Neurological involvement by Behçet's syndrome: clinical features, diagnosis, treatment and outcome.

Neurological involvement in Behçet's syndrome arises predominately through an inflammatory meningoencephalitis characterised by perivenular inflammation due to activation of Th-17 immunological pathways. The brainstem is involved in 50% of cases, the diencephalon and other areas of the brain in 30%, and the spinal cord in 10%. Movement disorders and epilepsy may occur. Psychiatric syndromes may arise with brain and brainstem involvement, and cognitive disorders relate to the brain disease, to circulating inflammatory factors, and to fatigue and despondency. Eighty per cent of cases begin with a relapsing disease course, of whom 70% have only one attack, and 30% have a progressive disease course either from onset or following an initially relapsing course. Venous thrombosis leading to intracranial hypertension and cerebral venous infarction is less common and caused by inflammation in affected veins and a circulating prothrombotic state. Arterial involvement is rare and relates to an arteritis affecting large-sized and medium-sized vessels within the brain leading to infarction, subarachnoid and parenchymal haemorrhage, aneurysm formation and arterial dissection. There is a newly recognised disorder of cerebral cortical hypoperfusion. Cranial neuropathy, peripheral neuropathy and myositis are rare. There has been significant progress in understanding the pathophysiology and treatment of the systemic disease, leading to improved outcomes, but there has been no randomised trial of treatment in the neurological disorder.

Osmotic demyelination syndrome despite appropriate gradual correction of moderate hyponatraemia.

Osmotic demyelination syndrome characteristically follows rapid correction of hyponatraemia. We present a young woman with a subacute progressive brainstem syndrome and diffuse pontine signal abnormality on MR imaging, diagnosed as osmotic demyelination syndrome. The case posed a diagnostic challenge due to comorbid Behçet's disease and the absence of significant fluctuation in her serum sodium concentration. Osmotic demyelination syndrome is not limited to patients with rapidly corrected hyponatraemia, especially when there are other risk factors. These factors, all present in this patient, include hypokalaemia, hypophosphataemia, malnutrition, harmful alcohol use and liver dysfunction.

Enfermedad de Behçet y embarazo: a propósito de un caso / Behçet's disease and pregnancy: a propos of a case

Introducción: La enfermedad de Behçet es una enfermedad crónica, multisistémica, de causa desconocida, caracterizada clínicamente por la presencia de aftas orales, genitales, manifestaciones sistémicas y prueba de patergia positiva. Es una enfermedad poco frecuente y predomina en el sexo masculino, por lo que su relación con el embarazo es escasamente reportada en la literatura. Objetivo: Mostrar las manifestaciones clínicas, evolución y complicaciones presentes durante el embarazo en una paciente con enfermedad de Behçet. Presentación del Caso: Se presenta el caso de una paciente de 26 años de edad con diagnóstico de Enfermedad de Behçet, la cual presentó complicaciones durante su embarazo que tuvieron como resultado un producto final de la gestación bajo peso. Conclusiones: La Enfermedad de Behçet puede provocar complicaciones sobre el embarazo como las presentadas en este caso, a su vez el embarazo puede producir actividad de la enfermedad, por lo que lograr un adecuado control de esta previo al embarazo y llegar a tener una correcta planificación familiar son vitales para llegar a un feliz término del embarazo en estas pacientes(AU)

Introduction: Behçet's disease is a chronic and multisystem illness, of unknown cause, clinically characterized by the presence of oral sores, genital, systemic manifestations and positive pathergy test. It is a not very frequent illness and prevails in the masculine sex; due to this its relationship with the pregnancy is barely reported in the literature. Objective: To show the clinical manifestations, evolution and complications that are present during the pregnancy in a patient with Behçets illness. Cases presentation: A case of a 26-year-old patient with diagnose of Behçes illness is presented, which show complications during its pregnancy that result at the end of her pregnancy; and underweight. Conclusions: The Behçets illness could cause complications during pregnancy like those presented in this case, in turn the pregnancy can produce activity of the illness, and for that reason, to achieve an appropriate control of the illness before the pregnancy with the aim of having a correct family planning, which is vital to arrive to a happy end of the pregnancy in these patients(AU)

Mielite longitudinal secundária à síndrome de Behçet: relato de caso / Longitudinal myelitis secondary to Behçet's syndrome: case report

JUSTIFICATIVA E OBJETIVOS: A síndrome de Behçet é uma doença inflamatória sistêmica idiopática e rara no Brasil. A ocorrência de manifestações neurológicas caracteriza a Neuro-Behçet e o envolvimento da medula espinhal é fator de mau prognóstico.O objetivo deste estudo foi relatar um caso de mielite longitudinal secundária a esta doença, e revisar a literatura acerca das manifestações neurológicas associadas à síndrome.RELATO DO CASO: Paciente do sexo masculino, adulto jovem,foi encaminhado para investigação de paresia nos membrosinferiores. Em menos de um mês, já havia se instalado paraplegia hiporreflexa e perda do controle esfincteriano, além de nível sensitivo em T5, paresia e hipoestesia nos membros superiores.A ressonância nuclear magnética (RNM) mostrou imagem compatível com mielite longitudinal. Sorologias virais e anticorposa ssociados a doenças reumatológicas foram negativos. O exame dermatológico detectou pequenas lesões cutâneas e uma úlcera genital cicatrizada. Genotipagem HLA mostrou positividade para B51, o maior fator de risco conhecido para a síndrome deBehçet. Instituiu-se teste terapêutico com metilprednisolona emalta dose, com boa resposta. O seguimento com ciclofosfamida eprednisona impediu a progressão do déficit neurológico, no entanto este já havia se tornado irreversível.CONCLUSÃO: O diagnóstico da síndrome de Behçet se baseia na história clínica e exame físico, com preenchimento de critérios definidos internacionalmente. O paciente em questão sofreu sequela neurológica grave devido ao atraso no diagnóstico. Nã oexistem testes específicos, portanto só o conhecimento da doença permitirá o diagnóstico precoce da síndrome e poderá evitar que outros pacientes evoluam com sequelas incapacitantes.

BACKGROUND AND OBJECTIVES: Behçet's syndrome is an idiopathic inflammatory disease and rare in Brazil. The occurrence of neurological symptoms characteristic of Neuro-Behçet's disease and spinal cord involvement is a bad prognostic factor. The aim of this study was to report a case of longitudinal myelitis secondary to this disease, and review the literature about the neurological manifestations associated with the syndrome.CASE REPORT: A male patient, young adult, was referred for investigation of weakness in lower limbs. In less than a month, was already installed hyporeflexia paraplegia and loss of sphincter control, and sensory level at T5, paresis and hypoesthesia in the upper limbs. Magnetic resonance imaging (MRI) showed an image compatible with longitudinal myelitis. Viral serology and antibodies associated with rheumatic diseases were negative. The dermatological examination detected a small skin lesions and genital ulcers healed. Genotyping was positive for HLA B51, the largest known risk factor for Behçet's syndrome. Was instituted therapeutic trial with high-dose methylprednisolone, with good response. The follow-up with cyclophosphamide and prednisone to prevent progression of neurological deficit, but this had become irreversible.CONCLUSION: The diagnosis of Behçet's syndrome is based on clinical history and physical examination, reaching internationally defined criteria. Our patient suffered severe neurological sequelae due to delay in diagnosis. There are no specific tests, so only the knowledge of the disease will allow early diagnosis of the syndrome and may avoid other patients evolve with disabling sequelae.

Doença de Behçet e tromboembolismo múltiplo / Behçet?s disease and multiple thromboembolism

A doença de Behçet (DB) é uma vasculite sistêmica, recorrente e crônica com manifestações que incluem úlceras orogenitais, uveíte, sinovite, tromboflebite e sintomas envolvendo o sistema nervoso central, trato gastrointestinal e pulmão. Apresenta etiologia desconhecida e diagnóstico baseado em critérios clínicos. A maioria dos casos apresenta manifestações benignas, no entanto, ocasionalmente cursa com trombose de grandes vasos que podem levar ao óbito. Os autores descrevem um caso peculiar, tanto pela dificuldade diagnóstica quanto pela gravidade das manifestações vasculares, e desfecho letal.