Allen NM, Dafsari HS, Wraige E, Jungbluth H. Neck-Tongue Syndrome: An Underrecognized Childhood Onset Cephalalgia. J Child Neurol. 2018 Jan 1:883073818756681. doi: 10.1177/0883073818756681. [Epub ahead of print]
Neck-tongue syndrome is a rarely reported headache disorder characterized by occipital and/or upper neck pain triggered by sudden rotatory head movement and accompanied by abnormal sensation and/or posture of the ipsilateral tongue. Although onset is thought to be in childhood, most of the limited number of cases reported so far were adults. Here the authors describe 3 cases, 2 girls and 1 boy, with neck-tongue syndrome. In each child additional headache symptoms occurred, headache improved over time in all, spontaneously in 2 and coinciding with gabapentin treatment in the other. Investigations were consistently unremarkable. Review of the literature reveals a usually self-limiting disorder, with early onset and variable additional features. Awareness of neck-tongue syndrome among pediatric neurologists and other practitioners is important, to allow for timely diagnosis and informed management of an underreported headache disorder with childhood onset.
Patient 1, an 11-year-old female, presented with a 5-month history of severe recurrent episodes of unilateral shooting and burning posterior neck pain lasting approximately 1 minute, triggered by head turning. Her first episode occurred while watering the garden and included a brief altered sensation affecting the right half of her body; there was no other history of trauma and initial symptoms resolved prior to presentation. Subsequently recurrent episodes of unilateral neck pain were rapidly followed by ipsilateral tongue hemianesthesia lasting 10 to 60 seconds. Occasional residual dull neck ache occurred, but no other headache or migraine symptomatology developed. Episodes alternated but were more right-sided. Frequency averaged once to twice weekly (up to 5/week) with episode-free weeks. Gabapentin treatment led to complete resolution, but was discontinued due to side effects (dizziness and weight gain despite dose reduction). Topical (neck) lidocaine patch 5% (nightly) reduced episodes to twice monthly with significantly less pain intensity at follow-up after 1 year. Examination revealed peripheral joint hypermobility, but was otherwise normal. Perinatal and developmental histories were unremarkable. Baseline laboratory investigations and contrast magnetic resonance imaging (MRI) of the brain and C-spine were normal…
The neck/occiput pain brought about by sudden head turning, in primary neck-tongue syndrome, is thought to be due to a temporary subluxation at the lateral atlantoaxial joint and compression/stretch of C2 and its roots, in particular impingement of the C2 ventral ramus. Tongue symptoms are explained by afferent proprioceptive fibers from the lingual nerve of trigeminal origin which anastomose with the hypoglossal nerve within the tongue, then travel through the ansa cervicalis to continue via the ventral ramus of C2.2-4 Where symptomatology is persistent, or follows minor trauma, secondary pathology (eg, arthritic disease) should be investigated…
The evidence-base for treating neck-tongue syndrome is anecdotal, due to only a very small number of cases reported to date (approximately 40). However in many cases such as patients 2 and 3 here, neck-tongue syndrome resolves or improves over time, requiring no or little prophylactic pharmacologic therapy. Patient 1 was treated with both gabapentin and topical lidocaine as preventive treatments, as symptoms had a greater impact on function. Some of her symptomatology was burning, and intense, so a trial of neuropathic pain medications appeared justified, given their benefit in related pain disorders,including neuralgiform headaches. The gabapentin response was immediate, but symptoms returned when the medication was discontinued due to other side effects. Symptoms subsequently resolved with lidocaine. While the authors discuss therapeutic benefit in this patient, it must be acknowledged that the observed improvement may just have reflected the natural history of the condition.
Reinstatement of neck-tongue syndrome in the International Classification of Headache Disorders may help clinicians recognize and manage this distinct headache disorder. Awareness that additional symptoms often occur in neck-tongue syndrome, and that onset is far more common in childhood may help improve diagnosis. Further reports are required to better understand the pathophysiology, epidemiology and natural history of neck-tongue syndrome.