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 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.

Marchiafava-Bignami Disease: A Rare Clinical Dilemma.

Marchiafava-Bignami Disease (MBD) is a progressive neurological disease, characterized by corpus callosal demyelination and necrosis and subsequent atrophy. It is usually seen in the context of alcoholism and malnutrition. Clinical diagnosis of this disease is quite challenging due to various presentations but a high degree of suspicion often leads to the correct diagnosis with help of neuroimaging. We report a case of MBD with a classical clinical course and typical radiological features. This case is highlighted in order to generate awareness regarding this uncommon but historic complication of chronic alcoholism.

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.

Vegetarian diet and excessive tea consumption: a dangerous association?

Rare metabolic diseases may sometimes arise acutely and endanger human life if not immediately recognized and treated. Marchiafava Bignami disease is an uncommon neurologic disorder described in alcohol abusers and characterized by an acute severe damage of brain white matter. Even more rarely, it has been reported in non-alcohol addicted patients, but never in vegetarian people. This is a case report of a young vegetarian woman, accustomed to drink high amounts of tea, who, three weeks after her first natural childbirth, developed serious motor and cognitive disturbances. A timely brain magnetic resonance (MR) allowed us to identify Marchiafava Bignami disease and she healed few hours after the administration of parenteral steroids and vitamins. We advise to suspect Marchiafava Bignami Disease in all patients presenting with non-obvious acute generalized motor and cognitive disturbances, also if non alcoholics, and to collect the nutritional habits in all patients with suspected symptoms. In these cases a timely brain MRI is warranted, since brain imaging is typical and patients may recover after a prompt treatment.

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.

Marchiafava-Bignami Disease with frontal cortex involvement and late onset, long-lasting psychiatric symptoms: a case report.

AIMS: To describe the case and management of a patient with Marchiafava-Bignami Disease (MBD) with frontal cortical lesions, no specific symptom at first referral to the Emergency Room, and late onset of atypical psychiatric symptoms. METHODS: We report the case of a 44-year-old patient with a history of chronic alcohol abuse, eventually diagnosed with MBD. RESULTS: Magnetic Resonance showed lesions in the splenium and the body of corpus callosum and bilateral lesions of the frontal cortex. The patient showed late-onset atypical psychiatric symptoms which were drug resistant. DISCUSSION: The case we describe seems to support the existing few ones describing cortical involvement in MBD, which suggest that this is associated with a poorer prognosis. Psychiatric symptoms may be challenging to treat because of drug resistance. CONCLUSIONS: The involvement of psychiatrists together with neurologists and radiologists, with a consultation-liaison approach proved important for the achievement of diagnosis and of the most appropriate management and treatment for this patient.

[A case of Marchiafava-Bignami disease suggesting vasogenic edema].

A 61-year-old alcoholic man was admitted to our hospital because of disturbance of consciousness. He also exhibited external ophthalmoplegia, diplopia and mild rigidity, but tendon reflex was normal. On brain MRI, diffusion weighted images (DWI) and apparent diffusion coefficient (ADC) map depicted high intensity in the splenium of the corpus callosum. DWI showed high intensity, but ADC map depicted iso-intensity in bilateral precentral gyri. Marchiafava-Bignami disease (MBD) was diagnosed. After intravenous drip of vitamin, his symptoms improved rapidly and the abnormal MRI findings in the splenium of the corpus callosum and bilateral precentral gyri disappeared gradually. MBD is pathologically characterized by demyelination and necrosis in the corpus callosum, which are generally caused by cytotoxic edema. Our case suggests that vasogenic edema may occur at the early stage of the MBD.

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.

[Diagnostic imaging of marchiafava-bignami disease].

Marchiafava-Bignami disease (MBD) is a rare alchol related disorder characterized by demyelination of the corpus callosum. Clinical features include impaired consciousness, seizure, dysarthria, limb hypertonus, frontal lobe symptoms in the acute stage and interhemispheric disconnection syndromes in the chronic stage. While autopsy was historically necessary for a definitive diagnosis of MBD, imaging systems such as X-ray computed tomography and magnetic resonance imaging have enabled in vivo diagnosis. We reviewed 91 reported MBD cases additional to our 9 own cases focusing on their imaging. The corpus callosum in MBD has irreversible necrosis secondary to edema and demyelination, often complicated by bleeding in the subacute stage and it becomes atrophic in the chronic stage. The observed patterns of corpus callosal lesions were highly variable, sometimes complicated by extra-callosal lesions in the frontal cortex, white matter, and putamen. Moreover, these lesions are sometimes detected in non-alcoholic patients. Although the underlying causes of MBD are still unknown, investigating its imaging characteristics together with clinical and pathological features will contribute to elucidating its etiology. Furthermore, MBD as an alcohol related disease is linked with neurological emergency presentation. Thus, early diagnosis and treatment are essential.

Marchiafava-Bignami disease mimics motor neuron disease: case report.

BACKGROUND: Marchiafava-Bignami disease (MBD) is a rare neurologic complication of chronic alcohol consumption that is characterized by callosal lesions involving demyelination and necrosis. Various reversible neurologic symptoms are found in patients with MBD. Dysarthria and dysphagia are found in various neurological diseases. CASE PRESENTATION: We report a 51-year-old man with chronic alcoholism and malnutrition who progressively developed dysarthria and dysphagia. On admission, the patient was alert with mild cognitive dysfunction. The facial expression was flat, and there was weakness of the orbicularis oris bilaterally. The patient's speech was slurred, there was difficulty swallowing, and the gag reflex and palate elevation were poor. The jaw jerk reflex was brisk and the snout reflex was positive. Neither tongue atrophy nor fasciculation were found. Bilateral upper and lower limb weakness with increased bilateral upper limb reflexes and Babinski reflexes were found. Because he had progressive dysarthria and dysphagia with upper and lower motor neuron signs, the initial diagnosis was motor neuron disease. However, electrophysiological analysis was normal. The vitamin B1 level was 14 ng/mL (normal: >24 ng/mL), and MRI revealed hyperintense lesions in the splenium of the corpus callosum and the primary motor cortices bilaterally. After vitamin B therapy for 17 days, the neurological disorders alleviated concurrently with disappearance of the lesions on MRI, which led to the definitive diagnosis of MBD. CONCLUSIONS: MBD presenting with these lesions can mimic motor neuron disease clinically.

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.

Alcoholic ketoacidosis coincides with acute Marchiafava-Bignami disease.

Alcoholism is the major cause of electrolyte and acid-base imbalance and nutrition deficiency. Ketoacidosis is one of major advised effect on alcoholism. Marchiafava-Bignami disease, a rare alcohol-related disorder, characterized by altered mental status, seizure, and multifocal central nervous system signs, which results from progressive demyelination and necrosis of corpus callosum. Here, we presented a 53-year-old man presented as decreased conscious level and 4 extremity rigidity on admission. Alcoholic ketoacidosis coincided with acute Marchiafava-Bignami disease had been confirmed by biochemistry examination and cranial magnetic resonance imaging. Aggressive hydration with 5% dextrose in normal saline and intravenous vitamin B complex were administered. The patient's symptoms completely recovered after ketoacidosis has been corrected 2 days later.

Acute Marchiafava-Bignami disease with selective involvement of the precentral cortex and splenium: a serial magnetic resonance imaging study.

INTRODUCTION: Marchiafava-Bignami disease (MBD) is defined pathologically as callosal degeneration associated with chronic alcoholism. We report a patient with MBD with acute lesions confined in the precentral cortex and splenium. Various magnetic resonance imaging (MRI) modalities were performed sequentially to elucidate the pathophysiology. CASE REPORT: A 52-year-old man with chronic alcoholism developed acute confusion and dysarthria. He improved incompletely with nutritional supplementation. Diffusion-weighted imaging MRI disclosed the presence of reversible lesions with low apparent diffusion coefficient values in the precentral cortex and splenium. Perfusion-weighted imaging revealed that the cerebral blood volume and flow decreased and subsequently increased, and the mean transit time and time to peak were persistently prolonged. On magnetic resonance spectroscopy, choline was initially normal and became elevated during the recovery period, whereas N-acetylaspartate was low initially and after the resolution of the lesion on conventional MRI. CONCLUSIONS: The precentral cortex and splenium are the most vulnerable areas in acute MBD. The lesions have reversible restricted diffusivity and hypoperfusion. The magnetic resonance spectroscopy findings correlate well with the clinical status.