International Journal of Research in Medical Sciences | November 2022 | Vol 10 | Issue 11 Page 2702
International Journal of Research in Medical Sciences
Jaganathan S et al. Int J Res Med Sci. 2022 Nov;10(11):2705-2705
www.msjonline.org pISSN 2320-6071 | eISSN 2320-6012
Case Report
Marchiafava-Bignami disease: an interesting case report!
Sriram Jaganathan
1
*, Medhini Rangarajan
2
INTRODUCTION
Marchiafava Bignami is a rare affliction of chronic
alcoholism primarily involving the corpus callosum
resulting in demyelination and necrosis.
1,2
The main
pathophysiology revolves around chronic alcoholism with
resultant hypovitaminosis, alteration in the
neurotransmitter activity and oxidative injury.
The clinical presentation of the disease encompasses a
wide range of symptoms ranging from reduced
consciousness, emotional and psychotic symptoms,
depression and apathy, aggression, seizures, hemiparesis,
ataxia, and apraxia.
1
The disease can present in 3 stages -
acute, subacute and chronic form.
3,4
However, if left untreated, these patients may land in coma
as well. The gold standard investigation is magnetic
resonance imaging (MRI) brain which may show typical
features of restricted diffusion in the corpus callosum and
helpful in appropriate clinical setting to guide in early
diagnosis.
4,5
A relatively accepted treatment for this
condition is intravenous thiamine (vitamin B1) infusion.
Some authors suggest usage of high dose steroids, folic
acid, and amantadine in the treatment protocol. Our patient
is a 57-year-old alcoholic who presented with acute
confusion and showed DWI abnormalities in corpus
callosum and periventricular white matter.
CASE REPORT
A 57-year-old chronic alcoholic presented to our hospital
acutely in a state of confusion, with disorientation and
aphasia. Patient had been consuming alcohol for almost 25
years.
We obtained the history from the patient’s relative. There
was no prior history of sensory or motor deficit, memory
loss or previous similar episodes. On examination, patient
was hemodynamically stable. CNS examination revealed
1
Department of Radiology, Global Hospitals and Health City, Chennai, Tamil Nadu, India
2
Department of Surgery, SRM Medical College, Kattangulathur, Chennai, Tamil Nadu, India
Received: 19 October 2022
Accepted: 26 October 2022
*Correspondence:
Dr. Sriram Jaganathan,
E-mail: sriramj.ach@gmail.com
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the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial
use, distribution, and reproduction in any medium, provided the original work is properly cited.
ABSTRACT
Marchiafava-Bignami Disease (MBD) is a rare neurodegenerative disease usually associated with chronic alcoholism
characterized by demyelination and necrosis of the corpus callosum. Here, we report a 57-year-old patient who
presented to our hospital with acute aphasia, disorientation and altered mental state. Diffusion weighted imaging (DWI)
magnetic resonance imaging (MRI) revealed areas of restricted diffusion in the corpus callosum, bilateral
periventricular white matter and right cerebellar peduncle. Given that the patient was a chronic alcoholic, a diagnosis
of Marchiafava-Bignami disease was made. Supportive management with intravenous Thiamine and multivitamins was
offered. The patient improved clinically after one week of treatment and a repeat DWI MRI revealed complete
resolution of the abnormalities. It is imperative to diagnose MBD promptly and distinguish this from other neurological
manifestations related to chronic alcoholism. An early diagnosis can aid in a better outcome and faster recovery.
Keywords: Chronic alcoholism, Corpus callosum, Demyelination, Intravenous thiamine
DOI: https://dx.doi.org/10.18203/2320-6012.ijrms20222887