Marchiafava–Bignami Disease: A Rare Finding in Alcohol Dependence Syndrome

Sir, Marchiafava–Bignami disease (MBD) is a rare demyelinating disorder, associated with alcohol consumption over a prolonged period of time and malnutrition. Diagnosis is often missed due to symptoms mimicking other acute neurological conditions and approximately 250 cases have been reported worldwide.[1,2] This disease is characteristically diagnosed on neuroimaging, which is demonstrated as demyelination and necrosis of the corpus callosum.[3] We present the case of a 27-year-old male who presented with a history of road traffic accident under the influence of alcohol. He sustained injuries to the face and head, which was also associated with a brief loss of consciousness followed by agitation, altered sensorium, slurred speech, and gait abnormality. There was no history of posttraumatic seizure, vomiting, headache, cerebrospinal fluid leak, and hemotympanum. However, the patient was agitated, ataxic, and dysarthric with mild impairment in consciousness and the Glasgow Coma Scale on presentation was 10/15 (E3V3M4). Pupils were noticed to be of normal size and were normally reacting. There was no autonomic instability and neurological deficits on clinical examination. Noncontrast computed tomography head revealed an undisplaced fracture of the body of the mandible near the left angle of the mandible. There was no evidence of traumatic brain injury on the computed tomography (CT) scan. Laboratory results revealed a normal hematological profile, but the liver function tests revealed increased Serum Gamma Glutamyl Transferase (SGGT) 163 U/L, Serum Glutamic- Oxaloacetic Transaminase (SGOT)-85 IU/L, Serum Glutamic Pyruvic Transaminase (SGPT)-322 IU/L, and ultrasonography of the abdomen revealed hepatomegaly with Grade-1 fatty liver. The patient demonstrated progressively increasing psychomotor action and worsening altered sensorium (mini-mental state examination [MMSE]-24/30 and Montreal cognitive assessment [MOCA]-19/30) with poor ideomotor recovery. A history revealed progressively increasing alcohol consumption over a period of 7-year fulfilling criteria of alcohol dependence syndrome as per the International Classification of Diseases-10. T2-weighted magnetic resonance imaging (MRI) scan revealed hyperintensity in the body and splenium of the corpus callosum on the left side suggestive of Type B MBD [Figure 1]. The patient was started on high doses of injectable thiamine hydrochloride 1.5 g/day in an intravenous infusion and injectable multivitamins along with oral anticraving drugs in the form of acamprosate (333 mg 2-2-2) in conjunction with forced abstinence. The patient responded satisfactory to treatment with improvement of sensorium and restoration of normal psychomotor activity. After stabilization, he was imparted with structured Yoga and Meditation (YogNidra and Pranayam) therapy by a trained yoga therapist. Cognitive retraining with cognitive restructuring App (Luminous) along with individual and group psychotherapy by a clinical psychologist in a deaddiction center aimed at relapse prevention. Over the next 3 months, the patient’s cognitive functions improved (MMSE - 30/30 and MOCA - 29/30). MRI brain repeated after 12 weeks of the treatment revealed complete resolution of lesions [Figure 2].Figure 1: MRI brain of the patient at initial presentation prior to the start of the treatment. (a) Axial T2-weighted MR image, (b) coronal T2-weighted MR image, and (c) axial FLAIR MR image reveal hyperintensities in the splenium of the corpus callosum on the left side, (d) sagittal T1-weighted MR image shows hypodensities in the splenium of the corpus callosum in the central part sparing peripheral most part showing “Sandwich sign.” MRI: Magnetic resonance imaging, MR: Magnetic resonance, FLAIR: Fluid-attenuated inversion recoveryFigure 2: MRI brain of the patient after 3 months of treatment. (a) Axial FLAIR image, (b) axial T1-weighted image, and (c) axial T2-weighted image show no abnormalities of the splenium. MRI: Magnetic resonance imaging, FLAIR: Fluid-attenuated inversion recoveryConsidering the history of road traffic accident under the influence of alcohol with facial trauma associated with mild impairment in consciousness, dysarthria, ataxic gait, and cognitive impairment in a patient with evidence of chronic alcohol use and the classical “Sandwich Sign” on MRI, the important differentials which considered were traumatic brain injury, Wernicke’s encephalopathy, and MBD. The history of trauma associated with mild impairment of consciousness and orientation pointed toward a structural abnormality such as an extradural/subdural hematoma or intracerebral hemorrhage and diffuse axonal injury, but these were ruled out by the initial CT scan and MRI brain. The background of chronic alcohol intake over the last 7 years and the presence of the classical “Sandwich sign” on MRI, however, tilted the scales in the favor of possible MBD. In case of chronic alcoholics, Wernicke’s encephalopathy needs to be considered; it presents with milder symptoms of cognitive dysfunction and a speedy recovery as compared to MBD. It further can be distinguished on MRI by the symmetrical involvement of the thalamus, hypothalamus, brain stem, and periaqueductal gray matter.[4] An absence of relapsing-remitting symptoms and absent periventricular white matter lesions on MRI excluded the diagnosis of Multiple sclerosis.[5] Studies suggested that B1-hypovitaminosis is the pathophysiology of the disease. Hence, early replenishment of thiamine may improve outcomes in MBD patients. Studies have shown that early initiation of treatment within 14 days of onset of symptoms with high-dose thiamine and multivitamins has proven to be statistically beneficial as compared to delayed treatment.[4] Better outcomes noted in MBD patient with parenteral administration of thiamine compared to oral administration might be due to delayed intestinal absorption and deranged hepatic uptake associated with chronic alcoholism.[6] In conclusion, MBD, though a rare clinical entity, is potentially reversible with an early diagnosis and prompt initiation of high-dose thiamine and supportive care along with adequate rehabilitation as seen in our patient. 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. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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