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Introduction: A first psychotic episode may be related to neurological diseases, especially encephalitis of infectious or autoimmune origin. It is remarkable that an immune-mediated encephalitis triggered by a confirmed subacute bacterial meningitis is documented, and this is the case we will present. Clinical case: A 22-year-old woman with no previous medical history, immunocompetent, with three months of behavioral, affective and cognitive symptoms with subsequent compromise of sensory perception and psychosis. Examination of cerebrospinal fluid showed inflammatory signs with positive FilmArray© for Streptococcus pneumoniae. She received anti-psychotic and antibiotic treatment for 2 weeks without clinical improvement. Postencephalitic syndrome with immune-mediated psychosis was considered as a diagnosis, and immunosuppressive management with corticosteroid and plasmapheresis was initiated with complete resolution of symptoms. After one year of follow-up no neurological relapse has been identified. Discussion: Encephalitis is a neurological syndrome due to brain parenchymal damage that can result in psychiatric symptoms including psychosis and behavioral changes. Its causes are usually infectious (usually viral) or autoimmune (Anti NMDA, AMPA, LGI1 or others). A psychiatric condition in bacterial meningitis without improvement with antibiotic treatment is remarkable, its presence should suggest an immune-mediated post-infectious syndrome that may respond to the use of immunomodulators even in the absence of identification of autoimmune encephalitis-associated antibodies. No similar cases have been reported in the literature. Conclusion: Immune-mediated psychosis may be a manifestation of post-encephalitic syndrome associated with bacterial meningitis and its treatment with immunosuppressants may offer benefit in cases where the use of antipsychotics and antibiotics shows no improvement.

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Abstract Sjogren's syndrome (SS) is a complex autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands, resulting in sicca symptoms. Additionally, SS presents with neurological manifestations that significantly impact the nervous system. This review aims to provide a comprehensive overview of the neurological aspects of SSj, covering both the peripheral and central nervous system involvement, while emphasizing diagnosis, treatment, and prognosis.

Resumo A síndrome de Sjogren (SS) é uma doença autoimune complexa caracterizada pela infiltração linfocítica das glândulas salivares e lacrimais, resultando em sintomas sicca. Além disso, a SS apresenta manifestações neurológicas que afetam significativamente o sistema nervoso. Esta revisão tem como objetivo fornecer uma visão abrangente dos aspectos neurológicos da SSj, abordando tanto o envolvimento do sistema nervoso periférico quanto do central, com ênfase no diagnóstico, tratamento e prognóstico.

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Abstract Precision medicine has revolutionized the field of neuroimmunology, with innovative approaches that characterize diseases based on their biology, deeper understanding of the factors leading to heterogeneity within the same disease, development of targeted therapies, and strategies to tailor therapies to each patient. This review explores the impact of precision medicine on various neuroimmunological conditions, including multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), optic neuritis, autoimmune encephalitis, and immune-mediated neuropathies. We discuss advances in disease subtyping, recognition of novel entities, promising biomarkers, and the development of more selective monoclonal antibodies and cutting-edge synthetic cell-based immunotherapies in neuroimmunological disorders. In addition, we analyze the challenges related to affordability and equity in the implementation of these emerging technologies, especially in situations with limited resources.

Resumo A medicina de precisão está revolucionando o campo da neuroimunologia, com uma abordagem inovadora caracterizada pela classificação de doenças com base em sua biologia, compreensão mais profunda dos fatores que levam à heterogeneidade dentro da mesma doença, desenvolvimento de terapias com alvos específicos e estratégias para adaptar as terapias a cada paciente. Esta revisão explora o impacto da medicina de precisão em várias condições neuroimunológicas, incluindo esclerose múltipla (EM), distúrbio do espectro da neuromielite óptica (NMOSD), doença associada ao anticorpo anti-glicoproteína da mielina do oligodendrócito (MOGAD), neurites ópticas, encefalites autoimunes e neuropatias imunomediadas. Discutimos avanços na subclassificação de doenças, reconhecimento de novas entidades, biomarcadores promissores e desenvolvimento de anticorpos monoclonais mais seletivos e imunoterapias de ponta baseadas em células sintéticas para as condições acima. Além disso, analisamos os desafios relacionados com acessibilidade e equidade na implementação dessas tecnologias emergentes, especialmente em ambientes com recursos limitados.

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The prevalence of neurological syndromes associated with antibodies to glutamic acid decarboxylase is increasing. While cognitive impairment is a common feature of this condition, it seldom emerges as the primary symptom. In this study, we discuss a case of refractory dementia associated with the glutamic acid decarboxylase spectrum disorder. Interestingly, this case showed a favorable outcome following autologous hematopoietic stem cell transplantation. We also provide an in-depth review of the current literature on the use of this therapeutic approach for the treatment of this disease.

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Background: Anti-LGI1 encephalitis is characterized by a pattern of inflammation that predominantly affects the limbic system It is part of the autoimmune encephalitis that attack neuronal surface antigens. It is characterized by the triad of subacute dementia, faciobrachial dystonic crises, and hyponatremia, presenting an excellent response to immunotherapy. The aim of this article is to describe the clinical evolution and functional outcome at 6 months of two patients with anti-LGI1 encephalitis using clinical cases. Clinical cases: Case 1: 62-year-old man with 8-week symptoms manifested by changes in mood, disorientation, and focal motor seizures. Case 2 A 72-year-old woman with a 5-month evolution of rapidly progressive dementia, hyponatremia and bitemporal hyperintensities on MRI. In both, due to clinical suspicion, acute dual immunotherapy with steroid and immunoglobulin was given with substantial improvement. Subsequently, the existence of anti-LGI1 antibodies in cerebrospinal fluid was confirmed. Although both patients received a dose of rituximab during their hospitalization, only the patient in the first case continued biannual doses of rituximab. The second patient was not initially considered to continue long-term immunomodulatory treatment and experienced a relapse. Conclusions: These clinical vignettes present the reader with the classic characteristics of this disease. This can facilitate its recognition and timely initiation of treatment, improving the functional prognosis of patients.

Introducción: la encefalitis anti-LGI1 se caracteriza por un patrón de inflamación que afecta de forma predominante al sistema límbico. Forma parte de las encefalitis autoinmunes que atacan a antígenos de superficie neuronal. Se caracteriza por la tríada de demencia subaguda, crisis distónicas faciobraquiales e hiponatremia, presentando una respuesta excelente a la inmunoterapia. El objetivo de este trabajo es describir por casos clínicos la evolución clínica y resultado funcional a 6 meses de dos pacientes con encefalitis anti-LGI1. Casos clínicos: caso 1: hombre de 62 años con cuadro de 8 semanas, manifestado por cambios en el estado de ánimo, desorientación y crisis focales motoras. Caso 2: mujer de 72 años con una evolución de 5 meses de demencia rápidamente progresiva, hiponatremia e hiperintensidades bitemporales en RMN. En ambos, ante la sospecha clínica, se otorgó inmunoterapia dual aguda con esteroide e inmunoglobulina con mejoría sustancial, posteriormente se corroboró la existencia de anticuerpos anti-LGI1 en líquido cefalorraquídeo. Pese a que ambos pacientes recibieron una dosis de rituximab durante su hospitalización, solo el primer caso continuó dosis semestrales de rituximab. El segundo no fue considerado inicialmente para continuar con tratamiento inmunomodulador a largo plazo y presentó una recaída. Conclusiones: estos casos, presentan al lector las características clásicas de esta enfermedad. Esto puede facilitar su reconocimiento y la instauración oportuna del tratamiento, mejorando el pronóstico funcional de los pacientes.

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RESUMEN Durante la infección aguda por el SARVS-CoV-2 se produce una desregulación del sistema inmune que puede durar hasta ocho meses después de controlado el cuadro agudo. Esto, sumado a otros factores, posiblemente este asociado con un aumento del riesgo de aparición y concurrencia de enfermedades autoinmunes. La aparición simultanea del síndrome de Guillain-Barré (SGB) y púrpura trombocitopénica (PTI) se ha reportado poco en la literatura, y el SGB raramente se asocia con otra enfermedad autoinmune. Presentamos el caso de un varón que luego de un mes de tener un cuadro agudo de COVID-19 moderado, presentó concurrentemente SGB y PTI con respuesta adecuada al tratamiento.

ABSTRACT During acute SARS-CoV-2 infection, there is persistent deregulation of the immune system that can last up to 8 months after the acute condition is controlled. This, added to other factors, is possibly associated with an increased risk of the appearance and concurrence of autoimmune diseases. The simultaneous occurrence of GBS and ITP has been rarely reported in the literature, and GBS is rarely associated with another autoimmune disease. We present the case of a man who, one month after his recovery from acute moderate COVID-19, presented concurrent GBS and ITP with an adequate response to treatment.

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RESUMEN La poliangitis microscópica es una vasculitis sistémica, asociada a la positividad de anticuerpos anticitoplasma de neutrófilos, caracterizada por el compromiso necrosante de los vasos de pequeño calibre. Las manifestaciones clínicas de la poliangitis microscópica son variadas y las formas más severas se manifiestan con glomerulonefritis rápidamente progresiva y capilaritis pulmonar. El compromiso nervioso afecta principalmente el sistema periférico. La afectación a nivel central no es común, y hallazgos clínicos de psicosis y alteración del comportamiento son bastante infrecuentes en el contexto de estas enfermedades autoinmunes.

A B S T R A C T Microscopic polyangiitis is a systemic anti-neutrophil cytoplasmic antibody-associated vasculitis, and is associated with the necrotising small calibre vessels. Its clinical manifestations are varied, and the most severe forms manifest with rapidly progressive glomerulonephritis and pulmonary capillaritis. In the nervous system, it mainly involves the peripheral system. Involvement of the central nervous system is not common, and clinical findings of psychosis and behaviour alterations are infrequent.

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Abstract Introduction: Epstein-Barr virus is an infectious agent used to immortalize and induce polyclonal activation of B cells. It has been widely described that this virus produces changes in the cells it infects and in the immune response, and stimulates the development of autoimmune diseases. Objective: To characterize the association between Epstein-Barr virus and multiple sclerosis described in current scientific literature. Materials and methods: A 59-years range literature search was conducted in the PubMed, ScienceDirect, Redalyc and SciELO databases using the following MeSH terms: "Epstein-Barr virus, multiple sclerosis autoimmune diseases, autoimmune diseases of the nervous system". Results: Many studies describe the association between Epstein-Barr virus and multiple sclerosis. It is believed that acute infection and viral reactivation promote the development of multiple sclerosis. Conclusions: It is necessary to conduct further research on the pathogenesis and morphophysiological and neuroimmunological changes -at the ecological, molecular, cellular, tissue, organic and systemic level- induced by the immune response and that favor the development of multiple sclerosis.

Resumen Introducción. El virus de Epstein-Barr (VEB) es un agente inmortalizador y activador policlonal de células B. Es conocido que este virus induce cambios en las células que infecta y en la respuesta inmune, y que favorece la presentación de enfermedades autoinmunes. Objetivo. Caracterizar la asociación entre el VEB y la esclerosis múltiple (EM) descrita en la literatura actual. Materiales y métodos. Se realizó una búsqueda bibliográfica con rango de 59 años mediante los términos DeCS "virus de Epstein-Barr, esclerosis múltiple, enfermedades autoinmunes del sistema nervioso" en las bases de datos PubMed, ScienceDirect, Redalyc y SciELO. Resultados. Hay muchos estudios que describen la asociación del VEB con la EM. Se cree que la infección aguda y la reactivación viral contribuyen al desarrollo de la enfermedad. Conclusiones. Es necesario realizar más estudios que indaguen sobre la patogénesis, los cambios morfofisiológicos y las alteraciones neuroinmunológicas en la ecología molecular, celular, tisular, orgánica y sistémica inducida por la respuesta inmune y que favorecen el desarrollo de la EM.

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ABSTRACT The year 2016 was the centennial anniversary of the recognition of the Guillain-Barré syndrome, which was first described by George Guillain, Jean-Alexandre Barré and André Strohl. In celebration of the centennial, this historical review describes aspects of the contributions of Guillain and the Spanish neurologist, Barraquer-Bordas and a brief account of the Fourth International Neurological Congress, which brought together Guillain and Barraquer-Bordas. There were many outstanding Brazilian physicians at that meeting. Finally, the author describes his interaction with Barraquer-Bordas and provides an account of his influence in shaping a generation of Brazilian neurologists, including himself.

RESUMO O ano de 2016 foi o aniversário do centenário do reconhecimento da síndrome de Guillain-Barré (GBS), que foi descrita pela primeira vez por George Guillain, Jean-Alexandre Barré e André Strohl. Em comemoração ao centenário, esta revisão histórica descreve aspectos das contribuições de Guillain e Barraquer-Bordas e uma breve descrição do IV Congresso Neurológico Internacional, que reuniu Guillain e o neurologista espanhol Barraquer-Bordas. Naquela reunião houve participação também de excelentes médicos brasileiros. Finalmente, o autor descreve sua interação com Barraquer-Bordas e fornece uma descrição de sua influência na formação de uma geração de neurologistas brasileiros, incluindo ele próprio.

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RESUMEN En 1976 se identificaron varios anticuerpos dirigidos contra el receptor de acetil colina en el suero de los pacientes con miastenia gravis (MG). Sin embargo, luego de unos años, se evidenció que aproximadamente el 20 % de los pacientes con MG generalizada y con evidencia electrofisiológica de un trastorno de la unión neuromuscular, no expresan dichos anticuerpos por radioinmunoensayo (RIA); éstos constituyen los casos de miastenia gravis seronegativa (MGSN). El diagnóstico en estos pacientes es difícil, dada la ausencia de autoanticuerpos detectables en suero y la falta de estudios neurofisiológicos sensibles. Recientemente un nuevo método basado en ensayos celulares muestra un aumento significativo en la detección de miastenia seropositiva, en casos diagnosticados previamente como seronegativos. Este artículo pretende dar un abordaje sobre la fisiopatología de la miastenia gravis seronegativa, así como una actualización de los últimos avances sobre su diagnóstico. También busca hacer una revisión sobre el contexto general actual de esta patología en Colombia.

SUMMARY In 1976 antibodies against acetyl-choline receptor were identified on the serum of patients with myasthenia gravis. However, some years later, it became clear that about 20% of patients with generalized MG and an electro physiologic disorder on the neuromuscular junction did not express these antibodies by radioimmunoassay (RIPA). These cases represent seronegative myasthenia gravis (SNMG). The diagnosis of these patients is difficult, given the absence of detectable autoantibodies on serum and the lack of sensitive neurophysiologic tests. Recently, a new method based on cellular assays shows an increase on detection of seropositive MG from cases, which were initially diagnosed as seronegative. This article reviews the physiopathology of seronegative MG and gives an update on the latest advances concerning its diagnosis. It also hopes to approach the current general context of the illness in Colombia.

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Background: Guillain-Barré Syndrome (GBS) is an acute polyneuropathy characterized by symmetrical weakness of the limbs with hyporeflexia or areflexia with a maximum progression within four weeks and can impair respiratory function and implies disability at a long. The aim of this paper was to describe the clinical, epidemiological and neurophysiological features of patients with GBS at the Hospital de Especialidades del Centro Medico Nacional Siglo XXI (HECMNSXXI) Methods: An observational, retrospective cross-sectional study, data were collected form clinical records of adults with GBS hospitalized in HECMNSXXI from March 2012 to March 2016. The recorded variables were demographics, previous infection, clinical presentation, disability scores, prognosis scores and neurophysiological subtypes. Results: Clinical records of 94 patients were analysed with a mean age of 53 years, 61% male, with previous infection in 80%. Albumin cytologic dissociation was present in 50%. Medical Research Council (MRC) sum scores mean was 32, the SGB disability score at admittance with a mean of 3.63. The axonal subtype was in 68%, and demyelinating in 29%, not conclusive in 3%. Conclusions: In this study the demographic and clinical features are similar to other previous reports, we documented a greater proportion of axonal subtype, which are related with important disability and worse prognosis.

Introducción: El síndrome de Guillain-Barré (SGB) es una polirradiculoneuropatía aguda caracterizada por debilidad simétrica progresiva de las extremidades con hipo o arreflexia, que progresa en un máximo de 4 semanas y que puede llevar a la falla respiratoria y a discapacidad a largo plazo. El objetivo de este trabajo fue describir las características clínicas, epidemiológicas y neurofisiológicas de pacientes con SGB del Hospital de Especialidades del Centro Médico Nacional Siglo XXI (HECMNSXXI). Métodos: Se realizó un estudio observacional de tipo transversal retrolectivo. Se obtuvieron datos de expedientes clínicos de adultos con diagnóstico de SGB hospitalizados en el HECMNSXXI en el periodo de marzo de 2012 a marzo de 2016. Se registraron variables demográficas, clínicas y neurofisiológicas. Resultados: Se incluyeron 94 pacientes con un promedio de edad de 53 años, en su mayoría hombres, con infección previa en 80%. La fuerza muscular medida con la escala del Medical Research Council (MRC) fue en promedio de 32, la escala de discapacidad del SGB (Hughes) al ingreso tuvo un promedio de 3.63. La variedad axonal se encontró en 68%, la desmielinizante en 29% y en 3% no concluyente. Conclusiones: Se documentan en esta muestra características demográficas y clínicas similares a lo reportado en la literatura, así como la mayor proporción de variedades axonales, las cuales tienen mayor severidad en la presentación clínica así como mal pronóstico.

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BACKGROUND: Guillain-Barré syndrome (GBS) has a cosmopolitan distribution. Its course is usually mild and tends to limit itself, but in severe cases it can cause death. The aim of this article is to describe the characteristics of a group of adults with GBS diagnosed and treated at a university hospital. METHODS: All cases of GBS that occurred between January 1, 2005 to December 31, 2009 were analyzed. The clinical records were reviewed through the implementation of a structured survey that included the following sections: patient identification, clinical data, history of infection, season of occurrence, rates of electrophysiological variants, and lethality. RESULTS: A total of 45 patients were included; the male to female ratio was 1.4: 1 and the mean age was 48.2 ± 16.0 years. The season with the highest number of cases was the summer. History of intestinal infection was present in 40 % of patients, and respiratory infection in 24.4%. The lethality associated with GBS was 11.1 % (95 % CI, 4.4-23.9), fatal cases occurred in patients older than the survivors (65.2 ± 15.0 versus 46.0 ± 14.9, p = 0.01). The most common variant was acute motor axonal neuropathy (64.4 %); there were four cases of Miller Fisher syndrome. CONCLUSIONS: The most common electromyographic variant of GBS was acute motor axonal neuropathy. The highest number of cases was observed during the summer season.

Introducción: el Síndrome de Guillain-Barré (SGB) tiene una distribución cosmopolita. Usualmente su curso es benigno y tiende a autolimitarse, pero en casos severos puede ocasionar la muerte. El objetivo de este artículo es describir las características de un grupo de adultos con SGB diagnosticados y tratados en un hospital universitario. Métodos: se analizaron todos los casos de SGB ocurridos del 1 de enero de 2005 al 31 de diciembre de 2009. Se revisaron los expedientes clínicos a través de la aplicación de una cédula estructurada que comprendió las siguientes secciones: identificación del paciente, datos clínicos, historia de infección, estación del año de ocurrencia, tipos de variantes electrofisiológicas y letalidad. Resultados: en total se incluyeron 45 pacientes; la relación hombre-mujer fue de 1.4:1 y la edad media del grupo fue 48.2 ± 16.0 años. La estación del año con mayor número de casos fue el verano. La historia de infección intestinal estuvo presente en 40 % de los pacientes, y la infección respiratoria en 24.4 %. La letalidad asociada con SGB fue de 11.1 % (IC: 95 %, 4.4-23.9), los casos fatales se presentaron en los pacientes con mayor edad que los que sobrevivieron (65.2 ± 15.0 frente a 46.0 ± 14.9, p = 0.01). La variante más frecuente fue la neuropatía axonal motora aguda (64.4 %); hubo cuatro casos de síndrome de Miller Fisher. Conclusiones: la variante electromiográfica más frecuente del SGB fue la neuropatía axonal motora aguda. Se observó mayor número de casos durante la estación de verano.

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Introdução: pesquisas recentes sugerem que os implantes dentários de titânio (Ti) podem ter mais efeitos colaterais do que se acreditava anteriormente. Somando-se aos comprometimentos estéticos dos metais, novas tecnologias com cerâmicas de zircônia (Zr) foram recentemente introduzidas na Odontologia, mantendo as características de sucesso do Ti, porém em reabilitações metal-free. Os resultados clínicos/histológicos com a cerâmica (ZrO2), impulsionados pela conscientização dos pacientes, que buscam estética sem metais, incrementaram sua demanda. Objetivo: encontrar uma alternativa viável aos implantes Ti e identificar os sistemas cerâmicos para uso em humanos, levando-se em conta a biocompatibilidade e a longevidade, apontando as suas vantagens e desvantagens. Métodos: foi realizado um amplo e detalhado levantamento bibliográfico. Conclusões: embora as normas ISO precisem ser revistas, verificou-se que os implantes de zircônia (Y-TZP) têm boas perspectivas para o futuro. O material apresenta maior longevidade nas reabilitações, pela menor adesão bacteriana. Os sistemas de implantes de Zr encontrados nos estudos foram: CeraRoot, Sigma, Z-Systems, Ziterion Zit-Z, Easy-Kon, Zeramex, Whitte Sky, Denti Circon Implants, Zimplant-Biosyr, Omnis-Creamed, White Implants e Ziraldent. Como desvantagens, encontram-se os custos elevados de produção, a necessidade de protetores no período de cura e a possível degradação hidrotérmica do material. Com base nas publicações científicas internacionais, conclui-se que os implantes dentários em Zr (Y-TZP) já são uma alternativa viável para substituir os de Ti, porém não ainda como rotina clínica.

Introduction: Recent research suggests that titanium (Ti) dental implants may have more side effects than previously believed.In addition to the fact that metals compromise aesthetics, emerging technologies involving zirconia(Zr) ceramics were recently introduced in dentistry, whichare proving as effective as Ti, but inmetal-free rehabilitation. The clinical/histological outcomes of ceramics (ZrO2), driven by the awareness of patients seeking aesthetics without metals, have increased their demand.Objective:To find a viable alternative to Ti implants and identify the ceramic systems amenable to use by humans, taking into account biocompatibility and longevity, while pointing out their advantages and disadvantages. Methods: Extensive and detailed bibliography. Conclusions: Although ISO standards need to be reviewed, it has been found that zirconia (Y-TZP) dental implantsshowa promising future. Zirconia increases the longevity oforal rehabilitation given its diminished bacterial adhesion.The following Zr implant systems were found in the studies :CeraRoot, Sigma, Z-Systems, Ziterion Zit-Z, Easy-Kon, Zeramex, White Sky, Denti Circon Implants, Zimplant-Biosyr, Omnis-Creamed, White Implants andZiraldent. Among the disadvantages are a high production cost, the need for protectors duringhealing, and potential hydrothermal degradation of the material. Based on international scientific publications, it was concluded thatZr (Y-TZP) dental implants are now a viable substitute for Ti, although not yet recommended forroutine clinical practice.

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Myasthenic crisis is a complication of myasthenia gravis characterized by worsening of muscle weakness, resulting in respiratory failure that requires intubation and mechanical ventilation. Advances in critical care have improved the mortality rate associated with myasthenic crisis. This article reviews the epidemiology of myasthenic crisis and discusses patient evaluation. Therapeutic options including mechanical ventilation and pharmacological and surgical treatments are also discussed.