Microangiopathy of brain, retina, and inner ear (Susac's syndrome) in an adolescent female presenting as acute disseminated encephalomyelitis.
Susac's syndrome is a rare disorder that consists of microangiopathy of the brain, retina, and inner ear and usually affects young women in young adulthood. The triad of clinical manifestations consists of acute encephalopathy with neurologic signs, branch retinal artery occlusion (BRAO), and sensorineural hearing loss. We present a case of an adolescent female who presented at age 16 years with clinical and neuroimaging features of acute disseminated encephalomyelitis (ADEM). The full triad did not develop until 2.5 years after the initial neurologic presentation.
Hahn
Jin S
JS
Department of Neurology, Stanford University School of Medicine, Stanford, California, USA. jhahn@stanford.edu
Lannin
W Craig
WC
Sarwal
Minnie M
MM
D000293:Adolescent; D001259:Ataxia / Q000209:etiology; D001921:Brain / Q000098:blood supply / Q000473:pathology; D007758:Ear, Inner / Q000098:blood supply; D004673:Encephalomyelitis, Acute Disseminated / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D015356:Retinal Artery Occlusion / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D013577:Syndrome