Clinical analysis of Marchiafava-Bignami disease

Abstract

Background: Marchiafava-Bignami disease (MBD) is an exceptionally rare condition, a fact that should pique the professional curiosity of medical practitioners. In recent years, case reports of this disease have been infrequent, and no comprehensive analysis or summary of the characteristics of the published cases has been conducted.

Methods: We collected the medical records of three patients treated at our hospital from March 2022 to March 2023. Furthermore, we searched PubMed for "case reports" from January 2017 to March 2023 and included 30 cases. By retrospectively analyzing these 33 cases, we summarized the characteristics of the disease.

Results: Based on our analysis, we found that MBD primarily affects middle-aged men and typically has an acute or subacute onset, with the primary clinical manifestations being disturbances of consciousness, speech disorders, cognitive impairment, and psychiatric or behavioral abnormalities, often leading to misdiagnosis of psychiatric disorders. Most patients have a history of alcohol consumption or malnutrition. Head CT or MRI revealed symmetric lesions in the corpus callosum, with the splenium being the most commonly affected area. Lesions might also involve white matter outside the corpus callosum, and a wider range of lesions suggested a poor prognosis. However, the prognosis is generally favorable with timely and adequate administration of B vitamins, providing reassurance to medical professionals and patients alike.

Conclusion: The early recognition and treatment of Marchiafava-Bignami disease are paramount, as they can significantly improve the prognosis. This underscores the critical need for prompt clinical intervention in the early stages of the disease, instilling a sense of urgency and significance in the work of medical professionals.

Keywords: Corpus callosum; Marchiafava-Bignami disease (MBD); Neurology.

Fig. 1

MRI imaging of a patient with MBD A DWI of the brain MRI shows hyperintense signals in the genu and splenium of the corpus callosum five days after MBD onset; B DWI of the brain MRI shows hyperintense signals in the periventricular white matter five days after MBD onset; C Sagittal T1-weighted MRI shows swelling of the splenium of the corpus callosum five days after MBD onset; D DWI of the brain MRI shows hypointense signals in the genu and splenium of the corpus callosum 84 days after MBD onset; E DWI of the brain MRI shows isointense signals in the periventricular white matter 84 days after MBD onset; F Sagittal T1-weighted MRI shows atrophy of the splenium of the corpus callosum 84 days after MBD onset