TABLE 1:

Clinical histories and presentations in patients with Susac syndrome

Clinical course	Symptoms	MR Examination	Auditory Findings	Retinal Findings	Treatment*
Patient 1
Initial admission, 2 wk after onset	Headaches, vomiting, R arm feeling loss, impaired balance, urinary incontinence, dysarthria, decreased attention span, gait ataxia	2 wk after onset	Normal	R branch retinal artery occlusion	IV SoluMedrol 1 g/d × 5, IVIG 0.4 g/d × 5, prednisone 60 mg/d (with taper)
Symptom relapse, 8 wk after initial onset	Headaches, cognitive decline, clumsiness, worsened gait ataxia, upper extremity ataxia	8 and 10 wk after initial onset	L high-frequency hearing loss	New L branch retinal artery occlusion	IVIG 0.4 g/d × 5, prednisone 50 mg/d (with taper)
Stable condition, 8–9 mo after initial onset	Difficult in tandem walking, mild dysmetria	8 mo after initial onset	No change	No new occlusion	Prednisone 20 mg/d
Patient 2
Initial admission, 2 wk after onset	Headaches, R partial vision loss	2 wk after onset	Normal	R branch retinal artery occlusion	Aspirin
Symptom relapse, 6 wk after initial onset	Headaches, R face and arm numbness	6 wk after initial onset	Normal	New L branch retinal artery occlusion	IV methylprednisolone 1 g × 1, prednisone 80 mg/d (with taper)
Symptom relapse, 4 mo after initial onset	L arm parethesias, R extremity weakness, staggering walking	4 mo after initial onset	R partial hearing loss	New R/L branch retinal artery occlusion	IVIG 0.4 g/d × 5 (monthly basis for 4 consecutive mo), prednisone 60 mg/d (with taper)
Worsening symptoms, 11 mo after initial onset	Worsening in vision, poor concentration, confusion, impaired balance	11 mo after initial onset	No change	No change	IVIG 0.4 gm/d × 5 (almost every 6–8 wk for 1 y)
Stable condition, 2 y later	Mild ataxia, poor tandem		Normal	Normal

Note.—IV indicates intravenous; IVIG, intravenous immunoglobulin.