Neck-Tongue syndrome: A systematic review

Abstract

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.

Facial Pain: A Comprehensive Review and Proposal for a Pragmatic Diagnostic Approach

“…Another rare disorder characterized by symptoms after head turning is the neck-tongue syndrome. Turning of the neck provokes unilateral neck and/or occipital pain, as well as an abnormal sensation and/or posture of the ipsilateral tongue [66]. Van…”

Neck–tongue syndrome

“…NTS is a condition rarely described in a clinical practice. In a review published in 2018, there were only 39 primary cases reported in the English literature 3. However, NTS may actually be more frequent than hitherto surmised 8–11…”

“…Neck–tongue syndrome (NTS) is a rarely reported disorder. Plausible speculations on its pathophysiology have recently been described in case reviews 1–3. It is characterised by brief attacks of occipital pain and ipsilateral hemiglossal dysaesthesia elicited by abrupt neck rotation 4.…”

Neck-Tongue Syndrome: Viewpoints on Etiology in a Patient with Bilateral Symptoms

“…In comparison to Gelfand et al's recent review, two aspects of this case are of note. Firstly, no cases of midline neck/occipital pain and only one case of bilateral tongue symptoms were reported in the review [ 1 , 3 ]. Bogduk, adding to Lance and Anthony's contributions to the anatomic explanation for this syndrome, argued that proprioceptive fibers from the lingual nerve returning via the C2 ventral ramus/cervical plexus are impacted against the edge of an articular process of the atlantoaxial joint.…”

“…Neck-Tongue Syndrome (NTS) is a type of cervicogenic headache recently reviewed by Gelfand et al whose proposed criteria entail unilateral neck and/or occipital pain accompanied by ipsilateral tongue dysesthesia and/or abnormal posture upon sudden turning of the neck. [ 1 ] These symptoms can last anywhere from 10 seconds to several minutes in duration and can occasionally be accompanied by various other symptoms involving the oropharynx and upper extremities [ 1 ]. The etiology of childhood/adolescent NTS, unlike adult NTS which is most often due to trauma or inflammatory diseases involving the cervical spine, remains unclear but invokes variations in osseous/ligamentous and nervous anatomy to explain this uncommon nexus of symptomology [ 2 ].…”