Marchiafava-Bignami disease mimics motor neuron disease: case report

Abstract

Background: Marchiafava-Bignami disease (MBD) is a rare neurologic complication of chronic alcohol consumption that is characterized by callosal lesions involving demyelination and necrosis. Various reversible neurologic symptoms are found in patients with MBD. Dysarthria and dysphagia are found in various neurological diseases.

Case presentation: We report a 51-year-old man with chronic alcoholism and malnutrition who progressively developed dysarthria and dysphagia. On admission, the patient was alert with mild cognitive dysfunction. The facial expression was flat, and there was weakness of the orbicularis oris bilaterally. The patient's speech was slurred, there was difficulty swallowing, and the gag reflex and palate elevation were poor. The jaw jerk reflex was brisk and the snout reflex was positive. Neither tongue atrophy nor fasciculation were found. Bilateral upper and lower limb weakness with increased bilateral upper limb reflexes and Babinski reflexes were found. Because he had progressive dysarthria and dysphagia with upper and lower motor neuron signs, the initial diagnosis was motor neuron disease. However, electrophysiological analysis was normal. The vitamin B1 level was 14 ng/mL (normal: >24 ng/mL), and MRI revealed hyperintense lesions in the splenium of the corpus callosum and the primary motor cortices bilaterally. After vitamin B therapy for 17 days, the neurological disorders alleviated concurrently with disappearance of the lesions on MRI, which led to the definitive diagnosis of MBD.

Conclusions: MBD presenting with these lesions can mimic motor neuron disease clinically.

Figure 1

Admission MRI. Representative images of brain MRI on admission including fluid attenuated inversion recovery (FLAIR) (A), diffusion-weighted image (DWI) (B), and the apparent diffusion coefficient (ADC) map (C), showing hyperintense lesions in the precentral gyrus bilaterally and the splenium of the corpus callosum.

Figure 2

MRI after therapy. Representative FLAIR images (A), DWI (B), and ADC map (C) 17 days after admission, showing disappearance of the hyperintense lesions.