Marchiafava-Bignami Disease

A 38-year-old man was admitted to our hospital with abdominal pain caused by acute pancreatitis. He had a history of alcohol abuse for over 10 years, consuming 1000 mL–1500 mL of 38% alcohol per day. Upon admission, he developed delirium, presenting with phobic hallucinations, dysphoria, and epileptic seizures. After 1 week, he exhibited an apathetic facial expression, poor mobility, reduced strength in his limbs. Physical examination revealed an indifferent expression, unclear speech, poor memory, calculation ability, and reflex responses. Strength in both upper limbs was graded at Grade I, while strength in the lower limbs ranged from Grade II to III. A brain CT and MRI showed swelling in the splenium of the corpus callosum, as well as in the bilateral frontal cortex and other cortical regions [Figure 1]. Marchiafava-Bignami disease (MBD) is an encephalopathy syndrome closely associated with chronic alcoholism.[1] It is mainly characterized by central demyelination and degenerative changes in the corpus callosum.[1] Clinically, it is often manifested by altered consciousness, cognitive impairment, and mental and behavioral disorders, including confusion, delirium, disorientation, and even coma.[2] Other symptoms may include ataxia, dysarthria, epilepsy, blurred vision, hearing loss, and urinary and fecal incontinence.[2] MBD can present with cerebral connection/disconnection syndrome, gaze palsy, pyramidal signs, myotonia, and other neurological signs.[2] Typical imaging findings include hyperintense signals in the midline, with significant rim enhancement and peripheral edema.[3] Lesions may also occur in the prefrontal and temporal lobes.[3] Patients with MBD are mainly treated with high-dose vitamin B (especially vitamin B1) and μ-opioid receptor antagonists to help manage alcohol withdrawal.[1]Figure 1: Brain imaging changes in patients with MBD. The following images shown in sequence are a (CT), b (T1WI), c (T2WI), d (DWI), and e (FLAIR)Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Author contributions Han Luo: Collected case data and wrote the first draft. Shanshan Li: Collected case data. Bo Liu: Conceived and designed, supervised, reviewed, and edited the manuscript. Informed consent Informed consent was obtained from the patient included in this report. Ethical approval All procedures performed in the studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Financial support and sponsorship General Program Sponsored by Basic Research Fund in Shenzhen Natural Science Foundation (JCYJ20240813153011015). Conflicts of interest There are no conflicts of interest.

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Journal
Neurology India
Published
2025-09-01
DOI
https://doi.org/10.4103/neurol-india.neurol-india-d-25-00023
Primary Topic
Infectious Encephalopathies and Encephalitis
Type
article
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article

Marchiafava-Bignami Disease

Han Luo, Shanshan Li, Bo Liu
Neurology India
Infectious Encephalopathies and Encephalitis
article

Marchiafava-Bignami Disease

Han Luo, Shanshan Li, Bo Liu
article en

Abstract

A 38-year-old man was admitted to our hospital with abdominal pain caused by acute pancreatitis. He had a history of alcohol abuse for over 10 years, consuming 1000 mL–1500 mL of 38% alcohol per day. Upon admission, he developed delirium, presenting with phobic hallucinations, dysphoria, and epileptic seizures. After 1 week, he exhibited an apathetic facial expression, poor mobility, reduced strength in his limbs. Physical examination revealed an indifferent expression, unclear speech, poor memory, calculation ability, and reflex responses. Strength in both upper limbs was graded at Grade I, while strength in the lower limbs ranged from Grade II to III. A brain CT and MRI showed swelling in the splenium of the corpus callosum, as well as in the bilateral frontal cortex and other cortical regions [Figure 1]. Marchiafava-Bignami disease (MBD) is an encephalopathy syndrome closely associated with chronic alcoholism.[1] It is mainly characterized by central demyelination and degenerative changes in the corpus callosum.[1] Clinically, it is often manifested by altered consciousness, cognitive impairment, and mental and behavioral disorders, including confusion, delirium, disorientation, and even coma.[2] Other symptoms may include ataxia, dysarthria, epilepsy, blurred vision, hearing loss, and urinary and fecal incontinence.[2] MBD can present with cerebral connection/disconnection syndrome, gaze palsy, pyramidal signs, myotonia, and other neurological signs.[2] Typical imaging findings include hyperintense signals in the midline, with significant rim enhancement and peripheral edema.[3] Lesions may also occur in the prefrontal and temporal lobes.[3] Patients with MBD are mainly treated with high-dose vitamin B (especially vitamin B1) and μ-opioid receptor antagonists to help manage alcohol withdrawal.[1]Figure 1: Brain imaging changes in patients with MBD. The following images shown in sequence are a (CT), b (T1WI), c (T2WI), d (DWI), and e (FLAIR)Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Author contributions Han Luo: Collected case data and wrote the first draft. Shanshan Li: Collected case data. Bo Liu: Conceived and designed, supervised, reviewed, and edited the manuscript. Informed consent Informed consent was obtained from the patient included in this report. Ethical approval All procedures performed in the studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Financial support and sponsorship General Program Sponsored by Basic Research Fund in Shenzhen Natural Science Foundation (JCYJ20240813153011015). Conflicts of interest There are no conflicts of interest.

Neurology IndiaVol. 73(6)
Longgang Central Hospital (CN)
Openalex Percentile: Top 72%
Infectious Encephalopathies and Encephalitis
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