Marchiafava-Bignami disease mimics motor neuron disease: Case report

BMC Neurology (Impact Factor: 2.04). 12/2013; 13(1):208. DOI: 10.1186/1471-2377-13-208
Source: PubMed


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

Download full-text


Available from: Hideki Shimura, Jan 14, 2014
  • Source

    Preview · Article · Jan 2014
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: A male, 62-year-old patient was admitted to hospital due to dizziness and gait disturbance for 10 days. The patient had fallen a few times due to the gait instability, which was associated with stiffness and memory loss. The patient had undergone cardiac carcinoma surgery three years previously and had no drinking history. Physical examination revealed that the patient was lucid when conscious but exhibited slurred speech, apathy and cognitive impairment. The finger-to-nose and rapid alternating movement tests showed the patient to be slightly clumsy. Magnetic resonance imaging revealed symmetric abnormal signals in the splenium of the corpus callosum, and the diagnosis was Marchiafava-Bignami disease (MBD). The patient recovered following the administration of vitamin B and other treatments. The patient had long-term appetite loss. A brain myelin metabolism disorder caused by long-term malnutrition and leading to demyelinating changes in the brain may have been the cause of the MBD of this patient. Clinicians should increase awareness of this disease and should not ignore the diagnosis of it, even when the patient lacks a drinking history. Early diagnosis and treatment can improve the prognosis of the patient.
    Full-text · Article · Feb 2015 · Experimental and therapeutic medicine