Marchiafava-Bignami disease in a non-alcoholic Indian male

doi:10.3109/00313027909061950

Pathology

Volume 11, Issue 2, 1979, Pages 241-249

https://doi.org/10.3109/00313027909061950 Get rights and content

Summary

Marchiafava-Bignami disease, a rare affliction of alcoholic males, is described in a severely malnourished Malaysian Indian male who took no alcohol. It is the second report of the disease in an Asian and represents one of the few cases which have occurred in non-alcoholics. Besides the pathognomonic demyelination of the central portion of the corpus callosum, there were striking demyelinative plaques in the subcortical white matter. In addition, neuropathological features of Wernicke's disease were found suggesting that severe malnutrition with thiamine deficiency was probably the cause of his demise.

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Cited by (36)

A patient with Marchiafava-Bignami disease as a complication of diabetes mellitus treated effectively with cortico steroid
2012, Journal of Clinical Neuroscience
Citation Excerpt :
The symptomatology is diverse, and MRI is helpful in diagnosis in the early stages of this disease.2 Although several cases of MBD have been reported in non-alcoholic patients, most instances of MBD have occurred as a result of malnutrition.3–9 We report a patient with MBD with no history of alcohol consumption or malnutrition with diabetes mellitus (DM).
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.
Neuroimaging in postinfectious demyelination and nutritional disorders of the central nervous system
2011, Neuroimaging Clinics of North America
Citation Excerpt :
Cortical involvement may indicate poor prognosis even when thiamine is promptly administered. Initially described in male Italian alcoholics consuming large quantities of red wine, this rare condition is now recognized worldwide among poorly nourished alcoholics as well as nondrinkers with severe nutritional deficiency.70–74 Patients present acutely with sudden altered consciousness, seizures, hypertonia, and pyramidal signs, progressing rapidly to unconsciousness and death.
A non-alcoholic patient with acute Marchiafava-Bignami disease associated with gynecologic malignancy: Paraneoplastic Marchiafava-Bignami disease?
2007, Clinical Neurology and Neurosurgery
Citation Excerpt :
It was first described in wine-drinking Italian men with bad nutritional habits and characterized by different neurological symptoms. However, MBD has since been observed among persons of many nationalities, with consumption of different types of alcohol and non-alcoholics [1–4]. MBD involves generally genu and body of the corpus callosum, but can also be seen in the extra-callosal structures, such as cerebral cortex, anterior commissure, centrum semiovale, cingulate gyrus, and middle cerebellar peduncles [5,6].
We report a 45-year-old, non-alcoholic woman with ovarian cancer who presented with acute impairment of consciousness. Cranial MRI revealed symmetrical and bilateral increased signal intensities of the corpus callosum and the dentate nucleus, without contrast enhancement. The findings are comparable with Marchiafava-Bignami disease (MBD), although pathological confirmation was not possible. Most of the reported cases of MBD are related to chronic ingestion of red wine and/or related with a nutritional cause. We suggest that this patient may suffer a MBD possible related to the ovarian cancer.
Pregnant, vomiting, and coma
1999, Lancet
An autopsy case of Marchiafava-Bignami disease with peculiar chronological CT changes in the corpus callosum: Neuroradiopathological correlations
1996, Journal of the Neurological Sciences
We conducted a comparative analysis of the autopsy findings in a case of Marchiafava-Bignami disease with the X-ray computed tomography (CT) and magnetic resonance imaging (MRI) findings obtained while the patient was alive. Histologically, massive deposits of hemosiderin were demonstrated in the astrocytes and macrophages located in the walls of the callosal cystic lesion, suggesting that the transient increase in density during the subacute stage observed on X-ray CT is secondary to significant hemorrhage.
Callosal bleeding in a case of Marchiafava-Bignami disease
1996, Journal of the Neurological Sciences

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Marchiafava-Bignami disease in a non-alcoholic Indian male

Summary

Prog. Brain Res

Hyponatremia and central pontine myelinosis

Neurology (Minneap.)

Marchiafava-Bignami disease: an anatomical study often cases (Fr.)

Rev. Neurol. (Paris)

Degeneration of white matter in hypoxia, acidosis and edema

J. Neuropathol. Exp. Neurol

Hypoxic-ischaemic leukoencephalopathy in man

Arch. Neurol

Terminological, etiological, anatomical and clinical considerations concerning Gayet-Wernicke encephalopathy. Its relationship with Marchiafava-Bignami syndrome and Korsakow's psychosis (Fr.)

Rev. Neurol. (Paris)

On the pathology of alcoholic encephalopathy (Fr.)

Rev. Neurol. (Paris)

Central demyelination of the corpus callosum (Marchiafava-Bignami disease). With report of a second case in Great Britain

Brain

Marchiafava-Bignami disease. Report of the first case in an Asian

Acta Neuropathol. (Berl.)