The Mystery of “Red Ear Syndrome”: Sign or Syndrome

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Non-invasive Brain Stimulation in Pediatric Migraine: A Perspective From Evidence in Adult Migraine

Pediatric migraine remains still a challenge for the headache specialists as concerns both diagnostic and therapeutic aspects. The less ability of children to describe the exact features of their migraines and the lack of reliable biomarker for migraine contribute to complicate the diagnostic process. Therefore, there's need for new effective tools for supporting diagnostic and therapeutic approach in children with migraine. Recently, promising results have been obtained in adult headache by means of application of neurostimulation techniques both for investigating pathophysiological mechanisms and also for therapeutical applications. Non-invasive brain stimulation (NIBS) techniques like tr…

Headache attributed to aeroplane travel: the first multicentric survey in a paediatric population affected by primary headaches

BACKGROUND: This multicentric survey investigates the prevalence and characteristics of Airplane Headache in children affected by primary headaches. METHODS: Patients with symptoms of Airplane Headache were recruited from nine Italian Pediatric Headache Centres. Each patient was handed a structured questionnaire which met the ICHD-III criteria. RESULTS: Among 320 children suffering from primary headaches who had flights during their lifetime, 15 (4.7%) had Airplane Headache, with mean age of 12.4 years. Most of the patients were females (80%). The headache was predominantly bilateral (80%) and localized to the frontal area (60%); it was mainly pulsating, and lasted less than 30 min in all c…

Red Ear Syndrome

The Red Ear syndrome (RES) is an intriguing syndrome originally described for the first time nearly 20 years ago. RES is characterized by unilateral/bilateral episodes of pain and burning sensation of the ear, associated with ipsilateral erythema. RES episodes are indeed isolated in some patients, but they can occur in association with primary headaches, including in particular migraine in the developmental age. Although the underlying pathophysiological mechanisms are still uncertain, in the recent years the described comorbidities have aroused increasing interest because of possible clinical implications. Moreover, RES seems to be more often associated with clinical features of migraine p…

The IHS classification criteria for migraine headaches in adolescents need minor modifications

The operational and diagnostic criteria for migraine and all other headache disorders released in 1988 by the International Headache Society are universally considered reliable and exhaustive. These criteria, however, cannot be considered as satisfactory for population-based studies on migraine prevalence, especially if adolescents are the subjects of the study. Using these diagnostic criteria, we conducted an epidemiological study in order to assess the prevalence of migraine headache in a student population aged 11 to 14 years. Our survey made it possible to code IHS 1.1 (migraine without aura) in 2.35%, IHS 1.2 (migraine with aura) in 0.62%, IHS 1.7 (migrainous disorders not fulfilling m…

"The Headache Week": a useful tool to highlight "invisible" migraineurs

Osmophobia as an early marker of migraine: a follow-up study in juvenile patients.

Background: Osmophobia is frequent in children with migraine (20–35%) but can also occur in up to 14% of cases with tension-type headache (TTH). So far, the prognostic role of this symptom in children with primary headaches has never been evaluated. Methods: A longitudinal prospective study was conducted on 90 young patients with TTH (37 with osmophobia, 53 without osmophobia). We evaluated whether osmophobia could predict the diagnosis transformation from TTH to migraine after a 3-year follow-up. Results and Discussion: In our cases the rate of diagnosis change was significantly greater in cases with osmophobia (62%) than in those without (23%). Osmophobia persisted at a 3-year follow-up …

Juvenile migraine and allodynia: results of a retrospective study.

Background There are only 2 small sample studies investigating allodynia in the pediatric population. The aim of this study was to evaluate the frequency of allodynia during cephalalgic attacks in a juvenile population with primary headaches and its association with other symptoms of migraine. Methods We reviewed all medical records of patients with primary headache consecutively seen during a 2-year period. Frequency of allodynia was evaluated, by means of a questionnaire, consisting of 6 questions (for example: Do you avoid touching your head when you have a migraine attack?). Results Two hundred thirty children suffering from primary headache were seen during the study period. Two hundre…

Migraine in Children Under 7 Years of Age: a Review

Purpose of review: Despite the accumulation of a significant amount of data on pediatric headache, few studies have been conducted on its occurrence in children under 7 years of age. Within primary headaches in this age, migraine especially, turns out to be a disorder affecting up to 4% of the general population. An underestimate of its true prevalence can be due to lack of specific diagnostic markers, the frequent difficulty of describing pain in childhood, and the necessity of reliable parents’ reports. Thus, migraine in children under 7 years of age represents an important challenge for clinicians. The objective of this manuscript is to provide a comprehensive review of epidemiologic, cl…

Migraine Headaches in a High School Student Population.

Is a digital platform useful in headache training? A 4-year Italian experience.

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Migraine and cranial autonomic symptoms in children and adolescents: a clinical study.

The frequency of cranial autonomic symptoms in children affected by primary headaches is uncertain. The aim of our study was to estimate the frequency of symptoms in pediatric headaches and correlate it with main migraine characteristics. A questionnaire investigating the presence of cranial autonomic symptoms was administered to all children with primary headache for 2 years. A total of 230 children with primary headache (105 males, 125 females) were included. Two hundred two children were affected by migraine and 28 (12.2%) by other primary headaches. Cranial autonomic symptoms were significantly complained by migraineurs (55% vs 17.8%) ( P < .001) and by children with higher frequenc…

Evolution of Pediatric Migraine Patients Admitted at an Emergency Department after a 10-Year Follow-Up

Background: Despite its high prevalence, the clinical course of pediatric migraine has not been fully understood, and previous studies present conflicting results. We present here the findings of a 10-year follow-up study involving children with severe migraine pain admitted to our emergency department. Furthermore, all studies were carried out on selected outpatient clinical case studies. Our aim was to evaluate a population of migraine children admitted to an emergency department because of increased severity or frequency of pain or even because of very anxious parents concerning their child’s headache in order to describe their long-term outcomes, whether it differed from that of o…

Transient topographical amnesia and cingulate cortex damage: A case report

Transient topographical amnesia (TTA) is the temporary inability to find one's way in familiar or unfamiliar surroundings due to the inability to use well known environmental landmarks for route finding. The syndrome has not been described as having any obvious aetiology and has been thought to be caused by a vascular deficit in right hemispheric structures which are crucial for topographic recognition, i.e. parietal association and parahippocampal cortex. The patient described in the present study complained of several critical episodes of TTA and tonic rigidity of the left limbs. Neuropsychological assessment was normal except for a deficit in spatial memory tasks. Magnetic resonance (MR)…

Survey on treatments for primary headaches in 13 specialized juvenile Headache Centers: The first multicenter Italian study

Abstract Aim The purpose of this retrospective multicenter study was to evaluate the use and the self-perceived efficacy and tolerability of pharmacological and non-pharmacological treatments in children and adolescents with primary headaches. Methods Study of a cohort of children and adolescents diagnosed with primary headache, consecutively referred to 13 juvenile Italian Headache Centers. An ad hoc questionnaire was used for clinical data collection. Results Among 706 patients with primary headaches included in the study, 637 cases with a single type of headache (migraine 76% – with and without aura in 10% and 67% respectively; tension-type headache 24%) were selected (mean age at clinic…

Prevalence of red ear syndrome in juvenile primary headaches.

Background: Previous studies have suggested a relationship between ‘red ear syndrome’ (RES) and pediatric migraine. Aims of this study were (i) to assess the frequency, specificity and sensitivity of RES in a population of pediatric migraineurs and (ii) to establish the pathophysiological mechanisms of RES associated with migraine. Methods and results: A total of 226 children suffering from headache (aged 4–17 years) were enrolled. One hundred and seventy-two (76.4%) were affected by migraine, the remaining 54 (23.6%) by other primary headaches. RES was followed significantly more frequently by migraine (23.3%; p < .0001), and was characterized by high specificity and positive predictiv…

Broad neurodevelopmental features and cortical anomalies associated with a novel de novo KMT2A variant in Wiedemann-Steiner syndrome.

Abstract Wiedemann-Steiner syndrome (WDSTS) is a rare genetic disorder including developmental delay/intellectual disability (DD/ID), hypertrichosis cubiti, short stature, and distinctive facial features, caused by mutation in KMT2A gene, which encodes a histone methyltransferase (H3K4) that regulates chromatin-mediated transcription. Different neurodevelopmental phenotypes have been described within the WDSTS spectrum, including a peculiar Autism Spectrum Disorder (ASDs) subtype in some affected individuals. Here, we report a 9-year-old Caucasian male found by next-generation panel sequencing to carry a novel heterozygous de novo KMT2A frameshift variant (NM_001197104.2:c.4433delG; p. Arg1…

Migraine in children under 6 years of age: A long-term follow-up study

Abstract Background Early starting of migraine seems predictive for less favorable outcome in later ages, however follow-up investigations are very few and all with short-term prospective period. We report here the longest follow-up study in a population of children presenting with migraine under the age of 6. Methods We followed-up 74 children under 6 years of age, referred for headache to our department between 1997 and 2003. The study was carried out between October 2016 and March 2018. Headache diagnoses were made according to the IHS criteria. Results 23/74 patients, 31% of the original cohort, were found at follow-up in a period ranging between 15 to 21 years after the first visit. Se…

EHMTI-0115. Migraine under 7 years: a clinical study

Migraine in children under 7 years has received limited attention and the few studies rarely report a careful description of clinical and therapeutic features.

New-onset headache following COVID-19: An Italian multicentre case series

Objective: To describe the characteristics of patients with new-onset headache following SARS-CoV-2 infection. Background: SARS-CoV-2 infection leads to several neurological manifestations, and headache is a frequent and disabling symptom, both exacerbating pre-existing headache syndromes and causing new-onset ones. Methods: Patients with new-onset headache after SARS-CoV-2 infection with consent to participate were included, while those ones with previous headaches were excluded. The temporal latency of headache after infection, pain characteristics, and concomitant symptoms were analysed. Moreover, the efficacy of acute and preventive medications was explored. Results: Eleven females (med…

Migraine headaches in adolescents: a five-year follow-up study

Background and Objectives.—Longitudinal studies of juvenile migraine are very few. We investigated the prevalence and evolution over 5 years of migraine without aura (MWOA) and migraineous disorder (MD) in an adolescent population. Methods.—Sixty-four subjects (34 girls and 30 boys, mean age 17.3±1.1 years) out of 80 selected in our 1989 epidemiological survey were included in the study. The diagnostic criteria of the International Headache Society were used in both studies. Results.—Thirty-two of 64 subjects (50%) had MWAO, 18 (28.1%) had MD, and 14 (21.9%) had headache not classifiable (HnC). Our results show that MWOA persisted in 56.2%, converted to MD or HnC in 9.4% and 3.1% of cases, …

Two cases of cluster headache effectively treated with levetiracetam.

Cluster headache (CH), which is characterized by recurrent attacks of short-lasting excruciating pain accompanied by signs of autonomic dysfunction (ICHD-II) (Headache Classification Subcommittee of the IHS, 2004), is the most severe of the primary headache syndromes. CH is the most common of the trigeminal autonomic cephalalgias (TACs), whose pathophysiology has not been completely elucidated ( Leone and Bussone, 2009). The treatment of CH is still debated. Verapamil (a calcium antagonist) and lithium are the first-option drugs. Several drugs have been proposed as alternative treatments, but only some of these showed limited efficacy. Recently, a new-generation antiepileptic drug, topirama…

Red ear syndrome in children: Review of literature and report of three cases

Abstract Background Red ear syndrome (RES) is a neurological syndrome that is characterized by attacks of redness and pain that is localized in the earlobe, accompanied by a burning sensation, swelling or otalgia. The exact pathophysiology of RES is not known. Several pediatric cases have been described. They show an extreme variability in clinical presentation and therapeutic response, and therefore there are numerous difficulties in the diagnostic-therapeutic approach and in the comprehension of the physiopathology. The goal of this report is to present three clinical cases of red ear syndrome in children. These cases show various characteristics that can give useful indications regarding…

Migraine headaches in adolescents: a student population-based study in Monreale.

We assessed the prevalence of migraine headaches in an epidemiological survey of an 11 to 14-year-old student population. Migraine headaches were classified on the basis of questionnaires and neurological examination using the operational diagnostic criteria of the International Headache Society. Prevalence of migraine without aura (IHS code 1.1) was 2.35%; that of migraine with aura (IHS code 1.2) was 0.62%. Migraine without aura was equally distributed among males and females, whereas migraine with aura was preponderant in the female cohort. The prevalence of migraine headaches in males was constant through the ages studied, whereas the prevalence of migraine headaches in females reached…

Five-year longitudinal study of juvenile migraine headaches

To evaluate the prevalence and the evolution over 5-year of juvenile migraine headaches. Sixty-four subjects selected in our 1989 epidemiological survey were included in the study. The criteria of the International Headache Society were used both in 1989 and 1994. Thirty-two out of 64 subjects (50%) had MWAO, 18 (28.1%) had MD and 14 (21.9%) had headache not classifiable (HnC). MWOA persists in 56.2%, becomes MD and HnC in 9.4% and 3.1% of cases respectively, changes to episodic tension-type headache (ETTH) in 12.5%, and remits in 18.8%. MD persists in 11.1%, becomes MWOA and HnC in 27.8% and 5.5% of cases respectively, changes to ETTH in 11.1, and remits in 44.5%. HnC persists in 14.3%, be…

Migraine and Sport in a Physically Active Population of Students: Results of a Cross‐Sectional Study

Abstract Objective: In this study, we explored the relationship between migraine and sport in a physically active population of students, analyzing the risk of migraine among sporty students. Background: The relationship between sport and migraine is controversial; moreover, several studies report on sport as a migraine trigger, but there is evidence that physical activity could have a relevant role in migraine prevention. Methods: A cross-sectional survey was conducted using the validated ID-migraine questionnaire including specific demo-anthropometric (gender, age, weight, height) and sports variables on a potentially active student population of the University of Palermo. Evaluation in p…

Are paediatric headaches in the emergency department increasing? An Italian experience

The aim of this study was to assess admissions, for headache, to the emergency department (ED) of the Di Cristina Children’s Hospital in Palermo over a decade. The total number of ED admissions for headache was retrospectively analysed considering two 24-month periods: 2009-2010 and 2017-2018. Total admissions to the ED decreased from 55,613 to 50,096 (-10%) between the two periods considered, while the number of admissions for headache increased by 63.56% (p < 0.0001). There was also a significant increase in the number of multiple ED admissions by single children (9.5% versus 17.98% of the patients accessing the ED for headache). This significant increase in admissions for paediatric h…

Migraine in a pediatric population: a clinical study in children younger than 7 years of age.

Aim Migraines in children younger than 7 years of age have received limited attention in the published literature. The aim of this study is to describe the characteristics of migraine phenotypes in children younger than 7 years, and to compare them with migraines in children older than 7 years of age. Method We reviewed all standard clinical files, collected over 4 years, related to children with a diagnosis of primary headache. We included all children younger than 7 years diagnosed with migraine in our study. Results A total of 374 children (188 males, 186 females) were affected by migraine with/without aura: 40 of these patients (10.7%; 20 males, 20 females; mean age 5y 7mo, SD 1y 2mo) w…

Idiopathic Non-Dental Facial Pain Syndromes in Italian Children: A Clinical Case Series

Background. The orofacial pain syndromes (OFPs) are a heterogeneous group of syndromes characterized by painful attacks involving the orofacial structures. They may be summarily subdivided into two great categories: (1) orofacial pain mainly attributed to dental disorders such as dentoalveolar and myofascial orofacial pain or temporomandibular joint (TM) pain; (2) orofacial pain mainly attributed to non-dental pain as neuralgias, facial localization of primary headaches or idiopathic orofacial pain. The second group is uncommon, often described by single case reports, can often show overlapping symptoms with the first group, and represents a clinical challenge, carrying the risk of underval…

Trigeminal Neuralgia and Cerebellopontine‐Angle Lipoma in a Child

Trigeminal neuralgia and cerebellopontine-angle lipomas are very rare in children. We describe the history and findings of an 8-year-old boy with right trigeminal neuralgia and a lipoma detected by magnetic resonance imaging at the level of the root-entry zone of the right seventh cranial nerve. We propose a possible mechanism of infiltration of the trigeminal rootlets by the lipoma.

Pediatric haedaches epidemiology in emergency department during COVID-19

Background and aims Recent studies have showed that in emergency department (ED) pediatric admissions for headache are increasing in the last years. However Covid-19 pandemic may have changed the use of health services for several reasons. Aim of this study is to analyze the rates of admission for pediatric headaches in ED before and during Covid19 Pandemic. Methods we have collected retrospectively the records of children (range of age 5–14) admitted on ED in 2012, 2019 and 2020. We selected the records including Headache and Headache associated to other symptoms (vomit, fever, dizziness, etc.), collecting further the use of computed tomography (CT) and neurological consultation. Results I…

Migraine and handedness.

Migraine is a typically unilateral disorder in adulthood; however, the reasons for painful lateralization have been little investigated. The possible influence of manual dominance was suggested. We aimed to investigate the localization of pain in migraine attacks in right-handed and left-handed subjects. The retrospective study collected 546 patients with migraine aged between 16 and 65 years, reporting the manual dominance to the Edinburgh test. We included 466 right-handed and 80 left-handed subjects with migraine. We registered 4215 unilateral painful attacks. The right-handers had 3412 unilateral episodes; 62.8% of the attacks were characterized by pain on the right side and 37.2% by pa…

Red Ear Syndrome and Migraine: Report of Eight Cases

We describe eight idiopathic cases of red ear syndrome in seven children and one adult. All were migraineurs with a history of paroxysmally painful and red ear, unilateral or alternating, in isolation or associated with migraine attacks. The reported duration of these episodes varied from 30 minutes to 1 hour. Neurologic examination, brain MRI and CT scans, and x-rays of the cervical spine were normal. The close temporal relationship between the "red ear episodes" and migraine attacks suggests an association between the two conditions.

Two distinct phenotypes, hemiplegic migraine and episodic Ataxia type 2, caused by a novel common CACNA1A variant

Abstract Background To investigate the genetic and environmental factors responsible for phenotype variability in a family carrying a novel CACNA1A missense mutation. Mutations in the CACNA1A gene were identified as responsible for at least three autosomal dominant disorders: FHM1 (Familial Hemiplegic Migraine), EA2 (Episodic Ataxia type 2), and SCA6 (Spinocerebellar Ataxia type 6). Overlapping clinical features within individuals of some families sharing the same CACNA1A mutation are not infrequent. Conversely, reports with distinct phenotypes within the same family associated with a common CACNA1A mutation are very rare. Case presentation A clinical, molecular, neuroradiological, neuropsy…