Marchiafava-Bignami Disease Research Articles

Alcoholic and Non-Alcoholic Marchiafava-Bignami Disease: A Comparative Study

Purpose: Marchiafava-Bignami Disease (MBD) is a rare condition mainly associated with alcoholism, although a few non-alcoholic cases have been reported. We performed a comparative study of demographic and clinical differences between alcoholic and non-alcoholic and assessed whether any treatment can be recommended. Methods: We reviewed 157 reports containing data on 168 subjects with Alcoholic MBD (AMBD) and 23 subjects with Non-Alcoholic MBD (NAMBD). The following data were extracted: demographic characteristics; delay from the onset of symptoms to admission; MRI features; location of the corpus callosum lesions; the presence of Wernicke’s disease; drug treatment (thiamine, other vitamins and steroids); outcome. Results: The subjects with AMBD were more frequently men (84.5% vs 47.8, P = 0.000); the ones with AMBD were frequently reported as suffering from malnutrition (81.3% vs 50%, P = 0.019), whereas the NAMBD was frequently reported as suffering from diabetes mellitus (30.4% vs 7.1%, P = 0.002). The lesions in the NAMBD are often located in the splenium (47.8%), whereas single splenial lesions are seen only in 18.7% of the AMBD. 43.5% (10/23) of the NAMBD was reported to have recovered completely, whereas only 15.4% (24/156) AMBD showed a complete recovery. Conclusions: It is important to diagnose NAMBD in the early stage with MRI and to treat the symptoms with thiamine and/or corticosteroids according to aetiology. We recommend prompt treatment of MBD with parenteral thiamine in subjects associated with malnutrition, anorexia nervosa and prolonged vomiting, and reduction of food intake. Corticosteroids may aid in recovery by reducing oedema in subjects associated with diabetes mellitus.

Marchiafava-Bignami Disease: A Less Known Neurological Complication of Alcoholism

Marchiafava-Bignami disease (MBD) is a disorder associated with chronic alcohol consumption and characterized by demyelination and necrosis of the corpus callosum. Patients may have reduced consciousness, psychotic symptoms, apathy, hemiparesis, ataxia and coma. Although some cases of recovery have been reported, the outcome is poor, with disability or dead. We reported a case of a 56-year-old woman with chronic alcoholism admitted to our hospital with impaired consciousness and a history of gait disturbance. On examination, she was in a coma, with increased muscle tone and left arm paresis. Laboratory studies showed a folic acid deficiency and the CT scan was normal. Magnetic resonance imaging revealed lesions in periventricular white matter and the entire corpus callosum with restriction of water diffusion consistent with the diagnosis of MBD. Despite treatment with B vitamin complex, the patient died. Coma and radiological involvement of the entire corpus callosum are compatible with type A MBD and associated with poor prognosis. Although being a rare alcoholism complication, it should not be forgotten since an early diagnosis and prompt supportive therapy may enable a better outcome. J Med Cases. 2016;7(5):171-173 doi: http://dx.doi.org/10.14740/jmc2477w

Marchiafava-Bignami and Alcohol Related Acute Polyneuropathy: The Cooccurrence of Two Rare Entities.

Objectives. The aim of this article is to represent the first reported case with cooccurrence of two rare alcohol related complications. Case Report. We report a 38-year-old man with chronic alcoholism who presented with both cranial and peripheral nerve palsy. On MRI examination characteristic findings of Marchiafava-Bignami disease were recognized. Discussion. Marchiafava-Bignami disease (MBD) is a rare complication of long-term, heavy alcohol abuse that has characteristic MRI findings. Acute alcohol related polyneuropathy (AARP) is another rare and not-well-understood complication of chronic alcohol abuse. We could not find any previous report of the cooccurrence of these two complications in the literature.

Marchiafava-Bignami disease: a case report

Marchiafava-Bignami disease: a case report

Rapid Recovery from Marchiafava-Bignami Disease after Intravenous Corticosteroid Administration

Background: Marchiafava-Bignami disease (MBD) is a rare disorder characterized by acute demyelination of the corpus callosum in people with chronic alcoholism. Early thiamine treatment can reduce neurological complications of MBD. However, the outcome of MBD is usually unfavorable. Here we report a case of MBD responded to intravenous corticosteroid administration. Case Report: 46-year-old woman with chronic alcoholism presented to our emergency department with two days of dysarthria, dysphagia, extremity weakness, and decreased consciousness. Diffusion-weighted image and fluid attenuated inversion recovery revealed high signal intensities of the left frontal cortex and the entire corpus callosum. After receiving high dose intravenous thiamine and multivitamin, she did not improve. At the third day of admission, intravenous steroid was started. Her mental status and weakness were rapidly improved. At 2 weeks later, she could walk independently. Follow-up MRI revealed only a small lesion in the splenium of corpus callosum. Conclusion: The MBD in our case is associated with thiamine deficiency with main pathology of edema and demyelination of corpus callosum. Corticosteroid stabilized blood brain barrier and reduced inflammation and edema. Therefore, corticosteroid therapy might have beneficial effects as an alternative therapy for MBD. Key Words: Marchiafava-Bignami disease; Corticosteroid; Extracallosal involvement

Marchiafava–Bignami disease – An alcohol related medical emergency

We report a case of Marchiafava–Bignami disease, an alcohol related neurological emergency.

The Etiology of Cortical Lesions in Marchiafava-Bignami Disease is Nicotinic Acid Deficiency: A Case Report and Review of the Literature (P6.024)

The Etiology of Cortical Lesions in Marchiafava-Bignami Disease is Nicotinic Acid Deficiency: A Case Report and Review of the Literature (P6.024)

Investigating the symptoms of white matter disorders

In this presentation, I will examine the intricate systemic connections in the white matter and the disturbances that occur due to diseases. In particular, I will discuss Marchiafava-Bignami disease, leukodystrophy with neuroaxonal spheroids, and myotonic dystrophy. Investigation of all three diseases reveals the crosstalk essential for the healthy brain and the resulting dysfunctions that follow lesions of different etiologies.

Bifrontal cortical lesions in Marchiafava–Bignami disease

Bifrontal cortical lesions in Marchiafava–Bignami disease

Marchiafava-Bignami disease with rare etiology: A case report.

A male, 62-year-old patient was admitted to hospital due to dizziness and gait disturbance for 10 days. The patient had fallen a few times due to the gait instability, which was associated with stiffness and memory loss. The patient had undergone cardiac carcinoma surgery three years previously and had no drinking history. Physical examination revealed that the patient was lucid when conscious but exhibited slurred speech, apathy and cognitive impairment. The finger-to-nose and rapid alternating movement tests showed the patient to be slightly clumsy. Magnetic resonance imaging revealed symmetric abnormal signals in the splenium of the corpus callosum, and the diagnosis was Marchiafava-Bignami disease (MBD). The patient recovered following the administration of vitamin B and other treatments. The patient had long-term appetite loss. A brain myelin metabolism disorder caused by long-term malnutrition and leading to demyelinating changes in the brain may have been the cause of the MBD of this patient. Clinicians should increase awareness of this disease and should not ignore the diagnosis of it, even when the patient lacks a drinking history. Early diagnosis and treatment can improve the prognosis of the patient.

Marchiafava-Bignami disease with asymmetric extracallosal lesions.

Marchiafava-Bignami disease (MBD) is a rare disease in patients with chronic alcoholism and is characterized by symmetric demyelination and necrosis of the corpus callosum. Clinical features include neuropsychiatric disorders, dysarthria, tetraparesis, astasia-abasia, impaired consciousness and symptoms of interhemispheric disconnection. The lesions in MBD are not restricted to the corpus callosum, but also occur in extracallosal regions. However, no case of MBD with extracallosal lesions confined to one side of the brain has been reported. We present a patient with MBD showing asymmetric extracallosal lesions and complete resolution of magnetic resonance imaging (MRI) abnormalities.

Akut Marchiafava-Bignami Hastalığı Olan Bir Hastada Manyetik Rezonans Görüntüleme Bulguları

Marchiafava-Bignami disease or syndrome (MBD) is a rare disorder that all cases had a history of chronic alcohol abuse. It is thought to be due to vitamin B complex deficiency. All cases had a symmetrical lesions in the corpus callosum and causes progressive demyelination and necrosis. It can spread to white matter of the neighboring tissues and rarely to the subcortical areas. Clinical symptoms include not only dysarthria, ataxia, tetraparesis, disphagia, dementia as a clinical outcome, cognitive deficits but also disturbed consciousness, seizure and coma. Some of them show asymmetrical hypointense areas in multiple regions on susceptibility-weighted imaging. Here, we report a 25-year-old man had progressive ataxia, dysarthria, and disphagia. This is a case of MBD that was diagnosed by magnetic resonance imaging (MRI).

Marchiafava-Bignami Disease (MBD): Rare Diagnosis With Poor Outcome in Alcoholic Cirrhosis

Marchiafava-Bignami Disease (MBD): Rare Diagnosis With Poor Outcome in Alcoholic Cirrhosis

Marchiafava-Bignami disease

A 57-year-old man with a history of chronic alcoholism was evaluated for seizures. MRI showed severe diffuse atrophy of the corpus callosum (CC). Clinical examination revealed bimanual coordination difficulties, left ideomotor apraxia, and tactile anomia. The genu and the splenium had central cystic degeneration, with sparing of the peripheral layers. Tractography showed the lack of central white matter tracts, an “acquired callosotomy” (figure). The imaging findings were compatible with the chronic phase of Marchiafava-Bignami disease, a rare demyelinating disorder, primarily affecting the central CC layers, thought to be due to chronic alcohol consumption.1,2

Poster 310 Proposing a Role for Inpatient Rehabilitation of Marchiafava-Bignami disease: A Case Report

Poster 310 Proposing a Role for Inpatient Rehabilitation of Marchiafava-Bignami disease: A Case Report

Lesions of the Corpus Callosum and Other Commissural Fibers: Diffusion Tensor Studies

The corpus callosum is the largest white matter tract in the brain, connecting the 2 hemispheres. The functions of the corpus callosum are many and varied, and lesions frequently cause only subtle clinical findings. The range of diseases that can affect the corpus callosum is vast and includes all potential white matter disease. The distribution of lesions in the corpus callosum is disease specific in only a few entities such as Susac syndrome and Marchiafava-Bignami disease. Group studies have found significant differences of diffusivity metrics in the corpus callosum in preterm infants, patients suffering seizure activity, and patients with early-onset Alzheimer's disease. Given the challenges that multiple orientation of fibers within the callosum presents, advanced postprocessing methods may be required to reveal ultrastructural disease.

Marchiafava Bignami Disease; an uncommon cause of confusion in a long-term alcohol abuser

The Galle Medical Journal is published by the Galle Medical Association. The journal is published quarterly in March, June, September and December each year. The submissions are accepted throughout the year. The aims of the Journal are to foster co-operation among the medical fraternity and to be a forum to make literary contributions, share experiences encountered in medical practice, update their knowledge and have debates on topics related to all aspects of medicine.

Neuropathology of Chronic Alcohol Abuse

Alcohol abuse and dependence are serious public health problems worldwide, resulting in significant morbidity, mortality, and legal implications. Therefore, sudden unexpected, suspicious, or violent death in alcohol consuming persons are frequently seen in the forensic setting. Alcohol-induced brain damage is predominantly caused by nutritional deficiencies and repeated withdrawal syndrome. Brain lesions associated with heavy alcohol consumption include brain atrophy, hepatic encephalopathy, and central pontine myelinolysis. In addition, the dementing conditions Wernicke-Korsakoff syndrome, acquired hepatocerebral degeneration, Marchiafava-Bignami disease, and pellagrous encephalopathy are closely associated with chronic alcohol consumption. In neuroradiological and autopsy studies, cerebral and cerebellar atrophy has been demonstrated in alcoholics. There is a regional selectivity, with the frontal lobes being particularly affected. Moreover, a disproportionate loss of cerebral white matter relative to cerebral cortex suggests that a major neurotoxic effect of chronic alcohol consumption predominantly affects the white matter.

Wernicke Encephalopathy and Ethanol-Related Syndromes

Ethanol causes diverse neurologic conditions caused by acute and chronic brain damage. This review provides an overview of Wernicke encephalopathy and other ethanol-related brain changes, such as chronic brain atrophy, Marchiafava-Bignami disease, osmotic demyelination syndrome, chronic hepatic encephalopathy, and acute alcohol withdrawal. As clinical symptoms of this spectrum of diseases have nonspecific neurologic alterations, radiologists should have current radiologic information and understand the imaging findings pertaining to the pathophysiology to support diagnosis.

Neurological complications of chronic alcoholism: study of 42 observations in Guinea

Neurologic disorders related to chronic alcoholism in traditional areas of Guinea are frequent, but reports about them are rare. We conducted the first study in Guinea on this subject and retrospectively collected 42 cases of neurologic manifestations related to alcoholism over a 7-year period. The standard findings of the literature were confirmed in our population: peak frequency after the age of 40 years (82.8%) and clear male overrepresentation (M/F sex ratio: 13/1). All the standard signs and symptoms are reported, with a clear predominance of alcoholic polyneuropathy and hepatic encephalopathy. The study of nutritional status by both body mass index (BMI) and the Detsky criteria showed that these patients were severely malnourished. The brain MRI was a crucial contribution for diagnosing the standard central nervous system complications of alcoholism: Gayet Wernicke encephalopathy, Marchiafava-Bignami disease, Korsakoff syndrome, central pontine myelinolysis, and cerebellar degeneration.