Clinicoradiologic subtypes of Marchiafava-Bignami disease

J Neurol. 2004 Sep;251(9):1050-9. doi: 10.1007/s00415-004-0566-1.


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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Aged
  • Female
  • Hemoglobinuria, Paroxysmal / classification*
  • Hemoglobinuria, Paroxysmal / diagnosis
  • Hemoglobinuria, Paroxysmal / diagnostic imaging*
  • Humans
  • Magnetic Resonance Imaging / methods
  • Male
  • Middle Aged
  • Radiography