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Case of Marchiafava Bignami Disease
Case of Marchiafava Bignami Disease
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This case describes a 28-year-old woman with heavy alcohol use disorder who presented with rapidly worsening mental status and gait instability. She had recently been treated for presumed Wernicke’s encephalopathy with IV thiamine and folate, but her neurologic status then deteriorated again, progressing from slowed speech and flat affect to mutism while still following commands. She also had poor hygiene, diffuse hyperpigmentation, and significant nutritional risk factors, including alcohol use and weight loss.<br /><br />Workup during the current admission revealed MRI findings consistent with Marchiafava-Bignami disease: restricted diffusion and FLAIR hyperintensities involving the corpus callosum, including the genu, rostrum, and splenium, with extension into periventricular white matter. This rare disorder is associated with chronic alcoholism and malnutrition and is caused by toxic-metabolic demyelination and necrosis of the corpus callosum. Because its presentation overlaps with other neurologic disorders, diagnosis can be delayed.<br /><br />The patient also had a complex medical background, including newly diagnosed systemic lupus erythematosus and prior positive autoimmune testing. Her treatment included a high-dose steroid burst, IVIG for five days, IV thiamine, folic acid, and penicillin G, along with supportive measures such as sensory stimulation, a windowed room, and family interaction with music.<br /><br />By discharge, she remained somewhat apathetic but was able to communicate in short answers and was transferred to inpatient rehabilitation with a PEG tube still in place. At one-month rheumatology follow-up, she had improved communication, was eating by mouth after PEG removal, and reported persistent memory gaps and balance problems but overall felt better.<br /><br />Key lessons from this case are that Marchiafava-Bignami disease should be suspected in patients with alcohol use disorder who worsen or fail to improve after standard Wernicke’s treatment, MRI corpus callosum abnormalities are an important clue, and early multidisciplinary care can support meaningful recovery.
Asset Subtitle
Katherine L. Ripley
Meta Tag
Author List
Abu Baker Sheikh, Eva Angeli, Katherine L. Ripley
Category
Clinical Vignettes
Concept
Marchiafava-Bignami Disease
Concept
Alcohol Use Disorder
Concept
Malnutrition
Concept
Corpus Callosum
Concept
Wernicke Encephalopathy
Distinguished
Non-Finalist
Presenter Organization
University of New Mexico
Presenting Author
Katherine L. Ripley
Track
Adult
Keywords
Marchiafava-Bignami disease
alcohol use disorder
Wernicke encephalopathy
corpus callosum
MRI restricted diffusion
malnutrition
toxic-metabolic demyelination
mutism
intravenous thiamine
systemic lupus erythematosus
Marchiafava-Bignami Disease
Alcohol Use Disorder
Malnutrition
Corpus Callosum
Wernicke Encephalopathy
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