Homonymous Hemianopsia and Visual Hallucination as a Manifestation of Sturge-Weber Syndrome without Facial Nevus

Sturge-Weber syndrome (SWS) is a rare neurocutaneous syndrome characterized by facial nevus and leptomeningeal angiomatosis with neurological and ophthalmological complications. In its rare variant, SWS type 3, the clinical feature of facial nevus is absent, posing a diagnostic challenge. We present the case of a patient with late-onset SWS without facial nevus who presented with homonymous hemianopsia and visual hallucinations.

104 Homonymous hemianopsia and visual hallucination as a manifestation of Sturge-Weber syndrome without facial nevus Ji-Yun Park 1, Kwang-Dong Choi 2, Hyung Lee 3, Hyun Ah Kim 4 1. Department of Neurology, Ulsan University Hospital, University of Ulsan college of Medicine, Ulsan, Republic of Korea, 2 Department of neurology, Pusan national university hospital, 3 Keimyung University, 4 Keimyung University School of Medicine 1 Introduction: Sturge-Weber syndrome (SWS) is a rare neurocutaneous syndrome characterized by facial nevus and leptomeningeal angiomatosis with neurological and ophthalmological complications. In its rare variant, SWS type 3, the clinical feature of facial nevus is absent, posing a diagnostic challenge. We present the case of a patient with late-onset SWS without facial nevus who presented with homonymous hemianopsia and visual hallucinations. Description of Cases: A 56-year-old man with diabetes presented with a five-day history of headache, visual disturbance and visual hallucinations. He had a headache that worsened when he sneezed or defecated, visual field loss localized to the left side, and visual hallucinations that occurred predominantly when he turned to the left. These hallucinations included images such as a person wrapped in a bandage, a television set, or a rainbow. A visual field analysis showed a bilateral field defect on the left hemifield. Brain MRI showed leptomeningeal enhancement in the right occipital leptomeninges. The cerebrospinal fluid study was normal. The electroencephalogram (EEG) performed on admission and during the first 72 hours of hospitalization showed no epileptic discharge. Cancer work-up and paraneoplastic antibody tests were all negative. He was started on steroid pulse therapy and levetiracetam. After 12 days, the intermittent visual hallucinations resolved, and the visual field defect mostly improved. One and a half years later, he experienced a recurrence of visual hallucinations. He described that everything looked like a square shape or body hair looked like black color. A follow-up brain MRI showed the same findings as the previous one. After increasing the dose of levetiracetam and stopping the immunosuppressant, the symptoms did not occur. Conclusions, including unique features of the case: We experienced a patient with leptomeningeal angiomatosis without facial angioma presenting with partial epilepsy and hemianopsia. Clinicians must keep SWS without facial veins in mind as a differential diagnosis of leptomeningeal enhancement with homonymous hemianopia and partial epilepsy. References: None provided. Keywords: Visual fields Financial Disclosures: The authors had no disclosures. Grant Support: None. Contact Information: Hyun Ah Kim, [e-mail] 160 | North American Neuro-Ophthalmology Society