Neck-Tongue syndrome: A systematic review

Amy A. Gelfand, Hannah Johnson, Marc E.P. Lenaerts, Jessica R. Litwin, Charles De Mesa, Nikolai Bogduk, Peter J. Goadsby

Research output: Contribution to journalReview articlepeer-review

10 Scopus citations


Objective: Neck-Tongue syndrome (NTS) is characterized by brief attacks of neck or occipital pain, or both, brought out by abrupt head turning and accompanied by ipsilateral tongue symptoms. As the disorder is rare, we undertook a systematic review of the literature to identify all reported cases in order to phenotype clinically the disorder and subsequently inform clinical diagnostic criteria. Methods: Two electronic databases were searched using the search term “neck tongue syndrome”. All English language references were reviewed in full. Cases were abstracted using a standardized abstraction form and the references of the retrieved articles were reviewed by hand to identify additional references and cases. Conference proceedings from recent headache meetings were searched. We also report six new cases from our centers. Results: There were 39 primary cases, 56% of which were female. Mean age (SD) at onset was 16 (12) years. Twenty (53%) experienced neck pain, seven (18%) occipital pain, and 11 (29%) both. Pain was most often sharp or stabbing and severe, lasting several seconds to several minutes. Eleven experienced numbness and/or tingling in the neck/occiput following the pain. Thirty-six had an accompanying tongue sensory disturbance and three a motor/posture disturbance; five had both. Thirteen had other headaches, and four a family history of Neck-Tongue syndrome. Conclusions: Neck-Tongue syndrome typically has pediatric or adolescent onset, suggesting that ligamentous laxity during growth and development may facilitate transient subluxation of the lateral atlantoaxial joint with sudden head turning. Familial cases suggest a genetic predisposition in some individuals. Neck-Tongue syndrome should be re-instated in the International Classification of Headache Disorders.

Original languageEnglish (US)
Pages (from-to)374-382
Number of pages9
Issue number2
StatePublished - Feb 1 2018


  • Neck-Tongue syndrome

ASJC Scopus subject areas

  • Clinical Neurology


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