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Migraine, the most frequent neurological ailment, affects visual processing during and between attacks. Most visual disturbances associated with migraine can be explained by increased neural hyperexcitability, as suggested by clinical, physiological and neuroimaging evidence. Here, we review how simple (e.g., patterns, color) visual functions can be affected in patients with migraine, describe the different complex manifestations of the so-called Alice in Wonderland Syndrome, and discuss how visual stimuli can trigger migraine attacks. We also reinforce the importance of a thorough, proactive examination of visual function in people with migraine.

Very few topics in the headache field engender more controversy than sinus headache. Patients often believe that their headaches are attributed to sinus disorders because they experience rhinitis symptoms (e.g. rhinorrhoea, nasal congestion, and postnasal drip) during their attacks. Primary care physicians, otolaryngologists, and allergists often diagnose sinus headaches and treat them with rhinitis medications, antibiotics, allergy shots, and surgical interventions, with varying levels of success. In contrast, most neurologists and headache specialists believe that many patients with a diagnosis of sinus headache are suffering from migraine, or another primary headache disorder.

While migraine headaches can be provoked, or predicted by the presence of an aura or premonitory symptoms, the prediction or elicitation of the aura itself is more problematic. Therefore, imaging studies directly examining the aura phenomenon are sparse. There are however interictal imaging studies that can shed light on the pathophysiology of the migraine with aura (MWA) cascade. Here, we review findings pointing to the involvement of cortical spreading depression (CSD) and neuroinflammation in MWA. Whether asymptomatic CSD also happens in some migraine without aura is still under debate. In addition, new evidence points to glial activation in MWA, indicating the involvement of astrocytes in the neuroinflammatory cascade that follows CSD, as well as dural macrophages, supporting the involvement of the trigeminovascular system in migraine pain.

A substantial body of research has shown that Parkinson disease (PD) patients display an enlarged hyperechogenic substantia nigra (SN) by transcranial sonography (TCS). Nonetheless, the categorization of SN echogenicity is still subjective. A method is described to quantify SN echogenicity based on the gray-level histograms of digitized TCS images. In 31 PD patients and 57 controls, the gray-scale mean (GSM) of the SN, mesencephalon, and basal cisterns and their ratios were assessed using image-editing computer software. The GSM values were compared between the groups and correlated with age, SN area, and disease duration. The SN GSM was significantly higher in the PD group ( P < .05) with a positive correlation with age but not with disease duration. In this cohort of participants, mesencephalon echogenicity was found to increase with disease duration. These results would suggest quantitatively SN hyperechogenicity in PD. Quantifying SN echogenicity is feasible and might minimize subjectivity in PD diagnosis by TCS. The method has the potential to disclose subtle changes in other midbrain structures’ echogenicity engendered by the disease process as well.

Given that the medial olivocochlear efferent system reduces the amplitude of otoacoustic emissions (OAE), the aim of this study was to establish whether such a pathway is affected in women with migraine and phonophobia by means of OAE suppression testing. In this prospective case-control study, 55 women (29 with migraine and phonophobia and 26 healthy women) were subjected to transient-evoked otoacoustic emission (TEOAE) testing at frequencies from 1 to 4 kHz. The amplitudes of the TEOAE response before and after exposure to contralateral noise and the magnitude of TEOAE suppression were assessed. The average TEOAE amplitudes in conditions with and without exposure to contralateral noise were not significantly different between the groups. However, the magnitude of TEOAE suppression was lower in the group with migraine; that difference was only statistically significant for frequencies 1 and 1.5 kHz (p = 0.042 and p = 0.004, respectively). In this study, women with migraine and phonophobia exhibited deficits in OAE suppression, which points to a disorder affecting the medial olivocochlear efferent system.

Made available in DSpace on 2021-07-14T10:18:35Z (GMT). No. of bitstreams: 0 Previous issue date: 2016-03. Added 1 bitstream(s) on 2021-07-14T11:26:49Z : No. of bitstreams: 1 S0004-282X2016000300017.pdf: 296980 bytes, checksum: 61532e35d8a3544c1e4f26fc993603b3 (MD5) Neste artigo um grupo de especialistas no tratamento de cefaleia da Sociedade Brasileira de Cefaleia através de um consenso elaborou medidas estratérgicas para tratar uma crise de migrânea tanto na criança como no adulto. Uma enfase particular foi dada no tratamento da migranea na mulher, incluindo gravidez, lactação e período perimenstrual. In this article, a group of experts in headache management of the Brazilian Headache Society developed through a consensus strategic measurements to treat a migraine attack in both the child and the adult. Particular emphasis was laid on the treatment of migraine in women, including at pregnancy, lactation and perimenstrual period. Universidade de São Paulo, Faculdade de Medicina de Ribeirão Preto Universidade Federal de São Paulo, Escola Paulista de Medicina Universidade Federal do Rio Grande do Norte Universidade do Federal do Paraná Faculdade de Medicina de Catanduva Universidade Federal Ciências da Saúde de Porto Alegre Hospital Mário Gartti Universidade Federal Fluminense Universidade Estadual de Campinas Unichristus Hospital Geral de Fortaleza Universidade Federal de Santa Catarina Sociedade Brasileira de Cefaleia Universidade Federal de Pernambuco Hospital Esperança Universidade Estadual Júlio de Mesquita Filho Universidade Federal do Rio de Janeiro Faculdade de Medicina de Barbacena Santa Casa de São Paulo Universidade Federal de Minas Gerais Universidade Estadual Júlio de Mesquita Filho

Vertigo symptoms and subclinical vestibular dysfunctions may occur in migraine. The Minimal Caloric Test (MCT), an easy-to-perform, convenient and yet informative procedure was used to test the vestibular function in 30 vertigo-free migraine patients outside attacks and 30 paired controls. Although not statistically significant, a right-to-left nystagmus duration asymmetry greater than 25% was present in both groups. This difference was greater in the patients group, suggesting the presence of subclinical vestibular imbalance in migraine. RESUMO Vertigem e alterações vestibulares subclínicas têm sido identificadas na enxaqueca. O teste calórico mínimo é simples de realizar, informativo e conveniente. Ele foi realizado em 30 pacientes com enxaqueca sem queixa de vertigem e 30 controles pareados. Embora não estatisticamente significativo, ocorreu assimetria direita-esquerda superior a 25% na duração do nistagmo em ambos os grupos, maior nos pacientes, o que sugere a presença de desequilíbrio vestibular subclínico na enxaqueca.

Objective The main goal of this study was to correlate migraine improvement, after prophylactic therapy, with cortical thickness changes. Methods Cortical thickness maps were obtained with magnetic resonance imaging (MRI) from 19 patients with migraine before (first scan) and after (second scan) prophylactic treatment, and these were compared with controls using the FreeSurfer MRI tool. Cortical changes were correlated with the headache index (HI). Results Anincrease incortical thickness was found in the right cuneus and precuneus, somatosensory and superior parietal cortices in both patient scans, compared with the controls. No changes were observed in the left hemisphere. Following correction for multiple comparisons, no areas changed from the first to the second scan. Regression analysis showed a significant negative correlation between the HI improvement and cortical thickness changes in the left posterior cingulate, a region involved with nociception and, possibly, the development of chronic pain. Conclusion There were changes in cortical thickness in patients with migraine relative to controls in areas involved with vision and pain processing. Left posterior cingulate cortical changes correlated with headache frequency and intensity. RESUMO Objetivos Correlacionar a melhora de pacientes enxaquecosos após tratamento preventivo com alterações na espessura do córtex cerebral. Métodos Espessura cortical foi determinada a partir de imagens de ressonância magnética (RM)em 19 pacientes com enxaqueca, antes (1ᵃ RM) e após (2ᵃ RM) o tratamento profilático, e comparada com controles, usando o programa FreeSurfer. Mudanças corticais foram correlacionadas com o índice de cefaleia (HI). Resultados O hemisfério direito apresentou aumento da espessura no córtex do cúneus e pré-cúneus, parietal superior e somatossensitivo na primeira RM e na segunda RM, em comparação aos controles. Após correção para comparações múltiplas, nenhuma região cortical se mostrou estatisticamente diferente entre a primeira e a segunda RM. A regressão mostrou correlação (negativa) significativa entre melhora do HI e mudanças na espessura cortical do cíngulo posterior esquerdo. Conclusão Existem alterações de espessura cortical em pacientes com enxaqueca em relação a controles em áreas envolvidas com processamento visual e com a dor. As alterações corticais no cíngulo posterior esquerdo variaram de acordo com a frequência e intensidade das crises.

Migraine and vertigo are common disorders, with lifetime prevalences of 16% and 7% respectively, and co-morbidity around 3.2%. Vestibular syndromes and dizziness occur more frequently in migraine patients. We investigated bedside clinical signs indicative of vestibular dysfunction in migraineurs. Objective To test the hypothesis that vestibulo-ocular reflex, vestibulo-spinal reflex and fall risk (FR) responses as measured by 14 bedside tests are abnormal in migraineurs without vertigo, as compared with controls. Method Cross-sectional study including sixty individuals – thirty migraineurs, 25 women, 19-60 y-o; and 30 gender/age healthy paired controls. Results Migraineurs showed a tendency to perform worse in almost all tests, albeit only the Romberg tandem test was statistically different from controls. A combination of four abnormal tests better discriminated the two groups (93.3% specificity). Conclusion Migraine patients consistently showed abnormal vestibular bedside tests when compared with controls. Enxaqueca e vertigem são desordens comuns, com prevalência de 16% e 7% respectivamente, e comorbidade em torno de 3,2%. Síndromes vestibulares e tonturas ocorrem mais frequentemente em enxaquecosos. Pesquisamos alterações vestibulares utilizando testes de beira-de-leito em enxaquecosos. Objetivo Verificar se as respostas dos reflexos vestíbulo-ocular, vestíbulo-medular e risco de quedas medidas por 14 testes de beira-de-leito são diferentes comparando-se enxaquecosos sem vertigem, e controles. Método Estudo transversal com sessenta pessoas, 30 enxaquecosos; 25 mulheres, 19-60 anos; e trinta controles saudáveis pareados por sexo e idade. Resultados Houve tendência de pior desempenho entre enxaquecosos em quase todos testes, porém apenas o teste de Romberg tandem foi estatisticamente diferente dos controles. Uma combinação de quatro testes anormais discrimina os grupos com especificidade de 93,3%. Conclusão O grupo de enxaquecosos mostrou consistentemente testes vestibulares de beira-de-leito anormais quando comparados a controles.

Background Migraine, particularly chronic migraine (CM), is underdiagnosed and undertreated worldwide. Our objective was to develop and validate a self-administered tool (ID-CM) to identify migraine and CM. Methods ID-CM was developed in four stages. (1) Expert clinicians suggested candidate items from existing instruments and experience (Delphi Panel method). (2) Candidate items were reviewed by people with CM during cognitive debriefing interviews. (3) Items were administered to a Web panel of people with severe headache to assess psychometric properties and refine ID-CM. (4) Classification accuracy was assessed using an ICHD-3β gold-standard clinician diagnosis. Results Stages 1 and 2 identified 20 items selected for psychometric validation in stage 3 ( n = 1562). The 12 psychometrically robust items from stage 3 underwent validity testing in stage 4. A scoring algorithm applied to four symptom items (moderate/severe pain intensity, photophobia, phonophobia, nausea) accurately classified most migraine cases among 111 people (sensitivity = 83.5%, specificity = 88.5%). Augmenting this algorithm with eight items assessing headache frequency, disability, medication use, and planning disruption correctly classified most CM cases (sensitivity = 80.6%, specificity = 88.6%). Discussion ID-CM is a simple yet accurate tool that correctly classifies most individuals with migraine and CM. Further testing in other settings will also be valuable.

Migraine is a central nervous system disorder frequently expressed with paroxysmal visual dysfunctions. Objective To test the hypothesis that normal visual input is vital for the migrainous aura and photophobia. Method We studied the migraine-related visual disturbances in 8 sightless migraineurs identified among 200 visually impaired subjects. Results The main findings were the visual aura and photophobia disappearance along with blindness development, the oddness of aura – too short, colourful (e.g. blue or fire-like), auditory in nature or different in shape (round forms) – and the lack of photophobia. Conclusion We propose that the aura duration should be accepted as shorter in visually impaired subjects. The changes in aura phenotype observed in our patients may be the result of both cerebral plasticity induced by the visual impairment and/or the lack of visual input per se. Integrity of visual pathways plays a key role in migraine visual aura and photophobia.

Physical therapy may be of great importance in Parkinson disease. The objective of the study is to analyze the gait in patients with Parkinson disease treated with an equipment of body weight support associated with a treadmill as compared with the conventional physical therapy. All subjects presented lower fatigue at the last session than at baseline. The body weight support associated with a treadmill group showed signifi cant improvement in Unifi ed Parkinson’s Disease Rating Scale motor scale score, while the conventional physical therapy group improved in the 6-minute walk test distance parameter. No technique proved universally better than another, and treatments led to good results considering

Background. Parsonage-Turner syndrome, or neuralgic amyotrophy (NA), is an acute brachial plexus neuritis that typically presents with unilateral shoulder pain and amyotrophy but also can affect other peripheral nerves, including the recurrent laryngeal nerve. Idiopathic vocal fold paralysis (VFP) represents approximately 12% of the VFP cases and recurrence is extremely rare.Methods and Results. We report a man with isolated recurrent unilateral right VFP and a diagnosis of NA years before.Conclusions. We emphasize that shoulder pain and amyotrophy should be inquired in any patient suffering from inexplicable dysphonia, and Parsonage-Turner syndrome should be considered in the differential diagnosis of idiopathic VFP.

Background Jumping to conclusions due to impulsivity has been shown to be a sensitive marker for dopamine dysregulation and addictive behaviour patterns in treated patients with Parkinson's disease (PD). It is unknown whether drug naive PD patients, who have never received dopaminergic therapy also have deficits in information sampling. Methods Twenty five de novo PD patients and twenty matched healthy controls were recruited and tested on the beads task, which is a validated information sampling task to assess reflection impulsivity and a temporal discounting questionnaire. Results Patients gathered significantly less information and made more irrational choices than matched controls. There was, however, no group difference on the temporal discounting questionnaire. Conclusions Poor information sampling and irrational decision making may be an inherent component of the neuropsychological deficit in Parkinson's disease. These findings suggest that underlying impulsivity detected by a metric task is common in de novo PD.

The prevalence of non-motor symptoms in Parkinson's disease (PD) is high. Depression varies from 20 to 50% of the PD patients, and is associated with increasing disability. The key characteristics of depression are anhedonia and low mood. The recommended scales for screening purposes are: HAM-D, BDI, HADS, MADRS and GDS. As for measurement of severity: HAM-D, MADRS, BDI and SDS. In cases with mild depression, non-pharmacological intervention is the treatment of choice. In moderate depression, antidepressants are required. The choice of an antidepressant should be based mainly on the comorbidities and unique features of the patient. Evidence for antidepressant effectiveness is seen mostly with amitriptyline and nortriptyline, but one should be cautious in elderly patients. Other antidepressants that can be prescribed are: citalopram, escitalopram, sertraline, bupropion, trazodone, venlafaxine, mirtazapine and duloxetin. The dopaminergic agonist pramipexole is a treatment option.

In Brazil there is no systematic study on Transcranial Sonography (TCS), a neuroimaging method that depicts echogenic deep brain structures using ultrasound. Objective: To establish the percentage of subjects with permissive temporal windows and to address the ability of TCS of the substantia nigra (SN) to distinguish parkinsonian patients in a Brazilian sample. Method: We performed TCS using the Acuson X300 (Siemens, Germany) in 37 individuals: 23 with Parkinson’s disease (PD) and 14 healthy controls. Results: 10.8% of subjects had insufficient temporal acoustic bone windows. SN echogenic areas were larger in patients (mean±SD, 0.31±0.08cm 2 ) compared to controls (mean±SD, 0.17±0.02cm 2 ). TCS accurately identified 88.2% of PD patients. Conclusion: A large proportion of Brazilians seem to be eligible for TCS. An expressive number of PD patients could be diagnosed by TCS based on an expanded SN echogenic area. However, the current data is preliminary and must be corroborated by larger studies.

Cervicogenic headache (CeH) is a well-recognized headache syndrome, distinguishable from other primary and secondary headaches. Although in some cases a cervical lesion may be detected in connection with the headache, many CeH patients have no demonstrable lesion. Besides, most of the frequent cervical diseases, such as spondylosis and disc herniations, do not present with headache of the cervicogenic type. This suggests that the neck is not an independent headache generator. CeH may depend in addition on a central predisposition counterpart, leading to the activation of the trigeminovascular system and pain generation.

Professor Leão described cortical spreading depression (CSD) in 1944 and changed the way migraine pathophysiology is scientifically conceived. This Brazilian professor and researcher developed his career in Rio de Janeiro. Because of him, the city once became the CSD world capital, attracting scientists from many laboratories around the globe. Aristides Leão was first and foremost a naturalist, a man genuinely interested in birds, fishes, plants, shells, and neuroscience. He could easily fascinate whoever would come for a chat, leaving no question without answers. He was born in 1914 and died in 1993. This report focuses on his life, family, habits, and hobbies, describing a little of Leão apart from CSD and the year of 1944.

The objective of this article is to highlight some of the most important pioneering books specifically focused on the neurological examination and their authors. During the XIX Century, Alexander Hammond, William Gowers and Charles Mills pioneered the neurological literature, followed in the XX Century by Aloysio de Castro, Monrad-Krohn, Derek Denny-Brown, Robert Wartenberg, Gordon Holmes, and Russel DeJong. With determination and a marked sense of observation and research, they competently developed and spread the technique and art of the neurological exam. O objetivo deste artigo é destacar alguns dos primeiros e mais importantes livros-texto interessados em difundir o ensino do exame neurológico e seus autores. Durante o século XIX, Alexander Hammond, William Gowers e Charles Mills foram pioneiros na literatura neurológica, seguidos por Aloysio de Castro, Monrad-Krohn, Derek Denny-Brown, Robert Wartenberg, Gordon Holmes e Russel DeJong no século XX. Com determinação, grande senso de observação e pesquisa, eles competentemente disseminaram a técnica e a arte de se realizar o exame neurológico.

A 14-year-old girl came to the office because of permanent and refractory headaches. She was symptom-free until the age of 12, when she started suffering from occasional headache attacks, some of them preceded by syncope. The first spell occurred at an airport toilet just prior to a family trip, during which no abnormality was present. However, attacks increased to 4–5 per week right after returning to town, precluding her from attending school. The present frequency of faints is around twice a month.

Trigeminal neuralgia is classically associated with neurovascular compression of the trigeminal nerve, at the root entry zone (REZ). However, patients are occasionally affected by intra-axial involvement of trigeminal sensory fibers caused by demyelinating diseases, strokes and, rarely, pontine cavernous malformations. We discuss the management strategies and decision-making process in a 55-year-old patient, affected by trigeminal neuralgia with 2 potential causative mechanisms: a neurovascular conflict at the trigeminal REZ and an ipsilateral cavernous malformation at the pontine nucleus of the trigeminal nerve.

Background Chronic pain disorders are presumed to induce changes in brain grey and white matters. Few studies have focused CNS alterations in trigeminal neuralgia (TN). Methods The aim of this study was to explore changes in white matter microstructure in TN subjects using diffusion tensor images (DTI) with tract-based spatial statistics (TBSS); and cortical thickness changes with surface based morphometry. Twenty-four patients with classical TN (37-67 y-o) and 24 healthy controls, matched for age and sex, were included in the study. Results Comparing patients with controls, no diffusivity abnormalities of brain white matter were detected. However, a significant reduction in cortical thickness was observed at the left cuneus and left fusiform cortex in the patients group. The thickness of the fusiform cortex correlated negatively with the carbamazepine dose (p = 0.023). Conclusions Since the cuneus and the fusiform gyrus have been related to the multisensory integration area and cognitive processing, as well as the retrieval of shock perception conveyed by Aδ fibers, our results support the role of these areas in TN pathogenesis. Whether such changes occurs as an epiphenomenon secondary to daily stimulation or represent a structural predisposition to TN in the light of peripheral vascular compression is a matter of future studies.

Introduction: Human T cell lymphotropic virus type I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) can impact the independence and motricity of patients. The aims of this study were to estimate the effects of physiotherapy on the functionality of patients with HAM/TSP during the stable phase of the disease using proprioceptive neuromuscular facilitation (PNF) and to compare two methods of treatment delivery. Methods: Fourteen patients with human T cell lymphotropic virus type I (HTLV-I) were randomly allocated into two groups. In group I (seven patients), PNF was applied by the therapist, facilitating the functional activities of rolling, sitting and standing, walking and climbing and descending stairs. In group II (seven patients), PNF was self-administered using an elastic tube, and the same activities were facilitated. Experiments were conducted for 1h twice per week for 12 weeks. Low-back pain, a modified Ashworth scale, the functional independence measure (FIM) and the timed up and go test (TUG) were assessed before and after the interventions. Results: In the within-group evaluation, low-back pain was significantly reduced in both groups, the FIM improved in group II, and the results of the TUG improved in group I. In the inter-group analysis, only the tone was lower in group II than in group I. Conclusions: Both PNF protocols were effective in treating patients with HAM/TSP.

More than 16 years after the first description of hemicrania continua (HC), its aetiology and pathogenesis remain obscure. Clinically, HC is considered a syndrome with two pivotal characteristics: (i) strictly unilateral (moderate, fluctuating, relatively long-lasting) headache; and (ii) absolute response to indomethacin. HC is further characterized by some ancillary, but mostly ‘negative’, features such as: (iii) relative paucity of accompaniments; and (iv) lack of precipitating factors. The female preponderance is also remarkable, although not diagnostic in the solitary case. Finally, a non-specific, but remarkable feature is the temporal pattern. HC may present as a remitting or chronic (continuous) headache. In HC, unilaterality and absolute response to indomethacin are considered crucial diagnostically. Existing controversy, such as regarding atypical features, particularly the so-called ‘HC resistant to indomethacin’, is discussed. The nature of hemicrania with negative indomethacin response remains most unclear; it may not belong to the HC cycle at all. Accordingly, we propose that the typical clinical picture of HC, including an absolute response to indomethacin, be termed Hemicrania continua vera. More or less analogous, but indomethacin-resistant, clinical pictures can provisionally be termed Hemicrania generis incerti (of undetermined nature), provided other diagnostic possibilities have been ruled out. The differential diagnosis of HC vs. other unilateral headaches is commented on. Previous attempts at classification of HC into the group chronic daily headache (CDH) are discussed. The only acceptable ‘link’ of HC with the other headaches classified as CDH is the temporal pattern (which is a non-specific feature). HC is probably pathophysiologically different from the others disorders classified under CDH. Conversely, HC and chronic paroxysmal hemicrania share many common features, including the absolute response to indomethacin. HC should probably be included in the IHS group 3.

Headaches are common disorders usually examined by nonneurologists. In order to assess how primary headache patients (IHS groups 1, 2, and 3) are generally managed by nonspecialists, 414 patients were asked about their previous headache care. Correct diagnosis had previously been made in only 44.9%, 6.7%, and 26.7% of the migraine, tension-type headache, and cluster headache patients, respectively. The patients underwent 501 investigative procedures motivated by the headache, averaging 1.21 examinations per patient, mostly EEGs. Preventive treatment was largely overlooked irrespective of the headache type. It is concluded that scientific improvements in headache care may be ineffective unless educational programs improve headache knowledge in general.

The social perception of headache, everywhere at low levels in industrialised countries, becomes totally absent in developing ones. Headache disorders came into the World Health Organization’s strategic priorities after publication of the 2001 World Health Report. Among the leading causes of disability, migraine was ranked 19th for adults of both sexes together and 12th for females. The Global Campaign (GC) to Reduce the Burden of Headache Worldwide was planned by the major international headache organizations together with WHO in order to identify and remove those cultural, social and educational barriers recognised as responsible factors for the inadequate treatment of headache disorders worldwide. Within the GC activities, the education of the medical body will represents a central pillar. An International Team for Specialist Education (ITSE) has been created to train physicians from all over the world through the acquisition of a university level Master Degree in Headache Medicine. Once trained as headache specialists, physicians will become trainers, offering education in this field to other health care providers in their own countries. In this way they will give life to a cultural chain raising awareness locally of headache, its burden and its medical control.

A migrânea crônica é uma condição com prevalência significativa ao redor do mundo e alto impacto socioeconômico, sendo que seu manuseio tem desafiado os neurologistas. Os avanços na compreensão de seus mecanismos e das condições a ela associadas, bem como nas novas terapêuticas, têm sido rápidos e importantes, fato que motivou as Sociedades Latino-americana e Brasileira de Cefaleia a elaborarem o presente consenso. O tratamento da migrânea crônica deve ser sempre precedido por uma revisão cuidadosa do diagnóstico, pela detecção de possíveis fatores de piora e das condições associadas, pela estratificação de gravidade/impossibilidade de se tratar e pelo monitoramento com um diário da dor. Este consenso apresenta abordagens farmacológicas e não-farmacológicas para tratar a migrânea crônica. Chronic migraine is a condition with significant prevalence all around the world and high socioeconomic impact, and its handling has been challenging neurologists. Developments for understanding its mechanisms and associated conditions, as well as that of new therapies, have been quick and important, a fact which has motivated the Latin American and Brazilian Headache Societies to prepare the present consensus. The treatment of chronic migraine should always be preceded by a careful diagnosis review; the detection of possible worsening factors and associated conditions; the stratification of seriousness/impossibility to treat; and monitoring establishment, with a pain diary. The present consensus deals with pharmacological and nonpharmacological forms of treatment to be used in chronic migraine. Universidad de Santiago de Chile and Sociedad de Neurologia Psiquiatria y Neurocirugia Universidad de Costa Rica Hospital San Juan de Dios Universidade Federal de Minas Gerais Hospital das Clinicas Universidade Jose do Rosario Vellano Universidade de São Paulo Faculdade de Medicina Barao de Maua Faculdade de Medicina de Ribeirao Preto Universidad Nacional de Cordoba Brazilian and International Headache Societies Academia Brasileira de Neurologia Centro Universitario de Volta Redonda Universidade Federal Fluminense (UFF) Universidad de la Republica Hospital Maciel Facultad de Medicina Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Sector of Investigation and Treatment of Headaches at Clinical Neurology in the Neurology Discipline Universidade Federal do Rio Grande do Norte Universidade Federal do Parana UFF Associacao Latino-Americana de Cefaleia Universidade Estadual de Campinas Faculdade de Medicina de Catanduva USP Faculdade de Medicina de Ribeirao Preto Universidade Federal de Ciencias da Saude de Porto Alegre Academia Brasileira de Neurologia Universidade de La Sabana USP Faculdade de Medicina Hospital Perez Carreno UFF Universidad Mayor de San Andres Universidad Central de Venezuela Hospital Vargas de Caracas Universidade de Santo Amaro Escola Paulista de Medicina da UNIFESP Universidad de Guadalajara UNIFESP Universidade Federal de Santa Catarina USP Faculdade de Medicina de Ribeirao Preto Centro Educacional Barao de Maua Universidade Federal de Pernambuco Universidad Mayor de San Andres Hospital das Clinicas Academia Mexicana de Neurologia Hospital Especialidades Centro Medico La Raza Sociedad Neurologica Hospital Israelita Albert Einstein UNIFESP Universidad de Concepcion Universidade Federal do Rio de Janeiro Hospital Universitario Clementino Fraga Filho Faculdade de Medicina University of Trondheim ASS Colombiana de Neurologia AHS Universidad de Buenos Aires Universidad Central de Venezuela Hospital Vargas Universidad Diego Portales Universidad de Santiago Instituto de Neurologia UFF Medicine School UNIFESP, EPM, Sector of Investigation and Treatment of Headaches at Clinical Neurology in the Neurology Discipline Universidade de Santo Amaro Escola Paulista de Medicina da UNIFESP, Universidade Federal de Santa Catarina Hospital Israelita Albert Einstein UNIFESP SciELO

The involvement of the cerebellum in migraine pathophysiology is not well understood. We used a biparametric approach at high-field MRI (3 T) to assess the structural integrity of the cerebellum in 15 migraineurs with aura (MWA), 23 migraineurs without aura (MWoA), and 20 healthy controls (HC). High-resolution T1 relaxation maps were acquired together with magnetization transfer images in order to probe microstructural and myelin integrity. Clusterwise analysis was performed on T1 and magnetization transfer ratio (MTR) maps of the cerebellum of MWA, MWoA, and HC using an ANOVA and a non-parametric clusterwise permutation F test, with age and gender as covariates and correction for familywise error rate. In addition, mean MTR and T1 in frontal regions known to be highly connected to the cerebellum were computed. Clusterwise comparison among groups showed a cluster of lower MTR in the right Crus I of MWoA patients vs. HC and MWA subjects (p = 0.04). Univariate and bivariate analysis on T1 and MTR contrasts showed that MWoA patients had longer T1 and lower MTR in the right and left pars orbitalis compared to MWA (p

Co-activation of cranial perivascular sensory and parasympathetic fibres in vivo induces simultaneous release of several vasodilatory substances with neurotransmitter or neuromodulatory roles. The role of the endothelium and possible interactions between such substances are poorly understood. The objective of this study was therefore to investigate these aspects with the sensory dilator calcitonin gene-related peptide (CGRP) and the parasympathetic dilators acetylcholine (ACh) and vasoactive intestinal peptide (VIP) in isolated porcine ophthalmic artery. Whilst ACh induced relatively rapid, endothelium-dependent dilation, CGRP and VIP induced slower dilations. Both CGRP and VIP were found to have partial endothelium-dependence in this artery. The simultaneous addition of ACh with CGRP potentiated the relaxation induced by CGRP, as has already been shown for substance P. ACh did not potentiate VIP relaxation, but the results generally indicate a potential role for ACh in initiating rapid dilation prior to strong, sustained relaxation by CGRP or VIP. The potential role of the endothelium and of substances like ACh or substance P in enhancing the rate of dilation of neuropeptides inducing strong and sustained relaxation is discussed.

The aim of this prospective study was to evaluate whether the cerebellopontine angle (CPA) cistern area and trigeminal nerve cisternal length play a role in the pathogenesis of trigeminal neuralgia (TN). High-resolution 1.5 T magnetic resonance imaging of the posterior fossa was performed in 26 patients with TN and 18 age-matched healthy controls. Axial T2-weighted, three-dimensional constructive interference in steady-state (3D-CISS) was used to measure bilaterally the cross-sectional area of the CPA cistern and trigeminal nerve cisternal length. In patients, the cross-sectional area of the CPA cistern and trigeminal nerve cisternal length was smaller on the affected side (p = 0.04). Healthy controls tended to have larger cisternal areas and longer trigeminal nerve lengths than patients (p = 0.059, p = 0.071, respectively). Larger CPA cisternal areas tended to be seen in older patients. There was a strong correlation between the cross-sectional area of the CPA cistern and the length of the trigeminal nerve (p = 0.000). Smaller CPA cisterns and short cisternal trigeminal nerves impact the pathogenesis of essential TN by facilitating the neurovascular conflict, especially in younger patients. Trigeminal nerve cisternal measurement provides an easy and direct estimation of the CPA area. This information can be used for surgical planning and potentially for outcome prediction.

Medication-overuse headache (MOH) is a relatively common and impactful disorder, affecting 1% to 2% of the population, characterized by daily or near-daily headache aggravated by chronic acute medication intake. Primary headache patients do not necessarily develop MOH after acute medication overuse, although a pre-existing primary headache is inevitably present. Likewise, headache patients may deteriorate in terms of frequency without medication overuse, or suffer from chronic headache in the presence of drug abuse without any causal relationship. To classify and define diagnostic criteria for MOH in the absence of objective biomarkers is a difficult task that is presently based on clinical grounds and is limited in part by the relative lack of research in this field. The present criteria are less restrictive but also less precise than the previous versions because they allow the diagnosis without the previously required MOH confirmation after medication withdrawal. MOH should remain as a distinct secondary disorder based on the available clinical and pathophysiological evidence.

The digiti quinti sign (DQS), described originally as a clinical indication of subtle motor deficit, consists of a relatively greater abduction of the fifth finger on the affected side when both arms are extended forwards. This sign was previously observed interictally in three consecutive hemiplegic migraine (HM) patients. Patients and methods: To verify whether the DQS specifically discriminates HM from non-hemiplegic migraine (nHM), the angle between the fourth and fifth fingers (ANG) was measured interictally in 10 HM patients, 44 migraine with aura and migraine without aura patients, and 45 healthy controls. Results: The ANG was significantly wider at the symptomatic side in HM as compared with nHM and controls. The differences between the symptomatic and non-symptomatic (for HM) or between the right and left sides (absolute values for nHM and controls) were, respectively, 10.10° ± 9.58°, 4.15° ± 3.95° and 5.37° ± 4.74° ( p = .007). The optimal cutoff point for ANG was 15° at the symptomatic side (sensitivity and specificity of 80.0% and 72.2%, respectively), 10.5° at the non-symptomatic side (sensitivity and specificity of 60.0% and 52.3%), and 3° for the difference between sides (sensitivity and specificity of 90.0% and 79.5%). Conclusion: Data show that the DQS discriminates HM from nHM and controls.

BACKGROUND AND PURPOSE Prior to modern neuroimaging, neurological treatment decisions were based on findings obtained from patient history and clinical examination. Despite the availability of sophisticated neuroimaging methods, to identify intracranial tumors the clinical recognition of associated subtle motor deficits is important for practice. Precise clinical tests are particularly advantageous, as some tumors may remain unnoticed for many. The purpose of this study was to determine the sensitivity and specificity of 13 clinical tests for detection of subtle motor deficits in patients with unilateral brain tumors. METHODS Sixty patients with unilateral brain tumors without obvious focal signs and 30 controls with normal magnetic resonance imaging were examined. Thirteen clinical maneuvers described to detect motor deficits were performed and their sensitivity, specificity, and positive and negative predictive values were estimated. RESULTS The test with greatest sensitivity and specificity (with 95% confidence interval) was the Digit Quinti Sign: 0.51 (0.41-0.61) and 0.70 (0.61-0.79), respectively. The agreement measurement among the 3 most sensitive signs (Digit Quinti Sign, Pronator Drifting Test, and Finger Rolling Test) was 21%. The Kappa index for these 3 tests indicated no significant concordance. CONCLUSIONS The Digit Quinti Sign, the Pronator Drifting Test, and the Finger Rolling Test are simple yet very useful maneuvers that clinicians can perform at bedside. Even without apparent motor deficits, when present, these signs suggest that comprehensive investigation for intracranial neoplams should be undertaken.

Patent foramen ovale (PFO), a relatively common abnormality in adults, has been associated with migraine. Few studies also linked PFO with cluster headache (CH). To verify whether right-to-left shunt (RLS) is related to headaches other than migraine and CH, we used transcranial Doppler following microbubbles injection to detect shunts in 24 CH, 7 paroxysmal hemicrania (PH), one SUNCT, two hemicrania continua (HC) patients; and 34 matched controls. RLS was significantly more frequent in CH than in controls (54% vs. 25%, p=0.03), particularly above the age of 50. In the HC+PH+SUNCT group, RLS was found in 6 patients and in 2 controls (p=0.08). Smoking as well as the Epworth Sleepiness Scale correlated significantly with CH, smoking being more frequent in patients with RLS. PFO may be non-specifically related to trigeminal autonomic cephalalgias and HC. The headache phenotype in PFO patients probably depends on individual susceptibility to circulating trigger factors. O forame oval patente (FOP), uma anormalidade cardíaca relativamente comum em adultos, tem sido associado à enxaqueca, mas raramente às cefaléias trigêmino-autonômicas (TACs). Utilizamos o Doppler transcraniano (DTC) para detecção de shunt direito-esquerdo (SDE) em 24 pacientes com cefaléia em salvas (CS), sete com hemicrania paroxística (HP), dois com hemicrania continua (HC) e um com SUNCT; alem de 34 controles. O SDE foi mais frequente nos pacientes com CS do que nos controles (54% vs. 25% p=0,03), particularmente acima de 50 anos. No grupo HP+HC+SUNCT, o SDE foi encontrado em seis pacientes e dois controles (p=0,08). O hábito de fumar, bem como sonolência excessiva diurna foram mais frequentes em paciente com CS. O FOP pode ter importância inespecífica na fisiopatologia das TACs e HC, na dependência da susceptibilidade individual a fatores desencadeantes.

Cervicogenic headache (CEH) is a well-recognized syndrome. Proposed diagnostic criteria differentiate CEH from migraine and tension-type headache (TTH) in most of the cases. The best differentiating factors include side-locked unilateral pain irradiating from the back and evidence of neck involvement--attacks may be precipitated by digital pressure over trigger spots in the cervical/nuchal areas or sustained awkward neck positions. Migrainous traits may be present in some cases. Cervical lesions are not necessarily seen, and most common cervical lesions do not produce CEH. Whiplash may occasionally induce headaches. This is suspected when the pain onset and the whiplash trauma are close in time. Whiplash-related headaches tend to be short-lasting, admitting mostly a TTH or a CEH-like phenotype. Neuroimaging abnormalities are not necessarily expected in CEH. Whiplash patients must undergo cervical imaging mostly in connection with the trauma, as no abnormalities are pathognomonic in chronic cases.

Peptides may function as neurotransmitters liberated antidromically by sensory nerve fibres, provoking vascular responses having potential importance in some neurological disorders. Dose-response relaxation curves induced by substance P (SP) and calcitonin gene related peptide (CGRP) have been studied in porcine ophthalmic arteries in vitro. Both peptides induced vasodilation when tested separately (CGRP much greater than SP). Because of the putative interactions between such peptides in this vascular territory, a computerised system was also used for analysing over time the response to a single addition of either 10(-8) M CGRP, 10(-8) M SP or a combination of 10(-8) M SP + 10(-8) M CGRP. SP did not augment the maximum relaxation induced by CGRP alone, but increased significantly the rate of relaxation during the initial phase of the response. The effect induced by the SP+CGRP combination was stronger than the sum of the individual SP and CGRP-induced relaxations during the first 4 min of the response, which suggests a SP-CGRP synergism in this artery.

The absolute indomethacin effect in some unilateral headaches may, at least partially, be cyclooxygenase inhibition-independent. Aspirin and indomethacin, for example, may inhibit the neurogenically induced plasma extravasation in rat dura mater. Given the putative involvement of trigeminal neuropeptides in the pathophysiology of these conditions, the influence of cyclooxygenase inhibitors (indomethacin, acetylsalicylic acid (ASA) and naproxen) has been studied upon substance P, calcitonin gene-related peptide and vasoactive intestinal peptide (VIP)-induced vasodilatation in PGF2a precontracted porcine ophthalmic arteries in vitro. None of the cyclooxygenase inhibitors significantly altered the effects of calcitonin gene-related peptide. The 10-10 mol/1 VIP-induced relaxation was inhibited significantly by all three cyclooxygenase inhibitors. Substance P-induced relaxation (from 10-10 to 10-8 mol/l) was enhanced by ASA and inhibited both by naproxen and, to a lesser extent, by indomethacin. The results suggest mainly that VIP-induced relaxations, particularly at lower concentrations, may be inhibited by all three cyclooxygenase inhibitors, and that naproxen, to a greater extent than aspirin or indomethacin, showed a tendency to inhibit vasodilatation induced by all peptides.

The digiti quinti sign (DQS) was described as an indication of mild hemiparesis. It consists of a wider space between the fourth and fifth fingers at the affected side when the patient extends both arms horizontally to the front with the palms down. The three successively examined sporadic hemiplegic migraine patients presented herein disclosed no other neurological abnormality except the interictally present DQS on the same side of the motor deficits. This sign is perhaps clinically useful for the diagnosis of hemiplegic migraine.

OBJECTIVE: To test the ability of a 5HT2a/c (trazodone) antagonist, to improve depression and motor function in Parkinson' disease (PD). METHOD: Twenty PD patients with and without depression were randomly assigned to receive trazodone (group 1) or not (group 2). They were evaluated through UPDRS and Hamilton Depression Rating Scale (HAM-D). RESULTS: For the UPDRS the mean score of group 2 was 33.1 ± 19.7 and 37.1 ± 18.0 at the end. For the group 1, the corresponding scores were 31.4 ± 11.3 and 25.9 ± 13.7. The variations in the Mann-Whitney test were 0.734 at the initial moment and 0.208 at the final moment. The variation in the comparison of the initial moment with the final moment was 0.005 providing statistical significance. For the HAM-D, the mean score went up 4 points in group 2, contrary to a 5.5 points decrease in group 1. CONCLUSION: Data analysis shows that this agent significantly improves depression, but the motor function improved only in the depressed patients. Because of the known anti-dopaminergic property of the 5-HT2c receptors, a possible approach for depression in PD could be the use of 5-HT2c antagonists, similarly to the use of atypical neuroleptics in case of psychotic symptoms.

Whiplash is a controversial concept. The real nature and prevalence of whiplash-related headaches are not known. To address this issue, the history of severe trauma was collected from occipital/nuchal headache-cervicogenic headache, migraine and tension-type headache patients attending a neurology office. The two last groups of patients had significantly less history of trauma in their medical records, with longer time span between the injury and the headache onset. Whiplash may induce preferably cervicogenic and tension-type headaches.

Migraine affects the cortical physiology and may induce dysfunction both ictally and interictally Although visual symptoms predominate during aura, other contiguous cortical areas related to less impressive symptoms are also impaired in migraine. Answers from 72.2% migraine with aura and 48.6% of migraine without aura patients on human faces and objects recognition, colour perception, proper names recalling and memory in general showed dysfunctions suggestive of prosopagnosia, dyschromatopsia, ideational apraxia, alien hand syndrome, proper name anomia or aphasia, varying in duration and severity. Symptoms frequently occurred in a successively building-up pattern fitting with the geographical distribution of the various cortical functions. When specifically inquired, migraineurs reveal less evident symptoms that are not usually considered during routine examination. Spreading depression most likely underlies the aura symptoms progression. Interictal involvement indicates that MWA and MWoA are not completely silent outside attacks, and that both subforms of migraine may share common mechanisms.

Concentration-response curves of isometric tension studies on isolated blood vessels are obtained traditionally. Although parameters such as Imax, EC50 and pA2 may be readily calculated, this method does not provide information on the temporal profile of the responses or the actual nature of the reaction curves. Computerized data acquisition systems can be used to obtain average data that represent a new source of otherwise inaccessible information, since early and late responses may be observed separately in detail.

The clinical manifestations of supraorbital neuralgia are apparently only incompletely known. The lack of awareness of this head pain may possibly be due to its rarity and problems with making the diagnosis. In the present work, the long-term result of minor, decompressive surgery of the supraorbital nerve in five patients is reported. The immediate improvement was good and, after a mean observation time of more than 6 years, an improvement of 50% to 100% was observed (mean, circa 85%). In the two patients with the longest postoperative observation time, approximately 8 years, pain has not recurred. The pain was severe, leading to suicidal thoughts in several patients. The long-term course was intermittent or continuous. The pain was generally unilateral, but was bilateral in one patient. Generally, there was lack of, or only minor benefit from drug treatment, including carbamazepine and indomethacin. There was clearly tenderness over the supraorbital nerve, especially at its outlet, and in some subjects occasionally, a slight local loss of sensation. Definite trigger zones were not present. Supraorbital nerve blockade generally provided instant and considerable pain relief. The persistence of protracted unilateral forehead/ocular pain, tenderness over the nerve, and repeated blockade effect strongly suggests the diagnosis.

The criteria for the confirmation of brain death (BD) using transcranial Doppler ultrasonography (TCD) state that flow should not be observed in the internal carotid artery (ICA). In 94 brain-dead patients examined by TCD, 19 (20%) had flow in the ICA despite cerebral circulatory arrest. There was no difference in the clinical characteristics of patients with or without isolated flow in the ICA. Shunting of blood from the ICA into the external carotid system or the arrest of blood flow at a higher level than the carotid siphon may explain our findings. Our results show that the current TCD criteria can result in a high rate of false negatives. We suggest that the TCD criteria for the diagnosis of brain death should be reviewed.

Proposals for the diagnostic criteria for hemicrania continua (HC) and also for the nosological status of HC are set forth. The clinical constellation of symptoms and signs making up HC consists of: unilaterality without side shift; absolute indomethacin effect; and long-lasting repetitive attacks of varying duration, eventually with a chronic pattern, the pain being mild to severe. For the typical clinical picture of HC, including a positive ‘indotest’, we propose the term hemicrania continua vera. More or less analogous, but ‘indotest-negative’ clinical pictures have provisionally been termed hemicrania generis incerti (of undetermined nature). At the present level of knowledge, the diagnosis of hemicrania generis incerti should be made mostly by exclusion. HC may possibly best be classified along with chronic paroxysmal hemicrania (CPH) as this is the only other headache absolutely responsive to indomethacin. The bond between these two headaches on the one hand and cluster headache on the other should, at most, be a loose one. Interrelationships of these four classifiable headaches are briefly discussed.

As manifestações neurológicas associadas à deficiência de vitamina B12 incluem polineuropatia, mielopatia, demência e neuropatia óptica. O diagnóstico laboratorial é feito através da dosagem sérica de cianocobalamina ou homocisteína e da excreção urinária de ácido metilmalônico. No estudo anatomopatológico observa-se na microscopia a destruição da mielina e de axônios vistos na substância branca. A região mais comumente afetada é o cordão posterior cervical e/ou torácico. O acometimento da coluna lateral é raro, ocorrendo em casos graves e avançados. O tratamento consiste na reposição de vitamina B12 e a resposta depende da gravidade do quadro e do tempo transcorrido entre o inicio dos sintomas e inicio do tratamento. Relatamos o caso de um paciente que apresentou, como manifestação de deficiência de vitamina B12, mielite transversa. O estudo morfológico da medula demonstrou comprometimento dos tractos cortico-espinhais lateral e anterior, da coluna dorsal e ainda do tracto espino-talâmico. Vitamin B12 deficiency may induce neuropathy, myelopathy, dementia and optic neuropathy. The diagnosis is established by vitamin B12, homocysteine and methylmalonic acid measurements. Myelin and axon destruction in the white matter of the spinal cord are observed. The posterior column of the cervical and thoracic level is the most common involved area. The involvement of the anterior column is restricted to advanced and relatively severe cases. Treatment is based on vitamin B12 injections, and the prognosis depends on the stage of vitamin deficiency and deterioration at treatment onset. We report a case with transverse myelitis due to vitamin B12 deficiency. This picture is relatively uncommon, however, we believe patients with transverse myelitis should have vitamin B12 studies as part of the diagnosis work up.

A doença de Creutzfeldt-Jakob (DCJ) é a encefalopatia espongiforme subaguda transmissível mais frequente nos seres humanos. Aproximadamente 85% dos casos pertencem à forma esporádica da doença. Os outros 15% consistem na forma genética e iatrogênica. Relatamos o caso de uma paciente com a forma esporádica da doença de Creutzfeldt-Jakob, com comprometimento medular e apresentação clínica caracterizada por síndrome demencial e cerebelar, miofasciculação com arreflexia difusa e crises convulsivas do tipo tônico-clônico generalizada. É rara a associação das duas últimas manifestações clínicas. O caso foi considerado como provável DCJ até confirmação por autópsia e imunohistoquímica. Concluímos que se deve sempre pensar na DCJ em pacientes que apresentam demência rapidamente progressiva e, na ausência de sinais piramidais ou extrapiramidais, pensar em acometimento periférico e/ou medular.