Korean J Pediatr Hematol Oncol.  2005 Apr;12(1):114-119.

A Case of Opsoclonus-Myoclonus-Ataxia Syndrome in Ganglioneuroblastoma

Affiliations
  • 1Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea. hsahn@snu.ac.kr
  • 2Department of Surgery, Seoul National University College of Medicine, Seoul, Korea.

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.

Keyword

Neuroblastoma; Ganglioneuroblastoma; Opsoclonus-myoclonus-ataxia

MeSH Terms

Adrenal Glands
Brain
Cerebrospinal Fluid
Child, Preschool
Extremities
Female
Gait
Ganglioneuroblastoma*
Homovanillic Acid
Humans
Magnetic Resonance Imaging
Myoclonus
Neuroblastoma
Paraneoplastic Syndromes
Spine
Homovanillic Acid
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