ABSTRACT
Multiple sclerosis (MS) is a chronic, neurological, immune-mediated disease that can worsen in the postpartum period. There is no consensus on the use of immunoglobulin for prevention of disease relapses after delivery. We have shown that the controversial beneficial effect of immunoglobulin given immediately after birth could not be observed in patients with MS.
Subject(s)
Immunoglobulins, Intravenous/therapeutic use , Mothers , Multiple Sclerosis, Relapsing-Remitting/prevention & control , Multiple Sclerosis/drug therapy , Postpartum Period/drug effects , Adult , Case-Control Studies , Female , Humans , Immunoglobulins, Intravenous/pharmacology , Multiple Sclerosis/complications , Multiple Sclerosis/physiopathology , Pregnancy , Pregnancy Complications/drug therapy , Pregnancy Complications/immunology , Pregnancy Outcome , Puerperal Disorders/prevention & control , Recurrence , Retrospective Studies , Risk Assessment , Risk Factors , Treatment OutcomeABSTRACT
We report four Brazilian siblings with Autosomal Dominant Hereditary Motor Sensory Neuropathy with Proximal Dominant Involvement (HMSN-P), a rare form of HMSN, that was characterized by proximal dominant muscle weakness and atrophy onset after the age of 30 years, fasciculation, arreflexia and sensory disturbances with autosomal dominant inheritance. Electrophysiological study and sural nerve biopsy were in the accordance with axonal sensory motor polyneuropathy and laboratorial analysis disclosed serum lipids and muscle enzymes abnormalities. Our report is the first done by a group outside Japan, where the disease initially seemed to be restricted and stressed the phenotypic variability from the original report.
Subject(s)
Hereditary Sensory and Motor Neuropathy/diagnosis , Pedigree , Siblings , Sural Nerve/pathology , Asian People , Biopsy , Brazil , Electromyography , Hereditary Sensory and Motor Neuropathy/genetics , Hereditary Sensory and Motor Neuropathy/pathology , Humans , Male , Middle Aged , PhenotypeABSTRACT
We report four Brazilian siblings with Autosomal Dominant Hereditary Motor Sensory Neuropathy with Proximal Dominant Involvement (HMSN-P), a rare form of HMSN, that was characterized by proximal dominant muscle weakness and atrophy onset after the age of 30 years, fasciculation, arreflexia and sensory disturbances with autosomal dominant inheritance. Electrophysiological study and sural nerve biopsy were in the accordance with axonal sensory motor polyneuropathy and laboratorial analysis disclosed serum lipids and muscle enzymes abnormalities. Our report is the first done by a group outside Japan, where the disease initially seemed to be restricted and stressed the phenotypic variability from the original report.
Relatamos os casos de quatro irmãos brasileiros com Neuropatia Sensitivo Motora Hereditária com Envolvimento Proximal Dominante (HMSN-P), uma forma rara de HMSN caracterizada por fraqueza muscular de predomínio proximal e atrofia de instalação após os 30 anos, fasciculações, arreflexia, distúrbios sensitivos e padrão de herança autossômica dominante. Os estudos eletrofisiológicos e de biópsia do nervo sural confirmaram o diagnóstico de polineuropatia sensitivo-motora com padrão lesional axonal. Laboratorialmente foram constatadas anormalidades séricas no metabolismo lipídico e enzimas musculares. Nosso relato é o primeiro feito por um grupo fora do Japão, onde a doença parecia restrita até então e ressalta a variabilidade fenotípica apresentada nos casos Brasileiros.
Subject(s)
Humans , Male , Middle Aged , Hereditary Sensory and Motor Neuropathy/diagnosis , Pedigree , Siblings , Sural Nerve/pathology , Asian People , Biopsy , Brazil , Electromyography , Hereditary Sensory and Motor Neuropathy/genetics , Hereditary Sensory and Motor Neuropathy/pathology , PhenotypeABSTRACT
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
Basilar artery (BA) dissecting aneurysms pose difficulties to treatment because both bleeding and thrombosis can happen in the same patient, clinical course is unpredictable and high morbidity is usual. We report the case of a 37-year-old woman with a BA aneurysm probably caused by arterial dissection, presenting embolic and hemorrhagic complications. The aneurysm was submitted to endovascular treatment with stenting and coil embolization. Clinical and radiological results were excellent and no complications were observed, suggesting that BA stenting and coil embolization may be a safe and effective treatment for this condition.
Subject(s)
Aortic Dissection/therapy , Basilar Artery , Embolization, Therapeutic/methods , Intracranial Aneurysm/therapy , Stents , Adult , Aortic Dissection/diagnosis , Female , Follow-Up Studies , Humans , Intracranial Aneurysm/diagnosis , Magnetic Resonance Imaging , Treatment OutcomeABSTRACT
Basilar artery (BA) dissecting aneurysms pose difficulties to treatment because both bleeding and thrombosis can happen in the same patient, clinical course is unpredictable and high morbidity is usual. We report the case of a 37-year-old woman with a BA aneurysm probably caused by arterial dissection, presenting embolic and hemorrhagic complications. The aneurysm was submitted to endovascular treatment with stenting and coil embolization. Clinical and radiological results were excellent and no complications were observed, suggesting that BA stenting and coil embolization may be a safe and effective treatment for this condition.
Aneurismas associados a dissecção da artéria basilar (AB) apresentam dificuldades terapêuticas, pois sangramentos e fenômenos trombóticos podem ocorrer no mesmo paciente, seu curso clínico é imprevisível e sua morbidade, alta. Relatamos o caso de uma paciente de 37 anos com aneurisma de artéria basilar provavelmente causado por dissecção arterial, apresentando-se com complicações hemorrágicas e embólicas. O aneurisma foi submetido a tratamento endovascular com colocação de "stent" e embolização com molas. Os resultados clínico e radiológico foram excelentes e nenhuma complicação foi observada, sugerindo que o tratamento dos aneurismas de AB dissecantes com colocação de "stent" e embolização com molas possa ser seguro e eficaz.
