ABSTRACT
Abstract Background Botulism is a rare and potentially fatal neuroparalytic syndrome caused by the gram-positive anaerobe spore-forming bacteriumClostridium botulinum. The microorganism produces a neurotoxin that inhibits the presynaptic release of acetylcholine at the neuromuscular junction, clinically leading to a myasthenic syndrome. Objective To describe the recent outbreak of botulism cases and its demographic, clinical, and laboratory characteristics. Methods We report 4 patients with botulism in the recent outbreak occurred between 2017 and 2019 in the state of Amazon. Results Out of four patients with botulism, three contracted it from eating contaminated food and one had wound botulism. We emphasize the excellent clinical outcome of the different disease presentations in our case series. Conclusion The temporal proximity of these reports may suggest a new rise in the number of cases in the upcoming years. A possible hypothesis is that the rarity of the disease decreased the awareness regarding the primary prevention or even a diagnosis by an untrained physician.
Resumo Antecedentes O botulismo é uma síndrome neuroparalítica rara e potencialmente fatal causada pela bactéria anaeróbia gram-positiva formadora de esporosClostridium botulinum. O microrganismo produz uma neurotoxina que inibe a liberação pré-sináptica de acetilcolina na junção neuromuscular, levando clinicamente a uma síndrome miastênica. Objetivo Descrever o recente surto de casos de botulismo e suas características demográficas, clínicas e laboratoriais. Métodos Relatamos quatro pacientes com botulismo em surto recente ocorrido entre 2017 e 2019 no estado do Amazonas. Resultados Dos quatro pacientes com botulismo, três o contraíram devido ao consumo de alimento contaminado e um por ferimento. Enfatizamos o excelente resultado clínico das diferentes apresentações da doença em nossa série de casos. Conclusão A proximidade temporal dessas notificações pode sugerir um novo aumento no número de casos nos próximos anos. Uma possível hipótese é que a raridade da doença diminuiu a conscientização quanto à prevenção primária ou mesmo ao diagnóstico por médico destreinado.
ABSTRACT
ABSTRACT. Normal-pressure hydrocephalus (NPH) is a potentially reversible syndrome characterized by enlarged cerebral ventricles (ventriculomegaly), cognitive impairment, gait apraxia and urinary incontinence. A critical review of the concept, pathophysiology, diagnosis, and treatment of both idiopathic and secondary NPH was conducted. We searched Medline and PubMed databases from January 2012 to December 2018 using the keywords "normal-pressure hydrocephalus" / "idiopathic normal-pressure hydrocephalus" / "secondary normal-pressure hydrocephalus" / "NPH" / "ventriculoperitoneal shunt". The initial search produced 341 hits. After careful selection, a total of 54 articles were chosen and additional relevant studies were included during the process of writing this article. NPH is an important cause of potentially reversible dementia, frequent falls and recurrent urinary infections in the elderly. The clinical and imaging features of NPH may be incomplete or nonspecific, posing a diagnostic challenge for medical doctors and often requiring expert assessment to minimize unsuccessful surgical treatments. Recent advances resulting from the use of non-invasive MRI methods for quantifying cerebral blood flow, in particular arterial spin-labeling (ASL), and the frequent association of NPH and obstructive sleep apnea (OSA), offer new avenues to understand and treat NPH.
RESUMO. A hidrocefalia de pressão normal (HPN) é uma síndrome potencialmente reversível marcada por ventrículos cerebrais alargados (ventriculomegalia), declínio cognitivo, apraxia da marcha e incontinência urinária. Revisar criticamente o conceito, a fisiopatologia, o diagnóstico e o tratamento da HPN idiopática e secundária. Os autores acessaram as bases de dados Medline e Pubmed entre janeiro de 2012 e dezembro de 2018, utilizando as palavras-chave "normal-pressure hydrocephalus" / "idiopathic normal-pressure hydrocephalus" / "secondary normal-pressure hydrocephalus" / "NPH" / "ventriculoperitoneal shunt". A busca inicial resultou em 341 artigos. Após cuidadosa seleção, 54 estudos foram escolhidos e pesquisas adicionais foram incluídas durante o processo de elaboração do manuscrito. A HPN é uma importante causa de demência potencialmente reversível, quedas frequentes e infecção urinária recorrente em idosos. As características clínicas e de imagem da HPN podem ser incompletas ou inespecíficas, de modo que este se torna um diagnóstico difícil para médicos. Não raro uma avaliação por especialista é necessária, visando minimizar tratamentos cirúrgicos ineficazes. Avanços recentes advindos do uso não invasivo de ressonância magnética para quantificação do fluxo sanguíneo cerebral, em particular arterial spin-labeling (ASL), assim como a usual associação entre HPN e apneia obstrutiva do sono representam novos meios de entender e de tratar a HPN.
Subject(s)
Humans , Spinal Puncture , Urinary Incontinence , Accidental Falls , Dementia , Hydrocephalus, Normal PressureABSTRACT
The brain represents 2% of the adult body mass; conversely, it is responsible for 20% to 25% of the glucose and 20% of the oxygen consumption, receiving 15% of the cardiac output. This substantial metabolic rate is associated with a significant production of biological debris, which is potentially toxic. Therefore, a complex and efficient clearance system is required to prevent the accumulation of byproducts and ensure optimal function. However, until today, there is little knowledge about this topic. The glymphatic system, also known as perivascular pathway, is a recently described glialdependent network that is responsible for the clearance of metabolites from the central nervous system (CNS), playing a role equivalent to the one played by the lymphatic vessels present in other organs. Studies have demonstrated that the glymphatic pathway has a paramount role in protein homeostasis, and that the malfunction of this system may be related to the development of neurodegenerative disorders such as Alzheimer disease and normal pressure hydrocephalus. They also showed that body posture, exercise and the state of consciousness influence the glymphatic transport. In this context, the understanding of this clearance system could not only clarify the pathophysiology of several diseases, but also contribute to future therapeutic interventions. In the present article, we will evaluate the glymphatic pathway, focusing on the factors that regulate its flow, as well as on its role in CNS physiology and in disease initiation and progression, including dementia, hydrocephalus, glaucoma and traumatic brain injury. Ultimately, this review also aims to encourage further research on novel therapeutic targets.