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Personal and professional rivalries involving prominent neurologists mark the history of nineteenth-century French neurology. One of the great examples is the feud between Pierre Marie and Jules Dejerine. The dispute between the two, nevertheless, did not prevent Pierre Marie's son, André Marie, and Gustave Roussy - one of Dejerine's favorite pupils, from collaborating on significant research that led to the doctoral dissertation by Andre Marie regarding sensory disturbances associated with painful hemiagnosia found in thalamic lesions.

As rivalidades pessoais e profissionais entre neurologistas proeminentes marcaram a história da neurologia francesa do século XIX. Um dos grandes exemplos é a rivalidade entre Pierre Marie e Jules Dejerine. A disputa entre os dois, no entanto, não impediu que o filho de Pierre Marie, André Marie, e Gustave Roussy, um dos pupilos preferidos de Dejerine, colaborassem numa investigação significativa que resultou na tese de doutorado de André Marie sobre os distúrbios sensoriais associados à hemiagnosia dolorosa encontrada nas lesões talâmicas.

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There is a clear need for experts with the requisite knowledge and experience to offer medicolegal opinions pertaining to various neuropsychiatric conditions. There is also an important distinction between clinical and medicolegal roles, and the need for training and expertise applicable to forensic assessment. But there remain few available experts with credentials spanning neuropsychiatry and forensic assessment. This creates a dilemma whereby parties involved in litigation featuring neuropsychiatric illness or injury are frequently forced to choose between experts with either knowledge and skills applicable to neuropsychiatric conditions or experts with skills and experience applicable to forensic assessment. Either choice introduces risk. Whether flawed medicolegal opinions are a consequence of deficient medical knowledge or an inadequate forensic evaluation process, the result remains the same, with triers of fact potentially being exposed to problematic testimony. There is, however, a more fundamental problem that implicates patient care more broadly: spurious dichotomies created by the historical segregation of psychiatry and neurology. Optimizing clinical care for patients with neuropsychiatric conditions, improving medical education in support of such care, and enabling forensic neuropsychiatric assessment must then start with more proactive efforts to reintegrate psychiatry and neurology.

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Spasmodic torticollis was an early designation used for cervical dystonia. The origin of this name is attributed to French physician and writer François Rabelais in the mid-sixteenth century. This early description of torticollis in the book Pantagruel was an inspiration for the understanding of cervical dystonia. The art expressed in Rabelais' literature ‒ which was immortalized by the drawings of Gustave Doré ‒ influenced poetry, art, and photography, and led to the adoption of the term torticollis in the neurological sciences.

Uma designação inicial usada para distonia cervical era torcicolo espasmódico. A origem desse termo é atribuída ao médico e escritor francês François Rabelais em meados do século XVI. Essa descrição inicial do torcicolo no livro Pantagruel foi uma inspiração para a compreensão da distonia cervical. A arte exibida na literatura de Rabelais ‒ imortalizada pelos desenhos de Gustave Doré ‒ influenciou a poesia, a arte e a fotografia, e levou à adoção do termo torcicolo nas ciências neurológicas.

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BACKGROUND: Charles Foix (1882-1927) may be mostly remembered today due to his contributions to vascular neurology and the syndromes that bear his name, such as the Foix-Alajouanine syndrome. However, he also developed a literary career and composed poetry and a vast collection of plays, often dealing with biblical themes or figures from Greek mythology. SUMMARY: His poetry was often inspired by his own experiences during the First World War, in which he was assigned to serve as a medical officer in Greece, becoming enamored with his surroundings and the classical lore. KEY MESSAGES: The authors explore Foix's poetry and drama and their relationship to his overall work as a neurologist, including his wartime experiences.

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One of the most important figures in the history of neurohistology, Giuseppe Levi (1872-1965) contributed in numerous ways to neuroscience, particularly in the fields of neuronal plasticity and the understanding of sensory ganglia. His daughter Natalia Ginzburg, née Levi (1916-1991), on the other hand, achieved fame as one of the most celebrated Italian writers of the twentieth century. Lessico Famigliare (Family Lexicon), from 1963, is a semibiographical account of her life in which she describes the life and character of her father in detail, providing depth and complexity to a seminal figures in the development of neuroscience. A thorough reading of the book enables modern neurologists to fully appreciate Levi's life and contributions, by means of humanizing him and giving context to his life and works. The present article provides a summary of Levi's and Natalia's lives and times as well as an analysis of the book and of the intimate, vivid descriptions of the neurohistologist's life.

Uma das figuras mais importantes da história da neuro-histologia, Giuseppe Levi (1872­1965) contribuiu de diversas maneiras para a neurociência, particularmente no campo da plasticidade neuronal e na compreensão dos gânglios sensitivos. Sua filha Natalia Ginzburg, nascida Levi (1916­1991), pelo contrário, adquiriu fama como uma das escritoras italianas mais célebres do século XX. Lessico Famigliare (Léxico familiar), de 1963, é um relato semibiográfico de sua vida, na qual ela descreve a vida e o comportamento de seu pai em detalhes, e confere profundidade e complexidade a uma figura seminal no desenvolvimento da neurociência. Uma leitura aprofundada do livro permite que neurologistas modernos apreciem a vida e as contribuições de Levi de forma mais completa, o humanizando e dando contexto a sua vida e suas obras. O autor resume as vidas e épocas de Levi e Natalia, bem como avalia o livro e as descrições íntimas, vívidas, da vida do neurohistologista.

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Narcolepsy is a primary disorder of the central nervous system resulting from genetic, environmental, and immunological interactions defined as excessive daytime sleepiness plus cataplexy, hallucinations, sleep paralysis, and sleep fragmentation. The pathophysiology is not entirely known, but the interaction among genetic predisposition, environmental exposition, and immune component with consequent hypocretin-1 deficiency is the model to explain narcolepsy type I. The mechanism of narcolepsy type II is less understood. There is a delay of over ten years for the diagnosis of narcolepsy around the world. Patients with narcolepsy have many comorbidities with a negative impact on quality of life. The treatment of narcolepsy must contain an educational approach for the family, coworkers, and patients. Scheduled naps and sleep hygiene are essential to minimize the dose of medications. Much progress has been seen in the pharmacological treatment of narcolepsy with new stimulants, different presentations of oxybate, and recent studies with orexin agonists. Narcolepsy is a rare disease that needs to be more understood and highlighted to avoid delayed diagnosis and severe disabilities in patients.

A narcolepsia é um distúrbio primário do sistema nervoso central resultante das interações genéticas, ambientais e imunológicas definidas como sonolência diurna excessiva mais cataplexia, alucinações, paralisia do sono e fragmentação do sono. A fisiopatologia não é completamente conhecida, mas a interação entre predisposição genética, exposição ambiental e componente imunológico com consequente deficiência de hipocretina-1 é o modelo para explicar a narcolepsia tipo I. O mecanismo da narcolepsia tipo II é menos compreendido. Há um atraso de mais de dez anos para o diagnóstico da narcolepsia em todo o mundo. Pacientes com narcolepsia apresentam muitas comorbidades com impacto negativo na qualidade de vida. O tratamento da narcolepsia deve conter uma abordagem educativa para a família, colegas de trabalho e pacientes. Cochilos programados e higiene do sono são importantes para minimizar a dose dos medicamentos. Muito progresso foi observado no tratamento farmacológico da narcolepsia com novos estimulantes, diferentes apresentações de oxibato e estudos recentes com agonistas de orexina. A narcolepsia é uma doença rara que precisa ser mais compreendida e destacada para evitar atrasos no diagnóstico e incapacidades graves nos pacientes.

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In pediatric patients, an acute altered mental status refers to a sudden and significant change in a child's brain function and level of consciousness. It may manifest as confusion, disorientation, agitation, lethargy or even a loss of consciousness. This condition is a medical emergency, and requires immediate evaluation and attention. There are several causes of acute altered mental status in children, including infections of the central nervous system such as meningitis or encephalitis, traumatic brain injury, metabolic disorders, seizures and poisoning, among others. The aim of this study was to analyse, prepare and classify the current literature in order to determine the best recommendations for the treatment of cases of acute altered mental status with various causes in pediatric patients. The study was based on opinions from experts in the field in order to classify the recommendations, and was submitted to the scientific committee of the Iberoamerican Academy of Pediatric Neurology for review. Our guide is an aid for the treatment of this non-specific symptom based on a basic and advanced approach, which can be applied by any pediatric neurologist.

TITLE: Guía de tratamiento de la alteración aguda del estado mental. Academia Iberoamericana de Neurología Pediátrica.La alteración aguda del estado mental en pediatría se refiere a un cambio repentino y significativo en la función cerebral y el nivel de conciencia de un niño. Puede manifestarse como confusión, desorientación, agitación, letargo o incluso pérdida de la conciencia. Esta condición es una emergencia médica, y requiere una evaluación y una atención inmediatas. Existen diversas causas de alteración aguda del estado mental en niños, algunas de las cuales incluyen infecciones del sistema nervioso central, como la meningitis o la encefalitis, los traumatismos craneoencefálicos, los trastornos metabólicos, las convulsiones o las intoxicaciones, entre otras. Este estudio tuvo como objetivo analizar, preparar y calificar la bibliografía actual para determinar las mejores recomendaciones sobre el tratamiento ante casos de alteración aguda del estado mental en pediatría de diferentes causas. El estudio se basó en la calificación de expertos en el campo para poder determinar la calificación de las recomendaciones, además de ser sometido a la revisión por parte del comité científico de la Academia Iberoamericana de Neurología Pediátrica. Nuestra guía representa una ayuda para el tratamiento de este síntoma inespecífico desde un enfoque básico y avanzado, aplicable por cualquier neurólogo pediatra.

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BACKGROUND AND OBJECTIVES: The field of vascular neurology has undergone significant advances over the last decade, and care has become more complex. However, vascular neurology training programs remain underdeveloped in many countries, despite stroke impact on health care. There are efforts towards building a nationally regulated curricula in some countries. Still, comprehensive planning and implementation of these programs may be needed on a global scale, especially in countries where stroke treatment is not fully implemented. We aim to comprehensively analyze vascular neurology trainees' profiles in Brazil to describe training program contents from trainees and program directors' perspectives. METHODS: We performed an observational, cross-sectional, web-based survey study to describe trainee and program-specific characteristics at vascular neurology fellowship training programs in Brazil. The study was conducted from June to September 2023 using a secure web-based survey sent to active fellows and program directors from all known vascular neurology fellowship programs in the country. All respondents were required to provide informed consent. RESULTS: We obtained a 100 % response rate of a total of 12 programs distributed in 7 federal states. Notably, 57 % of the 28 surveyed fellows were women, 60 % were aged 25-30, and 70 % self-identified as White. All fellows had prior neurology training, and 60 % engaged in the program just following residency. Exposure to various training experiences was favorable, except for simulation-based learning and telestroke training. Program directors perceived exposure to be sufficient for most components but similarly found deficiencies in telestroke and simulation-based learning. Scientific productivity was low, with about two-thirds of fellows having no publications or abstracts. Most fellows (92.6 %) reported performing non-fellowship medical activities to supplement their incomes. DISCUSSION: In conclusion, the number of vascular neurology training programs and trainees in Brazil is currently insufficient and exhibits an uneven geographic distribution. Despite this, the clinical training provided is extensive, and there is generally some funding available for fellows. These insights highlight the need for strategic improvements in Brazil's stroke education and could inform similar developments in other nations.

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Spinal muscular atrophy linked to chromosome 5 (SMA-5q) is an autosomal recessive genetic disease caused by mutations in the SMN1. SMA-5q is characterized by progressive degeneration of the spinal cord and bulbar motor neurons, causing severe motor and respiratory impairment with reduced survival, especially in its more severe clinical forms. In recent years, highly effective disease-modifying therapies have emerged, either acting by regulating the splicing of exon 7 of the SMN2 gene or adding a copy of the SMN1 gene through gene therapy, providing a drastic change in the natural history of the disease. In this way, developing therapeutic guides and expert consensus becomes essential to direct the use of these therapies in clinical practice. This consensus, prepared by Brazilian experts, aimed to review the main available disease-modifying therapies, critically analyze the results of clinical studies, and provide recommendations for their use in clinical practice for patients with SMA-5q. This consensus also addresses aspects related to diagnosis, genetic counseling, and follow-up of patients under drug treatment. Thus, this consensus provides valuable information regarding the current management of SMA-5q, helping therapeutic decisions in clinical practice and promoting additional gains in outcomes.

Atrofia muscular espinhal ligada ao cromossomo 5 (AME-5q) é uma doença genética de herança autossômica recessiva causada por mutações no gene SMN1. A AME-5q cursa com degeneração progressiva dos motoneurônios medulares e bulbares, acarretando grave comprometimento motor e respiratório com redução da sobrevida, especialmente nas suas formas clínicas mais graves. Nos últimos anos, terapias modificadoras da doença altamente eficazes, ou que atuam regulando o splicing do exon 7 do gene SMN2 ou adicionando uma cópia do gene SMN1 via terapia gênica, têm surgido, proporcionando uma mudança drástica na história natural da doença. Dessa forma, o desenvolvimento de guias terapêuticos e de consensos de especialistas torna-se importante no sentido de direcionar o uso dessas terapias na prática clínica. Este consenso, preparado por especialistas brasileiros, teve como objetivos revisar as principais terapias modificadoras de doença disponíveis, analisar criticamente os resultados dos estudos clínicos dessas terapias e prover recomendações para seu uso na prática clínica para pacientes com AME-5q. Aspectos relativos ao diagnóstico, aconselhamento genético e seguimento dos pacientes em uso das terapias também são abordados nesse consenso. Assim, esse consenso promove valiosas informações a respeito do manejo atual da AME-5q auxiliando decisões terapêuticas na prática clínica e promovendo ganhos adicionais nos desfechos finais.

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BACKGROUND: In 2012, the Neurocritical Care Society launched a compilation of protocols regarding the core issues that should be addressed within the first hours of neurological emergencies - the Emergency neurological life support (ENLS). OBJECTIVE: We aim to evaluate this repercussion through a bibliometric analysis. METHODS: We searched Scopus on October 2022 for articles mentioning ENLS. The following variables were obtained: number of citations; number of citations per year; number of publications per year; year of publication; research type; research subtype; country of corresponding author and its income category and world region; journal of publication and its 5-year impact factor (IF); and section where ENLS appeared. RESULTS: After applying eligibility criteria, we retrieved 421 articles, published from 2012 to 2022. The mean number of citations per article was 17.46 (95% Confidence Interval (CI) = 8.20-26.72), while the mean number of citations per year per article was 4.05 (95% CI = 2.50-5.61). The mean destiny journal 5-year IF was 5.141 (95% CI = 4.189-6.093). The majority of articles were secondary research (57.48%; n = 242/421) of which most were narrative reviews (71.90%; n = 174/242). High-Income countries were the most prominent (80.05%; n = 337/421 articles). There were no papers from low-income countries. There were no trials or systematic reviews from middle-income countries. CONCLUSION: Although still low, the number of publications mentioning ENLS is increasing. Articles were mainly published in journals of intensive care medicine, neurology, neurosurgery, and emergency medicine. Most articles were published by authors from high-income countries. The majority of papers were secondary research, with narrative review as the most frequent subtype.

ANTECEDENTES: Em 2012, a Neurocritical Care Society lançou uma compilação de protocolos sobre as questões centrais que devem ser abordadas nas primeiras horas de emergências neurológicas ­ Emergency neurological life support (ENLS). OBJETIVO: Avaliar a repercussão do ENLS por meio de uma análise bibliométrica. MéTODOS: A base de dados Scopus foi utilizada em outubro de 2022 para a busca por artigos mencionando o ENLS. As seguintes variáveis foram obtidas: número de citações; número de citações por ano; número de publicações por ano; ano de publicação; tipo de pesquisa; país do autor correspondente e sua categoria de renda; revista de publicação e seu fator de impacto de 5 anos (IF); e seção onde o ENLS apareceu. RESULTADOS: Os 421 artigos incluídos foram publicados de 2012 a 2022. A média de citações por artigo foi de 17.46 (intervalo de confiança (IC) 95% = 8.20­26.72), enquanto a de citações por ano por artigo foi de 4.05 (IC95% = 2.50­5.61). O IF médio por revista foi de 5.14 (IC95% = 4.19­6.09). A maioria dos artigos era de pesquisa secundária (57.48%; n = 242/421), dos quais a maioria eram revisões narrativas (71.90%; n = 174/242). Os países de alta renda foram os mais prolíficos (80.05%; n = 337/421 artigos). Não houve publicações de países de baixa ou média renda. CONCLUSãO: Embora ainda baixo, o número de publicações mencionando o ENLS vem aumentando recentemente. A maioria dos artigos foram publicados em revistas de medicina intensiva, neurologia, neurocirurgia e medicina de emergência. Artigos de pesquisa secundária foram os mais comuns, com revisões narrativas sendo o subtipo mais frequente.

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