Marchiafava-Bignami disease(MBD) involving bilateral handknob area: neuroimages.

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.

From Mild Gait Difficulties to a Sudden Coma: A Rare Case of Marchiafava-Bignami Disease.

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.

Marchiafava-Bignami disease presenting as reversible coma.

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.

Marchiafava-Bignami disease in a patient with schizophrenia and alcohol use disorder.

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.

NOTA CLÍNICA: Enfermedad deMarchiafava-Bignami en un paciente con esquizofrenia y trastorno por uso de alcohol / CLINICAL NOTE: Marchiafava-Bignami disease in a patient with schizophrenia and alcohol use disorder

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)

Comprehensive neuropsychological findings in a case of Marchiafava-Bignami disease.

OBJECTIVE: Marchiafava-Bignami disease (MBD) is a rare complication associated with chronic heavy alcohol use, with case reports documenting a range of cognitive outcomes. Given the variability in MBD presentation and outcomes, milder cases may remain undiagnosed and few studies or case reports have presented a comprehensive neuropsychological profile of these patients. The objective of this case study was to describe the neuropsychological presentation and findings of a case of likely MBD. METHOD: The patient was a 46-year-old, African American female with a complex history of malnutrition and alcohol abuse presenting for outpatient neuropsychological evaluation. She was administered a comprehensive battery of neuropsychological tests as part of routine clinical care. RESULTS: Neuropsychological data demonstrated severe deficits in executive functions, complex visuoconstruction, and motor dexterity, as well as an amnestic verbal and visual memory pattern. CONCLUSIONS: Overall, data and the patient's initial presentation of acute behavioral change were consistent with some reports of cognitive and behavioral sequela of MBD. Additionally, the patient's history of chronic poor nutritional intake with exacerbation from chronic heavy alcohol use, and imaging findings of severe cerebral/corpus callosum white matter loss and bilateral frontoparietal atrophy, were highly suggestive of MBD.

Diverse MRI findings and clinical outcomes of acute Marchiafava-Bignami disease.

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.

Rare case of Marchiafava-Bignami disease due to thiamine deficiency and malnutrition.

Marchiafava-Bignami disease (MBD) is a rare, toxic demyelinating disorder of the central nervous system associated with chronic alcoholism and malnutrition. The clinical presentation is varied and non-specific, including symptoms of acute dementia, impaired consciousness, dysarthria, hemiparesis, pyramidal tract signs, seizure activity, ataxia and signs of interhemispheric disconnection. The differential diagnosis of MBD may include Wernicke's encephalopathy, multiple sclerosis, encephalitis, infectious or paraneoplastic leucoencephalopathy, infarction, Alzheimer's disease, multi-infarct dementia and frontotemporal lobar degeneration (Pick) disease. The diagnosis of MBD is dependent on MRI findings of hyperintensity of the corpus callosum on T2 and fluid-attenuated inversion recovery T2 sequences, with or without extracallosal lesions. The use of MRI in diagnosis has allowed for early initiation of treatment with parenteral thiamine, and improved the prognosis of MBD from frequently fatal to a mortality of less than 8%. Administration of thiamine within 14 days of symptom onset has demonstrated statistically better outcomes over delayed treatment. We present a case report of MBD diagnosed in a 72-year-old woman who presented with ataxia and slurred speech, in an effort to highlight the importance of obtaining MRI in patients presenting with behavioural disturbance and neurological findings, as well as discuss the relationship between thiamine supplementation and demyelinating diseases in the central nervous system.

Marchiafava-Bignami disease: Report of three cases.

Marchiafava-Bignami disease (MBD) is a rare alcohol-associated disorder characterized by demyelination and necrosis of the corpus callosum. We report three patients who had history of chronic alcoholism, different clinical presentation and MRI findings consistent with the diagnosis of Marchiafava-Bignami disease.

Intermittent alien hand syndrome caused by Marchiafava-Bignami disease: A case report.

RATIONALE: Alien Hand syndrome (AHS) is characterized in most patients by seemingly purposeful, involuntary movements of the extremities. It is not well known among physicians on account of its diverse clinical manifestations. PATIENT CONCERNS: We present a 57-year-old Chinese man who could not stop or turn himself around as he involuntarily and uncontrollably walked forward, which had happened frequently in the month prior to treatment. He had been a heavy drinker for thirty years before the onset of the disease, with an alcohol intake of 600 to 800 ml/day. DIAGNOSES: History of alcohol intake and the brain magnetic resonance imaging findings indicated a diagnosis of Marchiafava-Bignami disease. The patient was additionally diagnosed with Alien Hand Syndrome according to his clinical symptoms. INTERVENTIONS: The patient was treated with high doses of vitamin B for 1 month. OUTCOMES: The patient's abnormal behaviors never appeared during the treatment, and no instance of recurrence was observed during the 6 months of follow-up. LESSONS: The clinical manifestation of AHS is non-specific. Only by considering its diverse manifestation can doctors better understand the disease and achieve early intervention.

Left hemispatial neglect with a splenial lesion.

With injury of the anterior two-thirds of the corpus callosum, each hemisphere's attentional bias to contralateral hemispace becomes manifest with each hand deviating ipsilaterally during line bisection tasks. Patients with infarctions in the right posterior cerebral artery distribution with occipital and splenial damage can also exhibit spatial neglect. The goal of this report is to learn the role of the splenium of the corpus callosum in mediating visuospatial attention. A right-handed woman with Marchiafava-Bignami disease and damage to the splenium of her corpus callosum without evidence of a mesial frontal, parietal, or occipital injury was assessed for spatial neglect with line bisections. When bisecting lines in her left hemispace with her right hand, she deviated to the right, but revealed no major deviations when the line was place in the midline, in right hemispace, or when bisecting lines with her left hand. This patient provides evidence that damage to the splenium can induce a special form of asymmetrical spatial neglect. This asymmetry might be related to the disconnected right hemisphere's ability to allocate attention to both right and left hemispaces with the disconnected left hemisphere's ability to allocate attention to the right but not left hemispace.

Marchiafava-Bignami Disease with Cortical Involvement.

BACKGROUND: Marchiafava-Bignami disease (MBD) is a neurological degenerative disorder with a pathognomonic hallmark of symmetric demyelination in the corpus callosum (CC). Most reported cases were chronic alcoholics and some showed cortical lesions related to poor clinical prognosis. Herein we report a case of a chronic alcoholic who presented with confusion and generalized weakness. METHODS: Nerve fiber integrity and metabolic changes were evaluated with Magnetic resonance imaging (MRI) sequences including diffusion tensor imaging (DTI) and MRS. RESULTS: MRI revealed the typical callosal lesions of MBD with bilateral frontoparietal cortical lesions. DTI and MRS showed both impaired myelin integrity and axonal density in the CC. The cortical lesions partly disappeared after intravenous administration of high-dose multivitamins and corticosteroids. The patient regained consciousness 3 months later while dysarthria and quadriplegia persisted. Three years later, the patient can interact occasionally with people and the functional activities of both upper and lower limbs have no improvement. CONCLUSIONS: To our knowledge, this is the first report of DTI together with MRS assisting in evaluating the prognosis of MBD.

Unilateral Apraxic Agraphia without Ideomotor Apraxia from a callosal lesion in a patient with Marchiafava-Bignami disease.

Apraxic agraphia can be caused by left hemispheric cerebral lesions in the area that contains the spatial representations of the movements required to write, from a lesion in, or connections to, the frontal premotor cortex that converts these spatial representations to motor programs (Exner's area).  A right-handed woman with Marchiafava Bignami disease and lesions of the genu and splenium of her corpus callosum had apraxic agraphia without ideomotor apraxia of her left. A disconnection of Exner's area in the left hemisphere from the right hemisphere's premotor and motor areas may have led to her inability to write with her left hand.

Simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis: A case report of a challenging diagnosis.

RATIONALE: Marchiafava-Bignami disease (MBD) is a rare disease characterized by demyelination of the corpus callosum. It is most commonly seen in patients with chronic alcoholism. The clinical diagnosis of MBD can be difficult due to its nonspecific manifestation. Central pontine myelinolysis (CPM) occurs mostly as a complication of severe and prolonged hyponatremia, especially when corrected too rapidly. However, CPM can be associated with chronic alcoholism and its clinical presentation can be heterogeneous. Because both MBD and CPM can have fatal outcomes, early recognition and treatment can result in a better prognosis. We present a very rare case of simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis in a patient with chronic alcoholism who was diagnosed unexpectedly using brain magnetic resonance imaging and improved after proper treatment. PATIENT CONCERNS: We presented a case of a 39-year-old patient who visited the hospital with general weakness and an altered neurologic condition after a week of vomiting. DIAGNOSIS: The patient was diagnosed with simultaneous acute Marchiafava-Bignami disease and central pontine myelinolysis using brain magnetic resonance imaging. INTERVENTION: Administration of a high dose of thiamine. OUTCOMES: The neurologic signs improved after a week of thiamine administration. LESSONS: This case suggests that Marchiafava-Bignami disease and central pontine myelinolysis might have a common pathogenesis, and brain magnetic resonance imaging is of crucial importance in chronic alcoholic patients presenting with nonspecific neurological deterioration. The appropriate administration of thiamine may prevent poor outcomes.

Manifestaciones neurológicas del alcoholism / Neurological manifestations of excessive alcohol consumption

En este artículo se revisan las distintas manifestaciones neurológicas del consumo excesivo de alcohol, que pueden ser agudas o crónicas y afectar al sistema nervioso central o periférico. El mecanismo por el cual se producen varía de un grupo de trastornos a otro. Destacan factores nutricionales, efectos tóxicos del alcohol, factores metabólicos e incluso inmunológicos. Estas manifestaciones pueden conllevar una gran morbilidad y un aumento significativo de la mortalidad, por lo que es importante reconocerlas y tratarlas precozmente (AU)

This article reviews the different acute and chronic neurological manifestations of excessive alcohol consumption that affect the central or peripheral nervous system. Several mechanisms can be implicated depending on the disorder, ranging from nutritional factors, alcohol-related toxicity, metabolic changes and immune-mediated mechanisms. Recognition and early treatment of these manifestations is essential given their association with high morbidity and significantly increased mortality (AU)

El cerebro adicto: imagen de las complicaciones neurológicas por el consumo de drogas / The addicted brain: imaging neurological complications of recreational drug abuse

Las drogas constituyen un gran problema sociosanitario. Tradicionalmente, la neuroimagen ha tenido un papel secundario limitado a la detección de eventos vasculares agudos. En la actualidad, el radiólogo ha adquirido gran relevancia en el diagnóstico de las complicaciones neurológicas agudas y crónicas, debido al avance en el conocimiento de la enfermedad y al desarrollo de las técnicas de imagen morfológicas y funcionales. Las principales complicaciones son la patología neurovascular, la infección, los trastornos tóxico-metabólicos y la atrofia cerebral. La sintomatología inespecífica y la negación del consumo hacen que la implicación del radiólogo pueda resultar fundamental en la atención de estos pacientes. La neuroimagen permite detectar alteraciones precoces y plantear el diagnóstico etiológico ante patrones de afectación específicos. Nuestro objetivo es describir el patrón de consumo y el mecanismo fisiopatológico de las drogas con mayor repercusión neurológica, así como ilustrar las complicaciones cerebrales agudas y crónicas mediante técnicas de imagen convencional y funcional (AU)

Recreational drug abuse represents a serious public health problem. Neuroimaging traditionally played a secondary role in this scenario, where it was limited to detecting acute vascular events. However, thanks to advances in knowledge about disease and in morphological and functional imaging techniques, radiologists have now become very important in the diagnosis of acute and chronic neurological complications of recreational drug abuse. The main complications are neurovascular disease, infection, toxicometabolic disorders, and brain atrophy. The nonspecific symptoms and denial of abuse make the radiologist's involvement fundamental in the management of these patients. Neuroimaging makes it possible to detect early changes and to suggest an etiological diagnosis in cases with specific patterns of involvement. We aim to describe the pattern of abuse and the pathophysiological mechanisms of the drugs with the greatest neurological repercussions as well as to illustrate the depiction of the acute and chronic cerebral complications on conventional and functional imaging techniques (AU)

Partial interhemispheric disconnection syndrome (P-IHDS) secondary to Marchiafava-Bignami disease type B (MBD-B).

A 53-year-old man with a 35-year history of excessive alcohol intake presents to our neurology department with 4-year history of progressive neurocognitive deterioration and disconnection syndrome. MRI head demonstrates extensive demyelination of the corpus callosum (and of extracallosal sites as well), leading to a diagnosis of Marchiafava-Bignami disease. He was given treatment with vitamin B complex (including folate) and was assessed and managed by psychology, occupational therapy and physiotherapy with initial signs of improvement.