Marchiafava-Bignami disease with haemophagocytic lymphohistiocytosis as a postoperative complication of cardiac surgery.

Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism; however, MBD in a non-alcoholic diabetic patient has rarely been reported. The aetiology or pathophysiology of MBD is still unknown. A 50-year-old man with a history of untreated diabetes mellitus underwent on-pump beating coronary artery bypass graft surgery (CABG) surgery for three-vessel and left main coronary disease. 3 days after the surgery, he developed a fever over 40°C and entered a coma state. MRI revealed multiple lesions, including in the corpus callosum, globus pallidus, brain stem and upper cervical spinal cord, which suggested MBD. The patient did not respond to thiamine therapy, but partly responded to steroid therapy. He ultimately died of respiratory failure. The autopsy revealed MBD and haemophagocytic lymphohistiocytosis. It is rare, but systemic inflammatory response syndrome induced by on-pump beating CABG could develop these complication.

Clinical and radiological features of Marchiafava-Bignami disease.

Marchiafava-Bignami disease (MBD) is a rare neurological disease usually associated with chronic alcoholism and characterized by demyelination and necrosis. Our aims were to describe the clinicoradiological features and identify factors that may affect the prognosis of patients with MBD.We examined clinical manifestations, laboratory results, and neuroradiological features of 9 patients with MBD. The patients were classified into 2 subgroups (favorable and poor outcome subgroups) based on the Modified Oxford Handicap Scale (MOSH). In addition, we compared the clinical and neuroimaging features between the 2 subgroups.Nine adult male patients (age of onset range 37-62 years, with a mean age of 47.00 ±â€Š14.50 years) were included in this study. According to MOSH, 4 patients were placed in the poor outcome subgroup (MOHS ≥ 3), 5 patients were placed in the favorable outcome subgroup (MOHS ≤ 2). Relatively high score of MAST-C (≥6) (P = .008), extracallosal lesions (P = .048), GCS (P = .026), cerebral lobe impairment (P = .048) was significantly more common in the poor outcome subgroup.Clinical manifestations of MBD are variable and lack specificity. Early diagnosis by relatively specific performance of bisymmetric lesions in corpus callosum of diffusion-weighted imaging (DWI) may affect the prognosis. The prognosis of patients with severe disturbance of consciousness, heavy alcohol consumption, extracallosal lesions, cerebral lobe impairment is probably unfavorable.

Marchiafava-Bignami disease: a rare entity with a poor outcome.

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.

Doença de Marchiafava-Bignami: uma rara entidade com prognóstico sombrio / Marchiafava-Bignami disease: a rare entity with a poor outcome

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.

A patient with Marchiafava-Bignami disease as a complication of diabetes mellitus treated effectively with corticosteroid.

We report a 37-year-old woman with no history of alcohol consumption or malnutrition who had Marchiafava-Bignami disease (MBD) as a complication of diabetes mellitus. The patient suddenly developed dizziness and could not speak words fluently. Neurological examination revealed acalculia, agraphia, left blepharoptosis, and mild left facial palsy. Her blood glucose was 391 mg/dL, and her glycated hemoglobin (HbA1c) was 16.0%. Her brain MRI revealed hyperintense changes in the corpus callosum on T2-weighted, fluid-attenuated inversion recovery and diffusion-weighted images. In addition to therapy for diabetes mellitus, prednisolone was commenced. All of the symptoms gradually improved, and after three months, only slight dysarthria, acalculia, and agraphia were observed. Brain MRI also revealed a reduction in lesion size. In conclusion, MBD may occur even with metabolic disorders. It is important to diagnose MBD in the early stages with MRI and to treat the symptoms with cortico steroids.

Marchiafava-Bignami disease.

Marchiafava- Bignami disease is the symmetrical demyelination of the middle portion of the corpus callosum observed in people with chronic alcoholism. We report two male patients who had history of chronic alcoholism, different clinical presentation and MRI findings consistent with the diagnosis of Marchiafava-Bignami disease.

[Marchiafava-Bignami disease in Uruguay]. / Enfermedad de Marchiafava-Bignami en Uruguay.

INTRODUCTION: Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism characterized by demyelination and necrosis of the corpus callosum that usually has a poor prognosis. It has an extensive clinical spectrum presentation and can presently be diagnosed in vivo with the computed tomography (CT) and magnetic resonance imaging (MRI). CLINICAL CASES: We describe two cases of MBD disease with fatal outcome that presented with acute onset coma. Impairment of consciousness, hypertonia, and frontal release signs were the dominant clinical signs. One of the patients had optic neuropathy and the other had neuropsychiatric symptoms before onset of coma and pellagra-like skin lesion. The CT scan and MRI showed lesions of the white matter that were fundamentally in the corpus callosum. CONCLUSIONS: The possibility of the existence of MBD should be investigated in all patients with chronic alcoholism who have prolonged confusional syndrome, stupor or coma. A prodromal stage with neuropsychiatric symptoms should be thoroughly investigated with a neuropsychological exploration including interhemispheric disconnection signs. Careful attention should be given to the corpus callosum in the image for the diagnosis of this probably underdiagnosed disease.