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Year : 2004  |  Volume : 52  |  Issue : 1  |  Page : 124-125

Atlantoaxial dislocation in idiopathic cervical dystonia

Department of Radiology, PO Box 12, Salmania Medical Complex, Manama

Correspondence Address:
Department of Radiology, PO Box 12, Salmania Medical Complex, Manama
[email protected]

  »  Abstract

We report a case of severe cervical spondylosis and atlantoaxial dislocation (AAD) in association with idiopathic cervical dystonia (ICD) in a middle-aged male. To our knowledge, this is the first case of ICD reported in association with AAD.

How to cite this article:
Kanekar S. Atlantoaxial dislocation in idiopathic cervical dystonia. Neurol India 2004;52:124-5

How to cite this URL:
Kanekar S. Atlantoaxial dislocation in idiopathic cervical dystonia. Neurol India [serial online] 2004 [cited 2021 Dec 8];52:124-5. Available from:

  »   Introduction Top

Dystonia is a type of movement disorder, which results in a twisting movement of the parts involved.[1] It may be focal or generalized. ICD is the most common form of adult onset focal dystonia.[2] When dystonia involves the cervical spine for a long time, it can result in degenerative changes leading to myeloradiculopathies.[2],[3],[4] A rare case with ICD is reported where, in addition to the gross degenerative changes in the cervical spine the patient had AAD.

  »   Case Report Top

A 40-year-old male was admitted with a history of progressive difficulty in walking and stiffness of both lower limbs for two months. He had repeated falls whilst walking during this period. He was able to perform all his routine work without assistance until 1 month prior to admission, at which time he needed a walking stick to move around. Since childhood, he had suffered from involuntary twisting movements involving the muscles of the neck and face, which later extended to the limbs. His family history was unremarkable.
On examination, he had dystonic movements involving predominantly the neck and trunk and, to a lesser extent the face and the limbs. He had spastic Grade 4 quadriparesis. Kinesthetic sensations were impaired in all four limbs. The rest of the neurological and systemic examination was normal. Routine blood investigations were normal. Screening for Wilson's disease was negative.
Investigations revealed degenerative changes in the cervical spine and AAD [Figure - 1]. However, there was no obvious compression noted at the cervicomedullary junction. The patient refused any surgical treatment.

  »   Discussion Top

Oppennheim[5] in 1911 coined the term dystonia, a condition characterized by uncontrollable muscular contractions that produce abnormal posture. Dystonia is defined as a syndrome of sustained muscle contractions, frequently causing twisting and repetitive movements or abnormal posture. Focal (cervical) dystonia is defined as an involuntary twisting and turning of the neck caused by abnormal involuntary muscle contracture.[3],[6] Abnormal postures frequently seen in cervical dystonia are torticollis, laterocollis, anterocollis, retrocollis or any combination of the above. In 1970, Levin and associates[7] noted that abnormal neck movements and posture caused premature spondylotic changes and degenerative changes were greater on the side of neck flexion. Nishihara and colleagues,[8] in their study of athetoid patients with cervical spondylotic myelopathy, found that C3-C4 and C4-C5 were the most frequently affected sites. It was suggested that patients with cervical movement disorders have a higher velocity during flexion or extension and an increased range of neck movements. This causes greater shearing forces and bending movement of the cervical structures, which predisposes the cervical spine to premature degenerative changes. In addition to these changes our patient also had AAD, which has not been described in the literature before. Cervical spondylotic changes with atlantoaxial instability in patients with abnormal movements have been previously described by Yashioka.[9] Greenberg et al[10] also reported AAI in patients with abnormal movement disorder.
The management of AAD in movement disorders is controversial and difficult as postoperative immobilization of the cervical spine is difficult due to the involuntary movements. 

  »   References Top

1.Ad Hoc committee: Ad Hoc committee of the dystonia Medical Research Foundation (Drs. A Barbeau, D B Calne, S Fahn, C D Marsden, J Menkes and G F Wooten) February 1984.  Back to cited text no. 1    
2.Chan J, Brin MF, Fahns S Idiopathic Cervical Dystonia: Clinical characteristics. Mov Disorder 1991;6:119-26.  Back to cited text no. 2    
3.Dauer WT, Burke RE, Greene P, Fahn S. Review Article: Current concepts on the clinical features, aetiology and management of idiopathic cervical dystonia. Brain 1998;121:547-60.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Jankovic J, Leder S, Warner D, Schwartz K. Cervical dystonia: Clinical findings and associated movements disorders. Neurology 1991;41:1088-91.  Back to cited text no. 4    
5.Oppenheim H. Dysbasia lordotica progressiva, Dystonia musculorum deformans. Neurol centrabl 1911;30:1090-7.   Back to cited text no. 5    
6.Fahn S, Marsden CD, Calne DB. Classification and investigation of Dystonia. In: Marsden CD, Fahn S, ed. Movement disorders 2. London: Butterworths; 1987. pp. 332-58.  Back to cited text no. 6    
7.Levin RA, Rosenbaum AE, Waltz JM, Scheinberg LC. Cervical Spondylosis and Dyskinesia. Neurology 1970;20:1194-9  Back to cited text no. 7    
8.Nobuharu Nishihara, Gozo Tonabe, Shinnosuke Nakahara, et al. Surgical treatment of cervical spondylotic myelopathy complicating Cerebral palsy. J Bone Joint Surgery1984;66:504-8.  Back to cited text no. 8    
9.Hideo Yoshioka, Hideaaki Kanai. Atlantoaxial instability in Athetoid-Dystonic Cerebral Palsy. Spine1992;1711:1434-7.  Back to cited text no. 9    
10.Greenberg AD. Atlanto-axial dislocation. Brain 1968;91:655-84.  Back to cited text no. 10    


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