[Susac syndrome: study of five cases].
BACKGROUND
Susac syndrome is a rare microangiopathy, responsible for small cerebral, retinal and cochlear infarcts. The classic clinical triad includes multiple neurologic signs (from headaches to coma), retinal branch occlusions and sensorineural hearing loss.


METHODS
We report a series of five patients with Susac syndrome followed in our department from 1997 to 2007.


RESULTS
There were four women and one man (mean age at onset: 35.2 years). Clinical symptoms at onset were neurological (n=1), ophthalmological (n=1), auditory (n=1) and clinical triad (n=2). Neurologic symptoms included encephalopathy (n=2), headache (n=5), transient ischemic attacks (n=1). Brain MRI showed T2 lesions in the white and grey matter, corpus callosum and gadolinium-enhanced punctiform lesions. Cerebrospinal fluid contained an elevated protein level in three cases. Immunologic treatments (steroids [n=4], cylophosphamid [n=3], intravenous immunoglobulins [n=5]) associated with aspirin and/or oral anticoagulants, despite early relapses (n=2), led to dramatic clinical improvement (n=5).


CONCLUSIONS
Due to its polymorphism the SS is difficult to diagnose when the clinical triad is lacking. In the absence of clinical trial and consensus treatment is empiric and based on supposed pathogenesis.
Maillart
E
E
Service de neurologie, fondation ophtalmologique Adolphe-de-Rothschild, 25-29, rue Manin, 75940 Paris cedex 19, France. elisabethmaillart@yahoo.fr
Deschamps
R
R
Moulignier
A
A
Vignal-Clermont
C
C
Obadia
M
M
Le Mer
Y
Y
Laloum
L
L
Gout
O
O
D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use; D001921:Brain / Q000473:pathology; D002544:Cerebral Infarction / Q000188:drug therapy / Q000473:pathology*; D015834:Cochlear Diseases / Q000188:drug therapy / Q000473:pathology*; D003128:Coma / Q000209:etiology; D038524:Diffusion Magnetic Resonance Imaging; D004596:Electroretinography; D005260:Female; D006261:Headache / Q000209:etiology; D034381:Hearing Loss / Q000209:etiology; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D018810:Magnetic Resonance Angiography; D008297:Male; D008875:Middle Aged; D012160:Retina / Q000473:pathology; D012164:Retinal Diseases / Q000188:drug therapy / Q000473:pathology*; D013577:Syndrome; D055815:Young Adult
D000925:Anticoagulants; D007155:Immunologic Factors