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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).)

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.

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 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.

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.

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)

The trigeminal autonomic cephalalgias (TACs) are characterized by the cardinal combination of short-lasting unilateral pain and autonomic phenomena affecting the head, including cluster headache, paroxysmal hemicrania and SUNCT. Infrequently reported have been cases with dissociation of these two cardinal features- usually with autonomic phenomena without pain, rather than the reverse. Herein is described the case of a 56-year-old man presenting with an unusual form of TAC resembling chronic paroxysmal hemicrania but with a lower frequency of occurrence, with temporal features not typical of previously described TACs, and consisting of severe unilateral head pain without any autonomic symptoms or signs, absolutely responsive to indomethacin.

Unruptured cerebral arterial aneurysms most often remain asymptomatic, but they may cause headache or other symptoms or signs. We describe herewith a case of headache attributed to an unruptured internal carotid artery aneurysm, clearly mimicking the phenotype of hemicrania continua. Potential pathophysiological explanations and recommendations for recognition of similar cases are discussed.