[Susac's syndrome: improvement with combined cyclophosphamide and intravenous immunoglobulin therapy].

Abstract

We report a case of Susac's syndrome characterized by subacute encephalopathy, bilateral hearing loss and multiple bilateral branch retinal artery occlusions in a forty-year-old-white-woman. Brain Magnetic Resonance Imaging showed on T2-weighted images multiple, punctate areas of increased signal intensity in periventricular white matter, gray matter and brainstem most of them being enhanced by gadolinium. Cerebrospinal fluid was acellular but with an increased protein level (1.66 g/l). Treatment with cyclophosphamid and intravenous immmunoglobulin resulted in dramatic improvement of the clinical status over the following months and CSF normalization.

• Authors: C Papeix, L Laloum, A Richet, D Ayache, A Moulignier, F Héran, P Bakouche, O Gout
• Journal: Revue neurologique (Rev Neurol (Paris)) Vol. 156 Issue 8-9 Pg. 783-5 (Sep 2000) ISSN: 0035-3787 [Print] France
• Vernacular Title: Syndrome de Susac: un cas d'évolution favorable sous cyclophosphamide et immunoglobulines intraveineuses.
• PMID: 10992124 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• Immunoglobulins, Intravenous
• Immunosuppressive Agents
• Cyclophosphamide

Topics

• Adult
• Arterial Occlusive Diseases (drug therapy)
• Brain (pathology)
• Brain Diseases (drug therapy)
• Cyclophosphamide (therapeutic use)
• Drug Therapy, Combination
• Female
• Hearing Loss, Sensorineural (drug therapy)
• Humans
• Immunoglobulins, Intravenous (therapeutic use)
• Immunosuppressive Agents (therapeutic use)
• Magnetic Resonance Imaging
• Retinal Vessels (pathology)
• Syndrome