CASE 15688 Published on 11.05.2018

Marchiafava-Bignami disease, a rare alcoholic encephalopathy

Section

Neuroradiology

Case Type

Clinical Cases

Authors

Vanhove F1, Lutin B, Seynaeve P

Department of Radiology, AZ Groeninge, President Kennedylaan 4, Kortrijk, Belgium
1Correspondence e-mail: vanhovefrederic93@gmail.com
Patient

46 years, female

Categories
Area of Interest Neuroradiology brain ; Imaging Technique CT, MR, MR-Diffusion/Perfusion
Clinical History

A 46-year-old alcoholic Caucasian female patient admitted at the emergency department with an encephalopathy that started 3 hours earlier. Symptoms were mutism, dysarthria, a partial left upper limb hemiparesis and a left facial nerve paralysis.

Imaging Findings

A non-enhanced brain computed tomography (CT) scan could exclude intracranial haemorrhage. After close examination, a hypodense corpus callosum (CC) was observed (Figure 1). A CT angiography scan showed no carotid stenosis and a normal circle of Willis. Perfusion CT scan reveiled no perfusion deficit or mismatch.
A cerebral Magnetic Resonance Imaging (MRI) scan performed two days after symptom onset showed significant cerebral atrophy and a hyperintense signal on fluid-attenuated inversion recovery (FLAIR) weighted sequences of the entire CC with diffusion restricted lesions on diffusion weighted imaging (DWI) (Figure 2). These lesions were not visualised on T1-weighted images and showed no contrast enhancement.
A follow-up MRI scan six days later showed a diminished FLAIR hyperintensity and diffusion restriction (Figure 3). On T1-weighted sequences, however, hypointense lesions emerged in the entire CC on a follow-up MRI scan after 26 days (Figure 4).

Discussion

Marchiafava-Bignami Disease (MBD) is a rare complication of chronic alcoholism, characterised by primary demyelination and necrosis of the CC. [1] Ettore Marchiafava and Amico Bignami, two pathologists, first described the disease in Italian heavy red wine drinkers in 1903. [2] This extremely rare condition has been published in approximately 300 case reports [e.g. 3, 4] with another 50 mentioned in older textbooks. [5] There is no geographic or ethnic predisposition. Mean age is about 47 years. [1] MBD occurs most frequently among alcoholic men, although MBD in non-alcoholic subjects has been reported. [6] The hypothesised pathophysiological mechanism is a synergism between malnutrition with hypovitaminosis B and oxidative stress in ethanol-induced neurotoxicity. [7] Abrupt fluctuation in serum osmolality (“callosal myelinolysis”) as a complication of diabetes mellitus or alcoholic ketoacidosis is another cause. [8]
The clinical presentation comprises a varying and nonspecific spectrum of symptoms. MBD patients frequently show an altered mental state, impaired walking, dysarthria, impaired memory, interhemispheric disconnection syndrome, pyramidal signs, mutism, hemiparesis or tetraparesis and facial palsy. [6] Heinrich et al. [1] differentiated two clinicoradiologic subtypes. Type A is characterised by major impairment of consciousness, affection of the entire CC, and poor outcome. Type B MBD presents with slight impairment of consciousness, partial callosal lesions, and a favourable prognosis.
Neuro-imaging is crucial to establish the diagnosis. A hypodense CC is observed on non-enhanced CT scan. Cerebellar and (sub)cortical atrophy is common in alcoholics. An MRI scan is the most sensitive diagnostic tool and shows a symmetrical hyperintense signal on FLAIR and T2-weighted sequences (due to demyelination and oedema), hypointense lesions on T1-weighted images (indicating cystic necrotic degeneration), and diffusion-restricted lesions on DWI with low apparent diffusion coefficient. The “sandwich sign” on sagittal T1-weighted sequences is pathognomonic for MBD with central layer abnormality and sparing of the dorsal and ventral layers. [9] The differential diagnoses of CC lesions in other clinical settings are multiple sclerosis, diffuse axonal injury, progressive multifocal leukoencephalopathy, and callosal infarction. MBD is distinguished by symmetrical, middle-layered and isolated CC lesions. Cortical involvement in MBD is rare. [10] Wernicke-Korsakoff involves other anatomical structures.
Treatment with thiamine administration is advised. [11] Our patient received parenteral thiamine and had a favourable course with complete clinical recovery.
In conclusion, isolated CC lesions in an alcoholic patient should raise suspicion of MBD, which is a rare complication of chronic alcoholism, characterised by primary toxic demyelination and necrosis of the CC.

Differential Diagnosis List
Marchiafava-Bignami disease
Multiple sclerosis
Diffuse axonal injury
Progressive multifocal leukoencephalopathy
Callosal infarction
Final Diagnosis
Marchiafava-Bignami disease
Case information
URL: https://eurorad.org/case/15688
DOI: 10.1594/EURORAD/CASE.15688
ISSN: 1563-4086
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