Original Article

Journal of Child Neurology

2018, Vol. 33(5) 347-350
Neck-Tongue Syndrome: An Underrecognized ª The Author(s) 2018
Reprints and permission:
sagepub.com/journalsPermissions.nav
Childhood Onset Cephalalgia DOI: 10.1177/0883073818756681
journals.sagepub.com/home/jcn

Nicholas M. Allen, MD1, Hormos S. Dafsari, MD2, Elizabeth Wraige, MD2,

and Heinz Jungbluth, PhD2,3,4

Abstract
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.

Keywords
cephalalgia, headache, pediatrics

Received September 26, 2017. Received revised December 31, 2017. Accepted for publication January 9, 2018.

Neck-tongue syndrome is a headache disorder characterized by Cases

the sudden onset of occipital and/or upper neck pain provoked
by sudden rotatory head movement and accompanied by abnor-
mal sensation and/or posture of the ipsilateral tongue.1 Attacks
are usually brief but severe. Neck-tongue syndrome was
included in a previous version of the International Classifica-
tion of Headache Disorders–2 version but removed from the
most recent 2013 beta version (International Classification of
Headache Disorders-3).1-3 Primary neck-tongue syndrome is
usually episodic and self-limiting, and hypothesized to result
from ligamentous laxity/subtle bony abnormalities leading to
transient ipsilateral lateral atlantoaxial subluxation.2 Compli-
cated neck-tongue syndrome reflects association with an under-
lying disease process, for example, significant degenerative
cervical disease, ankylosing spondylitis, myelopathy, inflam-
matory, or other (anatomical) disorders, mainly reported in
adults.3 Although onset is typically thought to be in late child-
hood or adolescence, few pediatric cases have been reported,
highlighting a lack of awareness among pediatric practitioners
not infrequently resulting in diagnostic delay.4 Here the authors
describe 3 additional children with neck-tongue syndrome
(Table 1) and review the neck-tongue syndrome literature. The
authors hope to raise awareness of this distinct but underre-
ported headache disorder among pediatric neurologists, to
inform investigations and management of what is often a
self-limiting disorder.
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,
1
Department of Paediatrics, National University of Ireland Galway, & Galway
University Hospital, Ireland
2
Department of Pediatric Neurology, Evelina Children’s Hospital, Guy’s & St.
Thomas’ Hospital NHS Foundation Trust, London, UK
3
Randall Division for Cell and Molecular Biophysics, Muscle Signalling Section,
London, UK
4
Department of Basic and Clinical Neuroscience, IoPPN, King’s College,
London, UK
Corresponding Author:
Heinz Jungbluth, PhD, Evelina Children’s Hospital, Guy’s & St Thomas’ NHS
Foundation Trust, Children’s Neurosciences Centre, F02, Becket House,
Lambeth Palace Rd, London SE1 7EU, UK.
Email: Heinz.jungbluth@gstt.nhs.uk
348 Journal of Child Neurology 33(5)

Table 1. Patient Characteristics.

Patient 1, female Patient 2, female Patient 3, male

Age of onset 11 years 11 years 12 years

Pain
Location Posterior neck (right/left) Posterior neck Occiput
Intensity Severe Severe Severe
Quality Shooting/burning Burning Burning
Duration 10 seconds to 1 minute 30 seconds to 2 minutes 30 seconds to minutes
Tongue symptoms Ipsilateral hemianesthesia/ Ipsilateral numbness Ipsilateral tightness/twisting
numbness (10-60 seconds
duration)
Side affected Alternating (right and left) Right Alternating (right more than left)
Frequency Several per week initially; 1 Usually one but up to several per week; 4-5/month at peak; gradual resolution
episode-free weeks gradually only monthly
Additional features Post tongue symptoms: dull Retroauricular, retroorbital, cheek flushing; Occasional nonspecific headaches;
ipsilateral neck pain/numbness brief frontal headache (post neck tongue nonspecific infrequent postural
symptoms) dizziness
Triggers/precipitants Head turning Sudden head turning/neck movement, Sudden head turning
fatigue
Family history Mother had migraine, depression, Father had migraine Nil
hypothyroidism, B12 deficiency
Outcome 2/month, less severe Much less over time (with symptom-free Much less over time (with symptom-
weeks) free months)

but no other headache or migraine symptomatology developed.
Episodes alternated but were more right-sided. Frequency aver-
aged 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 nor-
mal. Perinatal and developmental histories were unremarkable.
Baseline laboratory investigations and contrast magnetic reso-
nance imaging (MRI) of the brain and C-spine were normal.
Patient 2, an 11-year-old female, presented with episodic
retroauricular upper neck pain spreading laterally to the
ipsilateral orbit, with ipsilateral cheek flushing, followed
by brief ipsilateral tongue numbness. The pain, initially
“shooting,” would become “boiling hot” sometimes making
her “want to cry.” Episodes lasted from 30 seconds to
2 minutes, and were usually triggered by sudden head rota-
tion, or sometimes tiredness. Occasionally these symptoms
were followed by brief (minutes) nonthrobbing mild frontal
headache, without photophobia, phonophobia, or other
migrainous or autonomic symptoms. Episodes occurred
weekly, sometimes on successive days but decreased in fre-
quency (with mostly episode-free weeks) over the following
4 years not necessitating pharmacotherapy. There was no
preceding history of trauma or infection. Perinatal and
developmental history was unremarkable. Neurological and
systems examination were unremarkable. MRI brain
and routine laboratory investigations were normal.
Patient 3, a 12-year-old male presented with episodes of
right sided occipital pain, followed by stiffness of his tongue.
Onset was clearly remembered months previously while play-
ing basketball, while moving his arms upward prompting
him to stop playing. That episode resolved spontaneously
after 20 minutes. Subsequent episodes were characterized by
an intense burning sensation of the right occiput which
alternated. During episodes he developed an abnormal sen-
sation in the ipsilateral tongue, sometimes described as a
“twisting” or a “tightness.” Over a 2-year period, episodes
ranged from infrequent to 4-5 per month, varied in severity,
lasted from several to 30 minutes, and were usually trig-
gered by sudden head turning while sitting. Over the sub-
sequent 4 years episodes became less frequent, not requiring
prophylactic therapy, and he was discharged from follow-
up. Perinatal and developmental histories were unremark-
able and school performance was good. Neurological and
musculoskeletal examination was normal. Routine chemis-
try, hematological indices, and brain and cervical MRI were
normal. Doppler studies of cerebral and carotid arteries
were normal. X-ray cervical vertebrae was unremarkable.
Discussion
Neck-tongue syndrome is a distinct headache disorder attrib-
uted to a proposed underlying neuroanatomic pathomechan-
ism.2-5 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 impinge-
ment of the C2 ventral ramus. Tongue symptoms are explained
by afferent proprioceptive fibers from the lingual nerve of tri-
geminal origin which anastomose with the hypoglossal nerve
within the tongue, then travel through the ansa cervicalis to
Allen et al
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.
Despite its rarity, neck-tongue syndrome is an important
headache disorder. Although presentation almost always
occurs in childhood, based on the available literature it appears
that diagnosis is often delayed into adulthood.4 Due to the
unusual nature of symptoms and lack of awareness of the con-
dition, diagnostic uncertainty may often lead to avoidable anxi-
ety. A careful history should prompt the correct diagnosis, but
an extensive assessment is warranted in particular in the con-
text of substantial preceding trauma or when underlying
inflammatory or arthritic conditions are suspected. Given the
rarity of the disorder and the importance of excluding structural
pathology, investigation by head and cervical MRI is likely to
be considered an important part of the assessment in all cases.3
However, detailed imaging of the atlantoaxial system (ie, in
extremes of rotation) to determine stability (eg, using CT/bipla-
nar fluoroscopy) and neurovascular imaging are not routinely
advocated in primary/typical neck-tongue syndrome cases, and
where considered on an individual basis, this should be
arranged in close liaison with an experienced radiologist.4
It is worth pointing out that in the 3 current patients as well as
many others reported in the literature4, neck and tongue symp-
toms may be associated with additional headache manifesta-
tions, and other motor and sensory symptoms.4 In particular,
patient 1 experienced burning and occasional longer lasting dull
neck symptoms as well as unilateral somatosensory symptoms.
Patient 2 experienced facial hemianesthesia, cranial autonomic
symptoms (flushing) and frontal/eye pain that would not be
explained by an alternative headache diagnosis. Patient 3 expe-
rienced separate short lasting frontal headaches that did not meet
migraine diagnostic criteria. Although well described in the lit-
erature, and the precise mechanisms for this spectrum of symp-
toms are not always understood, in some cases of neck-tongue
syndrome neuro-anatomical explanations for additional sympto-
matology in neck-tongue syndrome can be postulated.6
Additional headache manifestations in the context of other-
wise typical neck-tongue syndrome inform the differential
diagnosis (including, for example, migraine and cervicogenic
headaches) but may also cause a particular diagnostic challenge
in individual cases. However a number of universally present
core features suggest neck-tongue syndrome as a distinct entity,
and include its unusual but typical location (upper nuchal/
occiput), the tongue manifestations, the unilaterality of neck
and tongue features in association with triggers of head turning,
and the typically short duration of episodes. There also appears
to be some commonality of risk factors, neuroanatomical asso-
ciation, and natural history. Therefore despite its removal from
the International Classification of Headache Disorders–3 beta
version in 2013,1 the authors’ cases, and a recent systematic
review4 highlight the importance of recognizing neck-tongue
syndrome as a distinct and established headache disorder, with
clear diagnostic criteria recently being reproposed (Table 2).4
The evidence-base for treating neck-tongue syndrome is anec-
dotal, due to only a very small number of cases reported to date
349
Table 2. Proposed Criteria for Neck-Tongue Syndrome4.
A. At least 2 episodes that fulfil 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 dysesthesia.
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 International
Classification of Headache Disorders–3 disorder.
(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.4
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,7 including neuralgi-
form headaches. The gabapentin response was immediate, but
symptoms returned when the medication was discontinued due
to other side effects. Symptoms subsequently resolved with lido-
caine. 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. Aware-
ness that additional symptoms often occur in neck-tongue syn-
drome, 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 his-
tory of neck-tongue syndrome.
Author Contributions
All authors (NMA, HSD, EW, HJ) significantly contributed to the con-
ception and design of the study, acquisition of data, interpretation of data,
drafting the article. All authors approved the final version to be submitted.
Declaration of Conflicting Interests
The authors declared no potential conflicts of interest with respect to
the research, authorship, and/or publication of this article.
Funding
The authors received no financial support for the research, authorship,
and/or publication of this article.
Ethical Approval
This retrospective clinical case review was conducted according to the
ethical guidelines of the participating institutions.
References
1. Headache Classification Committee of the International Headache
Society (2013). The International Classification of Headache Dis-
orders, 3rd edition (beta version). Cephalalgia. 2013;33(9):
629-808.
350
2. Bogduk N. An anatomical basis for the neck-tongue syndrome.
J Neurol Neurosurg Psychiatry. 1981;44(3):202-208.
3. Hu N, Dougherty C. Neck-tongue syndrome. Curr Pain Headache
Rep. 2016;20(4):27.
4. Gelfand AA, Johnson H, Lenaerts ME, et al. Neck-tongue
syndrome: a systematic review [published online ahead of print
January 18, 2017] Cephalalgia. doi:10.1177/0333102416681570.
Journal of Child Neurology 33(5)
5. Lance JW, Anthony M. Neck-tongue syndrome on sudden turning
of the head. J Neurol Neurosurg Psychiatry. 1980;43(2):97-101.
6. Dyer PV. An upper cervical spine injury producing paraesthesia in
the distribution of the mandibular division of the trigeminal nerve.
Br J Oral Maxillofac Surg. 1991;29(6):374-376.
7. Kitchener JM. Glossopharyngeal neuralgia responding to pregabalin.
Headache. 2006;46(8):1307-1308.