OBJECTIVE: This retrospective observational cohort study aimed to compare clinical characteristics and treatment responses in patients exclusively experiencing unifocal nummular headache (NH) with those who develop the bifocal variant.
METHODS: A retrospective study was conducted on patients diagnosed with NH who attended a neurology (headache) outpatient clinic between January 2018 and December 2022. The cohort was divided into two groups: Group 1, exclusive unifocal NH; and Group 2, those developing a secondary focal area of pain, i.e., bifocal NH. Data were collected on demographic characteristics, clinical features, other headache comorbidities, and treatment-related information.
RESULTS: A total of 23 patients were included in this study: 12 were categorized as unifocal NH (Group 1) and 11 as bifocal NH (Group 2). There were no differences between the two groups in terms of demographic characteristics, clinical features, or treatment response. Nonetheless, patients with bifocal NH exhibited spontaneous remission rates in the first pain area when compared to the unifocal NH group, with statistically significant differences (36% vs. 0%, p = 0.020).
CONCLUSIONS: In our sample, patients with bifocal NH demonstrated spontaneous remission rates in the initial pain area, a phenomenon not observed in patients with unifocal NH. It is worth noting the limited sample size in the present study, highlighting the need for larger cohorts to validate and further explore our findings.

BACKGROUND: Nummular headache (NH) is categorized as a primary headache in the International Classification of Headache Disorders, Third edition (ICHD-3) diagnostic criteria, but there are secondary etiologies as well. We present a case of secondary NH that associated with vascular lesion.
METHODS: We report on a 40-year-old man with a medical history of symptomatic intracranial arterial stenosis who developed a headache after percutaneous transluminal angioplasty and stenting because of Intracranial atherosclerotic stenosis(ICAS). This new-onset headache was a pinprick headache confined to the parietal part of the head and 5 cm in size. This headache most closely resembled the phenotype of a NH. And other causes of secondary headache were excluded. Thus, the diagnosis of NH was highly speculated. This patient represents a rare headache phenomenon after intracranial arterial stent placement.
CONCLUSIONS: This is the first report of NH after stent placement treatment in a patient with ICAS.

Since the first description of nummular headache (NH), more than 500 cases have been described, delineating its clinical phenotype and response to treatment. However, data on the natural history of NH and outcomes during long-term follow-up are not currently available. The present study aimed to describe the long-term outcomes and follow-up of a large series of patients with NH.
A descriptive observational ambisective study with a series of cases was conducted. The study population included adult patients with primary NH and a minimum of 12 months of follow-up. Demographic variables, previous medical history, clinical phenotype, diagnosis and treatment of NH, temporal pattern, and long-term evolution were analysed.
In total, 168 patients were enrolled and followed for a median [interquartile range (IQR)] of 80.5 (55-118.5) months. The temporal pattern after NH onset was chronic in 67.9% and, at diagnosis, the median (IQR) number of pain days per month was 20 [10-30] days with 138 (82.1%) patients with ≥8 days of pain per month. Preventive treatment was needed by 112 (66.7%) patients. The most frequently used drugs were gabapentin (69/112; 61.6%), onabotulinumtoxinA (38/112; 33.9%), amitriptyline (31/112; 27.7%) and lamotrigine (21/112; 18.7%). Response to preventive treatment was at least partial in 91/112 (81.3%) patients. At the end of follow-up, 81 (48.2%) patients had inactive NH. Of patients with active NH, the median (IQR) number of headache days per month was 3 (1-12) days and patients had ≥8 days of pain in 35 (20.8%) cases.
Long-term outcomes of NH were positive in most patients. After a median of 6.7 years of follow-up, 48% of cases were inactive. Two-thirds of patients required preventive treatment, and 80% of them were treatment-responsive. In NH cases that remained symptomatic, the headache frequency was lower, and the proportion of patients with chronic NH decreased from 68% to 11%.

Nummular headache is an unusual facial pain disorder with no evidence-based therapy recommendations. The ketogenic diet is an alternative therapy that demonstrated to be effective in migraineurs, but it was never used in the setting of nummular headache. We describe a 58-years old female patient with nummular headache successfully treated with a 6-months ketogenic diet and botulinum toxin type A injections. Ketogenic diet could be an effective alternative/complementary therapy in nummular headache patients although more studies are needed to confirm our results.

Nummular headache (NH) is a primary headache characterized by superficial coin-shaped pain. NUMITOR (NCT05475769) is an observational study evaluating the responder rate of preventive drugs in NH patients. The treatment response was assessed between weeks 8 and 12 compared with the baseline. Patients were included between February 2002 and October 2022. Demographic and clinical variables were assessed; treatment response was estimated by 50%, 30%, and 75% responder rates and treatment discontinuation due to inadequate tolerability. A total of 183 out of 282 patients fulfilled eligibility criteria and completed the study. Patients were aged 49.5 (standard deviation (SD): 16.8) years, and 60.7% were female. NH phenotype was a parietal circular pain of four centimeters\' diameter, moderate intensity, and oppressive quality. At baseline, patients had 25 (interquartile range) pain days per month. Preventive treatment was used by 114 (62.3%) patients. The highest 50% and 75% responder rates corresponded to onabotulinumtoxinA (62.5%, 47.5%), followed by gabapentin (43.7%, 35.2%). Oral preventive drugs were not tolerated by 12.9-25%. The present study provides class IV evidence of the effectiveness of oral preventive drugs and onabotulinumtoxinA in the treatment of primary NH. OnabotulinumtoxinA was the most effective and best-tolerated drug, positioning it as first-line treatment of NH.

BACKGROUND: Nummular headache (NH) is defined by the presence of localized pain circumscribed to a round or elliptical area of the scalp, with a well-defined contour and a diameter of 1-6 cm. Although some evidence supports a peripheral mechanism, its etiopathogenesis remains unclear.
METHODS: We report the case of a 64-year-old man with high-frequency episodic migraine who has used topiramate, beta-blockers, flunarizine, and amitriptyline without effect. In the last 8 years he also had continuous pain in an oval area of the scalp, consistent with NH. Triptans were ineffective for this new pain, and preventive therapy with gabapentin and onabotulinumtoxinA in the painful area had no effect. NH remitted when the patient received monthly treatment with subcutaneous galcanezumab for his migraine.
CONCLUSIONS: Monoclonal antibodies against calcitonin gene-related peptide (CGRP), in particular galcanezumab, might be an effective therapy in some patients with NH. CGRP may have a role in the etiopathogenesis of this headache, which warrants further investigation.

Nummular headaches are a rare and relatively newly characterized primary headache disorder. The epidemiology is largely unknown due to likely underdiagnosis and a small population of all headache patients in outpatient presentation. Though our understanding of nummular headaches continues to evolve, they remain a diagnostic challenge for physicians and the underlying pathophysiology is poorly understood. Hypotheses consider neuralgia stemming from epicranial tissues as well as undergoing observation of varying prevalence of autoimmune markers. Peripheral nociception versus central sensitization needs to be evaluated as well, with cases not having consistent direction. Selecting treatment options can be challenging due to limited efficacy, the vague nature of reported symptoms, the rarity of the diagnosis, and the range of presentations. Several treatment modalities have been utilized including non-steroidal anti-inflammatory drugs (NSAIDs), beta-blockers, botulinum toxin injection, transcutaneous nerve stimulation, or even simple reassurance. A case-by-case analysis must be undertaken to best develop treatment options for affected individuals as high-quality randomized quality trials for nummular headaches are very few. We detail two novel cases of patients presenting with nummular headaches that highlight the challenges and importance of making the diagnosis and weighing treatment options for improved levels of patient care, which is followed by a literature review.

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.

BACKGROUND: Nummular headache (NH) is defined in the International Classification of Headache Disorders (ICHD) by the presence of localized pain circumscribed to a small round area of the scalp, not better accounted by any other diagnosis. As in many other primary headache disorders, secondary cases might occur. To date, 13 secondary cases have been published. We aim to present a long series of secondary NH and review the literature of symptomatic NH.
METHODS: Retrospective analysis of an observational prospective cohort in a headache unit located in a tertiary hospital. We included patients that fulfilled ICHD criteria and were attributed to a secondary cause. We describe the clinical characteristics, the underlying causes, and the response to treatment.
RESULTS: We included 274 NH patients; eight of them (2.9%) were considered secondary. In one patient the underlying cause was subcutaneous, as for six cases the lesion was located in the bone (two hemangiomas, one osteoma, three different types of cysts), and in one was intracranial but closely related with internal diploe (cavernoma). Among our patients with secondary NH, a preventive therapy was not always needed and, when required, gabapentin or onabotulinumtoxinA were used with positive response.
CONCLUSIONS: Secondary NH phenotype overlaps primary NH. Therefore, we recommend routine imaging study in every NH patient. Concerning treatment, it was not necessary to remove the underlying lesion to control the pain and many cases responded to the same prophylactics as primary NH cases.

UNASSIGNED: Other primary headaches make up group 4.0 of the International Classification of Headache Disorders third edition (ICHD-3). This group includes a clinically varied group of headache entities whose pathophysiology is not well understood and whose treatments are based on open-label reports.
UNASSIGNED: To review and update the uncommon primary headaches included under group 4.0 in ICHD-3.
UNASSIGNED: We reviewed the headache disorders listed under group 4.0 of ICHD-3 for the clinical features, diagnostic criteria, and management, and described recent updates of these relatively rare disorders.
UNASSIGNED: The entities included in this group have a characteristic presentation in practice. Some of them are activity-related and can be provoked by Valsalva maneuver (primary cough headache), some by prolonged exercise (primary exercise headache), and some by sexual excitation (primary headache associated with sexual activity); primary thunderclap headache has also been included here because all the above-listed entities can sometimes present in thunderclap fashion too. Two of the entities in this group 4.0 are linked to direct physical stimuli viz. cold stimulus headache and external pressure headache. Also included in this group are primary stabbing headaches and nummular headaches that are presumed to be due to the involvement of the terminal branches of the sensory nerves supplying the scalp and are, therefore, termed \"epicranial headaches.\" Hypnic headache syndrome and new daily persistent headache are included here as \"miscellaneous headaches\" because we still do not know enough about their causation.
UNASSIGNED: The headache disorders included under group 4.0 in ICHD-3 are uncommon, heterogeneous entities, which may pose diagnostic and management challenges to the physicians. The diagnosis may be missed owing to a lack of familiarity.