Marchiafava-Bignami syndrome is a rare condition. However, with the advent of MRI, more and more of these cases are being diagnosed. Thus, it becomes essential for a radiologist to be familiar with its imaging features as well as clinical presentation. A 50-year-old chronic alcoholic presented to the emergency room with history of 3 episodes of seizures 2 days earlier. The patient had gait disturbances for the last few days. On examination, the patient was in a state of stupor. No neck rigidity was elicited. On MRI, the corpus callosum appeared diffusely hypointense on T1 weighted images and hyperintense on T2 weighted sequences without any evident enhancement after intravenous administration of Gadolinium. On fluid attenuation and inversion recovery images, central hypointensity with surrounding hyperintense rim involving the genu, body, and splenium of corpus callosum was noted. Additionally, cortical-subcortical signal intensity changes were also noted predominantly involving the right frontal lobe. On diffusion weighted imaging, all the above mentioned lesions showed restriction of diffusion. I am presenting here a case of Marchiafava-Bignami syndrome highlighting the role of MR imaging in diagnosing, prognosticating as well as in understanding the underlying pathophysiology of this rare clinical entity.
Marchiafava-Bignami syndrome (MBS) is a rare demyelinating disorder seen in chronic alcoholics that is characterized by corpus callosal necrosis and associated white matter changes [
I present the MRI features of MBS with an attempt to correlate them with the underlying pathophysiology. This report also highlights the role of imaging in predicting prognosis of the disease.
A 50-year-old male chronic alcoholic presented to the emergency room with history of 3 episodes of seizures 2 days earlier. No h/o fever was present. On further enquiry, history of gait disturbances was elicited.
On examination the patient was in a state of stupor. No neck rigidity was elicited.
The above observations prompted further imaging evaluation with MRI.
On MRI, the corpus callosum appeared diffusely hypointense on T1WI (Figure
Axial T1 weighted image showing hypointensity of the genu (white arrow) and splenium (white arrowhead) of corpus callosum.
Axial T2 weighted image showing hyperintensity of genu (white arrow) and splenium (white arrowhead) of corpus callosum.
Axial T2 weighted image revealing cortical-subcortical hyperintensity involving the right frontal lobe (white arrow).
Diffusion weighted images displaying restricted diffusion involving the subcortical right frontal white matter (black arrow) and genu and splenium (black arrowhead) of corpus callosum.
Post-Gadolinium axial T1 weighted image showing lack of enhancement of the hypointense lesions involving the corpus callosum (white arrow).
Sagittal fluid attenuation and inversion recovery image displaying central hypointensity (suggesting cavitation) with surrounding hyperintense rim (active inflammation) (white arrowheads) involving the genu, body, and splenium of corpus callosum.
On the basis of the clinical features and imaging appearances, a diagnosis of Marchiafava-Bignami syndrome was made.
The patient was admitted to the neurointensive care unit and succumbed 2 days later despite extensive supportive management.
Marchiafava-Bignami syndrome is characterized by corpus callosum necrosis and is observed predominantly in alcoholics. It usually affects the body of the corpus callosum, followed by the genu and splenium [
MBS can present as two main clinical forms: an acute form with severe disturbance of consciousness and neurocognitive deficits, often fatal, and a chronic form which usually presents as chronic dementia. The most important differential diagnosis considered in the current clinical context (acute presentation) was Wernicke’s encephalopathy. However, absence of typical clinical signs like ophthalmoplegia, nystagmus, and ataxia along with lack of typical MRI features of Wernicke’s encephalopathy such as involvement of mammillary bodies and periaqueductal grey matter excluded this differential. Both of these entities can sometimes coexist and happen to fall under the broad category of alcohol-related encephalopathies. The latter comprise a wide spectrum of CNS derangements caused by chronic alcohol intake, mediated through inflammation, DNA damage, and oxidative stress, sometimes exacerbated by accompanying thiamine deficiency (Wernicke’s encephalopathy) and altered plasma osmolality [
Clinical forms of MBS and their differentials [
Clinical form of MBS | Predominant symptoms | Clinical differentials and their MRI findings |
---|---|---|
Acute MBS | Mental confusion, disorientation, neurocognitive deficits, and seizures | (1) |
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Chronic MBS | Chronic dementia | (1) |
The usual chronology of pathological events in MBS is inflammation (acute phase) followed by demyelination and eventually necrosis and axonal loss (chronic phase) [
Correlation of imaging findings in MBS with pathophysiology [
MRI findings | Underlying pathophysiology | |
---|---|---|
1 | Hyperintensity on T2 weighted images | Edema and myelin damage |
2 | Hypointensity on T1 weighted images | Total loss of myelin with replacement of the region by a cyst |
3 | Hyperintense rims and hypointense cores on FLAIR images | Damage to the myelin at the rim with a central necrotic area |
4 | Uniformly hyperintense lesions on FLAIR | Mixture of demyelination and edema |
5 | Areas of restricted diffusion on DWI (acute phase) | Cytotoxic edema |
On MR imaging, patients with MBS show areas of low T1 signal intensity and high T2 and FLAIR signal intensity in the body of the corpus callosum with or without associated lesions in the cerebral white matter. These lesions are devoid of mass effect and may show peripheral contrast enhancement during the acute phase [
Hence diffuse corpus callosal involvement, presence of cortical involvement, and diffusion restriction, all of which were seen in this case, can be considered as poor prognostic factors while predicting the outcome of MBS.
Magnetic resonance imaging is a valuable tool in diagnosing, prognosticating as well as in understanding the underlying pathophysiology of Marchiafava-Bignami syndrome.
The author declares that there is no conflict of interests regarding the publication of this paper.