Use of high-dose cortisone in a patient with Marchiafava-Bignami disease

Gerlach, A; Oehm, E.; Wattchow, Jens; Ziyeh, Sargon; Glocker, F.X.; Els, Thomas

doi:10.1007/s00415-003-1072-6

Cited by 25 publications

(17 citation statements)

References 5 publications

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“…Hyperintense signal abnormality of the entire corpus callosum with a variable degree of swelling was reported in all of these patients. In three cases lacking results of sagittal MRI in the original manuscript, involvement of the entire corpus callosum was confirmed by autopsy [35] or sagittal [10] or coronal [21] imaging results provided to the authors of this article following direct communication. Focal areas of T2-shortening indicating callosal hemorrhage were found in 2 patients [11,31].…”

Section: Methodsmentioning

confidence: 98%

Clinicoradiologic subtypes of Marchiafava-Bignami disease

2004

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The clinical diagnosis of Marchiafava-Bignami disease (MBD) has considerably changed during recent decades with brain MRI providing the opportunity of a reliable in-vivo diagnosis. However, semiologic and neuroimaging characteristics of the currently known spectrum of MBD have not been investigated systematically, and knowledge of clinicoradiologic associations is sketchy. We report an illustrative case with limited callosal involvement on MRI and a favorable outcome and have reviewed literature on clinical and radiologic features in 50 cases of MBD diagnosed in vivo since 1985. Our reviewed data suggest the differentiation of two clinicoradiologic subtypes: Type A is characterized by major impairment of consciousness, T2-hyperintense swelling of the entire corpus callosum on early MRI and poor outcome. Type B shows at most slight impairment of consciousness, partial callosal lesions on MRI and a favorable outcome. Differentiation of these clinicoradiologic subtypes may help resolve inconsistencies of the established clinical classification resulting from new insights into the clinical course and prognosis of MBD by structural neuroimaging.

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Section: Methodsmentioning

confidence: 98%

Clinicoradiologic subtypes of Marchiafava-Bignami disease

2004

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“…Regarding treatment of suspected MBD, which is similar to treatment of the Wernicke‐Korsakoff syndrome, the most commonly applied medications include folate (B9), thiamine (B1), vitamin B 12, and other B vitamins . Other authors reported, that a high‐dose corticosteroid therapy led to clinical improvement . So far, however, there is no specific proven treatment for MBD.…”

Section: Conclusion and Review Of The Literaturementioning

confidence: 99%

Acute Marchiafava‐Bignami Disease with Extensive Diffusion Restriction and Early Recovery: Case Report and Review of the Literature

Wenz

Eisele

Artemis

et al. 2012

Journal of Neuroimaging

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Marchiafava-Bignami disease (MBD) is a neurological disorder that has been found to be associated with chronic alcoholism and malnutrition. MBD classically results in acute edema and demyelination of the corpus callosum. Edema of the complete corpus callosum has been described to be an unfavorable prognostic factor. We present an acute onset of MBD with diffusion restriction of the complete corpus callosum and symmetric bilateral extension into the semioval center, that almost completely resolved clinically as well as in MRI only 3 days later. With early detection and treatment, the prognosis of MBD may be good even in cases with severe diffusion restriction of the complete corpus callosum.

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“…Treatment of MBD was usually empirically initiated with multivitamins [1, 6, 13], corticosteroids [6, 14, 15], stabilization of plasma glucose [6], and supportive care. Early correct diagnosis and prompt appropriate management may be critical in reversing the underlying pathophysiology in the early stage.…”

Section: Discussionmentioning

confidence: 99%

Marchiafava-Bignami Disease in a Nonalcoholic Diabetic Patient

Yadala

Luo

2013

Case Reports in Neurological Medicine

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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.

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Use of high-dose cortisone in a patient with Marchiafava-Bignami disease

Cited by 25 publications

References 5 publications

Clinicoradiologic subtypes of Marchiafava-Bignami disease

Clinicoradiologic subtypes of Marchiafava-Bignami disease

Acute Marchiafava‐Bignami Disease with Extensive Diffusion Restriction and Early Recovery: Case Report and Review of the Literature

Marchiafava-Bignami Disease in a Nonalcoholic Diabetic Patient

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