Neck-Tongue Syndrome

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Written by: Dr Jeremy Steinberg – created: 26 March 2022; last modified: 4 May 2022

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Neck tongue syndrome fibres.jpg
The blue arrows show the proprioceptive pathways from the lingual nerve to the C2 ventral ramus. Copyrighted image.[1]
Neck-Tongue Syndrome
Definition The sudden onset of occipital and/or upper neck pain brought out by sudden rotatory head movement and accompanied by abnormal sensation and/or posture of the ipsilateral tongue.
Epidemiology Usually begins in childhood. Very rare.
Pathophysiology Subluxation of the atlantoaxial joint resulting in compression of the C2 ventral ramus
Treatment Supportive

Neck-Tongue Syndrome is a rare condition characterised by brief attacks of neck and/or occipital pain with neck rotation associated with sensory changes of the ipsilateral half of the tongue.

Pathophysiology

The relationship between the C2 dorsal root ganglion (g), spinal nerve (sn), ventral ramus (vr), the lateral atlanto-axial joint (j), posterior arch of the atlas (paa), the lamina of the axis (la), and the vertebral artery (va).
Top: Schematic illustrations of a dorsal view. Bottom: Dissection of a right dorsolateral view showing the C2 dorsal ramus cut at its point of branching (dr), cervical plexus (cp), third occipital nerve (ton), communicating branch C2 to C3 (c), and posterior atlantoaxial membrane (paam).
Copyright BMJ, reproduced with permission.[2]

Excessive rotation leads to subluxation of the lateral atlanto-axial joint leading to occipital pain. The C2 ventral ramus sits dorsal to the joint and is impinged against bone.

The symptoms can be explained as follows:[3]

Neck pain: The pain may come from joint capsule stretch.[3]

Tongue symptoms: The lingual afferents (trigeminal origin) from the tongue pass from the hypoglossal nerve into the C2 ventral ramus through the ansa cervicalis. C2 ventral ramus compression therefore causes tongue numbness. Most of the fibres are proprioceptive, and so patients may have pseudoathetosis of the tongue.[4] Bogduk contends that the numbness may not be true numbness but rather all due to proprioceptive loss and analogous to the sensory symptoms seen in Bell's palsy.[2]

Sensory symptoms in the neck: may come from compression of the C2 ventral ramus and/or tension of the C2 spinal nerve or roots.

Facial and Retro-auricular symptoms: The C2 ventral ramus innervates some of the posterior face and mastoid process and so some patients have symptoms in these regions.[2]

Upper extremity tingling: Unclear why some patients report this. It may be due to dura mater traction resulting in tension on the lower cervical nerve roots.[3]

Epidemiology and Risk Factors

Three quarters of patients have their onset in childhood or adolescence. There may be a family history which could relate to hypermobility. Previous trauma may be implicated. Patients may have another additional type of headache disorder other than Neck-Tongue Syndrome, especially migraine.[3]

A population based study in Norway found a prevalence of 0.2%.[5]

Clinical Features

Red Flags
  • Onset in adulthood
  • Post-traumatic onset
  • Persistent symptoms
  • Known rheumatic disease of the cervical spine


The symptoms; namely pain, numbness, and motor symptoms; of Neck-Tongue Syndrome are typically brief, in the order of seconds to minutes and are precipitated by sudden turning of the head.

The pain is in the C2 root distribution and is reproducible with neck rotation. Patients may report a popping or clicking sensation with the episodes.[3] There may be a decreased range of motion and tenderness of the upper cervical spine. [4]

There is associated numbness and/or paraesthesias of the ipsilateral half of the tongue with or without pseudoathetosis. There may be additional areas of sensory abnormalities in the neck and occiput.[3][4]

Diagnosis

Gelfand and colleagues proposed the following diagnostic criteria:[3]

Description. The sudden onset of occipital and/or upper neck pain brought out by sudden rotatory head movement and accompanied by abnormal sensation and/or posture of the ipsilateral tongue. The pain is usually severe in intensity.

Criterion Feature
A At least two episodes that fulfill criteria B through E
B Sharp or stabbing unilateral pain in the upper neck, or occipital region, or both, precipitated by sudden turning of the neck, with or without simultaneous dysaesthesia.
C Concurrent abnormal sensation, or posture, or both, of the ipsilateral tongue
D Duration from several seconds to several minutes
E Not better accounted for by another ICHD-3 disorder

Differential Diagnosis

Other causes of C2 ventral ramus compression are congenital fusion, stenosis, aneurysm, malignancy, or infection.

Neck-Tongue Syndrome DDX
  • Diseases of the eyes, ears, nose, throat, teeth
  • Tumours of the hypopharynx (tonsillar fossa, piriform sinus)
  • Cerebellopontine angle tumours
  • Demyelinating disease
  • Temporal arteritis
  • Glossopharyngeal neuralgia

Investigations

Practice Point
Imaging Indications
Consider imaging if symptoms are post-traumatic or occur in adulthood.

Imaging: Imaging is usually normal. The instability of the atlanto-axial joint is in the transverse plane in extremes of range and so normal sequences are unlikely to pick up any abnormalities. Undertaking axial CT scanning or biplanar fluoroscopy is not recommended as routine because of the extreme range.[3]

Indications for imaging are the development of symptoms following trauma and the initial occurrence in adulthood. Reporting should be done by experienced neuroradiologists.[3]

Blood tests: Blood tests are done to investigate for infection, inflammation (temporal arteritis), and malignancy. Consider FBC, UEC, CRP, and ESR.[4]

Other: Endoscopy of the hypopharynx looking at the piriform sinuses can be done to rule out malignancy of that area.[4]

Treatment

Treatment is usually not required if the attacks are uncommon and brief.

  • Immobilisation the cervical spine with a soft collar.
  • NSAIDs.
  • Manual therapy
  • Home exercises
  • C2 nerve corticosteroid injection
  • Atlanto-axial joint arthrodesis

References

  1. Niethamer, Lisa; Myers, Robin (2016-03). "Manual Therapy and Exercise for a Patient With Neck-Tongue Syndrome: A Case Report". The Journal of Orthopaedic and Sports Physical Therapy. 46 (3): 217–224. doi:10.2519/jospt.2016.6195. ISSN 1938-1344. PMID 26868897. Check date values in: |date= (help)
  2. 2.0 2.1 2.2 Bogduk, N. (1981-03). "An anatomical basis for the neck-tongue syndrome". Journal of Neurology, Neurosurgery, and Psychiatry. 44 (3): 202–208. doi:10.1136/jnnp.44.3.202. ISSN 0022-3050. PMC 490891. PMID 7229642. Check date values in: |date= (help)CS1 maint: PMC format (link)
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 3.8 Gelfand, Amy A.; Johnson, Hannah; Lenaerts, Marc Ep; Litwin, Jessica R.; De Mesa, Charles; Bogduk, Nikolai; Goadsby, Peter J. (2018-02). "Neck-Tongue syndrome: A systematic review". Cephalalgia: An International Journal of Headache. 38 (2): 374–382. doi:10.1177/0333102416681570. ISSN 1468-2982. PMID 28100071. Check date values in: |date= (help)
  4. 4.0 4.1 4.2 4.3 4.4 Waldman, Steven D. Atlas of uncommon pain syndromes. Philadelphia, PA: Elsevier Inc, 2020.
  5. Sjaastad, O.; Bakketeig, L. S. (2006-03). "Neck-tongue syndrome and related (?) conditions". Cephalalgia: An International Journal of Headache. 26 (3): 233–240. doi:10.1111/j.1468-2982.2006.00926.x. ISSN 0333-1024. PMID 16472328. Check date values in: |date= (help)

Literature Review