DOCSLIB.ORG

The Red Ear Syndrome Giorgio Lambru, Sarah Miller and Manjit S Matharu*

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
The Red Ear Syndrome Giorgio Lambru, Sarah Miller and Manjit S Matharu* Lambru et al. The Journal of Headache and Pain 2013, 14:83 http://www.thejournalofheadacheandpain.com/content/14/1/83 REVIEW ARTICLE Open Access The red ear syndrome Giorgio Lambru, Sarah Miller and Manjit S Matharu* Abstract Red Ear Syndrome (RES) is a very rare disorder, with approximately 100 published cases in the medical literature. Red ear (RE) episodes are characterised by unilateral or bilateral attacks of paroxysmal burning sensations and reddening of the external ear. The duration of these episodes ranges from a few seconds to several hours. The attacks occur with a frequency ranging from several a day to a few per year. Episodes can occur spontaneously or be triggered, most frequently by rubbing or touching the ear, heat or cold, chewing, brushing of the hair, neck movements or exertion. Early-onset idiopathic RES seems to be associated with migraine, whereas late-onset idiopathic forms have been reported in association with trigeminal autonomic cephalalgias (TACs). Secondary forms of RES occur with upper cervical spine disorders or temporo-mandibular joint dysfunction. RES is regarded refractory to medical treatments, although some migraine preventative treatments have shown moderate benefit mainly in patients with migraine-related attacks. The pathophysiology of RES is still unclear but several hypotheses involving peripheral or central nervous system mechanisms have been proposed. Keywords: Red ear syndrome; Migraine; Trigemino-autonomic reflex; Trigeminal autonomic cephalalgias; Parasympathetic system; Erythromelalgia Review The disorder has a slight female preponderance (54 Introduction women, 47 men), with a male to female ratio of 1:1.25. Red Ear Syndrome (RES) is a rare condition originally Interestingly, though, in the two series of young mi- described by Lance in 1994 [1] and further characterized graineurs with RES described by Raieli et al. [5,13], the in 1996 [2]. Since the initial description, approximately majority of patients were males (75% and 68% of patients 100 cases have been described in the literature [3-21]. respectively). From the available data (ages of onset not The syndrome itself has not been clearly defined. Apart provided by Raieli et al. [13]), the median age of onset of from its unique clinical presentation, characterised by RES is 44 years old, although a wide range of 4 to 92 years episodes of burning pain and reddening of the external is reported. ear, the condition lacks definition in terms of aetiology, pathophysiology and treatments. It is for these reasons Clinical features that it has yet to be included in the revised International Site of pain Classification of Headache Disorders (ICHD-IIIβ) [22]. Pain is felt over the pinna of the ear, often maximal on the This article reviews the case descriptions, pathophysio- ear lobe. It can radiate towards the cheek and mandible logical theories and effective treatments reported in the [16,20], as well as towards the occiput [4] and the involve- literature since the description of the first case in 1994. ment of the V1 area or whole hemicranium has also been described [1,10,12]. Epidemiology The prevalence and incidence of RES is unknown, al- Laterality of attack though the low number of reported cases would suggest From the original description, RE episodes were thought that it is rare. There are 100 relatively complete case de- to be predominantly unilateral and side-locked [1]. Subse- scriptions of RES found in the English language literature. quently, cases of unilateral attacks occurring on either side in the same patient [16] as well as bilateral attacks have * Correspondence: [email protected] Institute of Neurology and The National Hospital for Neurology and been described [5,11,17]. In total, 62 patients (62%) had Neurosurgery, Queen Square, London WC1N 3BG, UK strictly unilateral attacks, 31 (31%) bilateral attacks and 6 © 2013 Lambru et al.; licensee Springer. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Lambru et al. The Journal of Headache and Pain 2013, 14:83 Page 2 of 9 http://www.thejournalofheadacheandpain.com/content/14/1/83 (6%) either unilateral or bilateral attacks. Data is missing RE episodes occur most commonly during the daytime, for one patient. although Lance reported a case in whom RE attacks had Among patients with unilateral RE episodes, the pain is awakened the patient from sleep [2]. reported more frequently on the left (58%) than the right In most patients, RES seems to be a chronic problem side (42%) (left-sided attacks: 30 patients; right-sided at- without significant remission periods, although, in some tacks: 22 patients; data not available for 10 patients). cases the follow-up was too short to allow any meaningful conclusion to be drawn [7,15]. Some case reports clearly describe patients with episodic bouts of RES attacks al- Severity of pain ternating with remission periods. Donnet and Valade de- The degree of intensity of the pain episodes in RES has scribed a 92-year-old woman who experienced attacks of not been properly assessed. Generally, it is reported as an burning pain and red ear associated with lacrimation fol- annoying discomfort rather than an excruciating pain. lowing an episodic pattern. The episodes were short- However, authors do also report cases of patients com- lasting, occurring in bouts of daily attacks lasting 15 to plaining of moderate to severe attacks. Lambru et al. de- 45 days every 12–18 months [6]. Similarly, the case of a scribed a case of a patient with SUNA (Short-lasting 36-year-old woman who complained of episodes of ear, unilateral neuralgiform headache attacks with autonomic temple, cheek and upper neck pain associated with ear symptoms) and RES occurring on the same side. The se- redness and other autonomic symptoms such as conjunc- verity of the RE episodes were felt by the patient to be tival injection, tearing and nasal blockage lasting between moderate (VRS: 5/10) compared to the excruciating inten- 10–60 minutes was described. She had daily attacks occur- sity of the SUNA attacks (VRS: 8-10/10), suggesting a ring in “clusters”, presumably alternating with remission lower degree of severity of the pain of an RE episode com- periods [16]. Finally Boulton et al. described the case of a pared to that typical of a TAC attack [23]. However, some 66 year-old woman with a five year history of episodes of authors have reported cases of patients complaining of se- pain behind the left ear and ear redness, initially occurring vere pain. Boes et al. described a patient with episodes of in bouts of daily attacks lasting 1–2 weeks, followed by re- left ear pain attacks associated with reddening that the pa- missions lasing months but that subsequently became al- tient described as severe. The attacks were suppressed most daily [10]. completely with indometacin 75 mg/day, supporting a diagnosis of paroxysmal hemicrania-associated RES [3]. Associated features The key clinical feature of RES is the presence of burning Character of pain ear pain associated with marked ear reddening although Patients consistently described the pain as a burning sen- Hirsch reported five patients with episodes of bright red sation. Other characters that have been less often reported discoloration of the ears not associated with auricular pain include a dull ache [1,4], a stabbing pain [2,3], a sharp pain [18]. The erythema of the ear consistently follows the ear [2,10] and a jabbing pain [2]. pain and usually lasts as long as the burning pain does. Rare exceptions to this pattern exist. Selekler et al. de- scribed the case of a patient in whom the discoloration of Duration of the individual attacks the ear would disappear within 10 min from the onset of The duration of each RE episode can vary widely. Gener- the attack, whereas the burning pain would continue for a ally, the attacks are short-lasting with 63 RES patients further 5–10 minutes [11] and Boulton et al. reported a (63%) reporting attacks lasting up to four hours. The case of a 66 year-old woman in whom episodes of pain be- majority had attacks lasting 30 to 60 minutes [2,5,16]. hind the left ear were accompanied by reddening of the ip- However, short-lived attacks lasting seconds have been silateral ear only 50% of the time [10]. reported [7]. Only six patients (6%) had attacks lasting The area of reddening is usually limited to the external over 4 hours of whom two complained of constant pain ear and is most pronounced over the earlobe [2,5]. Simi- [2]. Data on duration of attacks in 32 patients was not larly to the pain pattern, the redness can extend beyond available from the case reported. the ear, most frequently involving the cheek [2,16]. In- volvements of the temple, upper neck and rarely the entire Attack frequency and remission periods face ipsilaterally to the affected ear have also been The attack frequency varies immensely both among suf- reported [2,12,16]. Given the complex sensory and auto- ferers and within individual sufferers. The majority of RES nomic innervation of the external ear, it would be import- patients report daily attacks ranging from one daily to up ant to obtain more precise information on the area of to 20 attacks a day [9]. A small minority of patients only reddening to help clarify the pathophysiological mecha- report infrequent attacks that occur 1 to 6 times per nisms at play. Unfortunately, besides some descriptions of month [5]. redness involving only the earlobe and one case in whom Lambru et al.
Load more

Recommended publications
  • Some Rare Headache Disorders, Including Alice in Wonderland Syndrome, Blip Syndrome, Cardiac Cephalalgia, Epicrania Fugax, Explo
    Some rare headache disorders, including Alice in Wonderland syndrome, blip syndrome, cardiac cephalalgia, epicrania fugax, exploding head syndrome, Harlequin syndrome, lacrimal neuralgia, neck–tongue syndrome, and red ear syndrome Oxford Textbook of Headache Syndromes Michel Ferrari, Andrew Charles, David Dodick, Fumihiko Sakai, and Joost Haan Publisher: Oxford University Press Print Publication Date: Apr 2020 Print ISBN-13: 9780198724322 Published online: Apr 2020 DOI: 10.1093/med/ 9780198724322.001.0001 Some rare headache disorders, including Alice in Wonderland syndrome, blip syndrome, cardiac cephalalgia, epicrania fugax, exploding head syndrome, Harlequin syndrome, lacrimal neuralgia, neck–tongue syndrome, and red ear syndrome Chapter: Some rare headache disorders, including Alice in Wonderland syndrome, blip syndrome, cardiac cephalalgia, epicrania fugax, exploding head syndrome, Harlequin syndrome, lacrimal neuralgia, neck–tongue syndrome, and red ear syndrome Author(s): Randolph W. Evans DOI: 10.1093/med/9780198724322.003.0028 Introduction Alice in Wonderland syndrome, blip syndrome, cardiac cephalalgia, epicrania fugax, exploding head syndrome, Harlequin syndrome, lacrimal headache, neck–tongue syndrome, and red ear syndrome are among the fascinating array of rare headache disorders. James W. Lance, Juan A. Pareja, and colleagues have first described or named six of them. Certainly, there are patients with additional rare headaches just waiting to be described by astute observers. Page 1 of 23 Some rare headache disorders, including Alice in Wonderland syndrome, blip syndrome, cardiac cephalalgia, epicrania fugax, exploding head syndrome, Harlequin syndrome, lacrimal neuralgia, neck–tongue syndrome, and red ear syndrome In case of a rare headache disorder, an appropriate diagnosis and treatment can be most reassuring to the patient. Alice in Wonderland syndrome History In 1952, Caro W.
    [Show full text]
  • Paroxysmal Hemicrania
    P1: KWW/KKL P2: KWW/HCN QC: KWW/FLX T1: KWW GRBT050-97 Olesen- 2057G GRBT050-Olesen-v6.cls August 17, 2005 1:30 ••Chapter 97 ◗ Paroxysmal Hemicrania Christopher J. Boes, Maurice Vincent, and David Russell PAROXYSMAL HEMICRANIA Sex Distribution PH was originally considered to be a female disease. Male International Headache Society (IHS) code and diag- cases, however, clearly occur (46,48). In initial series there nosis: was a female:male ratio of 7:1, but a 1989 review re- 3.2 Paroxysmal hemicrania ported a female:male ratio of 2.36:1 (1). Comprehensive 3.2.1 Episodic paroxysmal hemicrania and methodologically well-designed epidemiologic stud- 3.2.2 Chronic paroxysmal hemicrania ies are required to establish the actual extent of the female World Health Organization (WHO) code and diagnosis: preponderance in PH. G44.03 Chronic paroxysmal hemicrania Short description: Paroxysmal hemicrania (PH) suffer- ers have attacks with similar characteristics of pain Age of Onset and associated symptoms and signs to those of cluster PH may begin at any time, but its onset usually occurs headache (CH), but they are shorter lasting, are more during adulthood at the mean age of 34 years. The youngest frequent, occur more commonly in females, and re- age at onset was 1 year and the oldest 81 years (1,12). In spond absolutely to indomethacin. In episodic paroxys- the reviewed material from 1989, the mean age at diagnosis mal hemicrania (EPH), attacks occur in periods lasting was 47 and the mean illness duration 13 years. As far as 7 days to 1 year separated by pain-free periods lasting the subtypes are concerned, EPH seems to begin earlier 1 month or longer.
    [Show full text]
  • Red Ear Syndrome – Case Report and Review
    n e u r o l o g i a i n e u r o c h i r u r g i a p o l s k a 4 9 ( 2 0 1 5 ) 7 4 – 7 7 Available online at www.sciencedirect.com ScienceDirect journal homepage: http://www.elsevier.com/locate/pjnns Case report Red ear syndrome – Case report and review of literature Jedrzej Flicinski *, Jadwiga Wigowska-Sowinska, Anna Winczewska-Wiktor, Barbara Steinborn Chair and Department of Developmental Neurology, Poznan University of Medical Sciences, Poznan, Poland a r t i c l e i n f o a b s t r a c t Article history: Red ear syndrome is characterized by: paroxysmal, unilateral, recurrent pain, redness and Received 22 February 2014 discomfort of the ear lobe accompanied by a burning sensation. The duration and frequency Accepted 5 January 2015 of red ear syndrome attacks is very various and the episodes, usually occur spontaneously. Available online 14 January 2015 The pathophysiology is still unknown and also there are no medications with approved efficacy. The goal of this brief report is to present a 11-year old girls whose symptoms of red Keywords: ear syndrome preceded migraine without aura and the signs of redness of the ear occurred in clusters. The occurrence of symptoms of our case may have confirmed the observation that Red ear syndrome Headache red ear syndrome is associated with primary headaches particularly migraine and cluster headaches. The literature on this case report of pediatric idiopathic red ear syndrome has Great auricular nerve Migraine been reviewed.
    [Show full text]
  • A Dictionary of Neurological Signs.Pdf
    A DICTIONARY OF NEUROLOGICAL SIGNS THIRD EDITION A DICTIONARY OF NEUROLOGICAL SIGNS THIRD EDITION A.J. LARNER MA, MD, MRCP (UK), DHMSA Consultant Neurologist Walton Centre for Neurology and Neurosurgery, Liverpool Honorary Lecturer in Neuroscience, University of Liverpool Society of Apothecaries’ Honorary Lecturer in the History of Medicine, University of Liverpool Liverpool, U.K. 123 Andrew J. Larner MA MD MRCP (UK) DHMSA Walton Centre for Neurology & Neurosurgery Lower Lane L9 7LJ Liverpool, UK ISBN 978-1-4419-7094-7 e-ISBN 978-1-4419-7095-4 DOI 10.1007/978-1-4419-7095-4 Springer New York Dordrecht Heidelberg London Library of Congress Control Number: 2010937226 © Springer Science+Business Media, LLC 2001, 2006, 2011 All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, LLC, 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made.
    [Show full text]
  • A Patient's Guide To
    A Patient’s Guide to Erythromelalgia Our mission TEA’s mission is to empower all those affected by EM to improve their quality of life. TEA provides education, awareness and community through our website, member services program, newsletter, social media presence and networking programs. TEA promotes research through relationships with academic researchers, clinicians and pharma companies with interest in EM. TEA also raises funds to support EM research. 200 Old Castle Lane Wallingford, PA 19086 USA 610-566-0797 (messages only) [email protected] www.facebook.com/erythromelalgia www.erythromelalgia.org A Patient’s Guide to Erythromelalgia TABLE OF CONTENTS What is erythromelalgia?. .1 How common is EM?. 2 What are the different types of EM?. 2 How is EM diagnosed? . 3 Treatments for EM. 5 Coping with EM. .12 Looking ahead to the future. 19 References . .20 What is erythromelalgia? Erythromelalgia (EM) is a rare neurovascular condition that most commonly affects the feet, but may also occur in the hands, face, or other parts of the body. The term neurovascular means that both nerves and blood vessels are involved. The three hallmark symptoms of EM are: • Redness: This skin-color change is caused by excessive blood flow to the affected area. The medical term is erythema. • Heat: Skin will feel warm or hot to the touch due to the increased blood flow. • Pain: Discomfort may range from mild tingling to severe burning. For some, it can also involve sensations such as pins and needles or itching. Additional symptoms may include: • Swelling: The increased blood flow may cause a buildup of fluid in the affected body part.
    [Show full text]
  • A Review of Headache
    VIII HEADACHE Randolph W. Evans, MD Headaches are a near-universal experience, with a 1-year prevalence of 90% and a lifetime prevalence of 99%. Each Table 1 Major Categories of Headache year in the United States, 9% of adults see physicians for Disorders1 headaches and 83% self-medicate. Headaches are one of Primary Headaches the most common complaints of patients seen by primary Migraine care physicians and account for 20% of outpatient visits to Tension-type headache Cluster headache and chronic paroxysmal hemicrania neurologists. Miscellaneous headaches unassociated with structural lesion: The differential diagnosis of headaches is one of the idiopathic stabbing, external compression, cold stimulus, longest in medicine, with over 300 different types and benign cough, benign exertional, associated with sexual causes. Although most headaches are of benign (and still activity poorly understood) origin, some headaches can have serious Secondary Headaches and even potentially life-threatening causes. Thus, it is Headache associated with head trauma critical for the physician to diagnose headaches as precisely Headache associated with vascular disorder: acute ischemic as possible. cerebrovascular disorder, intracranial, hematoma, subarachnoid hemorrhage, unruptured vascular malforma- The International Headache Society (IHS) criteria, which tion, arteritis, carotid or vertebral artery pain, venous were introduced in 1988 and updated in 2004 (International thrombosis, arterial hypertension, associated with other Classifi cation of Headache Disorders Second Edition [ICHD-2]),1 vascular disorder are the worldwide standard for headache classifi cation. Headache associated with nonvascular intracranial disorder: high and low cerebrospinal fl uid pressure, intracranial IHS criteria categorize headaches as primary or secondary. infection, intracranial sarcoidosis and other noninfectious Primary headaches—those with no other underlying cause— infl ammatory disease, related to intrathecal injections, account for 90% of headaches.
    [Show full text]
  • Red Ear Syndrome – Chronic Pain Syndrome of Low Prevalence
    Acta Clin Croat 2010; 49:327-329 Case Report RED EAR SYNDROME – CHRONIC PAIN SYNDROME OF LOW PREVALENCE Duška Šklebar1, Ivan Šklebar2, Dragica Vrabec-Matković3 and Ljiljana Čengić4 1 Department of Neurology, 2Outpatient Pain Clinic, Bjelovar General Hospital, Bjelovar; 3Special Hospital for Medical Rehabilitation, Varaždinske Toplice; 4Department of Neurology, Vinkovci General Hospital, Vinkovci, Croatia SUMMARY – A case is described of a 72-year-old woman that presented to our outpatient neurology clinic complaining of relapsing attacks of severe pain in and around the left ear and ipsila- teral side of the face, accompanied by objectively visible cutaneous erythema with burning sensation throughout the attack. There was no alteration in her neurologic status. Comprehensive tests were done to exclude the possible primary diseases. The findings were within the limits of physiological values. Attempts at treating the pain with various analgesics and a combination of analgesics and first generation anticonvulsants did not prove effective. The pain was slightly relieved with prega- balin in maximum dosage. Because of various potential pathophysiological mechanisms, literature does not indicate any unique potential treatment approach, and some patients are resistant to any kind of therapy. Key words: Ear diseases – complications; Ear diseases – physiopathology; Erythema – etiology; Erythe- ma – pathology; Erythema – therapy; Syndrome Introduction thalamic syndrome2, part of trigeminal autonomic ce- phalgia, and may be, even without obvious structural Since 1994, when “the mystery of one red ear” was cause, precipitated by touch, movements, exposure to originally described in the literature1, and 1996, when 2 cold or warmth. It is more common in people suffer- the term ‘red ear syndrome (RES) was coined, about ing from migraine attacks5.
    [Show full text]
  • Red Ear Syndrome: a Case Presentation and Discussion Author Affiliations Are Listed at the End of This Article
    Brackenrich et al. HCA Healthcare Journal of Medicine (2020) 1:3 https://doi.org/10.36518/2689-0216.1053 Case Report Red Ear Syndrome: A Case Presentation and Discussion Author affiliations are listed at the end of this article. Jared Brackenrich, DO,1 Samantha Sternad, DO,1 Chad Johnston, DO2 Correspondence to: Abstract Jared Brackenrich, DO Introduction LewisGale Hospital - Mont- Red Ear Syndrome (RES) is a condition often considered to be a localized form of erythro- gomery melalgia. It can be related to structural cervical defects or idiopathic. RES is generally very 3700 S Main St difficult to treat. Blacksburg, VA 24060 ([email protected]) Discussion A 57-year-old male presented to the dermatology clinic complaining of a 4-month history of intermittent redness and severe burning of bilateral ears. On examination, the patient exhib- ited edematous erythema and tenderness to palpation affecting the right and left ear and right malar cheek. A skin biopsy revealed mild superficial perivascular lymphocytic infiltrate with hypertrophy of endothelial cells. The patient was found to have a normal lab work-up including complete blood count, metabolic panel, erythrocyte sedimentation rate, anti-nu- clear antibody and type II collagen antibody. A diagnosis of Red Ear Syndrome was made. After failing multiple medications over several months, the patient was started on aspirin and paroxetine which was gradually titrated until he was completely asymptomatic. To date, there is only one other case presentation illustrating the effectiveness of this treatment regimen. Conclusion There are a limited number of cases describing idiopathic RES with inconsistent results in treatment.
    [Show full text]
  • Coexistence of Migraine Headache and Red Ear Syndrome Kırmızı Kulak Sendromu Ile Birliktelik Gösteren Migren Baş Ağrısı
    Letter to the Editor / Editöre Mektup Doi: 10.4274/npa.y7619 Arc hi ves of Neu ropsy chi atry 2014; 51: 294 Nö rop si ki yat ri Ar fli vi 2014; 51: 294 294 Coexistence of Migraine Headache and Red Ear Syndrome Kırmızı Kulak Sendromu ile Birliktelik Gösteren Migren Baş Ağrısı Ali Ulvi UCA, Hasan Hüseyin KOZAK Necmettin Erbakan University Meram Faculty of Medicine, Department of Neurology, Konya, Turkey Dear Editor, some cases with the use of gabapentin, amitriptyline, imipramine, flunarizine, propranolol, verapamil and pregabalin (6). Red Ear Syndrome (RES) is a rare condition originally RES could be considered a potential accompaniment to described by Lance (1). RES episodes are characterised by migraine. Rarely, RES symptoms conceivably may represent the unilateral or bilateral attacks of paroxysmal burning sensations only clinical manifestations of an acute migraine attack (2). RES and reddening of the external ear. The duration of these episodes associated with migraine occurs more commonly in childhood, ranges from a few seconds to several hours. The attacks occur whereas the secondary forms prevail in later years. Amitriptyline with a frequency ranging from several daily to a few per year. treatment was found to be efficient in our case, compatible with An 11-year-old boy reported a 3-year history of migraine the literature. Hence, amitriptyline can be preferred for treatment without aura. During the previous 6 months, he had experienced in migraine headache patients with RES. episodes of painful burning in his right ear, lasting less than 30 minutes, and occurring three to five times per month; approximately half of these episodes were immediately followed by an acute References migraine attack.
    [Show full text]
  • Facial Pain in Childhood and Adolescence
    ISSN: 2689-3193 NEURO RESEARCH Review Article 2019; 1(1): 1 Facial Pain in Childhood and Adolescence D’Amico A¹, Quatrosi G¹, Lo Verde M¹, Bettiol C¹, Raieli V² ¹Child Neuropsychiatry School - University of Palermo, Piazzale delle Cliniche, Palermo, Italy ²Child Neuropsychiatry Unit - ISMEP - P.O. Cristina - ARNAS Civico, Via dei Benedettini 1, Palermo, Italy *Corresponding author: ABSTRACT Vincenzo Raieli Background: Facial pain syndromes are very disabling conditions. They are uncommon in children and adolescents Via dei Benedettini 1 - PO Di Cristina 90100 Palermo, and a lot of paediatric tests do not address them; also in Italy. literature only few cases are reported. A practitioner’s appropriate knowledge is very important in order to avoid misdiagnosis. Also there are symptomatic forms Received : June 15, 2019 that are secondary to other causes like tumors or vascular Published : August 12, 2019 malformation. Methods: This review updates the trigeminal neuralgia, nervous intermedius neuralgia, glossopharyngeal neuralgia, occipital nerve neuralgia, Bell’s palsy, red ear syndrome, neck- tongue syndrome, trigeminal autonomic cephalalgias, cluster headache, paroxysmal hemicrania, SUNA and SUNCT and the persistent and idiopathic facial pain. Conclusion: Knowledge of the clinical features of these facial pain syndromes in children allows physicians to establish the correct diagnosis and develop the optimal treatment plan. KEYWORDS: Red Ear Syndrome; Facial Pain; Childhood; Adolescence; Neuralgias. INTRODUCTION In this review we focused our attention on original articles reporting clinical features, etiology and treatment in pediatric Facial pain syndromes are a heterogeneous group of population. conditions characterized by pain in craniofacial district. On the basis of pain features (expression, duration, localization, etc.), Typical facial pain we distinguish typical and atypical facial pain syndromes.
    [Show full text]
  • Current Pain Management Strategies for Patients with Erythromelalgia: a Critical Review
    Journal name: Journal of Pain Research Article Designation: Review Year: 2018 Volume: 11 Journal of Pain Research Dovepress Running head verso: Tham and Giles Running head recto: Current pain management strategies for patients with erythromelalgia open access to scientific and medical research DOI: http://dx.doi.org/10.2147/JPR.S154462 Open Access Full Text Article REVIEW Current pain management strategies for patients with erythromelalgia: a critical review See Wan Tham1,2 Abstract: Erythromelalgia (EM) is a rare disorder characterized by erythematous, warm, Marian Giles1,2 painful extremities, which is often precipitated by cold conditions. The pathophysiology of EM is incompletely understood. Recent investigations have identified sodium channelopathy 1Seattle Children’s Research Institute, Seattle, WA, USA; 2Department of as a genetic cause for this pain condition, classified as primary inherited EM. Other subtypes Anesthesiology and Pain Medicine, are idiopathic EM and secondary EM. The management of pain in EM is challenging as no University of Washington School of single therapy has been found to be effective. There is varying response to pharmacotherapy Medicine, Seattle, WA, USA and significant variability within this clinical population, resulting in a stepwise trial and error approach. Consequently, EM is often associated with poorer health-related quality of life with higher morbidity. There is currently no consensus or guidelines on management of pain in EM. This is a review of the literature on management of pain using pharmacologic,
    [Show full text]