATION: A 23-year-old female presented with a chief complaint of right unilateral pain in the retro-bulbar, head and facial region for the past three years. An initial MRI of the brain was negative, whereas an MRI of the spine was advised to rule out a cervicogenic origin of the pain. The MRI revealed a well-defined mass lesion within right paravertebral region at T3 indicative of a neurogenic tumor. The patient was diagnosed with probable hemicrania continua secondary to neurogenic tumor impinging on adjacent sympathetic chain. A trial of indomethacin 75 mg/day was advised, which provided complete relief of the headache. The patient was referred to a neurologist for management of the neurogenic tumor. CONCLUSIONS: Headache disorders may be secondary to pathologies and comprehensive evaluation and accurate diagnosis are essential. Knowledge of neuroanatomy is paramount to understand and explain underlying pathophysiological mechanisms. Multidisciplinary management is essential in complex orofacial cases.

[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.

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.

Hemicrania continua in a family: A report of two cases.

OBJECTIVE: To report two cases of hemicrania continua (HC) in a mother and daughter. BACKGROUND: HC is a rare primary headache disorder belonging to the family of trigeminal autonomic cephalalgias (TACs). Unlike migraine, familial cases of TACs are rare, and we know relatively little of their inheritance pattern and genetic mechanisms. METHODS: We present a mother and daughter with HC. We compare the similarities and differences between this family and the first report of familial HC and discuss the implications for future studies. RESULTS: Both the mother and daughter presented with a constant, side-locked headache of moderate intensity, with episodic exacerbations of more severe pain that are associated with ipsilateral cranial autonomic activation. After negative workup, both patients were started on indomethacin and achieved absolute response at different doses, confirming HC. CONCLUSIONS: Our report further corroborates other reports of familial TACs that TACs are primary headaches possibly attributable to genetic factors, albeit detailed mechanisms remain elusive. Nevertheless, whether clinical presentation and treatment responses would be substantially different between sporadic and familial HCs remain unclear.

Indomethacin-responsive headaches-A narrative review.

BACKGROUND: Indomethacin is a nonsteroidal anti-inflammatory drug whose mechanism of action in certain types of headache disorders remains unknown. The so-called indomethacin-responsive headache disorders consist of a group of conditions with a very different presentation that have a particularly good response to indomethacin. The response is so distinct as to be used in the definition of two: hemicrania continua and paroxysmal hemicrania. METHODS: This is a narrative literature review. PubMed and the Cochrane databases were used for the literature search. RESULTS: We review the main pharmacokinetic and pharmacodynamics properties of indomethacin useful for daily practice. The proposed mechanisms of action of indomethacin in the responsive headache disorders, including its effect on cerebral blood flow and intracranial pressure, with special attention to nitrergic mechanisms, are covered. The current evidence for its use in primary headache disorders, such as some trigeminal autonomic cephalalgias, cough, hypnic, exertional or sexual headache, and migraine will be covered, as well as its indication for secondary headaches, such as those of posttraumatic origin. CONCLUSION: Increasing understanding of the mechanism(s) of action of indomethacin will enhance our understanding of the complex pathophysiology that might be shared by indomethacin-sensitive headache disorders.

The Promising Effect of Nerve Decompression in Trigeminal Autonomic Cephalalgias: Report of Case Series.

Trigeminal Autonomic Cephalalgias (TAC) are excruciating headaches with limited treatment options. The chronic forms of TACs, including chronic cluster, chronic paroxysmal hemicrania, and hemicrania continua, are disabling conditions. In addition to drug therapy, there are some studies regarding nerve blocking and nerve stimulation with acceptable results. Here we report four cases of decompression nerve surgery with promising results on pain control in these difficult to treat headaches.

Hemicrania continua: Indomethacin induced myoclonus.

Myoclonus has been described rarely as an adverse effect with some non-steroidal anti-inflammatory drugs, but never with indomethacin. Indomethacin is a common nonsteroidal anti-inflammatory drug used for various primary headache disorders, including hemicrania continua. We present a rare case of a 45-year-old male with hemicrania continua who developed myoclonus from indomethacin. These movements resolved completely following discontinuation of indomethacin. The disturbance on the serotonergic and GABAergic systems may be associated with indomethacin induced myoclonus. Clinicians and patients should be mindful with this potential side effect with indomethacin.

Paroxysmal Hemicrania: An Update.

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.

Hemicrania Continua: An Update.

BACKGROUND: Hemicrania continua (HC) is not uncommon in clinical practice, and several large case series have been published in the recent past. OBJECTIVES: This review provides an overview of the recent advancement in different aspects of HC. METHODS: We reviewed the articles published on HC in the last 2 decades. RESULTS: HC constitutes 1.7% of patients with headache in the clinics. It presents with unilateral continuous background pain with periodic exacerbations, usually accompanied by cranial autonomic features and restlessness. The continuous background headache is the most consistent and central feature of HC. Although the duration of exacerbations varies from a few seconds to a few weeks, the frequency ranges from >20 attacks/day to one attack in several months. The background pain is mild to moderate in intensity and does not hamper routine activity. Patients and physicians frequently ignore the basal pain, and a case of HC is misdiagnosed as other headaches, depending on the pattern of exacerbations. The exacerbation mimics several primary headaches and neuralgias. There are about 75 cases of secondary HC, due to 29 different pathologies. Although an absolute response to indomethacin is part of the diagnostic criteria, a subset of patients may respond to several other drugs. Headache reappears immediately on skipping a single dose of effective drug. Several surgical procedures have been tried in patients who are intolerant to indomethacin. CONCLUSION: Misdiagnosis of HC is common. Continuous background pain and response to indomethacin are two essential features for the diagnosis of HC.

Headache from clinically confirmed hemicrania continua arising from the sternocleidomastoid muscle: a case report.

BACKGROUND: A patient with a history of cluster headaches, now in remission, presented with confirmed hemicrania continua that resolved with a local anaesthetic injection into the Sternocleidomastoid (SCM) muscle. To the best of our knowledge, this is the first reported case of a trigeminal autonomic cephalalgia arising from a soft tissue source in the neck. CASE PRESENTATION: A 66-year-old man with a history of cluster headaches presented with a six-month history of a new constant right-sided headache. The new headaches were associated with tearing and redness of the right eye and responded to indomethacin, thus meeting the International Classification of Headache Disorders (ICHD-3) diagnostic criteria for hemicrania continua. The history and physical examination suggested a cervical source of the headache arising from the ipsilateral SCM muscle. Injection of the muscle with 1% lidocaine resulted in the elimination of the pain for 1 month without indomethacin. CONCLUSIONS: Due to the convergence of trigeminal, cervical and autonomic nerve fibres, various combinations of headache syndromes can result. This case report demonstrates how a meticulous examination is a crucial component of headache evaluation. Treatment directed to this muscle spared this patient further daily indomethacin and associated side effects.