Excessive blinking and ataxia in a child with occult neuroblastoma and voltage-gated potassium channel antibodies.

Hdl Handle:
http://hdl.handle.net/10147/233636
Title:
Excessive blinking and ataxia in a child with occult neuroblastoma and voltage-gated potassium channel antibodies.
Authors:
Allen, Nicholas M; McKeon, Andrew; O'Rourke, Declan J; O'Meara, Anne; King, Mary D
Affiliation:
Department of Pediatric Neurology, Children's University Hospital, Dublin 1, Ireland. nicholasallen@physicians.ie
Citation:
Excessive blinking and ataxia in a child with occult neuroblastoma and voltage-gated potassium channel antibodies. 2012, 129 (5):e1348-52 Pediatrics
Journal:
Pediatrics
Issue Date:
May-2012
URI:
http://hdl.handle.net/10147/233636
DOI:
10.1542/peds.2011-2690
PubMed ID:
22529275
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.
Item Type:
Article
Language:
en
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
ISSN:
1098-4275

Full metadata record

DC FieldValue Language
dc.contributor.authorAllen, Nicholas Men_GB
dc.contributor.authorMcKeon, Andrewen_GB
dc.contributor.authorO'Rourke, Declan Jen_GB
dc.contributor.authorO'Meara, Anneen_GB
dc.contributor.authorKing, Mary Den_GB
dc.date.accessioned2012-07-13T11:27:03Z-
dc.date.available2012-07-13T11:27:03Z-
dc.date.issued2012-05-
dc.identifier.citationExcessive blinking and ataxia in a child with occult neuroblastoma and voltage-gated potassium channel antibodies. 2012, 129 (5):e1348-52 Pediatricsen_GB
dc.identifier.issn1098-4275-
dc.identifier.pmid22529275-
dc.identifier.doi10.1542/peds.2011-2690-
dc.identifier.urihttp://hdl.handle.net/10147/233636-
dc.description.abstractA 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.en_GB
dc.language.isoenen
dc.rightsArchived with thanks to Pediatricsen_GB
dc.subject.meshAbdominal Neoplasms-
dc.subject.meshAntibodies, Antinuclear-
dc.subject.meshBlinking-
dc.subject.meshCerebellar Ataxia-
dc.subject.meshChild-
dc.subject.meshDiagnosis, Differential-
dc.subject.meshDysarthria-
dc.subject.meshFemale-
dc.subject.meshHumans-
dc.subject.meshNeoplasms, Unknown Primary-
dc.subject.meshNeuroblastoma-
dc.subject.meshParaneoplastic Syndromes-
dc.subject.meshPotassium Channels, Voltage-Gated-
dc.subject.meshTomography, X-Ray Computed-
dc.titleExcessive blinking and ataxia in a child with occult neuroblastoma and voltage-gated potassium channel antibodies.en_GB
dc.typeArticleen
dc.contributor.departmentDepartment of Pediatric Neurology, Children's University Hospital, Dublin 1, Ireland. nicholasallen@physicians.ieen_GB
dc.identifier.journalPediatricsen_GB
dc.description.provinceLeinsteren

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