Nummular Headache Research Articles

Are there differences between patients with unifocal nummular headache and those who progress to bifocal nummular headache? A retrospective observational study.

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. 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. 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). 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.

Cefaleas y neuralgias craneofaciales descritas por autores españoles

Spanish physicians have made important contributions to the nosology of headaches by describing various syndromes. In 1954, Eduardo Tolosa Colomer published the first case of Tolosa-Hunt syndrome, a clinical picture characterized by orbital pain and paralysis of extraocular muscles due to the presence of granulomatous inflammation in the cavernous sinus. In 1980, José Félix Martí Massó described pseudomigraine with cerebrospinal fluid pleocytosis, which manifests with episodes of migraine-type headache, transient neurological deficits, and elevated lymphocytes in the cerebrospinal fluid, usually with a benign prognosis. Since 2002, Juan Pareja and his group have identified several entities including: nummular headache, characterized by pain localized in a small coin-shaped area of the skull; primary trochlear headache, originating in the trochlea, in the superointernal angle of the orbit; epicrania fugax, which manifests with episodes of paroxysmal pain radiating along the surface of the head, following a linear or zigzag trajectory, and idiopathic ophthalmodynia and rhinalgia, which present with pain in the eyeball or nose with no apparent cause. Juan Pareja has also described several trigeminal terminal branch neuralgias, which are characterized by pain limited to the corresponding cutaneous territory and which typically respond to anesthetic blockade of the nerve. Finally, paroxysmal pressing headache, described by the same author, is manifested by brief episodes of unilateral pressing pain without accompanying symptoms. All these descriptions have been documented in scientific publications, and most of them have had a significant impact on clinical practice.

A case report: new-onset nummular headache after stenting of the middle cerebral artery

BackgroundNummular 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.Case presentationWe 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.ConclusionThis is the first report of NH after stent placement treatment in a patient with ICAS.

Long-term outcomes of nummular headache: A series of 168 patients and 1198 patient-years of follow-up.

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%.

Epicranial headaches part 2: Nummular headache and epicrania fugax.

Nummular headache and epicrania fugax are two primary headaches that fall under the heading of epicranial headaches. This article reviews the epidemiological and clinical features of nummular headache and epicrania fugax, proposed pathogenic mechanisms and state-of-the-art management, according to the literature. Nummular headache and epicrania fugax are generally viewed as rare headache disorders, but no population-based epidemiological data are available. Nummular headache is characterized by continuous or intermittent head pain, which remains circumscribed in a round or oval area of the scalp, typically one to six centimeters in diameter. Epicrania fugax manifests with brief paroxysms of pain that move along the surface of the head, following a linear or zigzag trajectory through different nerve territories. Nummular headache and epicrania fugax are mostly primary headaches, but some secondary cases have been reported. The pathogenesis of these headaches is not fully understood. Nummular headache could probably originate in epicranial tissues or adjacent intracranial structures, while the origin of epicrania fugax could be extracranial or intracranial. Diagnostic assessment requires careful examination of the symptomatic areas. Underlying disorders should be excluded by additional investigations, including neuroimaging and appropriate blood tests. No controlled clinical trials have been conducted in nummular headache or epicrania fugax. Analgesics and anti-inflammatory drugs, botulinum toxin and gabapentin are currently the most recommended treatment options for nummular headache. In epicrania fugax, the most used treatments are gabapentin, lamotrigine, and other antiseizure medications.

Trifocal Nummular Headache: A Case Report of Dramatic Response to Local Anesthetic Injection

Trifocal Nummular Headache: A Case Report of Dramatic Response to Local Anesthetic Injection

Нумулярний головний біль (клінічне спостереження, обговорення)

The purpose of the study was to show the importance of using international diagnostic criteria of headache in neurological practice through description of clinical case of nummular headache. Materials and methods. Complaints, medical history, life history, results of objective and neurological examination, data of laboratory and instrumental research methods in a patient with nummular headache. Results and discussion. Patient G., 53 years old, complained of constant, dull, local, moderate pain in the right parietal area. The headache appeared gradually 5–6 months ago, without any evident triggers. Patient denied autonomic trigeminal signs, nausea, photo- and phonophobia. Painkillers intake was accompanied by a temporary slight decrease of headache intensity. At examination patient pointed to local pain area in the right parietal region that had diameter about 3 cm, rounded shape and clear borders. Neurological status – without abnormalities, except for hyperalgesia in the pain area. The skin and hair in the pain area were not changed, touching the skin in this place caused unpleasant feeling. General and biochemical blood tests, blood glucose, general urine analysis were within reference values; magnetic resonance imaging of the brain was without abnormalities; magnetic resonance imaging of the cervical spine showed osteochondrosis, protrusion of intervertebral discs C5-C6, C6-C7. Additional examination was recommended to the patient: computer tomography of the head (skull bones were unchanged), consultation of a dermatologist (no pathology was found). Finally, it was concluded that patient's condition, according to the international classification of headache disorders of the 3rd revision, corresponds to the diagnosis of “nummular headache”. Gabapentin was prescribed 300 mg per day, followed by increasing of the drug dose by 300 mg every 3 days to a daily dose of 1200 mg or until a significant reduction (disappearance) of headache occurred at the lower gabapentin doses. After 1 week of gabapentin taking, patient reported significant decrease in headache intensity at a daily dose of 600 mg with subsequent headache disappearance at a daily dose of 900 mg. It was recommended to continue gabapentin taking at a dose of 900 mg per day for the next 3 months. After 3 months, the patient reported no headache during the entire period of gabapentin usage. It was recommended to reduce the daily dose of gabapentin by 300 mg every 5 days until the headache recurs or until total discontinuation of drug intake. 1 month later the patient reported no headache since stopping gabapentin intake. Conclusion. Neurologists need to know and actively use in their practice the international diagnostic criteria of nummular headache, that is the basis of adequate management of this rare primary headache

Treatment of Primary Nummular Headache: A Series of 183 Patients from the NUMITOR Study

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.

Pearls & Oy-sters: Postpipeline Headache Phenomenon: Nummular Headache Presenting After Intracranial Aneurysm Stenting.

We report on a 31-year-old right-handed woman with a medical history of presyncopal episodes and migraine headaches who presented to the outpatient clinic with a nummular headache after intracerebral stenting, which was different than her previous migraines. This represents postpipeline embolization headache phenomenon, which is a relatively new term to describe a new or different headache in individuals who recently underwent intracranial vascular stenting as a treatment for cerebral aneurysms.

Nummular headache in children: A case series and systematic literature review

Background: Nummular headache is a rare primary headache disorder potentially disabling and refractory to treatment. Of over 300 reported cases, only 9 are children. Design/Methods: We searched our institutional database and PubMed for “nummular headache” and synonyms and evaluated select articles from reference lists of substantial reviews. Results: Seven children were identified from our institution and nine from 107 unique publications. Mean age at onset was 9.7 and 11.9 years in our cohort and the literature, respectively, with a female:male ratio of 2:1. Location and quality varied, and allodynia was reported in five. Frequency ranged from every other week to continuous. Imaging was normal except in four cases. Migraine was often comorbid. Published cases were frequently refractory to treatment whereas four of our cohort improved with therapy for comorbid migraine. Conclusions: Nummular headache is rarely reported in children. We describe characteristics of seven new and nine previously published pediatric cases.

Diferencijalno dijagnostička distinkcija numularne glavobolje i Lihen planopilarisa

Introduction: Nummular headache is a type of primary headache of chronic character, with a large number of described varieties in the clinical picture, and therefore the differential diagnostic consideration must include a large number of disorders related to changes in epicranial structures. Certain dermatological disorders can also include pain in a limited area of the scalp with itching, burning or burning sensation, such as Lichen Planopilaris. Case report: This is a report of a patient who was initially diagnosed with Lichen planopilaris, but after determining the parameters of the distinction and the necessary additional diagnostic procedures, a diagnosis of Nummular headache was made. Conclusion: In this presentation, we have presented another important differential diagnostic item and we believe that the work is a small contribution to everyday practice, but also to further research.

Nummular Headache Successfully Managed With Percutaneous Electrical Nerve Stimulation: A Case Report

Nummular Headache Successfully Managed With Percutaneous Electrical Nerve Stimulation: A Case Report

Linear Headache: A Novel Entity or a Variant of Nummular Headache? Clinical Characteristics and Treatment Response in a Series of 16 Patients.

Linear headache has been recently described as an episodic or chronic unilateral pain distributed along a fixed linear trajectory, which combines some characteristics of epicrania fugax and nummular headache. The aim of this study was to describe the clinical characteristics and therapeutic response of a series of 16 new patients. This is an observational study with a series of cases. The study period encompassed June 2014 to June 2019. Demographic, clinical, and therapeutic response data were recorded. We included all consecutive patients who presented pain with the following characteristics: sharply contoured, fixed in size and shape, with linear shape, without movement along a trajectory, and not circumscribed to the territory of any nerve. Twelve patients were women, and four were men. The mean age at onset was 40.1 years. Pain was described as pressing in seven patients, burning in five, and electric or stabbing in two each. Symptomatic treatment had been used by 13 patients (81.2%), with analgesics being the most frequent treatment used. Thirteen patients received preventive treatment. The response to oral medications and anesthetic blockade was insufficient. OnabotulinumtoxinA was used in six cases, with an optimal (>75%) response observed in half. Linear headache appears to be a distinct headache syndrome from epicrania fugax or nummular headache. Preventive treatment is often required. The drug with the best response was onabotulinumtoxinA.

Other primary headaches. Cough headache, nummular headache and primary exercise headache: a secondary point of view.

Other primary headaches. Cough headache, nummular headache and primary exercise headache: a secondary point of view.

Cephalgia Alopecia.

In this review, we examine reported cases of cephalgia alopecia including the initial case report from 2006. The goal is to review the clinical description, pathophysiology, diagnosis, and treatment of cephalgia alopecia. The pathophysiology of the headache and hair loss in cephalgia alopecia is believed to be related to neuroregulation of skin and nerve. It is hypothesized that the headache causes recurrent activation of trigeminal and upper cervical branches that innervate the hair cells. The repetitive activation of C fibers results in depletion of substance P and calcitonin gene-related peptide (CGRP), which leads to loss of hair growth promotion and disruption of immune system regulation. A case report suggests that cephalgia alopecia and nummular headache with trophic changes may represent a spectrum of disease involving head pain and cutaneous changes. Cephalgia alopecia is a rare headache disorder described as recurrent burning, stabbing head, and neck pain that is followed by hair loss in the corresponding region of the scalp. The mainstay treatment for both pain and hair loss is OnabotulinumtoxinA (onabotA). A patient's clinical history and response to onabotA treatment is used to make the diagnosis. Future research is needed to examine the hypothesized disease continuum of head pain and cutaneous changes. It will also be beneficial to assess if the grid-like onabotA technique used in nummular headache is effective in cephalgia alopecia. In addition, further studies are needed to assess the proposed pathophysiology.

Nummular Headache and Its Surgical Treatment.

Background:Nummular headache (NH) is an uncommon primary headache characterized by pain limited to a precise small area of the scalp. There is no global consensus on its pathogenesis, but its extracranial origin is the most accepted theory. Moreover, peripheral mechanism is supported by the overlapping symptomatology of secondary forms of NH and is well described in the literature. However, a standard effective treatment is still lacking.Methods:A literature search according to Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines was conducted to evaluate surgical strategies for NH. Inclusion criteria were English language, diagnosis of primary NH according to International Classification of Headache Disorders, 3rd Edition, or of secondary NH, and follow-up at a minimum of 3 months. The treatment had to consist of peripheral surgery.Results:One hundred eighty-seven records were identified after duplicates were removed, 15 full-text articles were assessed for eligibility, and 4 records were selected for inclusion. A total of 53 patients were included in this review, 50 of whom were diagnosed with primary NH. The general positive response after surgery (>50% reduction in occipital migraine headaches) was about 70.0% for primary NH, while secondary NH always showed complete pain relief. However, many variations in patient selection and type of surgery were described.Conclusions:Neurovascular relationship in the extracranial tissues seems to be involved in the onset of NH. However, only limited data from meager literature and from few patients are currently available. Shared multicentric research protocols are badly required.

Nummular headache – three years later: What happened

Nummular headache – three years later: What happened

Characteristics and treatment effectiveness of the nummular headache: a systematic review and analysis of 110 cases

Background/objectiveNummular headache (NH) is a primary headache disorder characterised by intermittent or continuous scalp pain, affecting a small circumscribed area of the scalp. As there are limited data in the...

Clinical and pathophysiological description of nummular headaches: a case series

Nummular headache was initially described as an infrequent primary headache characterised by chronic circumscribed mild-to-moderate pain in a small area of the head that may present with exacerbations. Since its initial description, dozens of cases of nummular headache have been reported which broaden the clinical and pathophysiological spectrum. To review and establish the clinical characteristics of nummular headache, to analyse the epidemiology in our setting and to clarify some unanswered pathophysiological issues. The study involved 83 cases (42 women and 41 men) who were diagnosed with nummular headache from July 2008 and followed up until 2018 at the Fundacion Alcorcon Hospital. The mean age of onset was 46 years. The time to diagnosis was seven months. The pain was moderate-intense and oppressive, with exacerbations in 62.5% of cases. The average size of the symptomatic area was 2.4 ± 0.7 cm, with a parietal (47%), temporal (24%), frontal (12%) or occipital (6%) location. It presented a chronic (82%) or episodic (18%) pattern. Symptoms of local sensory dysfunction were observed in 25 patients and three others showed skin alterations. Spontaneous remission was observed in 36% of cases, and the most commonly used drugs were non-steroidal anti-inflammatory drugs, paracetamol and gabapentin. Botulinum toxin was effective. Nummular headache is a disorder with well-defined characteristics and is considered an entity in its own right. Our data indicate that its origin lies in the epicranial structures. The conjunction of pain, sensory dysfunction and trophic disorders suggests a local form of a complex regional syndrome.

Nummular headache: a gender-oriented perspective on a case series from the RegistRare Network.

Nummular headache (NH) is a rare headache disorder characterized by a small, circumscribed painful area of the scalp. The description of many cases in the last years has supported its re-classification as a primary headache from the International Headache Society, moving it from its previous placement in the Appendix of the International Criteria of Headache Disorders. Data were collected from a retro-prospective observational study about rare headaches promoted by the RegistRare Network, a collaborative group of seven Italian Headache Centres. According to the gender-biased profile of certain primary headaches, we have looked further NH patients from a gender perspective. Nineteen NH patients (11 men, 8 women) have been enrolled in the study. Headache onset was at 39 years and preceded approximately 8 years the diagnosis. No clinically evident differences between men and women have been found, including treatment prescriptions and headache resolution. Of note, the mean time from the onset of NH to the first visit in a Headache Centre was longer in men, compared with women (13.5 vs. 0.9 years). NH attacks were efficaciously treated with nonsteroidal anti-inflammatory drugs in 60% of patients receiving treatment. Headache prophylaxis with pregabalin and amitriptyline has been reported as effective in 40% and 67% of the treated patients, respectively. NH is a primary headache clinically heterogeneous in terms of temporal patterns and pain characteristics. Further research is needed to investigate the existence of male and female phenotypes, by clarifying whether it may be relevant for therapeutic purposes.