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August 8, 2005
Letter to the Editor

Marchiafava–Bignami disease: Diffusion-weighted MRI in corpus callosum and cortical lesions

August 9, 2005 issue
65 (3) 475-477

Abstract

The clinical diagnosis of Marchiafava–Bignami disease (MBD) can be difficult. Acute demyelination of the corpus callosum is characteristic of the disease. The authors report the use of MR diffusion-weighted imaging (DWI) in six cases of acute MBD. They show that apparent diffusion coefficient restriction of the corpus callosum and cortical lesions were associated with a higher mortality rate and more severe cognitive sequelae.

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References

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Heinrich A, Runge U, Khaw AV. Clinicoradiologic subtypes of Marchiafava-Bignami disease. J Neurol 2004;251:1050–9.
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Chang KH, Cha SH, Han MH, Park SH, Nah DL, Hong JH. Marchiafava-Bignami disease: serial changes in corpus callosum on MRI. Neuroradiol 1992;34:480–482.
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Bourekas E, Varakis K, Bruns D, et al. Lesions of the corpus callosum: MR imaging and differential considerations in adults and children. AJR Am J Roentgenol 2002;179:251–257.
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Gass A, Birtsch C, Oster M, Schwartz A, Hennerici MG. Marchiafava-Bignami disease: reversibility of neuroimaging abnormality. J Comput Assist Tomogr 1998;22:503–504.
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Castaigne P, Buge A, Cambier J, Escourolle R, Rancurel G. Clinical and neuropathological studies of 10 cases of Marchiafava-Bignami disease. Neurol Neurochir Pol 1973;7:183–4.
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Inagaki T, Saito K. A case of Marchiafava-Bignami disease demonstrated by MR diffusion-weighted image. No To Shinkei 2000;52:633–7.
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Letters to the Editor
6 December 2005
Marchiafava–Bignami disease: Diffusion-weighted MRI in corpus callosum and cortical lesions
Alexander V. Khaw, Dept. of Neurology, Ernst Moritz Arndt University
Alexander Heinrich

Ménégon et al recently reported abnormalities on diffusion-weighted MRI (DWI) in a series of six patients with Marchiafava-Bignami disease (MBD. [1]

Partial callosal signal abnormality is common on FLAIR and T2-weighted MRI in past reports.[2,4] In this study, DWI lesions involved the entire corpus callosum (CC) in all patients, prompting the authors to suggest that complete affection of the CC may be obligatory in MBD. Although sensitivity of DWI is likely superior to conventional MRI, this hypothesis appears questionable on the background of histopathological results reporting the sparing of callosal areas from demyelination or necrosis [3] and recent studies showing partial involvement on both conventional MRI and DWI. [4,5] Moreover, Ménégon et al reportedly acquired conventional and DWI images in axial slices only, an important limitation for comprehensive evaluation of the entire CC.

The authors proposed that markedly reduced callosal apparent diffusion coefficient (ADC) may indicate poor prognosis, which contradicts recent reports. [4] A prospective, multicenter MRI study including sagittal imaging may provide the most comprehensive results on the prognostic value of DWI in MBD. Correlative studies establishing a link between ADC changes and histological findings in MBD would be important but difficult due to the lack of an appropriate animal model and decreasing autopsy rates in this rare disorder.

The lateral-frontal predilection of cortical diffusion abnormalities [1,5] is consistent with the autopsy finding of Morel's laminar sclerosis in the same areas. [3,5] Nevertheless, evidence that diffusion abnormalities correspond to this highly specific form of cortical injury is insufficient as comparable DWI lesions consistent with cytotoxic edema have also been demonstrated following cerebral hypoxia, prolonged seizure activity or in Wernicke-Korsakoff disease which may accompany severe cases of MBD [1,4].

The discrepancy between MRI studies suggesting poor prognosis in cases with cortical involvement [1,5] and autopsy series reporting Morel's laminar sclerosis predominantly in cases with a mild course [3] also stresses the need for direct comparison of cortical diffusion abnormalities with histopathologic findings.

Assuming that the observed cortical MRI lesions reflect a pathology corresponding to Morel's laminar sclerosis, demonstration of the latter in the acute stage would indicate that cortical pathology in MBD may not result from secondary cortical neuronal degeneration due to callosal lesions as proposed earlier. [3]

References

1. Menegon P, Sibon I, Pachai C, Orgogozo JM, Dousset V. Marchiafava- Bignami disease: Diffusion-weighted MRI in corpus callosum and cortical lesions. Neurology 2005; 65:475-477.

2. Heinrich A, Runge U, Khaw AV. Clinicoradiologic subtypes of Marchiafava -Bignami disease. J Neurology 2004; 251:1050-1059.

3. Brion S. Marchiafava-Bignami disease; in: Vinken PJ, Bruyn GW (eds): Handbook of Clinical Neurology 1977, Amsterdam, North Holland: pp 317-329.

4. Hlaihel C, Gonnaud PM, Champin S, Rousset H, Tran-Minh VA, Cotton F. Diffusion-weighted magnetic resonance imaging in Marchiafava-Bignami disease: follow-up studies. Neuroradiology 2005; 47:520-24.

5. Johkura K, Naito M, Naka T. Cortical involvement in Marchiafava-Bignami disease. Am J Neuroradiol 2005; 26:670-673.

The authors report no conflicts of interest.

6 December 2005
Reply from Authors
Patrice Ménégon, Service de Neuroradiologie, Hôpital Pellegrin,
Igor Sibon , Chahin Pachai , Jean Marc Orgogozo, Vincent Dousset

We thank Drs. Khaw et al for their comments on our article where we describe MRI abnormalities in the sub-acute phase of MBD using conventional and DWI in axial and sagittal planes. [1] We observed diffuse corpus callosum abnormalities in all the cases but never suggested the complete lesion of the CC as a new mandatory diagnosis criteria of MBD.

The low number of subjects, the anatomo-pathological data, and the recent descriptions of partial corpus callosum diffusion hyper intensities in MBD do not allow us to draw this conclusion. [2] Our results also suggested a relationship between the values of the ADC/NAWM ratio and the long term prognosis but with possible bias related to the low number of subjects.

The gold standard for a such study would be to compare the MRI data to the brain anatomo-pathological lesions observed in subjects who died during follow-up. This would also answer questions concerning the molecular basis of the cortical lesions observed in our patients. Similar cortical lesions to those observed in our study have been reported in one case without history of seizure or cerebral anoxia. As in our report, this suggests a possible primary cortical involvement in MBD. [3]

Two arguments promote the hypothesis of poor prognosis associated with cortical lesions: the poor clinical outcome of patients in Johkura et al study (5) and of the three patients in our study; and the high frequency of cortical lesions in autopsy series. [6]

We agree with the authors that a multicenter MRI study using the uniform modalities of DWI acquisition and analysis would provide more comprehensive results on the prognosis value of MRI in the earlier phase of MBD.

References

6. Castaigne P, Buge A, Cambier J, Escourolle R, Rancurel G. Clinical and neuropathological studies of 10 caes of Marchiafava-Bignami diesase. Neurol Neurochur Pol 1973;7:183-184.

The authors report no conflicts of interest.

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Published In

Neurology®
Volume 65Number 3August 9, 2005
Pages: 475-477
PubMed: 16087921

Publication History

Published online: August 8, 2005
Published in print: August 9, 2005

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Authors

Affiliations & Disclosures

P. Méneégon, MD, PhD
From the Service de Neuroradiologie (Drs. Ménégon and Dousset), Hôpital Pellegrin and Federation of Clinical Neurosciences (Drs. Sibon and Orgogozo), CHU Bordeaux, France; and Theralys Inc. (Dr. Pachai), Lyon, France.
I. Sibon, MD, PhD
From the Service de Neuroradiologie (Drs. Ménégon and Dousset), Hôpital Pellegrin and Federation of Clinical Neurosciences (Drs. Sibon and Orgogozo), CHU Bordeaux, France; and Theralys Inc. (Dr. Pachai), Lyon, France.
C. Pachai, MD, PhD
From the Service de Neuroradiologie (Drs. Ménégon and Dousset), Hôpital Pellegrin and Federation of Clinical Neurosciences (Drs. Sibon and Orgogozo), CHU Bordeaux, France; and Theralys Inc. (Dr. Pachai), Lyon, France.
J. M. Orgogozo, MD, PhD
From the Service de Neuroradiologie (Drs. Ménégon and Dousset), Hôpital Pellegrin and Federation of Clinical Neurosciences (Drs. Sibon and Orgogozo), CHU Bordeaux, France; and Theralys Inc. (Dr. Pachai), Lyon, France.
V. Dousset, MD, PhD
From the Service de Neuroradiologie (Drs. Ménégon and Dousset), Hôpital Pellegrin and Federation of Clinical Neurosciences (Drs. Sibon and Orgogozo), CHU Bordeaux, France; and Theralys Inc. (Dr. Pachai), Lyon, France.

Notes

Address correspondence and reprint requests to Dr. Patrice Ménégon, Service de Neuroradiologie, Hôpital Pellegrin, Place Amélie Raba-Léon, 33076 Bordeaux, France; e-mail: [email protected]

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