Insights on the terminology and on some pathophysiological aspects of cluster headache and related conditions / Insights sobre a terminologia e alguns aspectos fisiopatológicos da cefaléia em salvas e condições relacionadas

Introduction: Cluster headache and the more recently reported "related conditions",namely Chronic Paroxysmal Hemicrania, Short lasting, unilateral, Neuralgiform Headache with Conjunctival Injection and Tearing (SUNCT) Syndrome and (possibly also) Hemicrania Continua, are one of the fascinating groups of conditions in Neurology whose cardinal features are the almost absolute unilaterality of pain, its excruciating severity, besides the prominent mainly facial autonomic disturbances and overall the intriguing biorhythmicity. Beyond any doubt, the progress of our knowledge about the mechanisms of these conditions has been considerable, but there is always room for reflection on where we are and where we can go. Objective: The objectives of the present study are to analyze the metrics of publications on the pathophysiology of these conditions, to explore in detail how proper the term "Trigeminal Autonomic Cephalgia" is, and to comment on their numerous synonyms. Comment: Much is needed to know the exact structures and circuitry involved in the pathophysiology of these conditions; accordingly, a non-compromising and just descriptive term might be useful. Along this line of reasoning and bearing in mind the cardinal points of such conditions, namely, pain in the trigeminal territory, prominent autonomic symptoms, prominent chronobiological features, and the excruciating character of the pain, a terminological possibility would be: Unilateral with Prominent Rhythmicity and Autonomic Symptoms Excrutiating Cephalgia (UPRASEC).

Introdução: Cefaleia em salvas e as "condições relacionadas" relatadas mais recentemente, nomeadamente Hemicrania Paroxística Crónica, Cefaleia Neuralgiforme unilateral de curta duração com Síndrome de Injecção e Lacrimejamento Conjuntival (SUNCT) e (possivelmente também) Hemicrania Continua, são um dos grupos fascinantes de condições em Neurologia cujas características cardinais são a unilateralidade quase absoluta da dor, sua gravidade excruciante, além dos proeminentes distúrbios autonômicos principalmente faciais e, em geral, da intrigante biorritmicidade. Sem dúvida, o progresso do nosso conhecimento sobre os mecanismos destas condições tem sido considerável, mas há sempre espaço para reflexão sobre onde estamos e para onde podemos ir. Objetivo: Os objetivos do presente estudo são analisar as métricas das publicações sobre a fisiopatologia dessas condições, explorar detalhadamente o quão adequado é o termo "Cefalgia Autonômica Trigeminal" e comentar seus numerosos sinônimos. Comentário: É necessário muito conhecimento das estruturas e circuitos exatos envolvidos na fisiopatologia dessas condições; consequentemente, um termo não comprometedor e apenas descritivo pode ser útil. Nessa linha de raciocínio e tendo em mente os pontos cardeais de tais condições, a saber, dor no território trigêmeo, sintomas autonômicos proeminentes, características cronobiológicas proeminentes e o caráter excruciante da dor, uma possibilidade terminológica seria: Unilateral com Ritmicidade Proeminente e sintomas autonômicos excruciantes da cefalgia (UPRASEC).

Paroxysmal Hemicrania-Trigeminal Neuralgia (PH-Tic) syndrome with no structural lesion in a Brazilian woman: the first case-report / Síndrome de Neuralgia Paroxística Hemicrânia-Trigêmea (PH-Tic) sem lesão estrutural em mulher brasileira: primeiro relato de caso

Introduction: In Brazil there is only one case report of a patient diagnosed with Paroxysmal Hemicrania-Trigeminal (PH-Tic) syndrome reported, however it was observed in a patient with Chiari I malformation. Objective: Here, we describe the first case of primary PH-Tic syndrome in the country. Method: Case report. CARE guideline was used to guide the structuring of this article. This case report was approved by the ethics committee and has been registered under the protocol number 70705623.7.0000.5440 on "Plataforma Brasil". Results:A 72-year-old woman with a five-month history of headaches was admitted at our headache outpatient clinic. The pain was sharp, intense, localized in the periorbital and left temporal regions. Blood counts, liver, renal and thyroid function were normal, as well as brain magnetic resonance imaging. Despite using carbamazepine, the patient had pain in only the left side of the face. Indomethacin was added until the dose of 100 mg a day and resulted in improvement of headache frequency. Conclusion: PH-Tic should be hypothesized in patients with short-lasting headaches associated with facial pain that partially improve with carbamazepine or indomethacin.

Introdução: No Brasil há apenas um relato de caso de paciente com diagnóstico de síndrome Paroxística Hemicrania-Trigeminal (PH-Tic), porém foi observado em um paciente com malformação de Chiari I. Objetivo: Descrevemos aqui o primeiro caso de síndrome PH-Tic primária no país. Método: Relato de caso. A diretriz CARE foi utilizada para orientar a estruturação deste artigo. Este relato de caso foi aprovado pelo comitê de ética e registrado sob o número de protocolo 70705623.7.0000.5440 na "Plataforma Brasil". Resultados: Uma mulher de 72 anos com história de cefaleias há cinco meses foi internada em nosso ambulatório de cefaleias. A dor era aguda, intensa, localizada nas regiões periorbital e temporal esquerda. Os hemogramas, as funções hepática, renal e tireoidiana estavam normais, assim como a ressonância magnética cerebral. Apesar do uso de carbamazepina, o paciente apresentava dor apenas no lado esquerdo da face. A indometacina foi adicionada até a dose de 100 mg ao dia e resultou em melhora da frequência da cefaleia. Conclusão: O PH-Tic deve ser hipotetizado em pacientes com cefaleias de curta duração associadas a dores faciais que melhoram parcialmente com carbamazepina ou indometacina.

Cefaleia Trigêmino-autonômica e Neuromielite Óptica / Trigemino-autonomic Headache and Neuromyelitis Optica

As cefaleias trigêmino-autonômicas compartilham os aspectos clínicos da cefaleia, além de proeminentes sintomas disautonômicos crânio faciais. A Neuromielite Óptica (NMO) ou Doença de Devic é uma doença inflamatória grave, desmielinizante e auto-imune do sistema nervoso central que acomete, preferencialmente, os nervos ópticos e a medula espinhal, causando neurite óptica aguda, uni ou bilateral, e mielite transversa. O objetivo desse trabalho é relatar um caso clínico de NMO, cuja manifestação inicial é atípica. Uma revisão de literatura com as palavras-chaves Neuromielite Óptica e Cefaleia Trigêminoautonômica foi realizada no PubMed e foram selecionados os artigos e relatos de casos mais relevantes sobre o assunto. Conclui-se que estas duas doenças podem ter em comum uma alteração hipotalâmica e uma doença desmielinizante grave pode se iniciar com uma cefaleia trigêmino-autonômica. (AU)

The Trigeminal-autonomic headaches share the clinical features of headache, as well as prominent facial skull disautonomic symptoms. The Neuromyelitis Optica (NMO) or Devic's disease is a severe inflammatory disease, demyelinating and autoimmune of the central nervous system that affects mainly the optic nerves and spinal cord, causing acute optic neuritis, unilateral or bilateral, and transverse myelitis. The objective of this study is to report a case of NMO, whose initial manifestation is atypical. A literature review with keywords Neuromyelitis Optica and Trigeminal-autonomic Headache has conducted in PubMed and we have selected the most relevant articles and case reports on the subject. In conclusion, these two diseases may have a common hypothalamic disturbance and a severe demyelinating disease can start with a trigeminal-autonomic headache. (AU)

Invasive Sphenoid Sinus Aspergillosis Presenting Hemicrania Continua-Like Headache

Hemicrania continua (HC) is an indomethacin-responsive primary headache. Owing to continuous unilateral headache and clinical rarity, a great attention should be paid during the diagnosis of HC to exclude secondary causes of headache. Various pathologies have been described for HC-like headache. We describe a 64-year old man with invasive sphenoid sinus aspergillosis who presented continuous unilateral headache, trigeminal autonomic symptoms and response to oral indomethacin 225 mg/day. He was treated with intranasal ethmoidectomy and antifungal agent, and his headache has greatly improved.

Clinical Features of Cluster Headache Patients in Korea

Cluster headache (CH) is a rare underdiagnosed primary headache disorder with very severe unilateral pain and autonomic symptoms. Clinical characteristics of Korean patients with CH have not yet been reported. We analyzed the clinical features of CH patients from 11 university hospitals in Korea. Among a total of 200 patients with CH, only 1 patient had chronic CH. The average age of CH patients was 38.1 ± 8.9 years (range 19–60 years) and the average age of onset was 30.7 ± 10.3 years (range 10–57 years). The male-to-female ratio was 7:1 (2.9:1 among teen-onset and 11.7:1 among twenties-onset). Pain was very severe at 9.3 ± 1.0 on the visual analogue scale. The average duration of each attack was 100.6 ± 55.6 minutes and a bout of CH lasted 6.5 ± 4.5 weeks. Autonomic symptoms were present in 93.5% and restlessness or agitation was present in 43.5% of patients. Patients suffered 3.0 ± 3.5 (range 1–25) bouts over 7.3 ± 6.7 (range 1–30) years. Diurnal periodicity and season propensity were present in 68.5% and 44.0% of patients, respectively. There were no sex differences in associated symptoms or diurnal and seasonal periodicity. Korean CH patients had a high male-to-female ratio, relatively short bout duration, and low proportion of chronic CH, unlike CH patients in Western countries.

Cluster Headache Presenting With Raeder's Syndrome

No abstract available.

Cefaléias inusitadas primárias reconhecidas pela sociedade internacional de cefaléias: revisão breve / Primary unsual headaches recognized by the international headaches society: brief review

As cefaléias são dores que assumem características clínicas, genéticas, fisiopatológicas e terapêuticas que as distinguem fundamentalmente das demais dores do ser humano. Dividem-se entre dores primárias ? as mais freqüentes ? e secundárias, de acordo com a presença ou não de desordens causadoras subjacentes. Nesta revisão salientamos os principais aspectos das cefaléias raras listadas pela Classificação Internacional das Cefaléias e que incluem: a hemicrania paroxística, SUNCT/SUNA, cefaléia primária em facadas, cefaléia primária da tosse, cefaléia primária do exercício, cefaléia primária associada à atividade sexual, cefaléia hípnica, cefaléia em trovoada, hemicrania contínua e a cefaléia nova diária e persistente. O diagnóstico depende da colheita de uma história cuidadosa e atenta, posto que reside no quadro clínico a chave para o diagnóstico diferencial.

Headaches are conditions fundamentally distinct from pain elsewhere due to clinical, genetic, pathophysiological and therapeutic reasons. They are first and foremost divided into primary - much more common - and secondary diseases according to the presence of underlying causes. In this short review, we highlight the most characteristic of the relatively rare primary headaches listed in the HIS Cassification of Headache Disorders, including proxysmal hemicrania, SUNCT/SUNA, pimary stabbing headache, pimary cough headache, pimary exertional headache, pimary sexual headache, hpnic headache, rimary thunderclap headache, hemicrania continua and new daily persistent headache. Diagnosis depends on a comprehensive case history, as the clue for differentiation among such conditions reside on clinical grounds.

A Case of Brain Stem Neuro-Behcet's Disease Presenting as Paroxysmal Headache With Autonomic Features

Migraine without aura was significantly frequent in Beh et's disease patients. While, the relationship of TAC(trigeminal autonomic cephalalgia) and Beh et's disease was not known. And, it is thought that pathogenesis of proxysmal headache with autonomic features in neuroBehcet's disease as our case may be different from those of clust headache and TAC. A 45-year-old male admitted with left sided periobital poxysmal headache accompained by ipsilateral conjunctival injection and lacrimination with autonomic features. T2WMR image showed about 1.5x2.6x2.6 cm sized mass with high signal intensity surrounded by a rim of low signal intensity in pons. Enhanced MR image showed enhancing mass-like lesion inside the T2 high signal intensity. CSF study revealed neutrophilic pleocytosis with normal pressure, protein and glucose concentration. No malignant cell were seen. Antiphospholipid antibody and cryoglobuline was significantly increased. 3 days after hospitalvisit, oral ulcerations appeared in the patient. He was treated with high-dose steroid for a week and his headache and abnormal findings of CSF study became fully recovered. Two weeks later, the mass was nearly reduced in the pons on follow-up MR image. We suggest to include a careful interviewfor 2ndary TAC as the first neurologic symptom in the diagnostic work-up of Behet's disease.