[Susac's syndrome: improvement with combined cyclophosphamide and intravenous immunoglobulin therapy].
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.
Papeix
C
C
Service de Neurologie, Fondation Ophtalmologique Adolphe de Rothschild, Paris.
Laloum
L
L
Richet
A
A
Ayache
D
D
Moulignier
A
A
Héran
F
F
Bakouche
P
P
Gout
O
O
D000328:Adult; D001157:Arterial Occlusive Diseases / Q000188:drug therapy*; D001921:Brain / Q000473:pathology*; D001927:Brain Diseases / Q000188:drug therapy*; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000188:drug therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D012171:Retinal Vessels / Q000473:pathology; D013577:Syndrome
D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide