Atypical Sturge-Weber Syndrome: A case report.

, Kim, Yong Jin; , Kim, Chul; , Ahn, Jae Ki; , Bang, In Keol; , Lee, Sung Min

Case Report

Journal of the Korean Academy of Rehabilitation Medicine 2002;26(6):811-814.

Kim, Yong Jin , Kim, Chul , Ahn, Jae Ki , Bang, In Keol , Lee, Sung Min

Department of Physical Medicine and Rehabilitation, Sanggye Paik Hospital, Inje University, Korea. oboist@orgio.net

비특이성 Sturge-Weber 증후군 ⁣증례 보고⁣

김용진ㆍ김철ㆍ안재기ㆍ방인걸ㆍ이성민

인제대학교 상계백병원 재활의학과

Abstract

Sturge-Weber syndrome is a congenital neurocutaneous disorder of the vessels of the face, the leptomeninges and the brain. Clinically SWS consists of symptoms and signs including a facial nevus (port-wine stain), seizure, hemiparesis, mental retardation. But only a few reports related to atypical Sturge-Weber syndrome without facial nevus have been published. We report a patient with atypical Sturge-Weber syndrome without any clinical feature except hemiparesis. In neuroimaging studies using brain CT scan and MRI, leptomeningeal angiomatosis was demonstated that is the characteristic feature of Sturge- Weber syndrome. In atypical Sturge-Weber syndrome, there may be late- developed complications such as hemiparesis. In conclusion, neuroimaging study is recommended to confirm diagnosis in suspicious atypical SWS patient. (J Korean Acad Rehab Med 2002; 26: 811-814)

Key Words: Atypical Sturge-Weber syndrome, Leptomeningeal angiomatosis, Hemiparesis, Late-developed complications