Diagnosis and management of Marchiafava-Bignami disease: a review of CT/MRI confirmed cases

Abstract

Objective Marchiafava-Bignami disease (MBD) is a rare condition mainly associated with alcoholism, although it may be mimicked by several other disorders that cause corpus callosum lesions. Our objective was to identify helpful features for differential diagnosis and assess whether any treatment can be recommended. Methods We reviewed 122 reports containing data on 153 subjects with confirmed MBD that was associated with either alcoholism or malnutrition and 20 reports with data on 53 subjects with conditions mimicking MBD. All the cases had been verified ante mortem by brain imaging. Unconditional logistic regression was used to demonstrate factors that were associated with the outcome of MBD. Results The mimicking conditions were differentiated from MBD by the occurrence of solitary and rapidly disappearing splenial lesions; fewer signs and symptoms with exception of seizures, hemiparesis, and tetraparesis; nystagmus; and rapid, complete recovery. MBD occurred most frequently among alcoholics, but it was also reported in 11 non-alcoholics (7·2% of all the MBD cases). A better outcome was observed among those who were treated within two weeks after onset of symptoms with parenteral thiamine (p=0·033). Conclusions As thiamine deficiency is frequently associated with alcoholism, malnutrition and prolonged vomiting, we recommend prompt treatment of MBD with parenteral thiamine in such subjects. Recovery should be followed by repeated neuropsychological and MRI examinations, preferably using diffusion tensor imaging.

The Whole Spectrum of Alcohol-Related Changes in the CNS: Practical MR and CT Imaging Guidelines for Daily Clinical Use

“…In the largest Marchiafava-Bignami study to date, patients with the non-alcohol-induced pseudoform fully recovered in over 80 % of cases. The reverse was true in alcohol-induced cases in which 89 % of patients had long-term neurological damage or died [35]. Etiologically the disease may be the result of a thiamine or vitamin B complex deficiency.…”

“…A rapidly progressing form in chronic alcohol abuse (approx. 90 % of all cases) can be differentiated from a non-alcohol-induced pseudoform with a significantly better prognosis [35]. The alcohol-induced form is not necessarily triggered by acute intoxication.…”

“…In contrast to the alcohol-induced form, the pseudoform usually exclusively affects the central splenium ( • " Fig. 6a-c) [35], Isolated edematous affection of the splenium is prognostically favorable in MarchiafavaBignami syndrome. However, extensive lesions, contrast enhancement, hemorrhages, and focal hypoperfusion are prognostically unfavorable as are extracallosal, e. g. cortical, lesions [35,38].…”

“…Etiologically the disease may be the result of a thiamine or vitamin B complex deficiency. However, the pathogenesis has not been sufficiently understood [35]. Diagnosis should be primarily made via MRI.…”

Marchiafava-Bignami Disease: Uncertain MRI Predictors of Outcome

“…Occasionally, similar lesions can involve other areas, such as anterior and posterior commissures, brachium pontis [8], optic chiasm, putamen [9], and frontal cortex [1,10]. In the chronic phase, because of the progression of demyelinating process, it possible to observe necrotic and cystic areas, haemosiderin stores, atrophy of corpus callosum [11,12].…”