title: Trombone tongue: a new clinical sign for significant medullary compression at the craniovertebral junction. Case report.
abstract: The authors describe a previously unreported clinical sign that may indicate the onset of significant compression of the medulla oblongata in cases of craniovertebral junction abnormalities. This 17-year-old boy presented with mild bilateral leg weakness. Imaging studies revealed severe basilar invagination and a marked Chiari malformation. While awaiting surgery, his tongue developed an involuntary constant protrusion-intrusion repetitive motion. The onset of this so-named "trombone tongue" sign was followed shortly afterward by rapidly progressive spastic tetraparesis. After the authors performed a transmaxillary clivectomy, foramen magnum decompression, and occipitocervical fusion, they noted that the abnormal tongue motion promptly resolved and the tetraparesis gradually improved. The authors discuss their current understanding of the central control of tongue movements and present a hypothesis on the pathogenesis of trombone tongue based on the neuroanatomical basis of another abnormal tongue movement sign, lingual myoclonus.
authors.affiliation: The National Hospital for Neurology and Neurosurgery, Queen Square, London; and Spinal Surgery Unit, Royal National Orthopaedic Hospital, Stanmore, United Kingdom.
authors.forename: Cheong H
authors.initials: CH
authors.lastname: Lee
authors.forename: Adrian T H
authors.initials: AT
authors.lastname: Casey
authors.forename: James B
authors.initials: JB
authors.lastname: Allibone
authors.forename: Ramesh
authors.initials: R
authors.lastname: Chelvarajah
mesh_terms: D000293:Adolescent; D001139:Arnold-Chiari Malformation / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D002574:Cervical Vertebrae; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008526:Medulla Oblongata / Q000473:pathology / Q000503:physiopathology*; D009069:Movement Disorders / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D009207:Myoclonus / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D014059:Tongue / Q000294:innervation / Q000503:physiopathology*