Marchiafava Bignami disease
Overview
Marchiafava-Bignami disease (MB) refers to a disorder resulting in demyelination of the corpus callosum. It was first described by two Italian pathologists who identified it in the autopsies of three patients who presented in status epilepticus and subsequently developed coma (1). All three patients were chronic alcoholics and had consumed considerable amounts of red wine. We present the MR imaging findings in two patients with MB, discuss the differential diagnosis, and review the disease.
Symptoms
* Mental changes * Excitement * Apathy * Convulsions * Tremors
Causes
It is classically associated with chronic alcoholics.
Diagnosis
Diagnosis is made on the basis of clinical findings in combination with imaging features. Patients present acutely with mental confusion, disorientation, neurocognitive deficits, and seizures. Muscle rigidity and facial trismus may be severe. Most patients presenting with the acute type of MB will go into coma and eventually die, although as illustrated in this report, some survive. Acute MB may be difficult to distinguish from Wernicke encephalopathy and may occur together with it. Patients with Wernicke encephalopathy have ataxia, ophthalmoplegia, nystagmus, and confusion. Patients with the subacute type of MB—which is characterized by dementia, disarthria, and muscle hypertonia—may survive for years. The chronic form of MB is characterized by a chronic dementia.
Prognosis
The 'prognosis' of Marchiafava-Bignami disease usually refers to the likely outcome of Marchiafava-Bignami disease. The prognosis of Marchiafava-Bignami disease may include the duration of Marchiafava-Bignami disease, chances of complications of Marchiafava-Bignami disease, probable outcomes, prospects for recovery, recovery period for Marchiafava-Bignami disease, survival rates, death rates, and other outcome possibilities in the overall prognosis of Marchiafava-Bignami disease. Naturally, such forecast issues are by their nature unpredictable.