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Open Access Case report

Marchiafava-Bignami disease mimics motor neuron disease: case report

Yasunobu Hoshino1, Yuji Ueno1*, Hideki Shimura1, Nobukazu Miyamoto1, Masao Watanabe1, Nobutaka Hattori2 and Takao Urabe1

Author Affiliations

1 Department of Neurology, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu, Chiba 279-0021, Japan

2 Department of Neurology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo, Tokyo 113-8421, Japan

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BMC Neurology 2013, 13:208  doi:10.1186/1471-2377-13-208

Published: 21 December 2013

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.

Keywords:
Marchiafava-Bignami disease; Motor neuron disease; Amyotrophic lateral sclerosis; Upper motor neuron signs; Lower motor neuron signs; Chronic alcoholism