Marchiafava-Bignami Disease in a Nonalcoholic Diabetic Patient

Abstract

Marchiafava-Bignami disease (MBD) is a rare neurological disorder mostly seen in alcoholic and malnourished patients with a pathognomonic hallmark of corpus callosum demyelination. MBD in nonalcoholics without malnutrition has rarely been reported. We report a case of MBD in a diabetic patient, without alcoholism or malnutrition, caused by a wide range of glycemic level fluctuations. A 38-year-old man presented with sudden onset of alteration in speech and multiple falls in three days. Neurologic examination showed dysarthria, dysmetria, and ataxia but, otherwise, normal cranial nerves, motor and sensory functions, and tendon reflexes. Brain MRI showed symmetric abnormalities in the splenium of the corpus callosum. In addition, demyelination was also observed in bilateral posterior limbs of the internal capsule and brachium ponti. His symptoms significantly improved after stabilization and normalization of his plasma glucose level and administration of multivitamins and corticosteroids. The underlying pathophysiology of the development of MBD in our case is likely to be osmotic stress from a wide range of glycemic fluctuations causing structural and functional disturbance of oligodendrocytes, which may be reversible in its early stage.

From Mild Gait Difficulties to a Sudden Coma: A Rare Case of Marchiafava-Bignami Disease

“…This is believed to originate from fluctuations in glycemia, causing osmotic stress in oligodendroglial cells and leading to callosal myelinolysis. [3][4][5] MBD has a variable clinical spectrum, and 2 types of classifications are currently in use. The first one recognizes 3 subtypes and is mostly based on the acuteness of onset and disease progression: (1) acute onset MBD generally presents with sudden loss of consciousness with seizures and rapid progression to coma, (2) subacute forms can have varying prodromes ranging from depression to ataxia or spasticity, and (3) chronic MBD presents as a progressive dementia, generally with behavior abnormalities, hallucinations, and delusions.…”

“…This is believed to originate from fluctuations in glycemia, causing osmotic stress in oligodendroglial cells and leading to callosal myelinolysis. 3 – 5 …”

Disconnected Motor Intention and Spatial Attention in a Case of Probable Marchiafava-Bignami Disease

“…Chronic alcoholism is the most common cause of MBD, but it can also result from malnutrition and glucose fluctuation (Kilinc et al, 2015; Yadala and Luo, 2013). Our patient’s hyperglycemia may have contributed to his MBD.…”

Marchiafava-Bignami disease in a trauma patient

“…[ 1 ] A relatively rare condition classically found in chronic alcoholics, a small number of cases of MBD has been reported in non-alcoholics. [ 2 3 4 5 6 7 8 9 10 11 12 ] Clinical features classically include reduced consciousness or coma, neuropsychiatric manifestations, seizures, hemiparesis, dysarthria, ataxia and apraxia, and symptoms of hemispheric disconnection. It was historically confirmed by pathological diagnosis; however, magnetic resonance imaging (MRI) has become the diagnostic method of choice by easily revealing the pathognomonic features.…”