A Case of Opsoclonus-Myoclonus-Ataxia Syndrome in Ganglioneuroblastoma.
- Author:
Eun Kyung KIM
1
;
Jeong Ah PARK
;
Eun Sil PARK
;
Hyoung Soo CHOI
;
Hee Young SHIN
;
Hyo Seop AHN
;
Kwi Won PARK
Author Information
1. Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. hsahn@snu.ac.kr
- Publication Type:Case Report
- Keywords:
Neuroblastoma;
Ganglioneuroblastoma;
Opsoclonus-myoclonus-ataxia
- MeSH:
Adrenal Glands;
Brain;
Cerebrospinal Fluid;
Child, Preschool;
Extremities;
Female;
Gait;
Ganglioneuroblastoma*;
Homovanillic Acid;
Humans;
Magnetic Resonance Imaging;
Myoclonus;
Neuroblastoma;
Paraneoplastic Syndromes;
Spine
- From:Korean Journal of Pediatric Hematology-Oncology
2005;12(1):114-119
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
The presence of rare paraneoplastic syndrome, the opsoclonus-myoclonus-ataxia syndrome (OMA), may strongly signal the presence of neuroblastoma. We report a case of ganglioneuroblastoma presented with OMA. A 26 month-old girl was admitted due to progressive ataxic gait and myoclonic jerking of the limbs. Brain and spine MRI scans were normal and cerebrospinal fluid analysis showed no specific abnormal finding. Abdominal computed tomography (CT) demonstrated about 3x1.5 cm sized well enhancing solid mass originated from the right adrenal gland. Urinary vanillyl mandelic acid (VMA) was mildly elevated and urinary homovanillic acid (HVA) was normal. After complete resection of the tumor, she was diagnosed with ganglioneuroblastoma and her symptomatology had disappeared.
