Magnetic Resonance Imaging in Marchiafava-Bignami Syndrome: A Cornerstone in Diagnosis and Prognosis

Abstract

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.

Clinical and radiological features of Marchiafava–Bignami disease

“…[ 15 – 19 ] MRI plays a dominant role in the early diagnosis of MBD, facilitates early invention of acute MBD, and also predicts its prognosis. [ 20 ] (Supplemental Figure 1).…”

Marchiafava-Bignami Disease: Uncertain MRI Predictors of Outcome

“…T2 and Fluid Attenuated Inversion Recovery (FLAIR) weighted sequences confirmed hyperintense lesions of the splenium and the body of corpus callosum, with relative sparing of the dorsal and ventral layers, which represented the typical "sandwich sign" (Figure 3) [2]. In FLAIR images, the frontal cortical-sottocortical lesions seen on the CT scan were evident as hyperintense areas (Figure 4).…”

Neuroimaging of corpus callosum in central nervous system demyelinating disorders

“…On the other hand, ADEM causes individually large, asymmetric lesions and involvements in marchiafava bignami disease were large, often symmetrically in the midline of the splenium and did not reach the edge of the CC. [5,27] Central CC volume along with medulla oblongata volume can help discriminate between different subtypes of MS. Various subtypes of MS affect different neuroanatomical regions of the CC differently.…”

“…General pattern of CC pathology in MBD is hyperintensity on flair imaging and sometimes hemispheric white matter, reflecting damage to myelin and vasogenic edema of the CC and extracallosal projections. [27] Because of pathological variety of this disorder there is no certain sequence and pattern of pathology of CC in MBD. Some studies showed that involvement of the CC in MBD was initially in the genu, without significant association with DTI changes of CC and NAWM.…”

“…Serial MRI and DTI method of evaluation in MBD cases have been found to be significantly helpful and that's why there will be a great need for further studies on this method of evaluation for such demyelinating disorders. [27] It suffices to briefly review MBD data because there is still significant potential for further new neuroimaging methods for such a rare disease and our main goal in this review is demyelinating diseases with autoimmune nature rather than the disorders caused by environmental factors.…”