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MARCHIAFAVA-BIGNAMI DISEASE? Dec, 24th, 2003 Hospital Virgen de las Nieves Servicio de Neurologia

This case study discusses a 42-year-old heavy drinker who presented with subacute encephalopathy. The patient showed no evidence of systemic disease and had no focal or lateralizing signs. Blood analysis revealed high MCV and serum gammaglobulins. CSF analysis was normal, and imaging studies (CT scan and MRI) showed mild improvement after a short course of metil-prednisolone. The considered diagnosis is Marchiafava-Bignami disease, but other possibilities such as demyelinating disease, neoplastic disease, vascular conditions, and Whipple disease are also suggested. Suggestions for further evaluation or diagnosis are welcomed.

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MARCHIAFAVA-BIGNAMI DISEASE? Dec, 24th, 2003 Hospital Virgen de las Nieves Servicio de Neurologia

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  1. MARCHIAFAVA-BIGNAMI DISEASE? Dec, 24th, 2003 Hospital Virgen de las Nieves Servicio de Neurologia Granada. Spain

  2. 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

  3. CT scan without contrast.-

  4. CT scan after contrast.-

  5. MRI sagital T1 w.-

  6. MRI axial T2 w and PD (1).-

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

  8. 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!

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