Jean-Martin Charcot: the polymath / Jean-Martin Charcot: o polímata

Abstract Jean-Martin Charcot, widely regarded as a leading founder of modern neurology, made substantial contributions to the understanding and characterization of numerous medical conditions. His initial focus was on internal medicine, later expanding to include neuropathology, general neurology, and eventually emerging fields such as neuropsychology and neuropsychiatry. Furthermore, Charcot's intellectual pursuits extended beyond medicine, encompassing research in art history, medical iconography, sociology, religious studies, and the arts, solidifying his status as a polymath.

Resumo Jean-Martin Charcot, amplamente considerado como um proeminente fundador da neurologia moderna, fez contribuições substanciais para a compreensão e a caracterização de várias condições médicas. Seu foco inicial era a medicina interna, expandindo-se posteriormente para incluir a neuropatologia, a neurologia geral e, por fim, campos emergentes como a neuropsicologia e a neuropsiquiatria. Além disso, as buscas intelectuais de Charcot foram além da medicina, abrangendo pesquisas em história da arte, iconografia médica, sociologia, estudos religiosos e artes, solidificando seu status de polímata.

Cannabinoids in Neurology - Position paper from Scientific Departments from Brazilian Academy of Neurology.

Cannabinoids comprehend endocannabinoids, phytocannabinoids, and synthetic cannabinoids, with actions both in the central and peripherical nervous systems. A considerable amount of publications have been made in recent years, although cannabis has been known for over a thousand years. Scientific Departments from the Brazilian Academy of Neurology described evidence for medical use in their areas. Literature is constantly changing, and possible new evidence can emerge in the next days or months. Prescription of these substances must be discussed with patients and their families, with knowledge about adverse events and their efficacy.

Cannabinoids in Neurology - Position paper from Scientific Departments from Brazilian Academy of Neurology / Canabinoides em Neurologia - Artigo de posicionamento dos Departamentos Científicos da Academia Brasileira de Neurologia

ABSTRACT Cannabinoids comprehend endocannabinoids, phytocannabinoids, and synthetic cannabinoids, with actions both in the central and peripherical nervous systems. A considerable amount of publications have been made in recent years, although cannabis has been known for over a thousand years. Scientific Departments from the Brazilian Academy of Neurology described evidence for medical use in their areas. Literature is constantly changing, and possible new evidence can emerge in the next days or months. Prescription of these substances must be discussed with patients and their families, with knowledge about adverse events and their efficacy.

RESUMO Os canabinoides compreendem os endocanabinoides, fitocanabinoides e os canabinoides sintéticos e desempenham ações no sistema nervoso central e periférico. Uma quantidade enorme de publicações tem sido lançada nos últimos anos, embora a cannabis seja conhecida por milênios. Os Departamentos Científicos da Academia Brasileira de Neurologia descreveram as evidências do uso médico em suas áreas. A literatura está em constantes mudanças e possíveis novas evidências podem surgir nos próximos dias ou meses. A prescrição dessas substâncias deve ser discutida com os pacientes e suas famílias, com conhecimento sobre eventos adversos e sua eficácia.

Dementia in elderly inpatients admitted to medical wards in Brazil: diagnosis and comorbidity with other clinical diseases.

BACKGROUND: Specific comorbidities affect older patients with dementia admitted to general hospitals and may complicate the recognition of dementia. The aim of the present study was to estimate the prevalence of dementia among elderly inpatients admitted to hospital medical wards and to identify its distribution across clinical and sociodemographic conditions. METHODS: From June 2011 to May 2012, a sample of elderly inpatients (≥60 years old) were screened for dementia with the Mini-Mental State Examination and the Bayer Activities of Daily Living Scale to identify cognitive and functional impairment (CFI). Subjects with CFI underwent a diagnostic procedure for dementia using the Cambridge Mental Disorders of the Elderly Examination and the Diagnostic and Statistical Manual of Mental Disorders, 4th edition. The elderly inpatients also completed a standard questionnaire to investigate sociodemographic and clinical variables and a screening procedure for depression and delirium. The data obtained were submitted to univariate and multivariate analyses. RESULTS: The sample of 224 subjects had a mean age of 71.5 years and was mostly men (62.2%), poorly educated (≤4 years of schooling: 74.6%), and married (53.4%). CFI was observed in 84 subjects (prevalence: 37.4%; 95% confidence interval: 31.1-43.7), and dementia was observed in 31 subjects (prevalence: 17.2%; 95% confidence interval: 12.3-22.1). Dementia was related to older age and the presence of delirium, stroke, and pneumonia. CONCLUSIONS: The prevalence of CFI and dementia was high among the elderly inpatients examined. The identification of medical and sociodemographic conditions associated with a dementia diagnosis in a general hospital may be useful in the development of preventative actions.

Treatment of Alzheimers disease in Brazil: II. Behavioral and psychological symptoms of dementia / Tratamento da doença de Alzheimer no Brasil: II. Dos sintomas comportamentais e psicológicos da demência

This article reports the recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology for the treatment of Alzheimers disease (AD) in Brazil, with special focus on behavioral and psychological symptoms of dementia (BPSD). It constitutes a revision and broadening of the 2005 guidelines based on a consensus involving researchers (physicians and non-physicians) in the . The authors carried out a search of articles published since 2005 on the MEDLINE, LILACS and Cochrane Library databases. The search criteria were pharmacological and non-pharmacological treatment of the behavioral and psychological symptoms of AD. Studies retrieved were categorized into four classes, and evidence into four levels, based on the 2008 recommendations of the American Academy of Neurology. The recommendations on therapy are pertinent to the dementia phase of AD. Recommendations are proposed for the treatment of BPSD encompassing both pharmacological (including acetyl-cholinesterase inhibitors, memantine, neuroleptics, anti-depressives, benzodiazepines, anti-convulsants plus other drugs and substances) and non-pharmacological (including education-based interventions, physiotherapy, occupational therapy, music therapy, therapy using light, massage and art therapy) approaches. Recommendations for the treatment of cognitive disorders of AD symptoms are included in a separate article of this edition.

Esse texto apresenta as recomendações da Academia Brasileira de Neurologia, por intermédio do seu Departamento Científico de Neurologia Cognitiva e do Envelhecimento, para o tratamento da doença de Alzheimer (DA) no Brasil, enfocando os sintomas comportamentais e psicológicos da demência (SCPD). Trata-se de uma revisão ampliada das diretrizes publicadas em 2005, resultada de um consenso envolvendo pesquisadores da área, médicos e não médicos. Os autores realizaram uma busca de artigos publicados a partir de 2005 nas bases MEDLINE, LILACS e Cochrane Library. A busca foi direcionada para tratamento farmacológico e não farmacológico dos sintomas comportamentais e psicológicos da DA. Os estudos foram categorizados em quatro classes e as evidências em quatro níveis, com base nas recomendações da Academia Americana de Neurologia publicadas em 2008. As recomendações terapêuticas referem-se à fase demencial da DA. Apresentam-se recomendações para o tratamento dos SCPD, tanto farmacológico (incluindo inibidores da acetilcolinesterase, memantina, neurolépticos, antidepressivos, benzodiazepínicos, anticonvulsivantes e outros fármacos e substâncias), como não farmacológico (incluindo intervenções educacionais, fisioterapia, terapia ocupacional, musicoterapia, terapia com luz, massagem e arterapia). As recomendações para o tratamento dos transtornos cognitivos da DA são apresentadas em outro artigo desse fascículo.

Treatment of Alzheimers disease in Brazil: I. Cognitive disorders / Tratamento da doença de Alzheimer no Brasil: I. dos transtornos cognitivos

This article reports the recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology for the treatment of Alzheimers disease (AD) in Brazil, with special focus on cognitive disorders. It constitutes a revision and broadening of the 2005 guidelines based on a consensus involving researchers (physicians and non-physicians) in the . The authors carried out a search of articles published since 2005 on the MEDLINE, LILACS and Cochrane Library databases. The search criteria were pharmacological and non-pharmacological treatment of cognitive disorders in AD. Studies retrieved were categorized into four classes, and evidence into four levels, based on the 2008 recommendations of the American Academy of Neurology. The recommendations on therapy are pertinent to the dementia phase of AD. Recommendations are proposed for the treatment of cognitive disorders encompassing both pharmacological (including acetyl-cholinesterase inhibitors, memantine and other drugs and substances) and non-pharmacological (including cognitive rehabilitation, physical activity, occupational therapy, and music therapy) approaches. Recommendations for the treatment of behavioral and psychological symptoms of dementia due to Alzheimers disease are included in a separate article of this edition.

Esse texto apresenta as recomendações da Academia Brasileira de Neurologia, por intermédio do seu Departamento Científico de Neurologia Cognitiva e do Envelhecimento, para o tratamento da doença de Alzheimer (DA) no Brasil, enfocando os transtornos cognitivos. Trata-se de uma revisão ampliada das diretrizes publicadas em 2005, resultada de um consenso envolvendo pesquisadores da área, médicos e não médicos. Os autores realizaram uma busca de artigos publicados a partir de 2005 nas bases MEDLINE, LILACS e Cochrane Library. A busca foi direcionada para tratamento farmacológico e não farmacológico dos transtornos cognitivos da DA. Os estudos foram categorizados em quatro classes e as evidências em quatro níveis, com base nas recomendações da Academia Americana de Neurologia publicadas em 2008. As recomendações terapêuticas referem-se à fase demencial da DA. Apresentam-se recomendações para o tratamento dos transtornos cognitivos, tanto farmacológico (incluindo inibidores da acetilcolinesterase, memantina e outros fármacos e substâncias), como não farmacológico (incluindo reabilitação cognitiva, atividade física, terapia ocupacional e musicoterapia). As recomendações para o tratamento dos sintomas comportamentais e psicológicos da demência da DA são apresentadas em outro artigo desse fascículo.

Tratamento da doença de Alzheimer / Alzheimer's disease Treatment

Esse texto apresenta as recomendações da Academia Brasileira de Neurologia (ABN), por intermédio do seu Departamento Científico de Neurologia Cognitiva e do Envelhecimento (DCNCE), para o tratamento da doença de Alzheimer no Brasil. Trata-se de uma revisão ampliada das recomendações publicadas em 2005. Os autores realizaram uma busca de artigos publicados a partir de 2005 nas bases MEDLINE (PubMed), LILACS e Cochrane Library. Os estudos foram categorizados em quatro classes e as evidências em quatro níveis, com base nas recomendações da Academia Americana de Neurologia publicadas em 2008. As recomendações terapêuticas referem-se à fase demencial da doença de Alzheimer. Apresentam-se recomendações para: farmacoterapia dos transtornos cognitivos, incluindo inibidores da acetilcolinesterase (IAChE), memantina e outros fármacos e substâncias; farmacoterapia dos sintomas comportamentais e psicológicos (SCPD), incluindo antipsicóticos, benzodiazepínicos, antidepressivos, anticonvulsivantes, IAChE, memantina e outros fármacos e substâncias; e tratamento não farmacológico dos transtornos cognitivos e dos SCPD.

Treatment of Alzheimer's disease in Brazil: I. Cognitive disorders.

This article reports the recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology for the treatment of Alzheimer's disease (AD) in Brazil, with special focus on cognitive disorders. It constitutes a revision and broadening of the 2005 guidelines based on a consensus involving researchers (physicians and non-physicians) in the field. The authors carried out a search of articles published since 2005 on the MEDLINE, LILACS and Cochrane Library databases. The search criteria were pharmacological and non-pharmacological treatment of cognitive disorders in AD. Studies retrieved were categorized into four classes, and evidence into four levels, based on the 2008 recommendations of the American Academy of Neurology. The recommendations on therapy are pertinent to the dementia phase of AD. Recommendations are proposed for the treatment of cognitive disorders encompassing both pharmacological (including acetyl-cholinesterase inhibitors, memantine and other drugs and substances) and non-pharmacological (including cognitive rehabilitation, physical activity, occupational therapy, and music therapy) approaches. Recommendations for the treatment of behavioral and psychological symptoms of dementia due to Alzheimer's disease are included in a separate article of this edition.

Esse texto apresenta as recomendações da Academia Brasileira de Neurologia, por intermédio do seu Departamento Científico de Neurologia Cognitiva e do Envelhecimento, para o tratamento da doença de Alzheimer (DA) no Brasil, enfocando os transtornos cognitivos. Trata-se de uma revisão ampliada das diretrizes publicadas em 2005, resultada de um consenso envolvendo pesquisadores da área, médicos e não médicos. Os autores realizaram uma busca de artigos publicados a partir de 2005 nas bases MEDLINE, LILACS e Cochrane Library. A busca foi direcionada para tratamento farmacológico e não farmacológico dos transtornos cognitivos da DA. Os estudos foram categorizados em quatro classes e as evidências em quatro níveis, com base nas recomendações da Academia Americana de Neurologia publicadas em 2008. As recomendações terapêuticas referem-se à fase demencial da DA. Apresentam-se recomendações para o tratamento dos transtornos cognitivos, tanto farmacológico (incluindo inibidores da acetilcolinesterase, memantina e outros fármacos e substâncias), como não farmacológico (incluindo reabilitação cognitiva, atividade física, terapia ocupacional e musicoterapia). As recomendações para o tratamento dos sintomas comportamentais e psicológicos da demência da DA são apresentadas em outro artigo desse fascículo.

Treatment of Alzheimer's disease in Brazil: II. Behavioral and psychological symptoms of dementia.

This article reports the recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology for the treatment of Alzheimer's disease (AD) in Brazil, with special focus on behavioral and psychological symptoms of dementia (BPSD). It constitutes a revision and broadening of the 2005 guidelines based on a consensus involving researchers (physicians and non-physicians) in the field. The authors carried out a search of articles published since 2005 on the MEDLINE, LILACS and Cochrane Library databases. The search criteria were pharmacological and non-pharmacological treatment of the behavioral and psychological symptoms of AD. Studies retrieved were categorized into four classes, and evidence into four levels, based on the 2008 recommendations of the American Academy of Neurology. The recommendations on therapy are pertinent to the dementia phase of AD. Recommendations are proposed for the treatment of BPSD encompassing both pharmacological (including acetyl-cholinesterase inhibitors, memantine, neuroleptics, anti-depressives, benzodiazepines, anti-convulsants plus other drugs and substances) and non-pharmacological (including education-based interventions, physiotherapy, occupational therapy, music therapy, therapy using light, massage and art therapy) approaches. Recommendations for the treatment of cognitive disorders of AD symptoms are included in a separate article of this edition.

Esse texto apresenta as recomendações da Academia Brasileira de Neurologia, por intermédio do seu Departamento Científico de Neurologia Cognitiva e do Envelhecimento, para o tratamento da doença de Alzheimer (DA) no Brasil, enfocando os sintomas comportamentais e psicológicos da demência (SCPD). Trata-se de uma revisão ampliada das diretrizes publicadas em 2005, resultada de um consenso envolvendo pesquisadores da área, médicos e não médicos. Os autores realizaram uma busca de artigos publicados a partir de 2005 nas bases MEDLINE, LILACS e Cochrane Library. A busca foi direcionada para tratamento farmacológico e não farmacológico dos sintomas comportamentais e psicológicos da DA. Os estudos foram categorizados em quatro classes e as evidências em quatro níveis, com base nas recomendações da Academia Americana de Neurologia publicadas em 2008. As recomendações terapêuticas referem-se à fase demencial da DA. Apresentam-se recomendações para o tratamento dos SCPD, tanto farmacológico (incluindo inibidores da acetilcolinesterase, memantina, neurolépticos, antidepressivos, benzodiazepínicos, anticonvulsivantes e outros fármacos e substâncias), como não farmacológico (incluindo intervenções educacionais, fisioterapia, terapia ocupacional, musicoterapia, terapia com luz, massagem e arterapia). As recomendações para o tratamento dos transtornos cognitivos da DA são apresentadas em outro artigo desse fascículo.

Morte encefálica: cinquenta anos além do coma profundo / Brain death: deep coma fifty years on

Mollaret e Goulon, há cinquenta anos, descreveram o coma dépassé, alteração irreversível da consciência que passou a ser chamada de morte encefálica e igualada à morte cardiorespiratória. Foram revisados a evolução dos critérios diagnósticos sobre morte encefálica, seus fundamentos éticos e científicos, e, principalmente, sua regulação ética e legal no Brasil.

Fifty years ago, Mollaret and Goulon described the dépassé coma, an irreversible alteration in consciousness that later came to be known as brain death and equated with heart and respiratory failure. This article presents an overview of the evolution of diagnostic criteria for brain death, its ethical and scientific basis, and, above all, its ethical and legal regulation in Brazil.

[The chorea of Zezé]. / A coréia do Zezé.

The authors analyse the correspondence between Eça de Queiroz, a well-known Portuguese writer, and his wife, in 1900, describing the neurological disease of their second son, José Maria d'Eça de Queiroz, know as Zezé. In those letters, the writer mentions chorea as his son diagnosis, explaining in detail its clinical picture. In addition, the writer reveals the medical thoughts of the time about the aetiology, treatment and natural history of Sydenham's Chorea.

Flutuação da atençao na doença de Parkinson / Fluctuation of attention in Parkinson's disease

Para avaliar a influencia em curto prazo da reposição dopaminérgica na flutuação da atenção em pacientes com DP, a latência média e o desvio-padrão da latência do tempo de reação simples e com escolha foram estabelecidos em 15 pacientes com DP antes e 90 minutos depois da administração da dose habitual matutina de levodopa e em 15 controles normais. Verificou-se , além de efeitos motores, maior sincronia nas latências de testes de tempo de reação complexos, mas não nos simples, sugerindo efeitos da dopamina em mecanismos atencionais e/ou de controle executivo que envolvam flexibilidade na identificação do estímulo e/ou na escolha da resposta / To evaluate short time effects of dopaminergic medication on fluctuation of attention in PD patients, simple and choice reaction latency and latency standard deviation was determined in 15 PD patients before and 90 min. after usual early morning levodopa dose and in 15 normal controls. Besides motor improvement, improved synchrony on complex but not on simple reaction time tests was observed, suggesting dopamine attention and executive control modulation, probably thru stimulus identification and action selection flexibility...

Congenital contractural arachnodactyly with neurogenic muscular atrophy: case report

We report the case of a 3-1/2-year-old girl with hypotonia, multiple joint contractures, hip luxation, arachnodactyly, adducted thumbs, dolichostenomelia, and abnormal external ears suggesting the diagnosis of congenital contractural arachnodactyly (CCA). The serum muscle enzimes were normal and the needle electromyography showed active and chronic denervation. The muscle biopsy demonstrated active and chronic denervation compatible with spinal muscular atrophy. Analysis of exons 7 and 8 of survival motor neuron gene through polymerase chain reaction did not show deletions. Neurogenic muscular atrophy is a new abnormality associated with CCA, suggesting that CCA is clinically heterogeneous

Doenças de Dupuytren e de Ledderhose associadas ao uso crônico de anticonvulsivantes: relato de caso / Dupuytren's and Ledderhose's diseases associated with chronic use of anticonvulsants: case report

Relatamos o caso de um paciente que após uso crônico de anticonvulsivantes, sem epilepsia definida, desenvolveu contraturas das aponeuroses palmar (doença de Dupuytren) e plantar (doença de Ledderhose). Discutimos as principais dessas complicações, os fatores predisponentes e sua estreita relação com o uso de anticonvulsivantes, particularmente de fenobarbital.

Rigid spine syndrome: case report

We describe a patient who had difficulty in walking since toddling stage and presented proximal upper and lower member weakness which have evolved to a progressive limitation of neck and trunk flexure, compatible with rigid spine syndrome. The serum muscle enzymes were somewhat elevated and the electromyography showed a myopatic change. The muscle biopsy demonstrated an active and chronic myopathy. The DNA analysis through PCR did not display any abnormality for dystrophin gene. The dystrophin by immnofluorescence was present in all fibers, but some interruptions were found in the plasma membrane giving it the appearance of a rosary. The test for merosin was normal.

Frequency of the different mutations causing spinocerebellar ataxia (SCA1, SCA2, MJD/SCA3 and DRPLA) in a large group of Brazilian patients

Spinocerebellar ataxia type 1 (SCA1), spinocerebellar ataxia type 2 (SCA2) and Machado-Joseph disease or spinocerebellar ataxia type 3 (MJD/SCA3) are three distinctive forms of autosomal dominant spinocerebellar ataxia (SCA) caused by expansions of an unstable CAG repeat localized in the coding region of the causative genes. Another related disease, dentatorubropallidoluysian atrophy (DRPLA) is also caused by an unstable triplet repeat and can present as SCA in late onset patients. We investigated the frequency of the SCA1, SCA2, MJD/SCA3 and DRPLA mutations in 328 Brazilian patients with SCA, belonging to 90 unrelated families with various patterns of inheritance and originating in different geographic regions of Brazil. We found mutations in 35 families (39 percent), 32 of them with a clear autosomal dominant inheritance. The frequency of the SCA1 mutation was 3 percent of all patients; and 6 percent in the dominantly inherited SCAs. We identified the SCA2 mutation in 6 percent of all families and in 9 percent of the families with autosomal dominant inheritance. The MJD/SCA3 mutation was detected in 30 percent of all patients; and in the 44 percent of the dominantly inherited cases. We found no DRPLA mutation. In addition, we observed variability in the frequency of the different mutations according to geographic origin of the patients, which is probably related to the distinct colonization of different parts of Brazil. These results suggest that SCA may be occasionally caused by the SCA1 and SCA2 mutations in the Brazilian population, and that the MJD/SCA3 mutation is the most common cause of dominantly inherited SCA in Brazil.

Neuropatia sensitiva aguda idiopática: relato de caso / Acute idiopathic sensory neuropathies: a case report

As neuronopatias sensitivas caracterizam-se por parestesis, ataxia sensitiva e arreflexia sem comprometimento motor, devidas a lesäo no gânglio sensitivo dorsal, com degeneraçäo axonal e do cordäo posterior da medula espinhal. Descrevemos o caso deuma paciente de 21 anos de idade, cuja doença se iniciou de modo agudo com parestesias distais, astasia, abasia e hiporreflexia. Força muscular normal. A conduçäo nervosa sensitiva estava ausente e a motora, discretamente reduzida. LCR com 2 leucócitos/mm3 e 1,06 g/dL de proteínas. A biópsia de músculo evidenciou atrofia de fibras tipo 2 e a biópsia do nervo sural, desmielinizaçäo axonal. Tratada com prednisona, permaneceu discreto déficit proprioceptivo nos pés após um ano e meio de evoluçäo. Säo discutidos aspectos clínicos do envolvimento dos gânglios sensitivos dorsais, possíveis etiologia tóxicas, a relaçäo com neoplasias e com a síndrome de Sjogren