MARCHIAFAVA-BIGNAMI DISEASE?

Dec, 24th, 2003

Hospital Virgen de las Nieves

Servicio de Neurologia

Granada. Spain

•42 years old gentleman.

•Heavy drinker

•Subacute encephalopaty (3 weeks)

•No evidence of systemic disease

•Absence of focal or lateralizing signs (alexia and agraphia not specifically sought)

•Blood analysis normal except a high MCV and serum gammaglobulines (without monoclonal peak); chest Rx and EKG normal

•CSF acelular, normal sugar; protein 0’7 gr/l

•CT scan and MRI

CT scan without contrast.-

CT scan after contrast.-

MRI sagital T1 w.-

MRI axial T2 w and PD (1).-

MRI axial T2 w and PD (2).- (No changes after contrast injection)

Mild improvement after short course of metil-prednisolone

CONSIDERED DIAGNOSIS.-

Marchiafava-Bignami disease (atypical)

Others:

•Demyelinating disease (PML, ADEM,...)

•Neoplastic disease (glioma, lymphoma,...)

•Vascular

•Some zebra: Whipple disease

SUGGESTIONS ARE WELCOME!