Marchiafava Bignami Disease in chronic alcoholic patient

Posted By tekwani par
Marchiafava Bignami Disease in chronic alcoholic patient

Images shows in a 45 years old chronic alcoholic patient presented with seizures, altered sensorium and bilateral lower limb paresis , T1W image shows hypointense corpus callosum and periventricular white matter with corresponding T2 and FLAIR images shows hyperintense signal with true restricted diffusion in DWI image. Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism characterized by demyelinization and necrosis of the entire length and middle layer of corpus callosum(1,4). Most accepted etiologic factor is the in multiple vitamin B deficiency(3). Clinical manifestations(6): 1) Acute state :Seizures, alterations of consciousness and death may occur. 2)Subacute state: Characterized by mental confusion, behavioural disorders, memory deficits, cerebellar signs and interhemispheric disconnection. 3) Chronic state: Mild dementia. Clinoco-radiological Subtypes of MBD(5,6): 1)Type A : Characterized by alterations of consciousness and diffuse swelling of the entire corpus callosum on imaging. 2) Type B : Mild impairment of consciousness and small callosal lesions associated with good prognosis. Early diagnosis and treatment can improve clinical outcome. MRI findings can help to differentiate MBD from other corpus callosal lesions and also from alcohol related disorders such as Wernicke encephalopathy . On MRI images (2) ( Figure 1): T1W: Shows confluent hypointense signal intensity in middle layer of corpus callosum. T2 and FLAIR : Shows hyperintense signal in middle layer of corpus callosum (Sandwich sign) . T1+C: No significant contrast enhancement. DWI : In the acute phase ,shows restricted diffusion because of cytotoxic edema caused by increase of extracellular glutamate that binds NMDA (N-methyl-D-aspartic acid) receptors inducing calcium entry and finally apoptosis without brain ischemia. ADC: Low apparent diffusion coefficient To differentiate MBD lesions to other possible causes of callosal damage we have to consider their specific localization inside the corpus callosum. The genu is usually the most involved structure followed by the splenium.. The entire corpus callosum may also be involved. In case of chronic stage, corpus callosum degenerates and separates into three layers with necrotic cavities mainly in the middle layer. Cortical involvement is extremely rare and when present, it is usually localized in lateral-frontal regions.(4) In conclusion even if it is not possible to identify pathognomonic characteristics of MBD lesions, the clinical aspects and neuroimaging pattern may helpful for the diagnosis. Final Diagnosis: Marchiafava-Bignami disease type A in chronic phase

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  • Roland Talanow 2016-04-13 20:52:38

    If interested in submitting to the JRCR, what makes this case report worthy? Thank you for showing.

    If showing DWI, I suggest showing also ADC maps.

    Reply