Miopatia induzida pelo vírus influenza A (H1N1): uma complicação extrapulmonar importante

Universidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina (EPM) Department of Neurology and NeurosurgeryUNIFESP, EPM, Department of Neurology and NeurosurgerySciEL

Sinal da língua brilhante: um marcador diagnóstico para esclerose lateral amiotrófica

Universidade Federal de São Paulo (UNIFESP) Departamento de Neurologia Unidade NeuromuscularUNIFESP, Depto. de Neurologia Unidade NeuromuscularSciEL

Clinical applications of immunoglobulin in neuromuscular diseases: focus on inflammatory myopathies

During recent years, an increasing number of neuromuscular diseases have been recognized either to be caused primarily by autoimmune mechanisms, or to have important autoimmune components. the involved pathophysiological mechanisms and clinical manifestations have been better recognized and many of these disorders are potentially treatable by immunosuppression or by immunomodulation with intravenous immunoglobulin (IVIg). IVIg has been tried in a variety of immune-mediated neurological diseases, being target of widespread use in central and peripheral nervous systems diseases. Objective: To give an overview of the main topics regarding the mechanism of action and different therapeutic uses of IVIg in neurological practice, mainly in neuromuscular diseases.Universidade Federal de São Paulo, Dept Neurol & Neurocirurgia, Div Doencas Neuromusculares, BR-04023900 São Paulo, BrazilUniversidade Federal de São Paulo, Dept Neurol & Neurocirurgia, Div Doencas Neuromusculares, BR-04023900 São Paulo, BrazilWeb of Scienc

C9orf72-related disorders: expanding the clinical and genetic spectrum of neurodegenerative diseases

Neurodegenerative diseases represent a heterogeneous group of neurological conditions primarily involving dementia, motor neuron disease and movement disorders. They are mostly related to different pathophysiological processes, notably in family forms in which the clinical and genetic heterogeneity are lush. in the last decade, much knowledge has been acumulated about the genetics of neurodegenerative diseases, making it essential in cases of motor neuron disease and frontotemporal dementia the repeat expansions of C9orf72 gene. This review analyzes the main clinical, radiological and genetic aspects of the phenotypes related to the hexanucleotide repeat expansions (GGGGCC) of C9orf72 gene. Future studies will aim to further characterize the neuropsychological, imaging and pathological aspects of the extra-motor features of motor neuron disease, and will help to provide a new classification system that is both clinically and biologically relevant.Universidade Federal de São Paulo, Dept Neurol & Neurocirurgia, Div Doencas Neuromusculares, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Neurol & Neurocirurgia, Div Doencas Neuromusculares, São Paulo, BrazilWeb of Scienc

Lumbago and alopecia in a patient with leukodystrophy: think on CARASIL

Univ Fed Sao Paulo, Div Doencas Neuromusculares, Dept Neurol & Neurocirugia, Sao Paulo, SP, BrazilUniv Fed Sao Paulo, Div Doencas Neuromusculares, Dept Neurol & Neurocirugia, Sao Paulo, SP, BrazilWeb of Scienc

Invaginação basilar em cefaleia associada ao esforço físico e torcicolo recorrente

Universidade Federal de São Paulo (UNIFESP)Universidade Federal de São Paulo (UNIFESP) Departamento de Neurologia e NeurocirurgiaUNIFESP, Depto. de Neurologia e NeurocirurgiaSciEL

Black coated tongue in integrative medicine: An alarm signal

Univ Fed Sao Paulo, Dept Neurol, Div Neuromuscular Dis Invest, Sao Paulo, SP, BrazilUniv Fed Sao Paulo Unifesp, Sao Paulo, SP, BrazilDivision of Neuromuscular Disease Investigation, Department of Neurology, Universidade Federal de São Paulo, São Paulo, SP, BrazilWeb of Scienc

Assistência nutricional nas doenças do neurônio motor/esclerose lateral amiotrófica

Patients with amyotrophic lateral sclerosis (ALS) often present changes in nutritional status. Based on weight loss and on difficulty in nutritional management, this study aims to review the different possibilities and to present guidelines concerning nutritional treatment to such patients. Diet characteristics, types of treatment and nutritional therapy indicating administration routes and discussing the details of the disease are described herein. Nutritional therapy has been a substantial therapeutic resource for ALS development.Pacientes com esclerose lateral amiotrófica (ELA) frequentemente apresentam alteração do estado nutricional. Baseado na perda ponderal de peso e na dificuldade no manejo nutricional, o objetivo desse estudo é rever as estratégias de tratamento nutricional para a manutenção do estado nutricional desses doentes. Nesse trabalho, descrevem-se as características da dieta, as formas de tratamento e terapia nutricional com indicação das vias de administração, bem como discuti-se as particularidades da doença. A terapia nutricional tem se mostrado um recurso terapêutico primordial atuante na evolução da ELA.UNIFESP-EPM Departamento de NeurologiaUNIFESP-EPMHospital Santa Marcelina Ambulatório Médico EspecialidadesUNIFESP, EPM, Depto. de NeurologiaSciEL

Paralisia de nervo fibular devido a síndrome compartimental após cirurgia plástica da face

A 25-year-old white man, right after bilateral rhytidoplasty, presented with agitation, necessiting use of haloperidol. Some hours after, he developed severe pain in his legs and a diagnosis of neuroleptic malignant syndrome (NMS) was considered. Even with treatment for NMS he still complained of pain. A diagnosis of lower limb compartment syndrome (CS) was done only 12 hours after the initial event, being submitted to fasciotomy in both legs, disclosing very pale muscles, due to previous ischemia. This syndrome was not explained only by facial surgery, his position and duration of the procedure. It can be explained by a sequence of events. He had a history of pain in his legs during physical exercises, usually seen in chronic compartment syndrome. He used to take anabolizant and venlafaxine, not previously related, and the agitation could be related to serotoninergic syndrome caused by interaction between venlafaxine and haloperidol. Rhabdomyolisis could lead to oedema and ischmemia in both anterior leg compartment. This report highlights the importance of early diagnosis of compartment syndrome, otherwise, even after fasciotomy, a permanent disability secondary to peripheral nerve compression could occur.Logo após ritidoplastia bilateral, um jovem de 25 anos apresentou agitação, necessitando uso de haloperidol. Algumas horas após, desenvolveu dor intensa em membros inferiores, e o diagnóstico de síndrome neuroléptica maligna foi considerado. Mesmo com o tratamento para tal, persistiu com dor. Após 12 horas do início do quadro, foi realizado o diagnóstico de síndrome compartimental de membros inferiores e o jovem foi submetido a fasciotomia bilateral. Uma seqüência de eventos desencadeou esta síndrome, já que sua ocorrência dificilmente seria justificada pela cirurgia facial e/ou posição do paciente durante o procedimento. O jovem apresentava previamente dor em membros inferiores aos exercícios, sugerindo a ocorrência de uma síndrome compartimental crônica. Ele fazia uso de anabolizantes e venlafaxina, não relatado no início do quadro, e a agitação poderia ser explicada por uma síndrome serotoninérgia desencadeada pela interação deste último medicamento e haloperidol. A rabdomiólise secundária a estes eventos causou edema e isquemia nos compartimentos anteriores de ambos os membros inferiores, levando a uma compressão secundária do nervo fibular. O caso em questão ilustra a importância do diagnóstico precoce da síndrome compartimental pois, caso contrário, mesmo com fasciotomia, uma complicação permanente devido à compressão de nervos periféricos pode se estabelecer.Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Department of Neurology and NeurosurgeryUniversidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Department of Vascular SurgeryUNIFESP, EPM, Department of Neurology and NeurosurgeryUNIFESP, EPM, Department of Vascular SurgerySciEL