RESUMO
Las enfermedades de Marchiafava-Bignami y de Wernicke Korsakoff, se consideran complicaciones neuropsiquiátricas causadas por el consumo crónico de bebidas alcohólicas. Son encefalopatías poco frecuentes caracterizadas por una desmielinización y necrosis del cuerpo calloso, con la subsiguiente atrofia por daño en las partes bajas del cerebro (tálamo e hipotálamo). Se presenta un paciente masculino de 29 años, con antecedentes de alcoholismo, el cual acude a consulta de Oftalmología por presentar disminución de la visión del ojo derecho durante un año. Se le realizaron, tomografía simple y resonancia magnética con contraste endovenoso de cráneo, donde se observaron hallazgos radiológicos compatibles con el síndrome de Wernicke Korsakoff (ocasiona afectación de la memoria y el aprendizaje) con estigmas de Marchiafava-Bignami (enfermedad poco conocida). Es necesario el dominio de la epistemología de estas enfermedades, porque, a pesar del mal pronóstico en su forma aguda, se reportan casos con buena evolución, si se le realiza un diagnóstico y tratamiento oportunos.
Marchiafava-Bignami and Wernicke-Korsakoff diseases are considered neuropsychiatric complications caused by the chronic consumption of alcoholic beverages. They are rare encephalopathies characterized by demyelination and necrosis of the corpus callosum, with subsequent atrophy due to damage in the lower parts of the brain (thalamus and hypothalamus). We present a 29-year-old male patient with a history of alcoholism who went to the Ophthalmology consultation due to decreased vision in his right eye for a year. Simple tomography and magnetic resonance imaging with intravenous contrast of the skull were performed, observing radiological findings of Wernicke -Korsakoff syndrome (affect memory and learning) with Marchiafava-Bignami stigmata (little-known disease). Mastery of the epistemology of these diseases is necessary, because, despite the poor prognosis in its acute form, cases with good evolution are reported, if an opportune diagnosis and treatment is made.
Assuntos
Encefalopatia de Wernicke , Doença de Marchiafava-Bignami , Imageamento por Ressonância Magnética Multiparamétrica , TomografiaRESUMO
RESUMEN La enfermedad de Marchiafava-Bignami ha sido reconocida como un síndrome caracterizado por necrosis y desmielinización focal del cuerpo calloso. Fue descrita por primera vez en 1903 por los patólogos Marchiafava y Bignami en tres pacientes alcohólicos italianos, bebedores de vino tinto. Se reporta el caso de una paciente de 55 años, alcohólica, que acudió con crisis epilépticas generalizadas y deterioro del nivel de conciencia. La resonancia magnética de cráneo reveló desmielinización del cuerpo calloso, sustancia blanca y gris de los lóbulos parietales y frontales con lo que se diagnosticó una enfermedad de Marchiafava-Bignami aguda tipo A. La paciente falleció a causa de una neumonía intrahospitalaria a los 15 días luego del ingreso. Es necesario el conocimiento de esta enfermedad, porque a pesar del mal pronóstico de su forma aguda, se reportan casos de buena evolución con un diagnóstico y tratamiento oportunos. Se presenta el caso con el objetivo de evidenciar el diagnóstico de una enfermedad poco frecuente que se puede expresar de forma variable y califica como una urgencia médica.
ABSTRACT Marchiafava-Bignami disease has been recognized as a syndrome characterized by necrosis and focal demyelination of the corpus callosum. It was first described in 1903 by the pathologists Marchiafava and Bignami in three Italian alcoholic patients who drink red wine. The case of a 55-year-old patient is reported, alcoholic, who attended with generalized epileptic seizures and impaired level of consciousness. Magnetic resonance imaging of the skull revealed demyelination of the corpus callosum, white and gray matter of the parietal and frontal lobes, leading to the diagnosis of acute Marchiafava-Bignami type A disease. The patient died of hospital-acquired pneumonia 15 days later of income. Knowledge of this disease is necessary, because despite the poor prognosis of its acute form, cases of good evolution with timely diagnosis and treatment are reported. The case is presented with the aim of evidencing the diagnosis of a rare disease that can be expressed in a variable way and qualifies as a medical emergency.
RESUMO
The Marchiafava-Bignami disease, characterized by demyelination and necrosis of the corpus callosum, has typically been associated with chronic alcohol intake, and clinically has various symptoms and signs. However, several cases have been reported without alcohol association, and these - according to several publications - have some common points, such as preference for female, related to malnutrition, and radiological involvement of the splenium of the corpus callosum. We report a case of a patient with the characteristics described above and whose clinical manifestation was Athetosis. The authors associate this manifestation with the somatotopic distribution of the corpus callosum, and contribute to the etiologic diagnosis of Athetosis as a manifestation of the Marchiafava-Bignami disease, which has not been reported in the medical literature according to our review of the database.
RESUMO
RESUMEN: Arch Med Interna 2013 - 35(3):105-108 La Enfermedad de Marchiafava-Bignami descripta a principios del siglo XX en bebedores de vino tinto y posteriormente en consumidores de otras bebidas alcohólicas, es una de las complicaciones menos frecuentes de los alcoholistas crónicos, su reconocimiento ha sido puesto en evidencia en los últimos tiempos debido a la Resonancia Magnética (RM). Lamentablemente el pronóstico, no ha variado sustancialmente debido a que no existe una terapéutica específica.
ABSTRACT: Arch Med Interna 2013 - 35(3):105-108 The Marchiafava-Bignami disease described at the beginning of the twentieth century, in red wine drinkers and consumers subsequently spirits, is a less common complication of chronic alcoholics, their recognition has been highlighted in recent times due to the Magnetic Resonance (MR). Unfortunately the prognosis has not changed substantially since there is no specific therapy.
RESUMO
La enfermedad de Marchiafava-Bignami descripta a principios del siglo XX en bebedores de vino tinto y posteriormente en consumidores de otras bebidas alcohólicas, es una de las complicaciones menos frecuentes de los alcoholistas crónicos, su reconocimiento ha sido puesto en evidencia en los últimos tiempos debido a la Resonancia Magnética (RM). Lamentablemente el pronóstico, no ha variado sustancialmente debido a que no existe una terapéutica específica.
Assuntos
Humanos , Doença de Marchiafava-Bignami/diagnóstico , Doença de Marchiafava-Bignami/etiologia , Doença de Marchiafava-Bignami/terapia , Alcoolismo/complicações , Corpo Caloso , Doenças Desmielinizantes/diagnóstico , Doenças Desmielinizantes/etiologia , Diagnóstico por ImagemRESUMO
A doença de Marchiafava-Bignami é uma entidade rara, caracterizada por uma degeneração primária do corpo caloso, associada com o consumo crônico do etanol. A doença pode, ocasionalmente, ocorrer em pacientes não etilistas cronicamente desnutridos. Uma deficiência de vitaminas do complexo B é considerada como a hipótese etiopatogênica principal, uma vez que muitos pacientes obtiveram uma melhora após a administração desses compostos. Algumas vezes, entretanto, tal resposta terapêutica não foi observada. O diagnóstico definitivo da doença de Marchiafava-Bignami pode ser problemático e depende das características de estudos de neuroimagem, especialmente a ressonância magnética. Seu tratamento, dessa forma, é ainda controverso, com resultados variáveis. Como estão implicados fatores nutricionais, analogamente à encefalopatia de Wernicke, alguns autores recomendam a reposição de vitaminas do complexo B, particularmente da B1. O presente artigo relata a forma aguda da doença de Marchiafava-Bignami em um paciente masculino dependente do álcool, que apresentou discreta melhora após a administração parenteral das vitaminas do complexo B. Como consequência de suas más condições neurológicas e imunológicas, ele desenvolveu infecções pulmonares múltiplas e permaneceu, por longo tempo, na unidade de terapia intensiva. Seu óbito ocorreu por sepse causada por um fungo raro, o Rhodotorula mucilaginosa. O artigo é um relato clínico da evolução desse paciente, com a apresentação de seus dados de neuroimagem, acompanhada por uma revisão sobre doença de Marchiafava-Bignami e sobre as infecções por Rhodotorula dentro da perspectiva da unidade de cuidado intensivo.
Marchiafava-Bignami disease is a rare affliction characterized by primary degeneration of the corpus callosum associated with chronic consumption of ethanol. The disease may occasionally occur in patients who are not alcoholics but are chronically malnourished. A complex deficiency of group B vitamins is the main etiopathogenic hypothesis, and many patients improve after the administration of these compounds. However, a good response is not always observed. The definitive diagnosis of Marchiafava-Bignami disease can be problematic and is based on features of neuroimaging studies, especially magnetic resonance imaging. Its treatment is still controversial and shows variable results. Because nutritional factors are implicated, as in Wernicke's encephalopathy, some authors claim that replacement of B vitamins is beneficial. The present article is a case report of a severe acute form of Marchiafava-Bignami disease in an alcohol-dependent male patient who improved after the administration of parenteral B vitamins. As a consequence of his neurological and immunologic conditions, he developed multiple pulmonary infections and had a protracted course in the intensive care unit. He eventually died of sepsis associated with an uncommon fungus, Rhodotorula mucilaginosa. The present article reports the clinical and neuroimaging data from this patient and contains a review of Marchiafava-Bignami disease and Rhodotorula infections in the intensive care unit.