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34838ournal ofNeurology, Neurosurgery, and Psychiatry 1994;57:348-352

The - syndrome J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.57.3.348 on 1 March 1994. Downloaded from

Richard W Orrell, C David Marsden

Abstract region. He attributed the unilateral numbness The neck-tongue syndrome, consisting of of the tongue (and of the skin behind the ) pain in the neck and altered sensation in to compression of the C2 ventral ramus by the ipsilateral half of the tongue aggra- movement or subluxation of the lateral vated by neck movement, has been atlantoaxial . He suggested that the attributed to damage to lingual afferent abnormal sensation on compression of pro- fibres travelling in the hypoglossal prioceptive afferents from the tongue is simi- to the C2 spinal roots. The lingual affer- lar to the altered sensation or numbness ents in the are thought recognised in Bell's palsy, despite intact to be proprioceptive. Two further cases trigeminal sensation, due to compression of of the neck-tongue syndrome are proprioceptive fibres in the . described, the spectrum of its clinical Since these original descriptions of the manifestations is explored, and the phe- neck-tongue syndrome, a few other cases nomenon of lingual pseudoathetosis is have been described. Lance4 briefly mentions illustrated as a result of the presumed a further four patients in whom the syndrome lingual deafferentation. started in late childhood or adolescence. Elisevich et all reported a 53-year-old woman (7 Neurol Neurosurg Psychiatry 1994;57:348-352) with assimilation of the to the occiput presenting with and ipsilateral paraesthesiae of the tongue on turning, Lance and Anthony' described four patients, in whom the symptoms became persistent aged 8 to 14 years at onset, with a brief (sec- and incapacitating. At operation, the C2 onds to a minute) sharp pain on one side of spinal. were found to be compressed by the upper neck or occiput on sudden rotation a protuberant atlantoaxial joint. Resection of of the neck, followed immediately by tran- the superficial parts of the C2 spinal nerves sient (seconds to minutes) ipsilateral numb- led to partial relief. ness of the tongue. Two of these cases had Webb et a16 described three cases with ipsi- congenital anomalies of the upper cervical lateral neck pain and tongue numbness pre- spine on radiography. Cyriax2 had previously cipitated by neck movement, first developing http://jnnp.bmj.com/ noted a similar syndrome in two patients, one symptoms at ages of 10 to 61 years. Degen- of whom was a 45-year-old woman with erative changes of the anterior atlantoaxial attacks of severe pain at the occiput and left joint were present in two of the cases. side of the neck and numbness of the tongue Restriction of neck movement, including use and back of the , precipitated by turn- of a surgical collar in two of the cases, con- ing her head sharply to the right. The other trolled the symptoms. patient had a cervical disc lesion and a sensa- Fortin and Biller7 described a 57-year-old

tion of pins and needles felt in the tongue woman with repeated episodes of numbness on September 29, 2021 by guest. Protected copyright. only. of the posterior right half of the tongue and Lance and Anthony' concluded that the right posterolateral oropharynx, an ipsilateral pain was due to irritation of the second (and "ear pressure", and a sharp aching pain radi- third) cervical root, which is vulnerable to ating along the border of the to the compression in its course between the atlas acromioclavicular joint. At the same time she and axis during neck rotation. They explained noticed a pressure sensation in the occiput the unilateral numbness of the tongue on the and paraesthesiae in the fourth and fifth fin- basis of afferent impulses (notably, propriocep- gers of the right . The episodes lasted noted no clear University tive) travelling from the via the one minute, and she herself Department of hypoglossal nerve to the second cervical root. precipitant, but they were precipitated by Clinical Neurology, Bogduk3 clarified the anatomical basis for extreme lateral rotation of the neck. The The National of the atlantoaxial joint was normal Hospital, Queen the syndrome by cadaveric dissection, region Square, London demonstrating that the C2 ganglion and radiologically. They also described a 29-year- WClN 3BG, UK lie dorsal to the lateral atlantoax- old man, with previous head injury and lower R W Orrell the C2 thoracic vertebral compression fracture after a C D Marsden ial joint; the joint is innervated by ventral ramus. He proposed that the pain was motorcycle accident, who complained of Correspondence to: Dr R W Orrell. due to temporary abnormal subluxation of mainly right, but sometimes left sided (never Received 14 April 1993 the lateral atlantoaxial joint on rotation of the both sides simultaneously), hemiglossal and and in final revised form This strains the capsule, and facial paraethesiae, sharp occipital pain shoot- 14 June 1993. head. joint Accepted 21 June 1993 thereby causes pain in the suboccipital ing to the periorbital area, dysarthria, nasal The neck-tongue syndrome 349

congestion, sialorrhoea, and paraesthesiae in exacerbations were associated with a tingling J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.57.3.348 on 1 March 1994. Downloaded from the ulnar distribution of the upper . The sensation of the left side of the tongue, and a symptoms in both patients were relieved by sensation of the tongue being pulled into the use of a surgical collar. back of the on the left, causing a feel- Bertoft and Westerberg8 reported a further ing of choking, with difficulty speaking. six patients with a complete syndrome, and Neurological examination was normal, and three with an incomplete syndrome, which in particular touch and pin prick sensation on they define as having characteristic attacks, both sides of the tongue was preserved. The but lacking the paroxysmal numbness of the tongue and palate appeared normal, with nor- tongue. We have not included these cases of mal movement. Cervical spine movement was an incomplete syndrome in the present dis- severely limited due to pain. cussion, but two of the patients with a com- A cervical spine radiograph showed degen- plete syndrome had radiological evidence of erative changes in the mid and lower cervical arthritis of the lateral atlantoaxial , and spine, with rotation at the atlantoaxial joint, in one the symptoms were abolished after but no horizontal subluxation. A cervical surgical atlantoaxial fusion. spine CT scan with the head turned to the Cassidy et a19 described three patients with right and left showed no structural lesion or the neck-tongue syndrome, who were suc- abnormal movement. Rotatory movement cessfully treated with rotational manipulation was equal to the right and left, with no dislo- of the cervical spine. Terrett et al'° described cation of the lateral atlantoaxial joint. The eight cases of the neck-tongue syndrome, rotation was about 40 degrees on each side, a which were treated with spinal manipulative little more than usually seen, but probably therapy. They also referred to an additional not pathological. A radioisotope scan, 54-year-old woman with , who, however, revealed increased uptake in the left after cervical manipulation, developed left atlantoaxial joint, attributed to . sided tongue numbness, persisting for two Injection of a local anaesthetic and steroid years, together with an intermittent into this joint led to brief relief of the pain. dysarthria. Hankey" described a more classical neck-tongue syndrome in, a 14-year- CASE 2 old girl with recurrent episodes of unilateral A 32-year-old woman accidentally fell down a upper nuchal pain radiating to the ipsilateral ffight of 12 stairs at night, hitting the right side of the tongue on ipsilateral rotation of frontoparietal region of her head against a the neck. table. This caused a laceration but no Noda and Umezaki"2 described a 35-year- loss of consciousness or immediately appar- old woman with paroxysmal episodes, lasting ent neurological deficit. The next day she up to one minute, of numbness of the left awoke with a continuous right sided occipital neck and occiput, with simultaneous numb- and neck pain exacerbated by ness of the left half of the tongue, preceded attempts at neck movement. She had slurring by tonic spasm of the left . They suggest of , continuous altered sensation of the this is a component of a spinal tonic seizure tongue and on the right with a feel- related to an upper cervical inflammatory ing of the tongue moving to the left, and diffi-

myelopathy involving the dorsal funiculus, culty manipulating food in the . http://jnnp.bmj.com/ proximal to the C2 and C3 dorsal root gan- On examination she had tenderness over glia, and hence a spinal neck-tongue syn- the upper cervical spine, and neck move- drome. ments were severely restricted by pain. The In total, 36 cases of this syndrome have tongue was rotated anticlockwise in the been previously described in the literature, mouth, with consequent appearance of some very briefly,48 although Bogduk13 com- decreased bulk of the left side of the tongue ments that on discussion with other neurolo- (fig A). On forward protrusion of the tongue gists and neurosurgeons it seems that the there was equal bulk of the two sides, but on September 29, 2021 by guest. Protected copyright. syndrome is not rare. Accordingly we great difficulty in maintaining a sustained describe two further cases of the syndrome, steady forward position, the tongue moving due in one case to atlantoaxial osteoarthritis, involuntarily to right and left (fig B). There and in the other to a traumatic fracture and were no visible fasciculations. There was full displacement of the occipital condyle. A strik- movement of the tongue to the left (fig C) ing feature was dysarthria and, in the second despite partial denervation of the right half of case, pseudoathetosis of the tongue. the tongue demonstrated by EMG. Tongue protrusion to the right was incomplete and poorly sustained although no weakness of the Patients left half of the tongue could be detected CASE 1 (fig D). On fibreoptic endoscopy the base of A 51-year-old woman complained of a pro- the tongue was more posterior than usual, gressive stiffness and pain in the neck for six and partially obstructing the . She had years. Three years previously she had noticed a mild lingual dysarthria. Sensation of the that the pain was exacerbated by movement tongue and pharynx to touch, , and pin of the neck to a critical position, with the prick was normal. Neurological examination down and to the right. She was aware of a was otherwise normal. grating sensation in the neck, and the pain Cervical spine radiography was normal. An appeared to be deep to the angle of the upper cervical CT scan was normal, but a CT on the left, radiating to the occiput. These scan of the base showed a fracture of the 350 Orrell, Marsden .F.',Ati>,t,_¢...... Sa.....sS.._...... __ii.''.__"wss...... ?_. o;vS'... J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.57.3.348 on 1 March 1994. Downloaded from tw'._.,.s x '.'.....>, ., ., ,, .m AF :, Ds

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ffi ...... '' _,8.,,S., ....J,,._Z,: .'S< s er iS i£ ifisl .:''' i_... io''.'>.i+ K -"+' 0., ?5'. '. .. _ MovementCofA/the tongue.S...... -in case;.v2;|Fposturesj...... A,B,C,Dand D...as described.inW%,,.the''.-text.....::. 1':>:''.. right occipital condyle, with slight medial dis- previous descriptions of neck-tongue syn- placement of the fragment just distal to the drome. A 15-year-old boy had a right occipi- right hypoglossal canal. tal pain and numbness of the right side of the tongue on sudden neck rotation, and a feeling that the tongue was twisted sideways in his Discussion mouth.' The symptoms lasted a few seconds The clinical spectrum of the neck-tongue syn- only. A 29-year-old man had ipsilateral occip- drome, including age at presentation, ital pain and tingling in his tongue, with chronicity, and disablement, has broadened dysarthria and , precipitated since the original description. The two by neck movement.7 A 60-year-old man, with patients we describe had a feeling of abnor- symptoms since the age of 10 years, had mal posturing or movement of the tongue as experienced a sensation of his tongue being a significant feature of their symptomatology. twisted sideways in the mouth during an In case 2 the positioning of the tongue was attack, "as though the whole tongue is cleav- clearly abnormal and she was unable to main- ing to the roof of the mouth".4 A 19-year-old tain a sustained normal posture of the tongue woman with pain in the left side of the neck in the absence of any specific weakness. In on sudden head turning also noticed devia-

particular there was no weakness of tongue tion of her tongue to the right for 10 seconds, http://jnnp.bmj.com/ movement to the left, which would be with anarthria.4 A 33-year-old woman affected by motor denervation of the right described paralysis of her tongue for about side of the tongue. We propose that this is a five seconds on sharp rotation of the neck.'0 A result of proprioceptive deafferentation of the 19-year-old woman described her tongue as tongue, with the clinical sign of lingual seeming to take up all the room in her mouth, pseudoathetosis. This is similar to pseudo- having a mind of its own, being uncontrol- athetosis as previously described in the lable, and resulting in dysarthria.'0 A 46-year- limbs.14 Pseudoathetoid movements of the old man experienced the symptoms of the on September 29, 2021 by guest. Protected copyright. tongue seem particularly complex in the neck-tongue syndrome, his tongue being absence of any restriction of direction of numb and rigid, with dysarthria.10 A 26-year- movement imposed by a joint. The associated old woman experienced the neck-tongue syn- dysarthria has features of both impaired lin- drome, with an inability to move her gual control and pharyngeal obstruction as tongue.'0 In these cases the dysarthria, as in manifestations of lingual pseudoathetosis pre- case 2, may be related to proprioceptive deaf- dominantly of the tip and base respectively. ferentation rather than a primary motor Proprioception in the tongue has been deficit. studied in animals and ,"5 with con- A 53-year-old woman had pain in the right troversy initially concerning whether the side of her neck and tingling of the ipsilateral tongue musculature has proprioception. As a side of her tongue, initially intermittent but generalisation, it seems that muscle spindles then constant. This was aggravated by head are not found in the tongue of , turning and talking, and when severe the pain except in moles, primates, and humans.16 radiated to the back of her head or right arm Muscle spindles have been demonstrated his- and caused mild related to a feeling tologically in the tongue.'7 18 The of muscle spasm in the throat.5 A 34-year-old afferent pathways seem to be in the hypoglos- man complained of intermittent left sided sal nerve,'6 19 which is otherwise thought of as neck pain radiating to the occiput, with ipsi- being a purely motor nerve. lateral aching and numbers of the tongue. Lingual pseudoathetosis is suggested in The symptoms were precipitated by rapid The neck-tongue syndrome 351

neck flexion and rotation to either side when atlantoaxial joint. In case 2, the site of deaf- J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.57.3.348 on 1 March 1994. Downloaded from breathing during competitive free-style swim- ferentation is uncertain, and could be at the ming. Occasionally the symptoms were so atlantoaxial joint related to the fall and twist- intense and associated with a choking sensa- ing of the neck, related to the displaced tion that he had to stop swimming during a occipital condyle and abnormal atlanto- race. (He had experienced the same symp- occipital joint, or in the hypoglossal nerve toms while swimming between the ages of 10 after exit from the hypoglossal canal related and 22 years).6 to the displaced bone fragment. Previous Most reported cases have had transient abnormalities of the atlanto-occipital joint symptoms and signs, but in case 2 these were were described in a 26-year-old man with constant, enabling the tongue posture and anteromedial bosses of bone on the condylar movement to be observed. The tongue processes of the occiput representing a minor seemed to be drawn back in the mouth, com- anomaly of the occipital assimilation.' The patible with the symptom described in case 1, spinous processes of the atlas were fused to and possibly explaining the muscle spasm in the adjoining occipital in a 15-year-old the throat of the 53-year-old woman5 and 34- girl,' and assimilation of the atlas to the year-old swimmer.6 occiput was noted in a 53-year-old woman.5 Of the cases of the neck-tongue syndrome Thus of the 38 cases now described, previously described, four involved abnormal- of the occipitoatlantoaxial joints ities at the atlantoaxial joints, two fusion of has been noted in 10. the atlas to the occiput, and another a minor The onset of symptoms in case 2 was abnormality of the occipital condylar clearly related to trauma, as were 10 previous processes. A cervical disc lesion was noted in cases. A 29-year-old man had a motorcycle another. In the other cases no clear pathology accident, with head and thoracic spine injury, of the upper cervical joints was noted two years before developing the neck-tongue (although in one an intrinsic myelopathy was syndrome.7 A 26-year-old man was hit in the suggested). In case 1 the structural abnormal- midfrontal region by a child's swing, with lac- ity was osteoarthritis at the left atlantoaxial eration of the . Shortly afterwards he joint demonstrated by radioisotope bone developed numbness and weakness of the scan. Irregular loss of the left lateral right hand lasting for several days. Ever since atlantoaxial joint space with reactive sclerosis he has had transient symptoms of numbness and osteophytic lipping was noted in a 36- of the right side of the tongue and right year-old man,6 and moderate degenerative fourth and fifth , and right sided neck changes at the anterior atlantoaxial joint in a pain, lasting a few seconds only, on sudden 65-year-old woman.6 rotation of the neck.' A 36-year-old man acci- Another possible example of the condition dentally jarred his neck, and subsequently, was described by Lees et al20 as "paroxysmal whenever jolted unexpectedly, experienced hemiglossal twisting". These authors reported high cervical pain radiating to the left side of two patients, and tentatively suggested dysto- his tongue "like the aftermath of a dental nia or tonic seizure as the cause. The first anaesthetic".6 A 28-year-old woman devel- case was a 61-year-old woman who, on oped the neck-tongue syndrome the day after

review of the original records, described a tin- a injury, which persisted for one http://jnnp.bmj.com/ gling sensation at the back of the tongue on year, but resolved with spinal manipulation.9 the left, the tongue then deviated to the right, Other cases associated with trauma include and she was unable to move her tongue to the four after a motor vehicle collision, and one left, associated with dysarthria. On other developing eight years after a water skiing occasions the tongue twisted from side to accident.10 side, and she had bitten the left side of her The transient relief of the symptoms in tongue several times. Touch sensation of the case 1 by injection of local anaesthetic and tongue was normal during an episode. She steroid into the lateral atlantoaxial joint is on September 29, 2021 by guest. Protected copyright. had repeated episodes, lasting for about one supportive of the symptoms being related to minute, which were infrequent, with no clear this joint, and raises the possibility of treat- precipitating neck movement, but appeared ment by permanent cervical fusion. to be related to exercise. The attacks could be Previously reported surgery with bilateral associated with discomfort down the left side resection of C2 spinal nerves with their dorsal of the neck and behind the left ear. A cervical and ventral rami, initially relieved the symp- spine radiograph showed no abnormality of toms, but after one week there was a recur- the upper cervical region, and CT scan of the rence of mild tingling sensations in the atlantoaxial joint was normal. Except for the tongue when the patient was tired. The neck absence of precipitation by neck movement, pain also recurred, but less severely than this case has features in common with the before the operation. The authors suggested neck-tongue syndrome, and the abnormal that the recurrence of tongue symptoms twisting of the tongue may be a lingual could be explained by facilitation of residual pseudoathetosis due to lingual proprioceptive proprioceptive fibres in the adjacent unin- deafferentation, with associated C2 pain. jured spinal roots. In another patient, surgical (The second patient had brief spontaneous atlantoaxial fusion abolished the symptoms.8 attacks of tongue twisting and dysarthria Other patients have had their symptoms without pain or paraesthesiae). relieved by spinal manipulation.910 Symptoms In case 1, the site of deafferentation is have been improved by preventing the precip- assumed to be in the neck, related to the itative neck movement with a surgical collar, 352 Onrell, Marsden

but the benefit is not necessarily permanent, 6 Webb J, March L, Tyndall A. The neck-tongue syndrome: J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.57.3.348 on 1 March 1994. Downloaded from occurrence with cervical arthritis as well as normals. and with no long term follow up of this syn- J Rheumatol 1984;11:530-3. drome the natural history is unclear. 7 Fortin CJ, Biller J. Neck-tongue syndrome. Headache 1985;25:255-8. Since the initial description by Lance and 8 Bertoft ES, Westerberg CE. Further observations on Anthony' of patients aged 15 to 26 years, the neck-tongue syndrome. Cephalgia 1985;5(suppl 3): 312-3. with an onset age of eight to 15 years, it has 9 Cassidy JD, Diakow PRP, De Korompay VL, Munkacsi I, become apparent that the same syndrome Yong-Hing K. Treatment of neck-tongue syndrome by spinal manipulation: a report of three cases. The Pain may present up to at least the age of 61 years, Clinic 1986;1:41-6. and may present as transient symptoms which 10 Terrett AGJ. Neck-tongue syndrome and spinal manipu- lative therapy. In: Vernon H, ed. Upper cervical syn- may persist, may resolve and relapse, or may drome: chiropractic diagnosis and treatment. Baltimore: become permanent and present as a perma- WiHiams and Wilkins, 1988:223-9. 11 Hankey GJ. "Neck-tongue" syndrome on sudden neck nent deficit. In a significant proportion there rotation. Aust NZJ Med 1988;18:181. is pathology at the atlantoaxial and atlanto- 12 Noda SN, Umezaki H. Spinal neck-tongue syndrome. J Neurol Neurosurg Psychiatry 1984;47:75 1. occipital joints, and in some cases the syn- 13 Bogduk N. Neck-tongue syndrome. Med,JAust 1980;2:4. drome may result from trauma. 14 Liversedge LA. Involuntary movements. In: Vinken PJ, Bruyn GW, eds. Handbook of clinical neurology. Vol 1. Amsterdam: North-Holland, 1969:277-292. 15 Tier GA, Rees RT, Rood JP. The sensory nerve supply to We thank Professor A E Harding for permission to include the tongue: a clinical reappraisal. Br Dent Jf 1984;157: details of the patient under her care. 354-7. 16 Kubota K, Narita N, Takada K, et al. Origin of lingual proprioceptive afferents in Japanese monkey, M fuscata 1 Lance JW, Anthony M. Neck-tongue syndrome on sud- fuscata. Studied by HRP-labelling technique. Anat Anz den turning of the head. J7 Neurol Neurosurg Psychiatry 1988;166: 141-8. 1980;43:97-101. 17 Cooper S. Muscle spindles in the intrinsic muscles of the 2 Cyriax J. Textbook of orthopaedic medicine. 4th ed. Vol 1. human tongue. J Physiol (Lond) 1953;122:193-202. London: Cassell, 1962:158. 18 Kubota K, Negishi T, Masegi T. Topological distribution 3 Bogduk N. An anatomical basis for the neck-tongue syn- of muscle spindles in the human tongue and its signifi- drome. J Neurol Neurosurg Psychiatry 198 1;44:202-8. cance in proprioception. Bull Tokyo Med Dent Univ 4 Lance JW. Unusual syndromes in neurological practice. 1975;22:235-42. MedJAust 1984;140:409-16. 19 Adatia AK, Gehring EN. Proprioceptive innervation of 5 Elisevich K, Stratford J, Bray G, Finlayson M. Neck- the tongue. JAnat 1971;110:215-20. tongue syndrome: operative management. J Neurol 20 Lees AJ, Blau JN, Schon F. Paroxysmal hemiglossal twist- Neurosurg Psychiatry 1984;47:407-9. ing. Lancet 1986;ii:812-3. http://jnnp.bmj.com/ on September 29, 2021 by guest. Protected copyright.