A case of marchiafavabignami disease was serially evaluated with mr imaging and mr spectroscopy at 1, 2, 4, and 11 months after the onset of symptoms. The genu and the splenium had central cystic degeneration, with sparing of the peripheral layers. Marchiafavabignami disease mbd is a rare neurological disease usually associated with chronic alcoholism and characterized by demyelination and necrosis. Objective marchiafavabignami disease mbd is a rare condition mainly associated with alcoholism, although it may be mimicked by several other disorders that cause corpus callosum lesions. Marchiafavabignami disease mbd is a very rare disorder that was first. He and angelo celli were the first to elucidate living malarial parasites in human. Marchiafavabignami disease mimics motor neuron disease. Marchiafavabignami disease mbd is a rare condition characterized by demyelination of the corpus callosum. Marchiafavabignami disease typically begins in the body of the corpus callosum and later involves the genu and then splenium 2. The process may extend laterally into the neighboring white matter and occasionally as far as the subcortical regions.
Pdf we postulated that disruption of callosal pathways as occurs in. Marchiafavabignami disease is a progressive neurological disease of alcoholism, characterized by corpus callosum demyelination and necrosis and subsequent atrophy. The marchiafavabignami disease, characterized by demyelination and necrosis of the corpus. Chronic alcoholic with acute headache and disorientation. Early symptoms may include depression, paranoia, psychosis, or dementia. With ct and, especially, mri it is possible to diagnose mbd in its early stages. It is seen most often in patients with chronic alcoholism. Marchiafavabignami disease mbd is the symmetrical demyelination of. Marchiafavabignami disease mbd, a rare disorder most commonly seen in patients with a history of alcohol consumption, involves progressive demyelination and subsequent necrosis of the corpus callosum. Marchiafavabignami disease with recovery diagnosed by ct. Lesions of cns structures other than the typical demyelination of the corpus callosum are described ante mortem in a patient with mbd. This book is well suited for the medical student, neurology resident, diagnostic radiology resident, neurosurgery resident, advanced practice provider, and practicing clinician who wishes to supplement their course work, show to patients in the clinical setting, or simply broaden their knowledge of. It is diagnosed by the imaging of demyelination at the corpus callosum with mri.
Marchiafavabignami disease mbd in a nonalcoholic patient. Pdf marchiafavabignami is a rare toxic disease associated with chronic alcohol. Other brain damage occasionally reported in alcoholics includes cortical laminar sclerosis. Mri showed severe diffuse atrophy of the corpus callosum cc. How onset occurs suddenly or chronically and the range of clinical symptoms vary among affected individuals.
Clinical examination revealed bimanual coordination difficulties, left ideomotor apraxia, and tactile anomia. Marchiafavabignami disease is a rare disorder mainly seen in chronic alcoholics. The disease process typically involves the corpus callosum and clinically often presents with altered sensorium, neurocognitive defects or seizures with acute cases often deteriorating to comatose state. Pdf on may 16, 2018, hiroki matsuura and others published marchiafavabignami disease find, read and cite all the research you need on researchgate. Subacute marchiafava bignami disease includes variable degrees of mental confusion, dysarthria, behavioural abnormalities, memory deficits. Two italian pathologists, e marchiafava and a bignami, first described it in 1903. Various reversible neurologic symptoms are found in patients with mbd. Marchiafavamicheli syndrome definition of marchiafava. We report a case of mbd in a diabetic patient, without alcoholism or malnutrition, caused by a wide range of glycemic level. Marchiafava bignami disease is defined by characteristic demyelination of the corpus callosum erosion of the protective covering of nerve. Clinical medicine from the new england journal of medicine marchiafava bignami disease. Generally, the most common presentation includes personality change and psychomotor impairment.
A rare disorder, marchiafavabignami disease is defined by characteristic lesions in the corpus callosum and a variable clinical spectrum. Marchiafavabignami disease mbd is a rare neurological disorder of unknown etiology, afflicting mostly middle or elderly males with a history of chronic alcohol consumption or chronic malnutrition. This is the fifth report of the disease in a native north american with no italian ancestry. The syndrome of interhemispheric disconnection was. Dysarthria and dysphagia are found in various neurological diseases. Marchiafavabignami disease radiology reference article. Two cases with magnetic resonance imaging and positron emission tomography scan findings a nalini 1, jerry m. Marchiafavabignami disease mbd is a rare neurological disease often associated with chronic, heavy alcohol consumption and malnutrition, and is characterized by callosal lesions consisting of necrosis and demyelination 14. Marchiafavabignami disease presenting as acute psychosis. Patients can present with acute, subacute, or chronic onset of mental status change varying from lethargy to coma, seizure, ocular movement dysfunction, memory loss, and gait disturbance. The disease was first described in 1903 by the italian pathologists amico bignami and ettore marchiafava in an italian chianti drinker. Marchiafava bignami disease definition of marchiafava. Over the past few years, magnetic resonance imaging mri findings of the callosal and cortical lesions, which are critical for the diagnosis. Ettore marchiafava 3 january 1847 22 october 1935 was an italian physician, pathologist and neurologist.
Marchiafavabignami disease may present in various clinical forms 9. Marchiafavabignami disease in a nonalcoholic diabetic patient. In marchiafavabignami disease, the speed of onset and the degree of physical findings vary. Marchiafavabignami disease mbd associated with chronic alcoholism is a fatal disorder characterized by demyelination of the corpus callosum. Our objective was to identify helpful features for differential diagnosis and assess whether any treatment can be recommended. Marchiafavabignami disease is a progressive marchiafav disease of alcoholism, characterized by corpus callosum demyelination and necrosis and subsequent atrophy. His works on malaria laid down the foundation for modern malariology.
Marchiafavabignami disease mbd is a rare neurologic complication of chronic alcohol consumption that is characterized by callosal lesions involving demyelination and necrosis. It classically involves the central layers with relative sparing of the dorsal and ventral extremes which may be seen as a sandwich sign on sagittal mri imaging. A 50yearold man with a history of untreated diabetes mellitus underwent onpump beating coronary artery bypass graft surgery cabg surgery for threevessel and left. This type is also associated with symptoms of the upper motor neurons. There may ensue seizures, fluctuating hemiparesis, aphasia, abnormal movements. Mbd in nonalcoholics without malnutrition has rarely been reported. Computed tomography and magnetic resonance imaging demonstrated the typical lesion, a necrosis of the middle portion of the corpus callosum. The lesion involved the corpus callosum and hippocampal commissure but spared the anterior commissure, middle cerebellar peduncles, optic chiasm. E kovoor 2, rose dawn 2, kumar g kallur 3 1 department of neurology, national institute of mental health and neuro sciences, bangalore, india 2 department of neuroimaging and interventional radiology, national institute of mental.
Marchiafavabignami disease mbd is a rare complication of chronic alcoholism. Acute marchiafava bignami disease includes seizures, impairment of consciousness, and rapid death. Marchiafavabignamis disease, as etiologic diagnosis of athetosis. Marchiafavabignami disease mbd is a rare condition mainly associated with alcoholism, although it may be mimicked by several other. In 1903, italian pathologists marchiafava and bignami described 3 alcoholic men who died after having seizures and coma. However, with the advent of mri, more and more of these cases are being diagnosed.
Clinical and radiological features of marchiafavabignami disease. Our aims were to describe the clinicoradiological features and identify factors that may affect the prognosis of patients with mbd we examined clinical manifestations, laboratory results, and neuroradiological features. A 50yearold chronic alcoholic presented to the emergency room with history of 3 episodes of seizures 2 days earlier. The first mr imaging study showed extensive abnormal signal intensity of the corpus callosum without macroscopic changes. Hillbom m, saloheimo p, fujioka s, wszolek zk, juvela s, leone ma. Marchiafava bignami disease statpearls ncbi bookshelf. Marchiafavabignami disease mbd is a form of toxic demyelinating disease more often seen in chronic alcoholics. Marchiafavabignami disease mbd was originally described as a rare, fatal disease affecting wine drinkers. Thus, it becomes essential for a radiologist to be familiar with its imaging features as well as clinical presentation. Other articles where marchiafavabignami disease is discussed. The aetiology or pathophysiology of mbd is still unknown. Magnetic resonance imaging in marchiafavabignami syndrome. Marchiafava bignami disease an overview sciencedirect. Marchiafavabignami disease special subjects msd manual.
Marchiafavabignami disease mbd is a rare neurological disorder mostly seen in alcoholic and malnourished patients with a pathognomonic hallmark of corpus callosum demyelination. We report two male patients who had history of chronic alcoholism. Marchiafavabignami disease, syndrome of interhemispheric. A 57yearold man with a history of chronic alcoholism was evaluated for seizures. Corpus callosum fibre disruption in marchiafavabignami. Marchiafavabignami is a rare toxic disease seen mostly in chronic alcoholics that results in progressive demyelination and necrosis of the corpus callosum. Marchiafavabignami disease in a patient with no alcohol abuse. Marchiafavabignami disease mahrkeafahvah benyahme, a disorder recognized primarily by its pathologic features, consisting of demyelination of the corpus callosum and cortical laminar necrosis involving the frontal and temporal lobes.
Clinical and radiological features of marchiafavabignami. Marchiafava bignami disease is defined by characteristic demyelination of the corpus callosum erosion of the protective covering. In this autopsy, marchiafava and bignami noticed that the middle twothirds of the corpus. Marchiafava bignami disease genetic and rare diseases. Objectives to report and discuss the neuropsychological deficits and neuroimaging findings in a patient with probable marchiafavabignami disease design and method a righthanded woman with chronic alcoholism demonstrated mutism, impaired comprehension of spoken language, alexia, and righthanded agraphia. Most individuals diagnosed with marchiafava bignami disease mbd have a history of alcoholism and poor nutrition.
Occurs predominantly in those with chronic alcoholism, particularly wine drinkers. Callosal bleeding in a case of marchiafavabignami disease epa0261 an atypical presentation of marchiafavabignami disease marchiafavabignami disease. Marchiafavabignami disease mbd is a rare neurological disease usually associated with chronic alcoholism and characterized by demyelination and. We present an original case of marchiafavabignami disease in a 47yearold lefthanded alcoholic man. Because clinical signs are nonspecific, the role of computed. Marchiafava bignami disease is defined by characteristic demyelination of the corpus callosum erosion of the protective covering of nerve fibers joining the 2 hemispheres of the brain. Most reported cases have been diagnosed at autopsy. Marchiafavabignami disease was diagnosed postmortem in a 39yearold man who drank excessive amounts of white port wine. Diagnosis and management of marchiafavabignami disease. Marchiafavabignami disease is a rare disorder of demyelination and necrosis of the corpus callosum and is usually associated with alcohol abuse.
615 428 1091 181 724 852 1418 738 1240 228 550 873 436 1498 449 1452 441 1270 485 133 363 714 480 688 425 834 889 1367 523 380 548 509 479 1295 551 321 196 885 725 857 444 1128 166 1414