Excessive blinking and ataxia in a child with occult neuroblastoma and voltage-gated potassium channel antibodies.
Allen, Nicholas M McKeon, Andrew O'Rourke, Declan J O'Meara, Anne King, Mary D
Allen, Nicholas M
McKeon, Andrew
O'Rourke, Declan J
O'Meara, Anne
King, Mary D
Advisors
Editors
Other Contributors
Date
2012-05
Date Submitted
Keywords
Other Subjects
Subject Mesh
Abdominal Neoplasms
Antibodies, Antinuclear
Blinking
Cerebellar Ataxia
Child
Diagnosis, Differential
Dysarthria
Female
Humans
Neoplasms, Unknown Primary
Neuroblastoma
Paraneoplastic Syndromes
Potassium Channels, Voltage-Gated
Tomography, X-Ray Computed
Antibodies, Antinuclear
Blinking
Cerebellar Ataxia
Child
Diagnosis, Differential
Dysarthria
Female
Humans
Neoplasms, Unknown Primary
Neuroblastoma
Paraneoplastic Syndromes
Potassium Channels, Voltage-Gated
Tomography, X-Ray Computed
Planned Date
Start Date
Collaborators
Principal Investigators
Alternative Titles
Publisher
Abstract
A previously healthy 9-year-old girl presented with a 10-day history of slowly progressive unsteadiness, slurred speech, and behavior change. On examination there was cerebellar ataxia and dysarthria, excessive blinking, subtle perioral myoclonus, and labile mood. The finding of oligoclonal bands in the cerebrospinal fluid prompted paraneoplastic serological evaluation and search for an occult neural crest tumor. Antineuronal nuclear autoantibody type 1 (anti-Hu) and voltage-gated potassium channel complex antibodies were detected in serum. Metaiodobenzylguanidine scan and computed tomography scan of the abdomen showed a localized abdominal mass in the region of the porta hepatis. A diagnosis of occult neuroblastoma was made. Resection of the stage 1 neuroblastoma and treatment with pulsed corticosteroids resulted in resolution of all symptoms and signs. Excessive blinking has rarely been described with neuroblastoma, and, when it is not an isolated finding, it may be a useful clue to this paraneoplastic syndrome. Although voltage-gated potassium channel complex autoimmunity has not been described previously in the setting of neuroblastoma, it is associated with a spectrum of paraneoplastic neurologic manifestations in adults, including peripheral nerve hyperexcitability disorders.
Language
en
ISSN
1098-4275
eISSN
ISBN
DOI
10.1542/peds.2011-2690
PMID
22529275