Marchiafava-Bignami Disease Clinical Presentation

Updated: Jun 27, 2017
  • Author: Cortney Lyford, MD; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
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Presentation

History

Most patients diagnosed with Marchiafava-Bignami disease (MBD) have a history of alcoholism and poor nutrition. The tempo of onset and the range of clinical symptoms vary. Some patients present to the hospital with sudden onset of stupor or coma, and some present with seizures. Other patients have acute, subacute, or chronic onset of dementia and/or gait problems. Spasticity often complicates the gait disorder. Psychiatric disturbances, incontinence, hemiparesis, aphasia, dysarthria, apraxia, and signs of interhemispheric disconnection are also observed.

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Physical Examination

Although the physical findings in MBD are typically nonspecific, a good physical examination may offer clues to the diagnosis. However, patients with severe alcoholism who have this syndrome frequently have other problems, such as subdural hemorrhage, Wernicke-Korsakoff syndrome, and alcoholic liver disease. Therefore, the diagnosis is not often clear.

General appearance and constitution

Patients later found to have MBD frequently present to an emergency department in a disheveled condition suggestive of chronic problems with alcohol.

Mental status

Patients can be lethargic, stuporous, or even unconscious (coma or seizures). If a patient is sufficiently alert for extensive neuropsychological testing, testing for ideomotor apraxia (ie, inability to perform motor activities that is not explainable by overt motor or sensory loss) may be revealing. [21]

Apraxia of the left (or nondominant) hand suggests interhemispheric disconnection (ie, impaired transfer of information from the left hemisphere to the right hemisphere). Damage to the fibers of the corpus callosum is the cause.

Inability to retain new information (ie, Korsakoff syndrome, the chronic phase of Wernicke-Korsakoff syndrome) and delirium tremens should suggest alcoholism and prompt the examiner to consider other alcohol-related problems, such as MBD. Dementia and aphasia have been noted in some patients with this disease.

Cranial nerves

Nystagmus or disconjugate eye movements, possibly together with confusion and/or ataxia, may indicate the acute/subacute encephalopathic Wernicke phase of the Wernicke-Korsakoff syndrome, which should prompt the examiner to consider MBD.

Motor function

Tremors, weakness, spasticity, and gait abnormalities, although nonspecific, have been seen in patients with MBD.

Delirium tremens is another alcohol-induced problem that patients with MBD may have. Currently, no evidence suggests that the presence of one is either positively or negatively correlated with the presence of the other.

Sensory function

Sensory loss may suggest an alcoholic neuropathy.

Cerebellar functions

Wide-based gait and truncal ataxia suggest alcoholism.

Reflexes

Alcoholic neuropathy can cause a loss of deep tendon reflexes and, therefore, prompt the consideration of MBD in some patients. The presence or absence of Babinski signs is not known to be specifically related to MBD.

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