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Neck-Tongue Syndrome

Neck-tongue syndrome (NTS) is defined by the International Classification of Headache Disorders (third edition) as two paroxysmal, short lived episodes of unilateral neck or occipital pain with concurrent ipsilateral tongue dysaesthesia, precipitated by ipsilateral neck rotation and no underlying cause^1,2. This syndrome is rare and underrecognized^1,3, with a mean age of onset at 16 years¹. Pathogenesis is attributed to the damage of the lingual afferent fibers traveling in the hypoglossal nerve to the C2 spinal root secondary to intermittent atlantoaxial subluxation¹. Even though NTS could occur without any predisposing conditions, seronegative arthropathies and degenerative cervical joint disease could cause it⁴. Differential diagnoses include cervicogenic headaches and vertebrobasilar insufficiency⁴. While there are no consensus guidelines for treatment, activity modification (e.g., cervical collar and avoiding triggers)¹ and medications (NSAIDs, amitriptyline, and tizanidine)⁴ have been beneficial in some cases.

References:

1. Gelfand AA, Johnson H, Lenaerts MEP, Litwin JR, De Mesa C, Bogduk N, et al. Neck-Tongue syndrome: A systematic review. Cephalalgia. 2017;38(2):374–82
2. The International Classification of Headache Disorders, 3rd edition. Cephalalgia. 2018;38(1): 176.
3. Allen NM, Dafsari HS, Wraige E, Jungbluth H. Neck-Tongue Syndrome: An Underrecognized Childhood Onset Cephalalgia. J Child Neurol. 2018 Apr;33(5):347-350. 
4. Hu N, Dougherty C. Neck-Tongue Syndrome. Curr Pain Headache Rep. 2016 Apr;20(4):27. 

Submitted by Dr. Nihas Raja Mateti, Medical graduate, Osmania Medical College (Hyderabad, India), Neurology Rotation, Houston Medical Clerkship, Sugar Land, Texas.

Dr. Nihas Raja Mateti reports no disclosures.