Year : 2014  |  Volume : 56  |  Issue : 1  |  Page : 76--78

Marchiafawa bignami disease possibly related to consumption of a locally brewed alcoholic beverage: Report of two cases

Jagdeo P Rawat1, Charles Pinto2, Kapil S Kulkarni1, M. Ananthi K Muthusamy1, Malay D Dave1,  
1 Department of Psychiatry, Jagjivan Ram Hospital, Mumbai, Maharashtra, India
2 BYL Nair Hospital and Topiwala National Medical College, Mumbai, Maharashtra, India

Correspondence Address:
Jagdeo P Rawat
11/6, Sky Scrapper Building, Western Railways Officers Flats, A. L. Nair Road, Mumbai Central, Mumbai - 400 008, Maharashtra


Marchiafava Bignami disease is a rare toxic disease seen mostly in chronic alcoholics, resulting in progressive demyelination and necrosis of the corpus callosum. Initially it was thought to be specific to individuals in central Italy, consuming large amounts of Chianti red wine; however, alcoholic beverages worldwide are presently implicated. In our case series of two cases, locally made DQillicitDQ liquor (Mahuwa Alcohol) could be the causative factor. In radiological point of view typically the corpus callosum is affected, with involvement of the body, genu, and splenium in order of occurrence. Occasionally the entire callosum may be also involved. Clinical presentation varies from case to case.

How to cite this article:
Rawat JP, Pinto C, Kulkarni KS, Muthusamy MK, Dave MD. Marchiafawa bignami disease possibly related to consumption of a locally brewed alcoholic beverage: Report of two cases.Indian J Psychiatry 2014;56:76-78

How to cite this URL:
Rawat JP, Pinto C, Kulkarni KS, Muthusamy MK, Dave MD. Marchiafawa bignami disease possibly related to consumption of a locally brewed alcoholic beverage: Report of two cases. Indian J Psychiatry [serial online] 2014 [cited 2021 Jan 16 ];56:76-78
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Marchiafava-Bignami disease (MBD) is a rare condition characterized by demyelination of the corpus callosum. MBD first described by Carducci in 1898 in Italian red wine drinkers and by Italian Pathologist Marchiafava and Bignami in 1903. Alcoholism remains the greatest risk factor for MBD, although rare cases have occurred in individuals who did not drink alcohol. Nutritional factors have been suspected in MBD, but no specific nutrient has been identified. Electrolyte disturbances (as in central pontine myelinolysis) may be important. In 2007, Celik et al., reported a case of a nonalcoholic patient with acute MBD that was associated with a gynecologic malignancy. The authors raised the question of a possible paraneoplastic MBD. [1] That same year, Rusche-Skolarus et al., described a case of MBD in a postoperative, nonalcoholic female who presented with an encephalopathy. [2] The tempo of onset and the range of clinical symptoms vary. Some patients present to the hospital with sudden onset of stupor or coma, and some present with seizures. Other patients have acute, subacute, or chronic onset of dementia and/or gait problems. Psychiatric disturbances, incontinence, hemiparesis, aphasia, and apraxia have been described. In the era before CT scanning, MBD was found almost exclusively at autopsy. However, modern CT scanning and MRI have allowed the detection of mild cases of the disease. Marchiafava and Bignami described three alcoholic men who died after having seizures and coma.

In each patient, the middle two-thirds of the corpus callosum was found to be severely necrotic. [3] Although the callosal lesions are the hallmark of the disease, few cases were known to be associated with cortical damage in addition to corpus callosum. Generally, the cortical damage was in the lateral frontal and the temporal lobes, mainly in the third (although sometimes also in the fourth) cortical layer. In these areas, the neurons degenerated and were replaced by glial cells. In 1939, Morel described this as cortical laminar sclerosis (now known as Morel cortical laminar sclerosis). [4]

Indian data till date shows that there are case reports in radiology, [5] neurology, [6],[7] and medicine [8],[9] journals. Some cases were chronic alcoholic while others were nonalcoholic. Along with corpus callosum degeneration, pontine myelinosis [5] was also reported in Indian cases.

In our two cases common factor was consumption of locally made Mahuwa Alcohol. Both cases were having different clinical presentation but radiological findings were similar.

 Case Reports

Case 1

Mr. A, 39-year-old married Hindu male from low socioeconomic class and urban background, a known case of chronic alcoholism, brought by wife and uncle with 3-month history of forgetfulness in form of forgetting his way back home, whether bath was taken, where he kept the things. It was progressive in nature affecting his daily routine and socio-occupational life. He used to consume locally made alcohol called Mahuwa Daru occasionally one pouch (100 ml) since 8 years, gradually increasing to one to two pouches three times a day since last one and half year. There is history of withdrawal symptoms in form of tremors, insomnia and irritability in past. No history of any other psychiatric illness. There is family history of alcohol dependence in father.

Patient was first taken to physician. His routine investigations were normal. MRI showed abnormal signal intensity with restricted diffusion in corpus callosum with demyelination in genu and splenium. He was treated for withdrawal and discharged. Patient restarted alcohol after discharge.

Patient was referred to our hospital for fitness. He was admitted. Last consumption was day prior to admission and he developed tremors next day. Routine investigations and neurological examination were normal. MMSE was 21/30. He was started on anti-withdrawal line of management with Inj. Thiamine (100 mg) OD, Tab. Multivitamin BD and Tab. Lorazepam 10 mg in divided doses. Repeat MRI brain showed hyper intense signal alterations involving posterior body and splenium, as well as anterior body and genu of the corpus callosum. This represent progressive demyelination and necrosis of the corpus callosum [Figure 1]. All these characteristic findings confirmed the diagnosis of MBD.{Figure 1}

Case 2

Mr. ABC, a 57-year-old male patient from low socio-economic status from rural area with history of chronic alcohol abuse since 35 years, gradually increasing to 4-5 (400-500 ml) pouches of Mahuwa Daru over 2 hours daily in the evening. Patient never abstained. Patient was forced to stop alcohol by relatives for financial and health reasons, the following day he developed tremors and sleep impairment and on third day he was found disoriented in disheveled state with fecal and urinary incontinence by the coworkers and was admitted. General examination showed malnourishment and thickening of post-auricular and ulnar nerve. Vital signs were normal. Mental state examination showed disorientation. Pupillary reactions were symmetrical. There were no lateralizing deficits and no signs of meningeal irritation. Laboratory tests showed a normal haemogram, liver and renal function tests and serum electrolytes. Patient was started on Inj Thiamine 100 mg and anti withdrawal line of management. He had four episodes of seizures over three days of admission. His EEG was normal. His MRI-Brain showed hyper intense signal intensities involving genu, body and splenium of corpus callosum on T2W and FLAIR [Figure 2]. Patient gradually improved. On 20 th day he was symptom free, MSE was clear, MMSE was 30/30 and was discharged and advised to follow up. After one month he resumed his work. Patient did not follow up in OPD for further evaluation.{Figure 2}


The old literature on MBD suggested that this condition was more common in Italians. This was solely an artifact of the initial cases having been found in Italy and the fact that, at first, Italian physicians were apparently the only investigators interested in finding such cases. MBD has since been found in persons from all over the world. It is now firmly believed that no national, geographic, ethnic, or racial predilection is known for MBD. It has been described that patients who consume at least 2 l of red wine for more than 20 years are likely to suffer from MBD. In our patients, both from same area, consuming same type of local made Mahuwa Alcohol (Botanical name-madhuca longifolia), which is made from flowers. [10] Both have history of different duration of consumption presenting with different features and difference in improvement.

In 2004, Heinrich et al., described two clinical subtypes of MBD as follows, based on a review of 50 radiological cases diagnosed in vivo. [11]

In both types T2-weighted axial images show high-signal lesions in the corpus callosum. The prognosis for MBD is correlated with the subtype [Table 1].{Table 1}


Special thanks to Dr. Karthik Ganesan (Radiologist) Jhankaria Imaging Centre, Mumbai.


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