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PEDIATRICS Vol. 114 No. 1 July 2004, pp. 276-281

EXPERIENCE AND REASON

Microangiopathy of Brain, Retina, and Inner Ear (Susac’s Syndrome) in an Adolescent Female Presenting as Acute Disseminated Encephalomyelitis

Jin S. Hahn, MD*,{ddagger},§, W. Craig Lannin, DO||, Minnie M. Sarwal, MD, PhD{ddagger},§

* Departments of Neurology
{ddagger} Pediatrics, Stanford University School of Medicine, Stanford, California
§ Lucile Packard Children’s Hospital, Palo Alto, California
|| Lucile Packard Children’s Hospital, Redding, California

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.

Abbreviations: BRAO, branch retinal artery occlusion • ADEM, acute disseminated encephalomyelitis • MRI, magnetic resonance image • CSF, cerebrospinal fluid • WBC, white blood cell • LPCH, Lucile Packard Children’s Hospital • SLE, system lupus erythematosus • MS, multiple sclerosis • CNS, central nervous system • FLAIR, fluid-attenuated inversion recovery

Received for publication Jul 11, 2003; Accepted Nov 4, 2003.


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