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1.
BMC Med Imaging ; 24(1): 100, 2024 Apr 29.
Artigo em Inglês | MEDLINE | ID: mdl-38684964

RESUMO

PURPOSE: To detect the Marchiafava Bignami Disease (MBD) using a distinct deep learning technique. BACKGROUND: Advanced deep learning methods are becoming more crucial in contemporary medical diagnostics, particularly for detecting intricate and uncommon neurological illnesses such as MBD. This rare neurodegenerative disorder, sometimes associated with persistent alcoholism, is characterized by the loss of myelin or tissue death in the corpus callosum. It poses significant diagnostic difficulties owing to its infrequency and the subtle signs it exhibits in its first stages, both clinically and on radiological scans. METHODS: The novel method of Variational Autoencoders (VAEs) in conjunction with attention mechanisms is used to identify MBD peculiar diseases accurately. VAEs are well-known for their proficiency in unsupervised learning and anomaly detection. They excel at analyzing extensive brain imaging datasets to uncover subtle patterns and abnormalities that traditional diagnostic approaches may overlook, especially those related to specific diseases. The use of attention mechanisms enhances this technique, enabling the model to concentrate on the most crucial elements of the imaging data, similar to the discerning observation of a skilled radiologist. Thus, we utilized the VAE with attention mechanisms in this study to detect MBD. Such a combination enables the prompt identification of MBD and assists in formulating more customized and efficient treatment strategies. RESULTS: A significant breakthrough in this field is the creation of a VAE equipped with attention mechanisms, which has shown outstanding performance by achieving accuracy rates of over 90% in accurately differentiating MBD from other neurodegenerative disorders. CONCLUSION: This model, which underwent training using a diverse range of MRI images, has shown a notable level of sensitivity and specificity, significantly minimizing the frequency of false positive results and strengthening the confidence and dependability of these sophisticated automated diagnostic tools.


Assuntos
Aprendizado Profundo , Imageamento por Ressonância Magnética , Doença de Marchiafava-Bignami , Humanos , Doença de Marchiafava-Bignami/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Masculino , Feminino , Pessoa de Meia-Idade , Adulto , Interpretação de Imagem Assistida por Computador/métodos , Sensibilidade e Especificidade
2.
J Int Med Res ; 52(4): 3000605241234555, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38587813

RESUMO

Among the various manifestations of COVID-19, the neurological implications of SARS-CoV-2 infection are of significant concern. Marchiafava-Bignami disease (MBD), a neurodegenerative disorder, exhibits a clinical spectrum ranging from mild progressive dementia in its chronic form to states of acute coma and varied mortality rates. Acute MBD primarily occurs in chronic alcoholics and malnourished individuals and is characterized by sudden loss of consciousness, seizures, confusion, and psychosis. We herein report a case of MBD presenting as acute loss of consciousness after the development of COVID-19. The patient presented with a history of fever and upper respiratory infection and was diagnosed with SARS-CoV-2 infection. He developed a neurological syndrome characterized by altered consciousness and convulsions, and brain magnetic resonance imaging revealed abnormal signals in the corpus callosum and frontoparietal lobes. Considering his alcohol intake history and the absence of other differential diagnoses, we diagnosed him with acute MBD triggered by COVID-19. After high-dose vitamin B1 and corticosteroid therapy, his clinical symptoms improved. In this case, we observed a temporal sequence between the development of COVID-19 and acute exacerbation of MBD. This case adds to the mounting evidence suggesting the potential effect of SARS-CoV-2 on the neurological system.


Assuntos
COVID-19 , Demência , Doença de Marchiafava-Bignami , Humanos , Masculino , Estado de Consciência , Doença de Marchiafava-Bignami/diagnóstico , Doença de Marchiafava-Bignami/diagnóstico por imagem , COVID-19/complicações , SARS-CoV-2 , Coma
3.
Neurol Sci ; 45(5): 2365-2366, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38291195

RESUMO

The Marchiafava-Bignami disease has a curious backstory, namely, the publication in 1898 of the Contribution to the Study of Nonsuppurative Encephalitis (Carducci A in Riv Psicol Psichiat Neuropat 8-9:125-135, 1898), in which the neo-graduate Agostino Carducci described the disease that the pathologists Ettore Marchiafava and Amico Bignami would report 5 years later.


Assuntos
Encefalite , Doença de Marchiafava-Bignami , Humanos , Doença de Marchiafava-Bignami/diagnóstico por imagem , Corpo Caloso , Imageamento por Ressonância Magnética
5.
Neurol Sci ; 45(1): 369-372, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37723370

RESUMO

Marchiafava-Bignami disease (MBD) is a metabolic disease of the nervous system. It mainly involves the Corpus callosum, but the handknob area is rarely involved. This article reports a MBD case involving the bilateral handknob area. The involvement of the bilateral handknob area contributes to the clinical presentation of convulsions of both hands. Through this case, more clinicians realize the bilateral handknob area involvement in MBD, which is helpful for the identification and diagnosis of MBD. To our knowledge, this is the first report on MBD involving the bilateral handknob area.


Assuntos
Alcoolismo , Doença de Marchiafava-Bignami , Humanos , Doença de Marchiafava-Bignami/complicações , Doença de Marchiafava-Bignami/diagnóstico por imagem , Imageamento por Ressonância Magnética , Corpo Caloso/diagnóstico por imagem
6.
Medicentro (Villa Clara) ; 27(4)dic. 2023.
Artigo em Espanhol | LILACS | ID: biblio-1534867

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 , Tomografia
9.
Sci Rep ; 13(1): 18516, 2023 10 28.
Artigo em Inglês | MEDLINE | ID: mdl-37898646

RESUMO

We aimed to investigate the clinico-radiologic features of acute Marchiafava-Bignami disease (MBD) and its evolutionary process after effective treatment through subgroup comparison. The clinical and MRI data of 23 patients with acute MBD were retrospectively analyzed and divided into type A (12 cases, with entire callosal involvement) and type B (11 cases, with focal callosal involvement). The clinical assessments and MRI findings (before and after treatment) were compared between the two subtypes. Compared with type B, type A had lower MoCA (Montreal Cognitive Assessment) scores at admission (16.50 ± 1.73 vs 18.27 ± 1.68, P = 0.021) and were more common with extracallosal involvement (66.67% vs 18.18%, P = 0.036) and longer illness duration (18.3 ± 2.1 days vs 15.6 ± 2.4 days, P = 0.012). During the treatment, the residual lesion in the splenium was more common in type A (58.33% vs 9.09%, P = 0.027). After treatment, the MoCa scores of both subtypes gradually increased (P < 0.001), and the callosal and extracallasal lesions disappeared completely. Clinico-radiologic typing of acute MBD is related to the severity of early symptoms, but not to the prognosis. Complete clinico-radiologic recovery is possible for both subtypes with combined treatment. The clinico-radiologic reversibility is helpful for accurate diagnosis and therapeutic evaluation.


Assuntos
Alcoolismo , Doença de Marchiafava-Bignami , Humanos , Doença de Marchiafava-Bignami/diagnóstico por imagem , Doença de Marchiafava-Bignami/patologia , Estudos Retrospectivos , Corpo Caloso/diagnóstico por imagem , Corpo Caloso/patologia , Imageamento por Ressonância Magnética , Prognóstico , Alcoolismo/patologia
12.
Ann Clin Transl Neurol ; 10(11): 2013-2024, 2023 11.
Artigo em Inglês | MEDLINE | ID: mdl-37649317

RESUMO

OBJECTIVE: Most patients with Marchiafava-Bignami disease (MBD) had unfavorable prognosis, with disability or death. We aimed to determine the risk factors of early unfavorable prognosis of MBD, and to develop a predictive nomogram for early unfavorable prognosis of MBD. METHODS: MBD patients admitted to our hospital between 1 January 2013 and 31 December 2021 were included. Unfavorable prognosis was defined as mRS score ≥3, the independent risk factors for unfavorable prognosis of MBD with the odds ratio (OR) and 95% confidential interval (CI) acquired by multiple logistic regression were included in development of the predictive nomogram for early unfavorable prognosis of MBD, and the area under curve (AUC) of the receiver operating characteristic curve was calculated. The published case reports of MBD were used as the external validation group to verify the predictive ability of the nomogram. RESULTS: Independent risk factors for early unfavorable prognosis of MBD included Glasgow Coma Scale score (OR = 0.636, 95% CI = 0.506-0.800, p = 0.004) and pneumonia (OR = 2.317, 95% CI = 1.003-5.352, p = 0.049). The AUC of the nomogram was 0.852. Ninety-four case reports, a total of 100 cases of MBD were included as the external validation group, its AUC was 0.840. The online dynamic nomogram for early unfavorable prognosis of MBD was constructed. INTERPRETATION: It is confirmed by external validation that the nomogram has a preferable predictive ability and clinical efficacy, and the dynamic online predictive nomogram is helpful for physicians to quickly assess the prognosis of MBD.


Assuntos
Doença de Marchiafava-Bignami , Nomogramas , Humanos , Prognóstico , Fatores de Risco , Resultado do Tratamento
13.
Top Magn Reson Imaging ; 32(1): 1-4, 2023 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-36648166

RESUMO

ABSTRACT: In this case report we describe the case of a 66-year old man with subacute gait difficulties, with a progression to confusion coma with multiple generalised epileptic seizures during the following days. Biochemical analysis showed hyperglycaemia, cerebrospinal fluid (CSF) testing showed a mild lymphocytic pleocytosis and an elevated protein and lactate. Broad-spectrum antibiotics and antiviral therapy where initiated. However, all other CSF testing remained negative. Magnetic resonance imaging of the brain showed remarkably symmetric hyperintense T2 white matter lesions most noticable in the corpus callosum. The lesion pattern was suggestive of a metabolic or toxic encephalopathy, the preponderance for the corpus callosum was furthermore suggestive for Marchiafava-Bignami disease (MDB), as was the clinical course since admission of the patient. A high dose IV substitution of vitamin B1, B6 and B12 was started and antibiotic and antiviral therapy was discontinued. After one day the patient showed progressive regaining of consciousness and he returned to premorbid functioning in a matter of 1-2 weeks. MRI of the brain after 1 week showed notable improvement of the white matter lesions. At routine follow-up two weeks later he presented with icterus and a diagnosis of Epstein-Barr virus (EBV) hepatitis was made, lymph node biopsies showed an EBV positive diffuse large cell B-cell lymphoma (DLCBL). MDB is mostly associated with severe alcoholism, with malnourishment being the second leading cause, however there are case reports describing MDB in patients with chronically poorly controlled diabetes mellitus. We hypothesize that his condition may have been precipitated by his poorly controlled diabetes mellitus. However it is also possible that weight loss (probably related to the DLCBL diagnosis) might have contributed to a state of malnourishment and therefore played a role in the aetiology as well.


Assuntos
Infecções por Vírus Epstein-Barr , Desnutrição , Doença de Marchiafava-Bignami , Masculino , Humanos , Idoso , Doença de Marchiafava-Bignami/complicações , Doença de Marchiafava-Bignami/patologia , Coma/complicações , Infecções por Vírus Epstein-Barr/complicações , Herpesvirus Humano 4 , Imageamento por Ressonância Magnética , Desnutrição/complicações , Antivirais
14.
BMJ Case Rep ; 15(4)2022 Apr 04.
Artigo em Inglês | MEDLINE | ID: mdl-35379680

RESUMO

Marchiafava-Bignami disease (MBD) is a rare demyelinating condition of the corpus callosum and subcortical white matter that is most commonly seen in alcoholic patients. The course of the disease varies with symptoms that range from dementia to complete coma; severe intermittent sympathetic storming with abnormal posturing is often reported in literature. It is presumably secondary to a deficiency of B complex vitamins, specifically thiamine and many patients have clinical improvement after repletion of B vitamins. We present a case of a 35-year-old man who developed MBD secondary to polysubstance misuse without history of alcohol use. His clinical course was complicated by persistent comatose state with autonomic dysfunction. After the administration of high-dose thiamine and vitamin C and E, the patient regained consciousness and was able to follow commands within 48 hours. Furthermore, this case showed recognising brain MRI findings for MBD is a crucial step in disease identification.


Assuntos
Alcoolismo , Doença de Marchiafava-Bignami , Adulto , Alcoolismo/complicações , Coma/etiologia , Corpo Caloso/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética/efeitos adversos , Masculino , Doença de Marchiafava-Bignami/complicações , Doença de Marchiafava-Bignami/etiologia
16.
Cogn Behav Neurol ; 34(3): 226-232, 2021 09 02.
Artigo em Inglês | MEDLINE | ID: mdl-34473675

RESUMO

Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism that typically causes demyelination and necrosis of the corpus callosum. Here, we report a man with probable MBD with callosal and right medial paracentral lesions who presented with abnormal reaching behavior and ideomotor apraxia of the left hand. He exhibited difficulty in reaching with the left hand when a target object was placed on his right-hand side, and he exhibited rightward bias when using his right hand in a line bisection task. These disturbances in reaching suggest disruption of the top-down control of motor intention and spatial attention at the corpus callosum.


Assuntos
Alcoolismo , Doença de Marchiafava-Bignami , Alcoolismo/complicações , Atenção , Corpo Caloso/diagnóstico por imagem , Humanos , Intenção , Masculino , Doença de Marchiafava-Bignami/diagnóstico por imagem
17.
Actas Esp Psiquiatr ; 49(5): 228-231, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34533206

RESUMO

Substance-related disorders are the most frequent comorbidity in schizophrenia. Concretely, alcohol is the most commonly consumed substance after tobacco. Patients with schizophrenia with this comorbidity have a worse clinical course and can develop serious neuropsychiatric complications. One of them, Marchiafava-Bignami disease (MBD) can be incorrectly diagnosed as a decompensation of their mental disorder.


Assuntos
Alcoolismo , Doença de Marchiafava-Bignami , Esquizofrenia , Alcoolismo/complicações , Corpo Caloso , Humanos , Imageamento por Ressonância Magnética , Doença de Marchiafava-Bignami/complicações , Esquizofrenia/complicações
18.
Actas esp. psiquiatr ; 49(5): 228-231, septiembre 2021. ilus
Artigo em Espanhol | IBECS | ID: ibc-207667

RESUMO

Introducción: Los trastornos por abuso de sustancias sonla comorbilidad más frecuente en la esquizofrenia, siendo elalcohol, concretamente, la sustancia más frecuentementeconsumida tras el tabaco. Los pacientes con esquizofreniaque presentan esta comorbilidad presentan una peor evolución clínica y pueden desarrollar graves complicaciones neuropsiquiátricas. Una de ellas, la enfermedad de Marchiafava-Bignami (EMB), puede ser erróneamente diagnosticadacomo una descompensación del trastorno mental, conllevando graves consecuencias.Método. Se presenta el caso de un varón de 51 años,diagnosticado de esquizofrenia y trastorno por consumode alcohol. Experimentó síntomas neuropsiquiátricos agudos por los que se sospechó una descompensación de suesquizofrenia. Dada la presentación clínica atípica y susantecedentes médicos, se sospechó una patología orgánicay se realizó una prueba de imagen cerebral en la que sediagnosticó una EMB.Conclusiones. La EMB es una entidad clínica infrecuenteque debe formar parte del diagnóstico diferencial en pacientes con trastorno por consumo de alcohol que experimenten síntomas neuropsiquiátricos atípicos. (AU)


Introduction: Substance-related disorders are the mostfrequent comorbidity in schizophrenia. Concretely, alcohol isthe most commonly consumed substance after tobacco. Patients with schizophrenia with this comorbidity have a worseclinical course and can develop serious neuropsychiatriccomplications. One of them, Marchiafava-Bignami disease(MBD) can be incorrectly diagnosed as a decompensation oftheir mental disorder.Methods. A case of a 51-year-old man, diagnosed withschizophrenia and alcohol use disorder is presented. He experienced acute neuropsychiatric symptoms for which schizophrenia decompensation was suspected. Based on his atypical symptoms and medical history, a brain imaging test wasperformed and MBD was diagnosed.Conclusions. MBD is an infrequent clinical entity thatshould be part of differential diagnosis in patients with alcohol abuse disorder experiencing atypical neuropsychiatricsymptoms. (AU)


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Alcoolismo/complicações , Corpo Caloso , Imagem por Ressonância Magnética de Flúor-19 , Doença de Marchiafava-Bignami/complicações , Esquizofrenia/complicações , Pacientes
20.
Acta Radiol ; 62(7): 904-908, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32718180

RESUMO

BACKGROUND: The acute onset of Marchiafava-Bignami disease (MBD) is difficult to capture, and its clinical manifestations are overlapped. Magnetic resonance imaging (MRI) is very useful in the diagnosis of acute MBD. PURPOSE: To investigate the MRI features and clinical outcomes of acute MBD. MATERIALS AND METHODS: Sagittal T2-weighted (T2W) or T1-weighted (T1W) imaging, axial T1W and T2W imaging, and axial FLAIR and diffusion-weighted imaging (DWI) sequences were performed in 17 patients with acute MBD on 1.5-T MR. According to the different ranges of callosal restricted diffusion, MBD was divided into Type I (n = 7, the completely involved), Type II (n = 5, the mostly involved), and Type III (n = 5, the partly involved). The MRI findings and outcomes of each type were retrospectively analyzed. RESULTS: With the reduced range of the callosal restricted diffusion, the callosal atrophy or cavitation was more common: no case of Type I; 1 (20%) case of Type II; and 3 (60%) cases of Type III. With the increased range of callosal restricted diffusion, the extracallosal involvement was more common: 6 (86%) cases of Type I; 3 (60%) cases of Type II; and 1 (20%) case of Type III. During the follow-up, five cases had neuropsychiatric sequelae: 1 (14%) case of type I; 1 (20%) case of Type II; 3 (60%) cases of Type III. CONCLUSION: The MRI findings and clinical outcomes of acute MBD are regular. The extensive restricted diffusion of acute MBD may present the curable condition. Callosal heterogeneity may affect the outcome of acute MBD.


Assuntos
Imageamento por Ressonância Magnética , Doença de Marchiafava-Bignami/diagnóstico por imagem , Doença Aguda , Adulto , Idoso , Corpo Caloso/diagnóstico por imagem , Corpo Caloso/patologia , Humanos , Masculino , Doença de Marchiafava-Bignami/complicações , Doença de Marchiafava-Bignami/terapia , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos
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