Susac syndrome in four male patients.
OBJECTIVE
To report the clinical and imaging features in four male patients presenting with Susac syndrome, a microangiopathy affecting the brain, the retina, and the cochlea.


METHODS
Retrospective review of clinical data, fluorescein angiograms, and magnetic resonance imaging findings in these four cases.


RESULTS
All four patients were young men (range, 20-35 years). The axiomatic triad of ocular, cochlear, and neurologic involvement was present in three patients. Neurologic symptoms were absent in the fourth one. Fluorescein angiography showed arteriolar wall hyperfluorescence in all four patients. Magnetic resonance images showed in three patients multifocal hyperintense lesions in the white matter and the corpus callosum with typical involvement of the central fibers. Therapeutic modalities and clinical course are described. Three patients had a follow-up of 3, 5, and 13 years with complete remission of the disease within 1 year in all three cases. One patient had severe neuropsychological sequelae.


CONCLUSIONS
Susac syndrome seems to be less unusual in men than previously reported. Though presenting as a self-limited monophasic course disease in most cases, it may result in severe neuropsychological sequelae. Early diagnosis of the syndrome is enabled by the combination of the ophthalmologic, audiometric, and brain magnetic resonance features.
Snyers
Bernadette
B
Department of Ophthalmology, Saint-Luc University Hospital, Brussels, Belgium. snyers@ofta.ucl.ac.be
Boschi
Antonella
A
De Potter
Patrick
P
Duprez
Thierry
T
Sindic
Christian
C
D000328:Adult; D001299:Audiometry; D015834:Cochlear Diseases / Q000175:diagnosis* / Q000188:drug therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005451:Fluorescein Angiography; D005938:Glucocorticoids / Q000627:therapeutic use; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D015356:Retinal Artery Occlusion / Q000175:diagnosis* / Q000188:drug therapy; D012189:Retrospective Studies; D013577:Syndrome; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000188:drug therapy; D014786:Vision Disorders / Q000175:diagnosis
D005938:Glucocorticoids; D003520:Cyclophosphamide