As contribuições neurológicas de William Osler

William Osler was one of the most remarkable clinicians worldwide, and his teachings in medical practice remain until the present days. Although Osler had never considered himself a neurologist, he contributed with clinical and pathological descriptions of numerous neurological diseases. He performed more than 800 autopsies studies of the nervous system and published over 200 articles in the field of Neurology. In this article, we described the main neurological contributions from William Osler.William Osler foi um dos médicos mais notáveis da história, e seus ensinamentos sobre a prática médica permanecem até os dias atuais. Embora Osler nunca tenha se considerado um neurologista, contribuiu com descrições clínicas e patológicas de numerosas doenças neurológicas. Osler realizou mais de 800 autópsias do sistema nervoso e publicou mais de 200 artigos na área da Neurologia. Neste artigo, descre-vemos as principais contribuições neurológicas de William Osler.Universidade Federal de São Paulo (UNIFESP) Division of General Neurology and Ataxias Department of NeurologyUNIFESP, Division of General Neurology and Ataxias Department of NeurologySciEL

Atrofia da medula espinhal na ataxia espinocerebelar do tipo 1

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

As contribuicoes de Babinski para os sinais cerebelares: construindo as bases do exame neurologico

Several assumptions about the function of the cerebellum and semiotic signs have been described over the centuries. Among the long list of famous researchers who have provided a strong contribution and who have left their names on the highway of cerebellar research, Joseph Babinski appears as a prominent name. The description of various forms of cerebellar symptomatology was a major part of Babinski's work, and clinical terms that he introduced, namely hypermetry , diadochokinesia , and asynergy , remain part of contemporary clinical vocabulary. Babinski studied cerebellar signs in many patients and was able to conduct longitudinal studies that permitted him to understand the evolution of cerebellar dysfunction. Babinski contributions to cerebellar symptomatology continue to influence the most modern theories, including functional and neuropathological studies.Diversas teorias sobre a função do cerebelo e a semiologia dos sinais cerebelares têm sido descritas ao longo dos séculos. Dentre a longa lista de pesquisadores renomados que apresentaram importantes contribuições nesse assunto e que deixaram seus nomes na história, Joseph Babinski aparece com destaque. A descrição dos mais variados sintomas e sinais cerebelares foi peça importante do trabalho de Babinski e algumas nomenclaturas introduzidas por ele, tais como hipermetria , disdiadococinesia e assinergia , continuam a fazer parte do vocabulário contemporâneo. Babinski estudou os sinais cerebelares em muitos pacientes e foi capaz de realizar pesquisas que lhe permitiram compreender a disfunção cerebelar. As contribuições de Babinski para a caracterizar a sintomatologia cerebelar continuam a influenciar as teorias mais modernas, a despeito de estudos funcionais e neuropatológicos.Universidade Federal de São Paulo (UNIFESP) Setor de Neurologia Geral e Ataxias Departamento de Neurologia e NeurocirurgiaRush University Medical Center Department of Neurological SciencesUNIFESP, Setor de Neurologia Geral e Ataxias Depto. de Neurologia e NeurocirurgiaSciEL

Lentiform "Fork Sign" and Parkinsonism After Acute Myocardial Infarction and Cardiac Failure

Univ Fed Sao Paulo, Dept Neurol, Sao Paulo, SP, BrazilUniv Fed Sao Paulo, Dept Neurol, Sao Paulo, SP, BrazilWeb of Scienc

Facial grimacing como pista para o diagnóstico de gangliosidose GM1 tipo 3

Universidade Federal de São Paulo (UNIFESP) Departamento de Neurologia e Neurocirurgia Ambulatório de Neurologia GeralUNIFESP, Depto. de Neurologia e Neurocirurgia Ambulatório de Neurologia GeralSciEL

Brain SPECT imaging in Sydenham's chorea

The objective of the present study was to determine whether brain single-photon emission computed tomography (SPECT) imaging is capable of detecting perfusional abnormalities. Ten Sydenham's chorea (SC) patients, eight females and two males, 8 to 25 years of age (mean 13.4), with a clinical diagnosis of SC were submitted to brain SPECT imaging. We used HMPAO labeled with technetium-99m at a dose of 740 MBq. Six examinations revealed hyperperfusion of the basal ganglia, while the remaining four were normal. The six patients with abnormal results were females and their data were not correlated with severity of symptoms. Patients with abnormal brain SPECT had a more recent onset of symptoms (mean of 49 days) compared to those with normal SPECT (mean of 85 days) but this difference did not reach statistical significance. Brain SPECT can be a helpful method to determine abnormalities of the basal ganglia in SC patients but further studies on a larger number of patients are needed in order to detect the phase of the disease during which the examination is more sensitive.Universidade Federal de São Paulo (UNIFESP) Departamento de Neurologia Setor de Distúrbios do MovimentoUniversidade Federal de São Paulo (UNIFESP) Departamento de Diagnóstico por Imagem Setor de Medicina NuclearUNIFESP, Depto. de Neurologia Setor de Distúrbios do MovimentoUNIFESP, Depto. de Diagnóstico por Imagem Setor de Medicina NuclearSciEL

Sinal da cruz mimetizando atrofia de multiplos sistemas em um paciente com doenca de Machado-Joseph

Universidade Federal de São Paulo (UNIFESP) Department of Neurology Ataxia UnitUniversidade Federal de São Paulo (UNIFESP) Department of RadiologyDelboni Auriemo, Diagnosticos da AmericaUNIFESP, Department of Neurology Ataxia UnitUNIFESP, Department of RadiologySciEL

The relationship between Marcel Proust and Joseph Babinski: the encounter of two geniuses

Marcel Proust was one of the greatest French writers of all times. Since early in his life, Proust was interested in arts and particularly literature. He also demonstrated a great knowledge of medicine, particularly neurology. His father was a doctor, and contributed to neurology through studies on aphasia, stroke, hysteria, and neurasthenia. During his childhood, Proust had the first asthma attack, initially considered a manifestation of neurasthenia. Regarding his illness, Proust was in touch with several renowned neurologists, such as Brissaud, Babinski and Sollier, and other disciples of Charcot. Proust spent the last three years of his life mostly confined to his bedroom since his health had badly deteriorated. in one moment, Babinski was called, examined Proust and after leaving his bedroom, announced to his brother that Proust was practically dead. Few hours later, Proust developed vomica and died.Fundação de Amparo à Pesquisa do Estado de Minas Gerais (FAPEMIG)Universidade Federal de São Paulo, Dept Neurol, Setor Neurol Geral & Ataxias, São Paulo, BrazilUniv Fed Parana, Hosp Clin, Serv Neurol, Setor Disturbios Movimento, BR-80060000 Curitiba, Parana, BrazilUniv Fed Minas Gerais, Dept Interno Med, Serv Neurol, Setor Disturbios Movimento, Belo Horizonte, MG, BrazilUniversidade Federal de São Paulo, Dept Neurol, Setor Neurol Geral & Ataxias, São Paulo, BrazilWeb of Scienc

Síndrome de Sneddon: relato de caso e revisão sobre a relação com a síndrome do anticorpo antifosfolipídio

The Sneddon's syndrome is a rare disorder characterized by the occurrence of cerebrovascular disease associated with livedo reticularis. The antiphospholipid syndrome is the most frequent type of acquired thrombophilia, defined by the occurrence of thrombosis or pregnancy morbidity in the presence of persistently positive antiphospholipid antibodies. Approximately 80% of Sneddon's syndrome patients have an antiphospholipid antibody marker. These antibodies may play a pathogenetic role in some cases of Sneddon's syndrome, and many authors consider these two syndromes as the same entity. Although clinical features of antiphospholipid syndrome and Sneddon's syndrome may overlap, there is a distinction between clinical and laboratory evidence suggesting that these two entities are different diseases. A recent finding of coagulopathies, including elevated levels of coagulation factor VII, decreased levels of protein S, and activated protein C in Sneddon's syndrome patients suggested a possible biological link between the vasculopathy and a primary coagulopathy. Moreover, the clinical course seems to be progressive in Sneddon's syndrome patients and includes increase of disability and cognitive deterioration, more arterial involvement, and the antiphospholipid syndrome shows a more benign course. Both syndromes share clinical and laboratory features, and whether Sneddon's syndrome represents a spectrum of antiphospholipid syndrome remains unclear. Sneddon's syndrome patients have a worse prognosis and may represent a subgroup of patients who demands more rigorous follow-up. It is important to recognize the Sneddon's syndrome, particularly because stroke episodes may be prevented through appropriate treatment.A síndrome de Sneddon é um distúrbio raro caracterizado pela ocorrência de doença cerebrovascular associada a livedo reticular. A síndrome do anticorpo antifosfolipídio é o tipo mais frequente de trombofilia, definida pela ocorrência de trombose ou morbidade gestacional na presença de anticorpos antifosfolípides persistentemente positivos. Aproximadamente 80% dos pacientes com síndrome de Sneddon apresentam um marcador de anticorpo antifosfolipídio. Esses anticorpos podem exercer um papel fisiopatológico em alguns casos de síndrome de Sneddon, e muitos autores consideram essa síndrome e a síndrome do anticorpo antifosfolipídio a mesma entidade. Apesar de os quadros clínicos das suas síndromes poderem se sobrepor, há evidência clínica e laboratorial distintiva, sugerindo que as duas entidades são doenças diferentes. Um achado recente de coagulopatia, incluindo níveis elevados do fator VII de coagulação, diminuição dos níveis da proteína S, e proteína C ativada em pacientes com síndrome de Sneddon, sugeriu uma possível ligação biológica entre a vasculopatia e coagulopatia primária. Além disso, o curso clínico pareceu ser progressivo em pacientes com síndrome de Sneddon, visto que há aumento de incapacidade e deterioração cognitiva, além de maior envolvimento arterial, enquanto a síndrome do anticorpo antifosfolipídio apresenta um curso mais benigno. Ambas as síndromes compartilham características clínicas e laboratoriais; até qual ponto a síndrome de Sneddon representa um espectro da síndrome do anticorpo antifosfolipídio permanece desconhecido. Os pacientes com a primeira síndrome apresentam pior prognóstico e podem representar um subgrupo de pacientes que requer um seguimento mais rigoroso. É importante reconhecer a síndrome de Sneddon já que os episódios de acidente vascular cerebral podem ser prevenidos com a terapia apropriada.Universidade Federal de São Paulo (UNIFESP) Departament of Neurology and NeurosurgeryInstituto do Cérebro Hospital Israelita Albert EinsteinUNIFESP, Departament of Neurology and NeurosurgerySciEL