A Case of Silver-Russell Syndrome Diagnosed in the Patient Admitted with Hypoglycemic Seizure.

Lee,  Jung Yong; Kim,  Young A; Lee,  Kyung Yeon; Oh,  Ki Won; Kim,  Ja Hyeong; Kim,  Joon Sung; Jeong,  Jin Young; Park,  Sang Kyu

Case Report

Journal of the Korean Child Neurology Society 2010;18(1):117-122.
Published online May 30, 2010.

A Case of Silver-Russell Syndrome Diagnosed in the Patient Admitted with Hypoglycemic Seizure.

Jung Yong Lee, Young A Kim, Kyung Yeon Lee, Ki Won Oh, Ja Hyeong Kim, Joon Sung Kim, Jin Young Jeong, Sang Kyu Park

Department of Pediatrics, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Korea. pdsnoopy@yahoo.co.kr

Abstract

The Silver-Russell syndrome(SRS) is a clinically heterogeneous syndrome characterized by intrauterine and postnatal growth retardation with spared cranial growth, characteristic facial features, and body asymmetry. Although mild to moderate hypoglycemic symptoms occasionally appear in children with SRS especially those who are not fed frequently and regularly, hypoglycemic seizures rarely occur. We report a rare case of SRS which was diagnosed in a 4-year-old female who admitted with hypoglycemic seizure. The patient showed the characteristic features of SRS. Endocrinologic studies were normal except for partial growth hormone insufficiency. To prevent seizures and chronic neurologic deficits in children with SRS, the early recognition and appropriate management of hypoglycemia is critical.

Key Words: Silver-Russell syndrome, Hypoglycemia, Seizures