COVID-19-induced acute loss of consciousness in Marchiafava-Bignami disease: a case report.

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.

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 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.

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.

Thiamine deficiency, oxidative metabolic pathways and ethanol-induced neurotoxicity: how poor nutrition contributes to the alcoholic syndrome, as Marchiafava-Bignami disease.

Ethanol is an important risk factor for the occurrence of several brain disorders that depend on the amount, period and frequency of its consumption. Chronic use of ethanol often leads to the development of neurodegenerative syndromes, which cause morphological and functional impairments such as foetal alcohol syndrome in newborns exposed to ethanol during pregnancy, Wernicke-Korsakoff Syndrome and, more rarely, Marchiafava-Bignami disease (MBD). MBD is characterized by primary degeneration of the corpus callosum, without inflammation and is associated with oxidative stress and hypovitaminosis, as well as altered mental status, to mention dementia, seizures, depression and so on. This review discusses MBD and poor nutrition as a risk factor for the development of such alcoholic syndrome, with focus on diagnosis, pathogenic aspects, signs and symptoms, as well as therapeutic perspectives. On the basis of the inclusion/exclusion criteria adopted, the performed search in scientific databases (Pubmed, Scielo and Google Scholar) resulted in 100 studies that are being presented and discussed in the present work. Review, case-control and cohort studies on alcoholism-associated hypovitaminosis, oxidative stress, MBD and ethanol metabolism pathways were admitted as relevant. We highlight that MBD is a poorly described, diagnosed, insidious and progressive condition, for which evidence suggests a synergism between ethanol-induced neurotoxic effects and hypovitaminosis B. Present treatment consists of vitamin B1(thiamine) supplementation. Nonetheless, other strategies such as the inclusion of antidepressants or steroidal anti-inflammatories as add-on therapies have been employed as an attempt to improve the damage. Indeed, both the diagnosis and treatment are difficult, and death occurs within few years.

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.

Altered Sensorium in a Young Male: Marchiafava Bignami Disease Revisited.

Marchiafava Bignami disease (MBD) is a rare and devastating complication of chronic alcoholism. Degeneration of the corpus callosum is the hallmark feature of MBD. Early diagnosis of MBD by its typical "Sandwich Sign" on magnetic resonance imaging (MRI). Prompt institution of treatment and strict alcohol abstinence can cause regression of changes and hence, clinical improvement. Here we report the case of a young chronic alcoholic male admitted with altered sensorium and his further course in ward.

Atrophy of the corpus callosum in heavy alcoholic patients

Atrophy of the corpus callosum among alcoholics was classically restricted to patients affected by Marchiafava-Bignami (MB) disease. It was further observed in patients with thiamine and/or niacin deficiency, or in alcoholics who had consumed alcoholic beverages for a long period. A 42-year-old alcoholic patient was admitted with a full-blown alcohol withdrawal syndrome. After recovery, unstable gait and marked pyramidal signs were observed. A brain magnetic resonance was performed, which revealed corpus callosum atrophy. At discharge the patient was placed under ambulatory care. Nevertheless, he never attended his appointments and he was readmitted several times with withdrawal syndrome. Repeated MRI studies showed no remarkable changes besides progressive atrophy of the corpus callosum. Indeed, the area of corpus callosum was markedly reduced when compared with that of 20 alcoholics and 5 further patients with Wernicke´s encephalopathy. Therefore, the clinical picture is consistent with classic MB disease, and the more severe atrophy than that observed in the remaining alcoholics suggests that additional mechanisms may play a role in MB disease

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[Marchiafava-Bignami disease (Case-report)]. / Marchiafava-Bignami-betegség (Egy 57 éves nobeteg esetének ismertetése).

Marchiafava-Bignami disease (MBD) is caused by damage of the corpus callosum. There are acute, subacute and chronic forms, it occurs most frequently among alcoholic patients. A variety of neurological symptoms, epileptic seizures, and coma may be associated with the disease, but the chronic form may start with acute confusion and dementia, interhemispherial disconnection syndrome or with slow progressive changes in behavior. In 2001, only 250 cases were reported, of which 200 died, 30 cases contributed to severe dementia or bed rest, and favorable outcome occured in only 20 cases. The MBD diagnosis of our patient was based on the anamnesis and cranial MRI and the treatment consisted of administration of B vitamin complex, folic acid, memantine, piracetam and haloperidol. Reviewing the international literature currently recommended therapeutic options are thiamin and folic acid. According to some authors the immediate administration of thiamine affects the outcome of the disease, and there are case reports of beneficial effects of amantadine and steroids.

Neurologic Manifestations of Chronic Liver Disease and Liver Cirrhosis.

The normal functioning of brain is intimately as well as intricately interrelated with normal functioning of the liver. Liver plays a critical role of not only providing vital nutrients to the brain but also of detoxifying the splanchnic blood. Compromised liver function leads to insufficient detoxification thus allowing neurotoxins (such as ammonia, manganese, and other chemicals) to enter the cerebral circulation. In addition, portosystemic shunts, which are common accompaniments of advanced liver disease, facilitate free passage of neurotoxins into the cerebral circulation. The problem is compounded further by additional variables such as gastrointestinal tract bleeding, malnutrition, and concurrent renal failure, which are often associated with liver cirrhosis. Neurologic damage in chronic liver disease and liver cirrhosis seems to be multifactorial primarily attributable to the following: brain accumulation of ammonia, manganese, and lactate; altered permeability of the blood-brain barrier; recruitment of monocytes after microglial activation; and neuroinflammation, that is, direct effects of circulating systemic proinflammatory cytokines such as tumor necrosis factor, IL-1ß, and IL-6. Radiologist should be aware of the conundrum of neurologic complications that can be encountered in liver disease, which include hepatic encephalopathy, hepatocerebral degeneration, hepatic myelopathy, cirrhosis-related parkinsonism, cerebral infections, hemorrhage, and osmotic demyelination. In addition, neurologic complications can be exclusive to certain disorders, for example, Wilson disease, alcoholism (Wernicke encephalopathy, alcoholic cerebellar degeneration, Marchiafava-Bignami disease, etc). Radiologist should be aware of their varied clinical presentation and radiological appearances as the diagnosis is not always straightforward.

Enfermedad de Marchiafava-Bignami / Marchiafava-Bignami disease

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