Structural mri studies have shown that subcortical and brainstem structures known to be affected in severe neurological syndromes such as marchiafava bignami disease, 5 central pontine myelinolysis, alcoholic cerebellar degeneration, and korsakoffs syndrome, which are all associated with excessive alcohol consumption or associated nutritional deficiency, also are affected in patients with. Marchiafavabignami disease mbd was originally described as a rare, fatal disease affecting wine drinkers. Dec 05, 2012 marchiafava bignami disease mbd is characterized by primary degeneration of the corpus callosum associated with chronic alcohol consumption. Only about 200 cases have been reported in the medical literature. Marchiafava bignami disease typically begins in the body of the corpus callosum and later involves the genu and then splenium 2. Pontes cm, neurology department, hospital federal dos servidores do estado, rua sacadura cabral n. Wernickes syndrome korsakoffs amnestic syndrome cerebellar degeneration stroke hemorrhagic, ischemic marchiafavabignami corpus callosum confusion, language, dementia, seizures breast cancer depression. It is characterized pathologically by demyelination and necrosis of the corpus callosum.
We report a 51yearold man with chronic alcoholism and malnutrition. Background and purpose rapidly progressive dementia rpd is an emergency in cognitive neurology, defined as cognitive impairment affecting the daily living activities developed over less than 1 year. 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. Other articles where marchiafavabignami disease is discussed.
Thus, it becomes essential for a radiologist to be familiar with its imaging features as well as clinical presentation. A nonalcoholic japanese patient with wernickes encephalopathy and marchiafavabignami disease. Marchiafavabignami disease a progressive neurological syndrome is a rare condition seen in chronic alcoholics. Some patients present to the hospital with sudden onset of stupor or coma, and some present with seizures. Transcranial magnetic stimulation shows impaired transcallosal inhibition in marchiafava bignami syndrome. In some diseases, cc involvement is typical and sometimes isolated, while in other diseases cc lesions are seen only occasionally in the presence of other typical extracallosal abnormalities. It is seen most often in patients with chronic alcoholism. Paulo eduardo mestrinelli carrilho1 marchiafavabignami.
Dysarthria and dysphagia are found in various neurological diseases. Oct 12, 2016 marchiafava bignami disease mbd is a rare condition characterized by demyelination of the corpus callosum. Magnetic resonance imaging in marchiafavabignami syndrome. 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. Ppiinniioonn1440 north chase street athens, ga 30601. From research to practice richard saitz md, mph, facp, fasam. Objective marchiafavabignami disease mbd is a rare condition mainly. However, it can occur in patients who do not use alcohol. Pdf marchiafavabignami disease mbd in a nonalcoholic.
The majority of patients have complete resolution of brain mri. The process may extend laterally into the neighboring white matter and occasionally as far as the subcortical regions. Cortical involvement in marchiafava bignami disease. Marchiafavabignami disease mbd is a rare condition characterized by demyelination of the corpus callosum.
Mbd presents with impaired consciousness and multifocal nonspecific neurological deficits. Currently at least three different kinds are proposed based on the presence of autoantibodies thought to produce the disease. Objective marchiafava bignami disease mbd is a rare condition mainly associated with alcoholism, although it may be mimicked by several other disorders that cause corpus callosum lesions. However, with the advent of mri, more and more of these cases are being diagnosed. Marchiafava bignami disease mbd is a rare neurological disorder mostly seen in alcoholic and malnourished patients with a pathognomonic hallmark of corpus callosum demyelination. We report a case of mbd in a diabetic patient, without alcoholism or malnutrition, caused by a wide range of glycemic level fluctuations. In patients with hyperchloremic nongap metabolic acidosis with previous exposure to ifosfamide further workup is indicated to rule out fanconi s syndrome. 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. This case illustrates what are probably features of prior marchiafava bignami syndrome. It is diagnosed by the imaging of demyelination at the corpus callosum with mri. Marchiafavabignami syndrome mbd is a progressive neurological.
Dec 21, 20 marchiafava bignami disease mbd is a rare neurologic complication of chronic alcohol consumption that is characterized by callosal lesions involving demyelination and necrosis. The pathological findings were symmetric necrosis of the middle laminae of the corpus callosum and signs of general lesion of the bloodbrainbarrier, the. Original history of the deficiency diseases of the nervous. Subsequent history obtained of the patient, confirmed the presence of a period of severe binge drinking while depressed. Marchiafavabignami disease triggered by poorly controlled. Marchiafavabignami disease with widespread case report. The patient also had polyuria and polydipsia which are frequently seen in fanconi s syndrome urine volumes of 34 l per day.
Most patients diagnosed with marchiafavabignami disease mbd have a history of alcoholism and poor nutrition. Marchiafava bignami disease mbd was originally described as a rare, fatal disease affecting wine drinkers. Intermediate syndrome 449 diagnosis, treatment of opc poisoning 450 oximes 450, 451 carbamates 451, 452 organochlorines 452 endrine 453 paraquat 453 pyrethrins and pyrethroids 454 zinc phosphide 454 aluminium phosphide 455, 456 7. We report a 51yearold man with chronic alcoholism and malnutrition who. Kosaka k, aoki m, kawasaki n, adachi y, konuma i, iizuka r. Marchiafavabignami disease mbd is a rare neurological disorder mostly seen in alcoholic and malnourished patients with a pathognomonic hallmark of corpus callosum demyelination. Most individuals diagnosed with marchiafava bignami disease mbd have a history of alcoholism and poor nutrition. The marchiafavabignami disease, characterized by demyelination and. Nutritional encephalopathies, including the thiamineresponsive wernickekorsakoff syndrome. The main symptoms are fits, stupor, coma and dementia. The syndrome was first described by marchiafava and bignami two italian pathologists in 1903.
Aug 14, 2016 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. Refractory nonconvulsive status epilepticus with favorable outcome in a patient with marchiafavabignami disease ryul kim, a heejin cho, b howon lee, b, c and jinsun jun a, d a department of neurology, seoul national university hospital, seoul, korea b department of neurology, school of medicine, kyungpook national university, kyungpook national university chilgok hospital, daegu, korea. It is a progressive neurological disease most frequently seen in middleaged or elderly alcoholic males. Lesions of the corpus callosum cc are seen in a multitude of disorders including vascular diseases, metabolic disorders, tumours, demyelinating diseases, trauma and infections. A progressive neurological disease most frequently seen in middleaged or elderly alcoholic males but also affecting some nonalcoholic subjects. A case report find, read and cite all the research you need on researchgate. Marchiafavabignami disease was first described in 1903 by 2. Various reversible neurologic symptoms are found in patients with mbd. 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. Fahd ali alkhamis imam abdulrahman bin faisal university. Marchiafavabignami disease radiology reference article. The syndrome is made up of two separate, but related disorders. Marchiafavabignami disease in a nonalcoholic diabetic patient.
This study investigated the profile of patients with rapidly progressive dementia at first presentation. Diagnosis and management of marchiafavabignami disease. Pdf on may 16, 2018, hiroki matsuura and others published marchiafava bignami disease find, read and cite all the research you need on. Case report marchiafavabignami disease with widespread lesions and complete recovery c. Marchiafava bignami disease is a progressive neurological disease of alcoholism, characterized by corpus callosum demyelination and necrosis and subsequent atrophy. How onset occurs suddenly or chronically and the range of clinical symptoms vary among affected individuals. Acute marchiafava bignami disease with selective involvement of the precentral neurologist. Rivista di patologia nervosa e mentale, 1903, 8 12. The patient received treatment with intravenous thiamine 300mgday for 3. Marchiafava bignami is a rare toxic disease seen mostly in chronic alcoholics that results in progressive demyelination and necrosis of the corpus callosum. The tempo of onset and the range of clinical symptoms vary.
Due to demyelination of the corpus callosum and cortical laminar. The syndrome is named after the two people who first described the conditions. Occurs predominantly in those with chronic alcoholism, particularly wine drinkers. Several mr findings have shown lesions not only in the corpus callosum but also in the hemispheric white matter. Pdf marchiafavabignami is a rare toxic disease associated with chronic alcohol intake and characterized by progressive demyelination and. Generally, the most common presentation includes personality change and psychomotor impairment. Marchiafavabignami disease mbd is a rare form of toxic demyelination of the corpus callosum associated with chronic alcoholic consumption. Marchiafavabignami disease triggered by poorly controlled diabetes mellitus. The importance of brain mri in the diagnosis of marchiafava. Marchiafava bignami disease with widespread lesions and complete recovery c. Similar to wernickekorsakoff syndrome, this condition is caused by a vitamin b1 deficiency, and the treatment is based on nutritional. A l c o h o l c o n c e r n wernickekorsakoffs syndrome.
Prior to this, there was no history of alcohol abuse which would have made marchiafava bignami syndrome unlikely. Diffuse hyperintensity was seen limited to the central region of the corpus callosum bilaterally at the sections of t2 weighted cranial mri performed after 10 days. Marchiafavabignami disease is a progressive neurological disease of alcoholism, characterized by corpus callosum demyelination and necrosis and subsequent atrophy. Other brain damage occasionally reported in alcoholics includes cortical laminar sclerosis, cerebellar degeneration. 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. Early symptoms may include depression, paranoia, psychosis, or dementia. Northholland publishing company, amsterdam new york oxford, pp. In 1903, italian pathologists marchiafava and bignami described 3 alcoholic men who died after having seizures and coma. Marchiafava bignami disease a condition that occurs in chronic alcoholics, clinically characterised by progressive neurological disease and histologically characterised by corpus callosum demyelination, necrosis and atrophy.
Marchiafava bignami disease genetic and rare diseases. Most patients diagnosed with marchiafava bignami disease mbd have a history of alcoholism and poor nutrition. Methods retrospective case analysis was done in 187 patients with rapidly progressive. Pdf on jan 1, 2010, santosh b murthy and others published marchiafavabignami disease mbd in a nonalcoholic patient. Wernickekorsakoff syndrome in established or suspected wernickekorsakoff syndrome, two pairs of pabrinex iv ampoules should be given three times daily for 2 days, followed by one pair of pabrinex iv or v im ampoules daily for 5 days if there is a m response. Marchiafava bignami disease with widespread lesions and complete recovery.
A reversible isolated lesion in the splenium of corpus. Vinken pj, bruyn gw eds handbook of clinical neurology, vol 28. A rare clinical dilemma nitesh pansari 1, ravi goyal, manish aswani 1, shruti agrawal, heeralal verma 2, hemant mahur3, mahesh dave4 introduction m archiafavabignami disease mbdaggressive and irritable during the is a progressive neurological disease, characterized by corpus callosal. Neuromyelitis optica nmo is a heterogeneous condition consisting of the inflammation and demyelination of the optic nerve optic neuritis and the spinal cord myelitis. It is very difficult to diagnose and there is no specific treatment.
Marchiafavabignami disease mbd is characterized by primary degeneration of the corpus callosum associated with chronic alcohol consumption. Marchiafavabignamis disease, as etiologic diagnosis of athetosis. In my own inaugural case report mentioned above, my teacher jurgen peiver and i, hypothesized that the pleomorphic xanthoastrocytoma is not a glial tumor and we argued that the two eminent neuropathologists john. Feb 20, 2012 marchiafava bignami disease, genetic and rare diseases information center gard, 2011. Mbd is a rare disorder strongly associated with alcoholism. Marchiafavabignami disease mimics motor neuron disease. Neuromyelitis optica nmo is a heterogeneous condition consisting of the inflammation and demyelination of the optic nerve optic neuritis and the spinal cord. Ppiinniioonn1440 north chase street athens, ga 30601 return. The disease seems to most often affect severe and chronic alcoholics in their middle or late adult life. The disease was first described in 1903 by the italian pathologists amico bignami and ettore marchiafava in an italian chianti drinker.
Marchiafava bignami disease statpearls ncbi bookshelf. Primary degeneration of the corpus callosum marchiafavabignami. The second case of marchiafavabignamisyndrome in central europe is reported of a 44yearold man, chronic drinker of regional wines with characteristic terminal clinical picture. A 43 year old man presents because he bumped his head after slipping and falling. Marchiafavabignami disease mbd is a very rare disorder that was first. Mbd in nonalcoholics without malnutrition has rarely been reported.
In this autopsy, marchiafava and bignami noticed that the middle twothirds of the corpus callosum were necrotic. Marchiafavabignami disease mbd is a rare neurologic complication of chronic alcohol consumption that is characterized by callosal lesions involving demyelination and necrosis. Pdf marchiafava bignami disease is the symmetrical demyelination of the middle portion of the corpus callosum observed in people with chronic. The second case of marchiafava bignami syndrome in central europe is reported of a 44yearold man, chronic drinker of regional wines with characteristic terminal clinical picture. Transcranial magnetic stimulation shows impaired transcallosal inhibition in marchiafavabignami syndrome. Marchiafava bignami disease mbd associated with chronic alcoholism is a fatal disorder characterized by demyelination of the corpus callosum. History of the deficiency diseases of the nervous system 159 a toxic aetiology led korsakoff to name it toxaemic cerebropathy. Because clinical signs are nonspecific, the role of computed. Marchiafavabignami disease definition of marchiafava. University of oklahoma college of medicine curriculum vitae bhrugav gautam raval, md assistant professor, department of neurology williams pavilion, g. A 50yearold chronic alcoholic presented to the emergency room with history of 3 episodes of seizures 2 days earlier. A rare clinical dilemma nitesh pansari 1, ravi goyal, manish aswani 1, shruti agrawal, heeralal verma 2, hemant mahur3, mahesh dave4 introduction m archiafavabignami disease mbdaggressive and irritable during the is a.