RESUMO
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.
RESUMO
BACKGROUND AND OBJECTIVES: Brain magnetic resonance is a major exam to evaluate thunderclap headache, after excluding subarachnoid hemorrhage. This study aimed at reporting a case of brainstem cavernous angioma (cavernoma) where clinical presentation and computerized tomography have suggested intraventricular hemorrhage. CASE REPORT: Female patient, 55 years, was referred to the hospital with a history of new headache 10 days ago. Pain onset was sudden, pressure-type, severe, located in the occipital region with irradiation throughout the head, followed by severe photophobia, nauseas, diplopia and blurred vision. Neurological evaluation has revealed anisocoria, complete ophthalmoplegia and right eyelid ptosis. Cranial CT has shown blood in the third ventricle. Conventional brain arteriography has not shown aneurysm, arteriovenous malformation or venous sinus thrombosis. At lumbar puncture, an opening water pressure of 45cm was found and liquor analysis was normal. Brain resonance has shown oval lesion (1.0x1.0x0.6cm) of exophytic aspect in the interpeduncular cistern and third ventricle, compatible with brainstem cavernoma. CONCLUSION: In this case, magnetic resonance was essential for the diagnosis, since routine exams (brain tomography, liquor puncture and arteriography) could not define it. Further studies are needed to explain how magnetic resonance impacts investigation. .
JUSTIFICATIVA E OBJETIVOS: A ressonância magnética cerebral é um exame importante na investigação da cefaleia em trovoada, após a exclusão de hemorragia subaracnoidea. O objetivo deste estudo foi relatar um caso de angioma cavernoso (cavernoma) no tronco cerebral, em que a apresentação clínica e tomografia computadorizada sugeriram uma hemorragia intraventricular. RELATO DO CASO: Paciente do gênero feminino, 55 anos, foi encaminhada ao hospital com uma história de cefaleia nova há 10 dias. A dor teve início súbito, do tipo pressão, de forte intensidade, localizada na região occipital com irradiação para toda a cabeça, acompanhada de fotofobia intensa, náuseas, diplopia e visão turva. O exame neurológico revelou anisocoria, oftalmoplegia completa e ptose palpebral à direita. Tomografia computadorizada de crânio mostrou sangue no terceiro ventrículo. Arteriografia cerebral convencional não apresentou aneurisma, malformação arteriovenosa ou trombose de seios venosos. Na punção lombar, uma pressão de 45cm de água de abertura foi encontrada e a análise do líquido cefalorraquidiano foi normal. A ressonância de crânio revelou lesão oval (1,0x1,0x0,6cm) de aspecto exofítica na cisterna interpeduncular e terceiro ventrículo compatível com cavernoma de tronco cerebral. CONCLUSÃO: No caso descrito, a ressonância magnética foi essencial para o diagnóstico, uma vez que os exames de rotina (tomografia de crânio, punção de líquor e arteriografia) não conseguiram defini-lo. Maiores estudos são necessários para esclarecer como a realização de ressonância magnética impacta a investigação. .
RESUMO
The frontal assessment battery (FAB) is a bedside cognitive scale designed to measure executive functions. Huntington's disease (HD) is a neurodegenerative disorder characterized by motor, behavioral, and cognitive dysfunction. The aim of this study was to check the validity of the FAB for the evaluation of cognitive impairment in patients with HD. Forty-one patients diagnosed with HD and 53 healthy controls matched by education, sex and age were evaluated with a validated Brazilian version of the UHDRS, the VFT, the SDMT, the SIT, the MMSE, and the FAB. The diagnosis of HD was made by DNA analysis. FAB scores were lower in patients than in the controls (p < 0.001) and had significant correlations with the VFT (r = 0.79; p < 0.05), the SDMT (r = 0.80; p < 0.05), the SIT (r = 0.72; p < 0.05), the MMSE (r = 0.83; p < 0.05), the FCS (r = 0.79; p < 0.05) and the motor section of the UHDRS (r = -0.80; p < 0.05). The FAB differentiated between HD patients in the initial and later stages of the disease. The one-year longitudinal evaluation revealed a global trend toward a worsening in the second score of the FAB. The results demonstrate that the FAB presents good internal consistency and also convergent and discriminative validity; therefore it is a useful scale to assess executive functions and to evaluate cognitive impairment in patients with HD.
