ABSTRACT
BACKGROUND: Autoimmune encephalitis (AIE) is a group of inflammatory diseases characterized by the presence of antibodies against neuronal and glial antigens, leading to subacute psychiatric symptoms, memory complaints, and movement disorders. The patients are predominantly young, and delays in treatment are associated with worse prognosis. OBJECTIVE: With the support of the Brazilian Academy of Neurology (Academia Brasileira de Neurologia, ABN) and the Brazilian Society of Child Neurology (Sociedade Brasileira de Neurologia Infantil, SBNI), a consensus on the diagnosis and treatment of AIE in Brazil was developed using the Delphi method. METHODS: A total of 25 panelists, including adult and child neurologists, participated in the study. RESULTS: The panelists agreed that patients fulfilling criteria for possible AIE should be screened for antineuronal antibodies in the serum and cerebrospinal fluid (CSF) using the tissue-based assay (TBA) and cell-based assay (CBA) techniques. Children should also be screened for anti-myelin oligodendrocyte glucoprotein antibodies (anti-MOG). Treatment should be started within the first 4 weeks of symptoms. The first-line option is methylprednisolone plus intravenous immunoglobulin (IVIG) or plasmapheresis, the second-line includes rituximab and/or cyclophosphamide, while third-line treatment options are bortezomib and tocilizumab. Most seizures in AIE are symptomatic, and antiseizure medications may be weaned after the acute stage. In anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, the panelists have agreed that oral immunosuppressant agents should not be used. Patients should be evaluated at the acute and postacute stages using functional and cognitive scales, such as the Mini-Mental State Examination (MMSE), the Montreal Cognitive Assessment (MoCA), the Modified Rankin Scale (mRS), and the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). CONCLUSION: The present study provides tangible evidence for the effective management of AIE patients within the Brazilian healthcare system.
ANTECEDENTES: Encefalites autoimunes (EAIs) são um grupo de doenças inflamatórias caracterizadas pela presença de anticorpos contra antígenos neuronais e gliais, que ocasionam sintomas psiquiátricos subagudos, queixas de memória e distúrbios anormais do movimento. A maioria dos pacientes é jovem, e o atraso no tratamento está associado a pior prognóstico. OBJETIVO: Com o apoio da Academia Brasileira de Neurologia (ABN) e da Sociedade Brasileira de Neurologia Infantil (SBNI), desenvolvemos um consenso sobre o diagnóstico e o tratamento da EAIs no Brasil utilizando a metodologia Delphi. MéTODOS: Um total de 25 especialistas, incluindo neurologistas e neurologistas infantis, foram convidados a participar. RESULTADOS: Os especialistas concordaram que os pacientes com critérios de possíveis EAIs devem ser submetidos ao rastreio de anticorpos antineuronais no soro e no líquido cefalorraquidiano (LCR) por meio das técnicas de ensaio baseado em tecidos (tissue-based assay, TBA, em inglês) e ensaio baseado em células (cell-based assay, CBA, em inglês). As crianças também devem ser submetidas ao rastreio de de anticorpo contra a glicoproteína da mielina de oligodendrócitos (anti-myelin oligodendrocyte glycoprotein, anti-MOG, em inglês). O tratamento deve ser iniciado dentro das primeiras 4 semanas dos sintomas, sendo as opções de primeira linha metilprednisolona combinada com imunoglobulina intravenosa (IGIV) ou plasmaférese. O tratamento de segunda linha inclui rituximabe e ciclofosfamida. Bortezomib e tocilizumab são opções de tratamento de terceira linha. A maioria das crises epilépticas nas EAIs são sintomáticas, e os fármacos anticrise podem ser desmamadas após a fase aguda. Em relação à encefalite antirreceptor de N-metil-D-aspartato (anti-N-methyl-D-aspartate receptor, anti-NMDAR, em inglês), os especialistas concordaram que agentes imunossupressores orais não devem ser usados. Os pacientes devem ser avaliados na fase aguda e pós-aguda mediante escalas funcionais e cognitivas, como Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment (MoCA), Modified Rankin Scale (mRS), e Clinical Assessment Scale in Autoimmune Encephalitis (CASE). CONCLUSãO: Esta pesquisa oferece evidências tangíveis do manejo efetivo de pacientes com EAIs no sistema de saúde Brasileiro.
Subject(s)
Consensus , Encephalitis , Humans , Encephalitis/diagnosis , Encephalitis/therapy , Encephalitis/immunology , Brazil , Child , Adult , Hashimoto Disease/diagnosis , Hashimoto Disease/therapy , Delphi Technique , Autoantibodies/bloodABSTRACT
INTRODUCTION: Although neurological complications are well recognized in patients with sickle cell disease, myelopathy has been rarely described, with few reported cases of compressive and ischemic myelopathy. We present the first case report of longitudinally extensive myelitis (LETM) in SCD and review the differential diagnosis of myelopathy in these patients. CASE PRESENTATION: We report the case of a 29-year-old African-Brazilian man with SCD, who experienced a subacute flaccid paraparesis, with T2 sensory level and urinary retention. Cerebrospinal fluid analysis showed a lymphocytic pleocytosis and increased protein levels. MRI disclosed a longitudinally extensive spinal cord lesion, with a high T2/STIR signal extending from C2 to T12. We searched Medline/PubMed, Embase, Scopus, and Google Scholar databases for myelopathy in SCD patients. DISCUSSION: Spinal cord compression by vertebral fractures, extramedullary hematopoietic tissue, and Salmonella epidural abscess have been reported in SCD. We found only three case reports of spinal cord infarction, which is unexpectedly infrequent compared to the prevalence of cerebral infarction in SCD. We found only one case report of varicella-zoster myelitis and no previous report of LETM in SCD patients. Specific and time-sensitive causes of myelopathy should be considered in SCD patients. In addition to compression and ischemia, LETM is a possible mechanism of spinal cord involvement in SCD patients.
Subject(s)
Anemia, Sickle Cell , Myelitis , Spinal Cord Compression , Spinal Cord Diseases , Adult , Anemia, Sickle Cell/complications , Humans , Magnetic Resonance Imaging , Male , Myelitis/diagnosis , Myelitis/etiology , Spinal Cord Diseases/diagnosis , Spinal Cord Diseases/etiologySubject(s)
Brain/diagnostic imaging , Cyclophosphamide/administration & dosage , Diabetes Insipidus, Neurogenic , Lupus Erythematosus, Systemic/complications , Methylprednisolone/administration & dosage , Myelitis, Transverse , Spinal Cord/diagnostic imaging , Antirheumatic Agents/administration & dosage , Diabetes Insipidus, Neurogenic/diagnosis , Diabetes Insipidus, Neurogenic/drug therapy , Diabetes Insipidus, Neurogenic/etiology , Diabetes Insipidus, Neurogenic/physiopathology , Diagnosis, Differential , Female , Humans , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/immunology , Magnetic Resonance Imaging/methods , Myelitis, Transverse/diagnosis , Myelitis, Transverse/etiology , Myelitis, Transverse/physiopathology , Myelitis, Transverse/therapy , Patient Acuity , Plasma Exchange/methods , Spinal Cord/pathology , Symptom Flare Up , Treatment Outcome , Young AdultABSTRACT
Dissection of cervical arteries constitutes a medical emergency. Although relatively rarely, activities classified as sports and recreation may be a cause of arterial dissection independently of neck or head trauma. The purpose of the present paper was to present a series of cases of cerebrum-cervical arterial dissection in individuals during or soon after the practice of these sports activities. Methods Retrospective data on patients with arterial dissection related to sports and recreation. Results Forty-one cases were identified. The most frequently affected vessel was the vertebral artery. A large variety of activities had a temporal relationship to arterial dissection, and jogging was the most frequent of these. This is the largest case series in the literature. Conclusion Arterial dissection may be a complication from practicing sports.
A dissecção das artérias cervicais é uma emergência médica. Embora de forma relativamente rara, certas atividades descritas como esportes e recreação podem ser a causa de dissecção arterial independentemente de trauma de crânio ou cervical. O propósito do presente estudo é apresentar uma série de casos de dissecção de artérias cérebro-cervicais em indivíduos durante ou logo após a prática destas atividades desportivas. Métodos Dados retrospectivos de pacientes com dissecção arterial relacionada à prática de esportes e recreação. Resultados Quarenta e um casos foram identificados. A artéria mais frequentemente afetada foi a vertebral. Uma grande variedade de atividades teve relação temporal com a dissecção arterial, sendo a corrida a mais frequente delas. Esta é a maior série de casos da literatura. Conclusão Dissecção arterial pode ser uma complicação da prática de esportes.
Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Athletic Injuries/complications , Carotid Artery, Internal, Dissection/etiology , Recreation , Sports/statistics & numerical data , Vertebral Artery Dissection/etiology , Cerebral Angiography , Carotid Artery, Internal, Dissection/pathology , Headache/etiology , Retrospective Studies , Risk Factors , Stroke/etiology , Vertebral Artery Dissection/pathologyABSTRACT
UNLABELLED: Dissection of cervical arteries constitutes a medical emergency. Although relatively rarely, activities classified as sports and recreation may be a cause of arterial dissection independently of neck or head trauma. The purpose of the present paper was to present a series of cases of cerebrum-cervical arterial dissection in individuals during or soon after the practice of these sports activities. METHODS: Retrospective data on patients with arterial dissection related to sports and recreation. RESULTS: Forty-one cases were identified. The most frequently affected vessel was the vertebral artery. A large variety of activities had a temporal relationship to arterial dissection, and jogging was the most frequent of these. This is the largest case series in the literature. CONCLUSION: Arterial dissection may be a complication from practicing sports.
Subject(s)
Athletic Injuries/complications , Carotid Artery, Internal, Dissection/etiology , Recreation , Sports/statistics & numerical data , Vertebral Artery Dissection/etiology , Adolescent , Adult , Aged , Carotid Artery, Internal, Dissection/pathology , Cerebral Angiography , Female , Headache/etiology , Humans , Male , Middle Aged , Retrospective Studies , Risk Factors , Stroke/etiology , Vertebral Artery Dissection/pathology , Young AdultABSTRACT
OBJECTIVE: To describe characteristics and provision of care for patients admitted with cerebrovascular disorders (CVD), focusing on ischemic stroke (IS), in a large, public, academic hospital in São Paulo, Brazil. METHOD: We retrieved information about 357 patients with CVD admitted to the Neurology Emergency Department (NED) and Neurology Ward (NW) of our institution. We described patient characteristics and management of IS in NED and in NW. RESULTS: IS was diagnosed in 79.6% of CVD patients admitted to NED; 2.7% were submitted to thrombolysis. Extent of IS investigation and management were significantly different in NED and NW. CONCLUSION: IS patients in our center were younger than in developed countries. IS management was significantly influenced by patient characteristics. This information can aid in planning strategies to decrease stroke burden.
Subject(s)
Emergency Medical Services/standards , Hospitalization/statistics & numerical data , Outcome and Process Assessment, Health Care , Stroke/drug therapy , Thrombolytic Therapy , Adolescent , Adult , Aged , Brazil/epidemiology , Developed Countries , Emergency Medical Services/statistics & numerical data , Emergency Service, Hospital , Epidemiologic Methods , Female , Hospitals, University , Humans , Ischemic Attack, Transient/drug therapy , Male , Middle Aged , Stroke/epidemiology , Young AdultABSTRACT
OBJECTIVE: To describe characteristics and provision of care for patients admitted with cerebrovascular disorders (CVD), focusing on ischemic stroke (IS), in a large, public, academic hospital in São Paulo, Brazil. METHOD: We retrieved information about 357 patients with CVD admitted to the Neurology Emergency Department (NED) and Neurology Ward (NW) of our institution. We described patient characteristics and management of IS in NED and in NW. RESULTS: IS was diagnosed in 79.6 percent of CVD patients admitted to NED; 2.7 percent were submitted to thrombolysis. Extent of IS investigation and management were significantly different in NED and NW. CONCLUSION: IS patients in our center were younger than in developed countries. IS management was significantly influenced by patient characteristics. This information can aid in planning strategies to decrease stroke burden.
OBJETIVO: Descrever características e manejo de pacientes internados com diagnóstico de doença cerebrovascular (DCV), enfocando principalmente o acidente vascular cerebral isquêmico (AVCI), em um hospital público universitário em São Paulo. MÉTODO: Coletamos informações de 357 pacientes com DCV internados no Pronto-Socorro de Neurologia (PSN) e na Enfermaria de Neurologia (EN) de nossa instituição. Descrevemos características dos pacientes e manejo do AVCI no PSN e na EN. RESULTADOS: O AVCI foi diagnosticado em 79,6 por cento dos pacientes com DCV admitidos no PSN; 2,7 por cento foram submetidos a trombólise. A extensão da investigação e o manejo da doença foram significativamente diferentes no PSN e na EN. CONCLUSÃO: os pacientes com AVCI em nosso centro foram mais jovens que em países desenvolvidos. O manejo do AVCI foi influenciado significativamente pelas características dos pacientes. Estas informações podem auxiliar no planejamento de estratégias para diminuir as conseqüências das DCV em nosso meio.
Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Emergency Medical Services/standards , Hospitalization/statistics & numerical data , Outcome and Process Assessment, Health Care , Stroke/drug therapy , Thrombolytic Therapy , Brazil/epidemiology , Developed Countries , Emergency Service, Hospital , Epidemiologic Methods , Emergency Medical Services/statistics & numerical data , Hospitals, University , Ischemic Attack, Transient/drug therapy , Stroke/epidemiology , Young AdultABSTRACT
O acometimento do sistema nervoso central pela tuberculose pode se manifestar de duas formas principais: tuberculose meníngea ou tuberculoma. Esta forma é uma condição que vinha decrescendo progressivamente, mas que encontrou nos dias atuais condições ideais para o seu desenvolvimento. O presente estudo discorre sobre três pacientes que desenvolveram expansão paradoxal de lesões de tuberculose cerebral durante o uso de tuberculostáticos, o que demonstra ser importante o acompanhamento tomográfico de pacientes que apresentem sinais neurológicos no curso do tratamento para a tuberculose. Os dois primeiros pacientes apresentaram o acometimento neurológico associado a tuberculose pulmonar, enquanto no terceiro havia história de tuberculose meníngea prévia. A tomografia computadorizada foi o exame utilizado para o acompanhamento desses pacientes. O tratamento constou do uso de tuberculostáticos e corticóides.