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

Nicholas M. Allen, Andrew B McKeon, Declan J. O'Rourke, Anne O'Meara, Mary D. King

Research output: Contribution to journalArticle

3 Citations (Scopus)

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.

Original languageEnglish (US)
JournalPediatrics
Volume129
Issue number5
DOIs
StatePublished - May 2012

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Voltage-Gated Potassium Channels
Blinking
Ataxia
Neuroblastoma
Antibodies
Oligoclonal Bands
Paraneoplastic Syndromes
Dysarthria
Cerebellar Ataxia
Myoclonus
Neurologic Manifestations
Autoimmunity
Peripheral Nerves
Abdomen
Autoantibodies
Signs and Symptoms
Cerebrospinal Fluid
Adrenal Cortex Hormones
Tomography
Serum

Keywords

  • Autoimmune
  • Cerebellar ataxia
  • Excessive blinking
  • Neuroblastoma
  • VGKC antibody

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Arts and Humanities (miscellaneous)

Cite this

Excessive blinking and ataxia in a child with occult neuroblastoma and voltage-gated potassium channel antibodies. / Allen, Nicholas M.; McKeon, Andrew B; O'Rourke, Declan J.; O'Meara, Anne; King, Mary D.

In: Pediatrics, Vol. 129, No. 5, 05.2012.

Research output: Contribution to journalArticle

Allen, Nicholas M. ; McKeon, Andrew B ; O'Rourke, Declan J. ; O'Meara, Anne ; King, Mary D. / Excessive blinking and ataxia in a child with occult neuroblastoma and voltage-gated potassium channel antibodies. In: Pediatrics. 2012 ; Vol. 129, No. 5.
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