Severe headache in primary Sjögren's syndrome treated with intrathecal rituximab

A severe and persistent migrainous headache in a patient with primary Sjøgren's syndrome unresponsive to treatment with immunosuppressive drugs, triptans, opioids, and NSAIDs, responded successfully to intrathecal B‐cell depletion with rituximab. We hypothesize that brain‐resident autoreactive B cells were involved in headache pathogenesis and were eliminated by this procedure.publishedVersio

A REVIEW ON CLASSIFICATION, PATHOPHYSIOLOGY, DIAGNOSIS, AND PHARMACOTHERAPY OF HEADACHE

Headache disorders, characterized by recurrent headache, are among the most common disorders of the nervous system. Headache disorder is classified mainly into two major types, primary headache and secondary headache by the International Classification of Headache Disorders. Most types of headache are diagnosed by the clinical history and from headache classification committee of the International Headache Society (IHS). A number of intrinsic or extrinsic factors can trigger headache attack which release neurotransmitters and activate trigeminal vascular system. The grading of headache intensity is done by headache severity scale of IHS. Headache management includes pharmacological and non-pharmacological treatment

Migraine

Migrena je primarna glavobolja, čija prevalencija u općoj populaciji Europske unije iznosi 15%. Može se javiti epizodički, a može biti i kronična. Klinički je karakterizirana onesposobljavajućom, unilateralnom, pulsirajućom glavoboljom, trajanja 4 do 72 sata, praćenom mučninom i povraćanjem te fotofobijom, fonofobijom i osmofobijom. Godišnji troškovi migrene, direktni vezani uz dijagnostiku i terapiju te indirektni vezani uz izostanke s posla, procjenjuju se u Europskoj uniji na 5 milijardi eura. Stoga migrena predstavlja ne samo medicinski, već i socioekonomski problem. Točan uzrok migrenske glavobolje nije poznat, a zbog učestale pozitivne obiteljske anamneze smatra se da su u podlozi i genetski čimbenici. Prema današnjem shvaćanju u patogenezi migrenske glavobolje središnje mjesto zauzima aktivacija trigeminovaskularnog sustava s posljedičnom neurogenom inflamacijom i dilatacijom intrakranijskih krvnih žila inerviranih od C vlakana trigeminalnog živca. Dijagnoza migrene postavlja se na temelju kliničke slike i simptoma, a prema kriterijima Međunarodnog društva za glavobolju (International Headache Society) svrstava se u jednu od podvrsta - migrenu bez aure i migrenu s aurom. Terapija migrene je prema preporukama Europske federacije neuroloških društava (European Federation of Neurological Societies) abortivna – usmjerena na prekidanje glavobolje i ublažavanje pratećih simptoma te profilaktička – usmjerena prevenciji nastanka istih. U abortivnoj terapiji mogu se koristiti lijekovi iz skupina nesteroidnih antiinflamatornih lijekova ili triptana, dok se u profilaktičkoj terapiji koriste lijekovi iz skupina antihipertenziva, antidepresiva ili antiepileptika. Osim farmakoterapije, u liječenju odnosno prevenciji migrene mogu se koristiti komplementarne metode kao što su promjena životnih navika, biofeedback, akupunktura i transkutana električna stimulacija živaca.Migraine is a primary headache, whose prevalence in the general population of the European Union is 15%. It can be episodic but it can also be a chronic condition. It is characterized by a unilateral, pulsating headache and can last between 4 and 72 hours. Associated symptoms may include nausea, vomiting and sensitivity to light, sound or smell. The direct and indirect annual costs of migraine are estimated to be 5 billion euros in the European Union. Thus, migraine is not only medical but also a socio-economic problem. The exact cause of migraine headaches is unknown, but frequent positive family history indicates a connection with genetic factors. The central place in the pathogenesis of migraine belongs to the activation of the trigeminovascular system, with consequent vasodilatation and neurogenic inflammation of intracranial blood vessels that are innervated by C fibers from the trigeminal nerve. The diagnosis of migraine should be based on clinical presentation and symptoms, and pursuant to the criteria set by the International Headache Society, it may be more closely classified as one of the types of migraine – migraine without aura and migraine with aura. According to the European Federation of Neurological Societies, the treatment of migraine can be abortive - the removal and alleviation of symptoms, and prophylactic - prevention of the occurrence of symptoms. Non-steroidal anti-inflammatory drugs or triptans are used in the abortive therapy, and antihypertensive medication, antidepressants or antiepileptics are used in prophylactic therapy. In addition to pharmacotherapy, complementary methods, such as changing habits, biofeedback, acupuncture and transcutaneous electrical nerve stimulation, can also be used to treat or prevent migraine headache

Migraine

Migrena je primarna glavobolja, čija prevalencija u općoj populaciji Europske unije iznosi 15%. Može se javiti epizodički, a može biti i kronična. Klinički je karakterizirana onesposobljavajućom, unilateralnom, pulsirajućom glavoboljom, trajanja 4 do 72 sata, praćenom mučninom i povraćanjem te fotofobijom, fonofobijom i osmofobijom. Godišnji troškovi migrene, direktni vezani uz dijagnostiku i terapiju te indirektni vezani uz izostanke s posla, procjenjuju se u Europskoj uniji na 5 milijardi eura. Stoga migrena predstavlja ne samo medicinski, već i socioekonomski problem. Točan uzrok migrenske glavobolje nije poznat, a zbog učestale pozitivne obiteljske anamneze smatra se da su u podlozi i genetski čimbenici. Prema današnjem shvaćanju u patogenezi migrenske glavobolje središnje mjesto zauzima aktivacija trigeminovaskularnog sustava s posljedičnom neurogenom inflamacijom i dilatacijom intrakranijskih krvnih žila inerviranih od C vlakana trigeminalnog živca. Dijagnoza migrene postavlja se na temelju kliničke slike i simptoma, a prema kriterijima Međunarodnog društva za glavobolju (International Headache Society) svrstava se u jednu od podvrsta - migrenu bez aure i migrenu s aurom. Terapija migrene je prema preporukama Europske federacije neuroloških društava (European Federation of Neurological Societies) abortivna – usmjerena na prekidanje glavobolje i ublažavanje pratećih simptoma te profilaktička – usmjerena prevenciji nastanka istih. U abortivnoj terapiji mogu se koristiti lijekovi iz skupina nesteroidnih antiinflamatornih lijekova ili triptana, dok se u profilaktičkoj terapiji koriste lijekovi iz skupina antihipertenziva, antidepresiva ili antiepileptika. Osim farmakoterapije, u liječenju odnosno prevenciji migrene mogu se koristiti komplementarne metode kao što su promjena životnih navika, biofeedback, akupunktura i transkutana električna stimulacija živaca.Migraine is a primary headache, whose prevalence in the general population of the European Union is 15%. It can be episodic but it can also be a chronic condition. It is characterized by a unilateral, pulsating headache and can last between 4 and 72 hours. Associated symptoms may include nausea, vomiting and sensitivity to light, sound or smell. The direct and indirect annual costs of migraine are estimated to be 5 billion euros in the European Union. Thus, migraine is not only medical but also a socio-economic problem. The exact cause of migraine headaches is unknown, but frequent positive family history indicates a connection with genetic factors. The central place in the pathogenesis of migraine belongs to the activation of the trigeminovascular system, with consequent vasodilatation and neurogenic inflammation of intracranial blood vessels that are innervated by C fibers from the trigeminal nerve. The diagnosis of migraine should be based on clinical presentation and symptoms, and pursuant to the criteria set by the International Headache Society, it may be more closely classified as one of the types of migraine – migraine without aura and migraine with aura. According to the European Federation of Neurological Societies, the treatment of migraine can be abortive - the removal and alleviation of symptoms, and prophylactic - prevention of the occurrence of symptoms. Non-steroidal anti-inflammatory drugs or triptans are used in the abortive therapy, and antihypertensive medication, antidepressants or antiepileptics are used in prophylactic therapy. In addition to pharmacotherapy, complementary methods, such as changing habits, biofeedback, acupuncture and transcutaneous electrical nerve stimulation, can also be used to treat or prevent migraine headache

Migraine

Migrena je primarna glavobolja, čija prevalencija u općoj populaciji Europske unije iznosi 15%. Može se javiti epizodički, a može biti i kronična. Klinički je karakterizirana onesposobljavajućom, unilateralnom, pulsirajućom glavoboljom, trajanja 4 do 72 sata, praćenom mučninom i povraćanjem te fotofobijom, fonofobijom i osmofobijom. Godišnji troškovi migrene, direktni vezani uz dijagnostiku i terapiju te indirektni vezani uz izostanke s posla, procjenjuju se u Europskoj uniji na 5 milijardi eura. Stoga migrena predstavlja ne samo medicinski, već i socioekonomski problem. Točan uzrok migrenske glavobolje nije poznat, a zbog učestale pozitivne obiteljske anamneze smatra se da su u podlozi i genetski čimbenici. Prema današnjem shvaćanju u patogenezi migrenske glavobolje središnje mjesto zauzima aktivacija trigeminovaskularnog sustava s posljedičnom neurogenom inflamacijom i dilatacijom intrakranijskih krvnih žila inerviranih od C vlakana trigeminalnog živca. Dijagnoza migrene postavlja se na temelju kliničke slike i simptoma, a prema kriterijima Međunarodnog društva za glavobolju (International Headache Society) svrstava se u jednu od podvrsta - migrenu bez aure i migrenu s aurom. Terapija migrene je prema preporukama Europske federacije neuroloških društava (European Federation of Neurological Societies) abortivna – usmjerena na prekidanje glavobolje i ublažavanje pratećih simptoma te profilaktička – usmjerena prevenciji nastanka istih. U abortivnoj terapiji mogu se koristiti lijekovi iz skupina nesteroidnih antiinflamatornih lijekova ili triptana, dok se u profilaktičkoj terapiji koriste lijekovi iz skupina antihipertenziva, antidepresiva ili antiepileptika. Osim farmakoterapije, u liječenju odnosno prevenciji migrene mogu se koristiti komplementarne metode kao što su promjena životnih navika, biofeedback, akupunktura i transkutana električna stimulacija živaca.Migraine is a primary headache, whose prevalence in the general population of the European Union is 15%. It can be episodic but it can also be a chronic condition. It is characterized by a unilateral, pulsating headache and can last between 4 and 72 hours. Associated symptoms may include nausea, vomiting and sensitivity to light, sound or smell. The direct and indirect annual costs of migraine are estimated to be 5 billion euros in the European Union. Thus, migraine is not only medical but also a socio-economic problem. The exact cause of migraine headaches is unknown, but frequent positive family history indicates a connection with genetic factors. The central place in the pathogenesis of migraine belongs to the activation of the trigeminovascular system, with consequent vasodilatation and neurogenic inflammation of intracranial blood vessels that are innervated by C fibers from the trigeminal nerve. The diagnosis of migraine should be based on clinical presentation and symptoms, and pursuant to the criteria set by the International Headache Society, it may be more closely classified as one of the types of migraine – migraine without aura and migraine with aura. According to the European Federation of Neurological Societies, the treatment of migraine can be abortive - the removal and alleviation of symptoms, and prophylactic - prevention of the occurrence of symptoms. Non-steroidal anti-inflammatory drugs or triptans are used in the abortive therapy, and antihypertensive medication, antidepressants or antiepileptics are used in prophylactic therapy. In addition to pharmacotherapy, complementary methods, such as changing habits, biofeedback, acupuncture and transcutaneous electrical nerve stimulation, can also be used to treat or prevent migraine headache

Association Between Vestibular Migraine and Migraine Headache: Yet to Explore.

ObjectivesTo evaluate if patients with a diagnosis of vestibular migraine (VM) by the International Classification of Headache Disorders (ICHD) criteria have meaningful differences in symptomatology and disease characteristics when compared to patients with concurrent vestibular symptoms and migraine that do not meet ICHD criteria.MethodsPatients who presented for the evaluation of vertigo were provided a detailed questionnaire about dizziness and migraine symptoms. Patients were assigned to either VM cohort (met ICHD criteria for VM) or migraine headache (MH) cohort (met ICHD criteria for migraine with or without aura but not VM). Disease characteristics, symptomatology, quality of life, and perceived stress score were compared between the cohorts.ResultsThe VM cohort demonstrated a shorter duration of vertigo episodes, 11 ± 22 hours versus 84 ± 146 hours in the MH cohort. In the VM cohort, 81% reported experiencing migraine headaches during episodes of vertigo, versus 61% in the MH cohort. All patients in the VM cohort reported a previous diagnosis of migraine headache, whereas 9% of the MH cohort had not been previously diagnosed by another physician. There was no difference in quality of life or perceived stress scores between the cohorts.ConclusionsA large proportion of vertigo patients with migrainous features do not meet the ICHD criteria for VM. The differences between cohorts represent selection bias rather than meaningful features unique to the cohorts. As such, VM and MH with vestibular symptoms may exist on a spectrum of the same disease process and may warrant the same treatment protocols

Clinical Features of Pediatric Idiopathic Intracranial Hypertension and Applicability of New ICHD-3 Criteria

Idiopathic intracranial hypertension (IIH) is characterized by intracranial pressure >28 cmH2O in the absence of identifiable causes. Aim of this paper is to describe the clinical phenotype of pediatric IIH and to analyze the applicability of ICHD-3 criteria in comparison to the ICHD-2. We conducted a retrospective analysis of full clinical data of pediatric patients diagnosed with IIH between January 2007 and June 2018. Diagnostic evaluation included neuroimaging (all patients) and ultrasound-based optic nerve sheath diameter measurement (9 patients). Diagnosis of IIH was verified according to both ICHD-2 and ICHD-3 criteria for headache attributed to IIH, to verify the degree of concordance. We identified 41 subjects with suspected IIH; 14 were excluded due a diagnosis of secondary IH or lack of data. We therefore selected 27 subjects (age 4-15 years, mean 11). All patients presented with headache and bilateral papilloedema. Headache was daily in 22% cases, with diffuse gravative pain in 41%. In 4%, pain was exacerbated by cough, stress or tension. The most common presentation symptoms, in addition to headache, were blurred vision or diplopia (70%), vomiting (33%), and dizziness (15%). Twenty patients (74%) were obese. In 6 patients (22%) neuroimaging showed empty sella. Optic nerve sheath distension was detected in 6 out of 9 patients. Regarding the applicability of the ICHD-2 criteria, 18/27 (71%) patients have criterion A; 24/27 (89%) criterion B; 27/27 (100%) criterion C; 27/27 (100%) criterion D. When the ICHD-3 criteria were used, 27/27 (100%) fitted criterion A; 24/27 (89%) criterion B; 27/27 (100%) criterion C; and 27/27 (100%) criterion D. Our study suggests that, as compared with the ICHD-2, the new ICHD-3 criteria for headache attributed to IIH are better satisfied by pediatric patients with IIH. This is mainly due to the fact that qualitative headache characteristics are no longer considered in ICHD-3. Although the risk of under-rating the symptom of headache in IIH should not be disregarded, in pediatric population headache characteristics are usually less defined than in adults and obtaining a precise description of them is often very difficult

Cryopyrin-associated periodic fever syndrome manifesting as Tolosa-Hunt syndrome

Background Tolosa-Hunt syndrome (THS) is characterized by unilateral orbital pain, ipsilateral oculomotor paresis and a prompt response to treatment with corticosteroids. Several reports have demonstrated that the clinical features of THS are not specific to one causal aetiology and can lead to misdiagnosis. Case report We report the case of a patient diagnosed with THS after an episode of unilateral orbital pain and diplopia with demonstration of granulomatous inflammation of both cavernous sinus on cerebral magnetic resonance imaging and an immediate response to treatment with corticosteroids. Progression of the disease over the following years, accompanied by increasing signs of inflammation on cerebral magnetic resonance imaging and cerebrospinal fluid pleocytosis, led to further diagnostic tests. Genetic analyses revealed a heterozygote low-penetrance mutation (Q703K) of the cryopyrin/NLRP3 gene compatible with a cryopyrin-associated periodic fever syndrome. Discussion This case report demonstrates that THS can be a central nervous system manifestation of cryopyrin-associated periodic fever syndrome, which therefore represents a differential diagnosis of THS, even in elderly patients

Algometría en migraña: variación en sus diferentes situaciones clínicas

Cada vez existen nuevas pruebas que sugieren que la sensibilidad al dolor no se distribuye de forma uniforme sobre los músculos, indicando la existencia de cambios topográficos en la sensibilidad dolorosa a la presión. El objetivo de este estudio es evaluar la sensibilidad mecánica dolorosa cefálica para diferenciar a los pacientes migrañosos de la población control, así como sus modificaciones en diferentes situaciones clínicas de la migraña y ante el tratamiento preventivo.Incluimos pacientes atendidos en la Unidad de Cefaleas del Hospital Clínico Universitario de Valladolid, diagnosticados de Migraña Episódica (ME) y Migraña Crónica (MC) de acuerdo con los criterios de la III edición de la Clasificación Internacional de Cefaleas (CIC-3), así como una población control. En todos ellos se llevó a cabo un estudio utilizando un algómetro de presión para medir los umbrales de dolor o “pressure pain thresholds” (PPT), sobre 21 puntos dibujados sobre el cráneo según el sistema 10-20 de la colocación de EEG. En cada punto se llevaron a cabo 3 mediciones analizándose la media aritmética. En 25 pacientes con Migraña Crónica refractaria tratada con OnabotulinumtoxinA (OnabotA) se realizaron estudios basales (previos al tratamiento) y un mes después de las 3 primeras sesiones. Incluimos 171 pacientes migrañosos, entre los que 86 fueron diagnosticados de migraña episódica y 85 de migraña crónica, así como 40 controles. En todos los puntos analizados la sensibilidad mecánica fue superior en migrañosos que en controles. Al comparar los umbrales de dolor a la presión entre ME y MC observamos que eran inferiores, es decir, una mayor sensibilidad mecánica, en pacientes con MC respecto a los ME, principalmente en los puntos de la calota frontal y temporal anterior. Al analizar los cambios tras el tratamiento con OnabotA en los 25 pacientes, apreciamos una tendencia a la disminución de la sensibilidad mecánica pero sin alcanzar significación estadística. Concluimos que la algometría como técnica de medición de la sensibilidad mecánica cefálica caracteriza a los pacientes migrañosos respecto a lapoblación control. Además, muestra diferencias entre pacientes con ME y MC sobre todo en regiones frontal y temporal anterior de la calota. Por último, podría ser útil para monitorizar la respuesta al tratamiento con OnabotA en pacientes con migraña crónica refractaria.Grado en Medicin

Diagnosis of primary headache in children younger than 6 years: A clinical challenge

Background: Criteria defined by the International headache Society are commonly used for the diagnosis of the different headache types in both adults and children. However, some authors have stressed some limits of these criteria when applied to preschool age. Objective: Our study aimed to describe the characteristics of primary headaches in children younger than 6 years and investigate how often the International Classification of Headache Disorders (ICHD) criteria allow a definitive diagnosis. Methods: This retrospective study analysed the clinical feature of 368 children younger than 6 years with primary headache. Results: We found that in our patients the percentage of undefined diagnosis was high when either the ICHD-II or the ICHD-III criteria were used. More than 70% of our children showed a duration of their attacks shorter than 1 hour. The absence of photophobia/phonophobia and nausea/vomiting significantly correlate with tension-type headache (TTH) and probable TTH. The number of first-degree relatives with migraine was positively correlated to the diagnosis of migraine in the patients (p<0.001). Conclusions: Our study showed that the ICHD-III criteria are difficult to use in children younger than 6 years. The problem is not solved by the reduction of the lowest duration limit for the diagnosis of migraine to 1 hour, as was done in the ICHD-II