RESUMEN
Myasthenia gravis (MG) is an autoimmune disorder affecting neuromuscular transmission leading to generalized or localized muscle weakness due most frequently to the presence of autoantibodies against acetylcholine receptors in the postsynaptic motor end-plate. Myasthenic crisis (MC) is a complication of MG characterized by worsening muscle weakness, resulting in respiratory failure that requires intubation and mechanical ventilation. It also includes postsurgical patients, in whom exacerbation of muscle weakness from MG causes a delay in extubation. MC is a very important, serious, and reversible neurological emergency that affects 20–30% of the myasthenic patients, usually within the first year of illness and maybe the debut form of the disease. Most patients have a predisposing factor that triggers the crisis, generally an infection of the respiratory tract. Immunoglobulins, plasma exchange, and steroids are the cornerstones of immunotherapy. Today with the modern neurocritical care, mortality rate of MC is less than 5%.
Miastenia grave (MG) é um distúbio autoimune que afeta principalmente a transmissão neuromuscular, levando a fraqueza muscular generalizada ou localizada. É devida mais frequentemente à presença de auto-anticorpos anti-receptores de acetilcolina na fenda pós-sináptica da placa motora. A crise miastênica (CM) é uma complicação da MG caracterizada por piora da fraqueza muscular, resultando en falência respiratória, o que requer entubação endotraqueal e ventilação mecânica. Isto ocorre também em pacientes pós-cirúrgicos, em que há piora da fraqueza muscular devido à MG, causando um atraso na extubação. MC é uma emergência neurológica importante, séria e reversível que afeta 20–30% dos pacientes miastênicos, usualmente duranteo primeiro ano de enfermidade, podendo a crise miastênica ser a manifestação inicial da MG. A maioria dos pacientes tem fatores predisponentes que desencadeiam a crise, geralmente uma infecção do trato respiratório. Imunoglobulina, plasmaférese e esteróides são a pedra angular da imunoterapia. Hoje, dentro da terapia neurocrítica, a taxa de mortalidade na CM é menor que 5%.
Asunto(s)
Humanos , Miastenia Gravis , Diagnóstico Diferencial , Miastenia Gravis/diagnóstico , Miastenia Gravis/fisiopatología , Miastenia Gravis/terapia , Factores de Riesgo , Índice de Severidad de la EnfermedadRESUMEN
In patients with acute cerebral injury, polyuric states can potentially trigger, maintain and aggravate the primary neurological damage, due to hypovolemia, arterial hypotension and alterations of osmolarity. The true incidence of the condition in this population is unknown. A widely validated definition of polyuric state is lacking and its etiology is multifactorial. There are two principal classes of polyuria: a) aqueous polyuria with diabetes insipidus as the main cause; and b) osmotic polyuria in which sodium, glucose or ureaplay the main role. Polyuric states are in close association with disorders of water and sodium metabolism and with alterations in acid-base balance. A detailed analysis of the history, clinical picture and simple laboratory determinations in blood and urine, are required for an adequate assessment of these polyuric states. The problem must be faced with pathophysiological reasoning and a systematic and sequential approach, because each disorder needs a specific therapy.