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    Topic review

    Neck-Tongue Syndrome

    Subjects: Physics, Applied
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    Neck-tongue syndrome (NTS) is rare, and characterized by unilateral upper neck or occipital pain and paresthesia in the ipsilateral hemisphere of the tongue due to neck movement. Treatment for NTS is mainly conservative. 

    1. Introduction

    Neck-tongue syndrome (NTS) is a very rare clinical condition defined by the International Headache Society (IHS) as “painful lesions of the cranial nerves and other facial pain” [1]. Characteristic symptoms of NTS include unilateral upper neck or occipital pain, and paresthesia of the ipsilateral half of the tongue due to neck movement [2]. Past studies of prevalence have shown that NTS is a rare case, estimated to occur in 0.22% of adults [3].
    According to the reported characteristics of NTS, it can be divided into uncomplicated NTS and complicated NTS [4]. Uncomplicated NTS is related to idiopathic, hereditary, and trauma, and complicated NTS is reported to appear in patients with underlying diseases [5]. For this reason, differential diagnosis is considered very important, and related diseases include cervical artery dysfunction, vertebrobasilar insufficiency (VBI), transient ischemic attack, migraine, ligamentous insufficiency, and Chiari-1 malformation [2][6][7][8].
    In the study of Lewis et al. [9], an autosomal dominant genetic pattern was reported through three cases as a genetic trait, and although symptoms were present in adolescence, the symptoms disappeared in adulthood. For this reason, it can be suggested that the pathogenesis according to age may be different. In other words, structural abnormalities in NTS appearing in adults are found, but rarely reported in adolescents. The pathogenesis reported so far is that stimulation of the C2 ventral branch, which is transmitted through the afferent proprioceptive nerve from the lingual nerve to the C2 nerve root and cervical plexus through anastomosis with the sublingual nerve, causes tongue paresthesia [5][10].
    Classification of reported treatments for NTS can be divided into pharmacological and non-pharmacological treatments. Pharmacological treatments include antiepileptic drugs, antidepressants, neuropathic pain modulators, and nonsteroidal anti-inflammatory drugs [7][11][12]. Non-pharmacological treatments include cervical collar, physical therapy, exercise, and neuromuscular re-education [4][11][13].

    2. Neck-Tongue Syndrome 

    Table 1 summarizes the characteristic symptoms of the neck and tongue for NTS and the results of the interventions and treatments. In the six case reports [4][13][14][15][16][17], symptoms and treatments were relatively similar. Although patients’ ages were similar, most patients were in their 20s or younger, and the number of women was relatively high.
    As defined in the study of Lance and Anthony [2], there were unilateral upper neck or occipital pain and paresthesia in the ipsilateral hemisphere of the tongue due to neck movement. This caused neck pain due to the sudden rotation. In addition, accompanying symptoms not reviewed in this study included dysarthria, numbness of the mastoid process region, facial paresthesia, and numbness in the upper extremities [2][7][9]. As a further note, in a systematic review reported in 2018, 39 patients were analyzed [18]. In this study, most of these patients had NTS onset at childhood or adolescence, and those affected were mostly women (56%). Ligament laxity during growth and development and some genetic factors were reported to be causes of the early onset age. In other studies on NTS episodes, there were cases of cervical osteoarthritis [3][7][19], and in magnetic resonance imaging (MRI) studies, there were cases of retrocerebellar arachnoid cysts [20].
    Although non-surgical methods for managing NTS are common, a study on the surgical management of NTS confirmed that symptoms were relieved by resolving the compression on the C2 spinal nerve by the protuberant atlantoaxial joint [21]. In the pharmacological and non-pharmacological interventions included in non-surgical interventions, pharmacological interventions were performed only in the Allen et al. [17] study. In the other five case reports, manual therapy, exercise, and cervical collar were found to be effective.
    Differential diagnoses of idiopathic and post-traumatic NTS can be made by considering the accompanying symptoms. Treatment may include using a neck collar or medication to relieve symptoms, and manual therapy and exercise appear to be appropriate as an intervention. However, in a review by Gelfand et al. [18], pathologically, when ankylosing spondylitis and rheumatoid arthritis are accompanied by chronic pain and joint instability in the cervical spine (C1, C2), it is difficult to explain the pain of NTS neurologically. Furthermore, what can be said to be different from our study is that there is no advantage of spinal manipulation therapy for the type of suspected instability. However, in childhood and adolescence cases, there are cases of compression due to shortness of the scalenus and congenital abnormalities or shortness of the sternocleidomastoid muscle. At this time, symptoms are improved through the effect of manual therapy for the absence of neuromuscular control development of muscles and ligaments due to neurophysiological pathology and structural instability [22]. Therefore, it is suggested that interventions such as spinal manipulation therapy, manual therapy, exercise, and neuromuscular re-education can be helpful as a conservative treatment method.
    Although it is a syndrome with a relatively low incidence and long-term discomfort, randomized controlled trials are difficult to conduct, but it is worth studying the most effective management through a long-term cohort study. In addition, in items b, c, and d of ICHD-3, first, diagnosis of intermittent pain rather than chronic pain when there is no neck movement, second, sharp pain during rotation, accompanied by paresthesia of the tongue, and third, there is partial agreement as it is essential for the diagnosis of NTS pain that the duration of treatment ranges from a few seconds to a few minutes.
    Table 1. Characteristics of the case reports.
    Study Sex Age Symptoms Interventions Results Author’s Conclusion
    Triggers Neck Tongue NTS Duration Types Intensity
    Roberts. [14] Female 34 Rotation of the head to the left side Mild pain in the left suboccipital region Described as “tingling,” “shooting,” “piercing,” and “electric shock-like” on the left side tongue >2 years Spinal manipulative therapy, myofascial release, and exercises Two weeks Symptoms improved after 2 weeks of intervention and persisted after 2 years Chiropractic care is helpful for NTS
    Niethamer and Myers. [13] Female 13 Turning head to one side or the other or looking up Sharp pain Numbness/tingling of the ipsilateral tongue > 6 months Manual therapy, exercise (including postural awareness) Eight visits Patient-specific functional scale, cervical ROM, postural normalization An effective known treatment for cervicogenic headache may be the basis for treating NTS
    Borody. [15] Female 24 Brisk active rotation Upper neck (right) ‘‘sharp’’ or ‘‘piercing’’ pain Ipsilateral tongue numbness Recurrent pain after 8 years of age Diversified spinal manipulative therapy performed to the restrictions throughout the cervical spine Various intervals Reduction in the frequency and intensity of the symptoms In the absence of other additional symptoms and upper cervical instability, spinal manipulative therapy is effective
    Chirchiglia et al. [4] Female 42 Rotation of the head to the right side Severe neck pain Paresthesia of the right tongue A month later, reported a whiplash injury, following a car accident Topiramate combined with non-steroidal anti-inflammatory drugs and cervical collar Two weeks Improved pain both in the neck and the tongue, reducing the episodes in frequency and intensity Pharmacological treatment is prophylactic
    Chu and Lin. [16] Male 47 Head-turning to the right, forward neck glide or chewing right suboccipital pain Tingling of the right face and ipsilateral hemitongue >3 months Chiropractic regimen Three times a week for four weeks The painful episodes had disappeared and the active range of neck motion had increased Conservative management, including physiotherapy and minor cervical adjustment, may be the preferred initial treatment
    Allen et al. [17] Female 11 Head-turning Severe shooting/burning pain in the posterior neck Ipsilateral hemianesthesia >5 months Gabapentin and topical lidocaine - Less pain intensity at follow-up after 1 year It is characterized by additional symptoms of headache; headache was spontaneously resolved in 2 patients, and 1 patient was relieved of headaches by gabapentin
    Female 11 Sudden head-turning/neck movement, fatigue Severe burning pain in the posterior neck Ipsilateral numbness After symptom onset - 4 years follow-up Relieve symptoms naturally
    Male 12 Sudden head-turning Severe burning pain in the Occiput Ipsilateral tightness/twisting Some months 6 years follow-up

    This entry is adapted from 10.3390/medicina57101097


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