Neurol India Home 

Year : 2021  |  Volume : 69  |  Issue : 2  |  Page : 517--519

Chronic Cervical Dystonia Complicating Cervical Myelopathy—A Therapeutic Challenge Managed by Botulinum Toxin and Surgical Intervention

SP Chaithra, Vikram Holla, Akshay Kulkarni, Pramod K Pal, Ravi Yadav, Nupur Pruthi 
 Department of Neurology and Neurosurgery, National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India

Correspondence Address:
Ravi Yadav
Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Hosur Road, Bangalore - 560 029, Karnataka

How to cite this article:
Chaithra S P, Holla V, Kulkarni A, Pal PK, Yadav R, Pruthi N. Chronic Cervical Dystonia Complicating Cervical Myelopathy—A Therapeutic Challenge Managed by Botulinum Toxin and Surgical Intervention.Neurol India 2021;69:517-519

How to cite this URL:
Chaithra S P, Holla V, Kulkarni A, Pal PK, Yadav R, Pruthi N. Chronic Cervical Dystonia Complicating Cervical Myelopathy—A Therapeutic Challenge Managed by Botulinum Toxin and Surgical Intervention. Neurol India [serial online] 2021 [cited 2021 Jun 23 ];69:517-519
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Full Text


Cervical dystonia (CD), most common form of focal dystonia can cause various neck-related orthopedic and neurological complications.[1] Cervical myelopathy (CM) is one such challenging complication occurring because of the repetitive twisting movements at the neck. Surgical management for myelopathy is challenging in such cases due to the continuous neck movements resulting in difficult perioperative stabilization of the neck and poor post-surgical outcome. We report a case of cervical myelopathy in a patient with dystonic cerebral palsy managed with botulinum toxin injection before and after surgery.

A 41-year-old working male with global developmental delay and untreated generalized dystonia since childhood presented with gradually progressive weakness of both upper limbs of 2 years which started in left hand followed by right hand with reduced dexterity and hand grip. For the past 3 months there was weakness of the lower limbs also. At presentation he was wheel chair bound. He had no history of mental sub-normality, cranial nerve impairment, radicular neck pain, sensory impairment, or urinary symptoms. He had history of a trivial fall 2 years back. On examination, patient had short neck. Motor system examination revealed spastic quadriparesis of grade 4 power with exaggerated deep tendon reflexes in all four limbs and mute plantar reflex. He was able to stand and walk with support of one person. He had facial dyskinesia, hyperkinetic speech, and cervical dystonia in the form of retrocollis and right sided torticollis with intermittent dystonic spasms. He also had mild dystonia of the distal upper limbs. TWSTRS (Toronto Western Spasmodic Torticollis Rating Scale) score for severity of cervical dystonia was 79 out of 95. MRI and CT cervical spine showed compressive myelopathy at C1-2 level [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d. Surgery was planned for relieving cord compression, stabilizing cranio-cervical junction, to restore the height of left C1-C2 joint and perform excisional biopsy of the soft tissue mass. Three weeks prior to surgery, 500 units of Abobotulinum toxin A (Dysport) was injected (250 units in right Splenius Capitis, 100 units in the left Splenius Capitis, and 150 units in the left Sternocleidomastoid) to reduce dystonia, aid in traction for aligning the C1-2 joint and facilitate surgery. Improved alignment and increased height of C1-2 joint was achieved with traction post botulinum toxin injection [Figure 1]e. During surgery, the soft tissue lesion was noted to be a left C2 root neurofibroma and was excised completely. Right Side C1 lateral mass and C2 transpedicular screw fixation was done. Left side C1 lateral mass and end plate cartilage was eroded and very thin rim was remaining. Attempted drilling through left C1 lateral mass resulted in brisk bleeding and was controlled with bone wax. Therefore, on the left side, occipito-C2 translaminar screw fixation was done. Bilaterally rib graft was placed in C1-2 joint space [Figure 1]f. He did not have any new neurological deficit post-surgery. Post-operative CT showed good alignment between occiput -C1-2 and increased C1-2 joint space with bone graft in situ. Delayed CT angiography showed bilateral proximal and distal patent vertebral arteries. The postoperative TWSTRS score was 35, however it could be confounded by the fact that surgical fixation was done. Botulinum toxin injection was repeated at 4 weeks postoperative period to prevent worsening of dystonia and further repeated every 3–4 months. At 4 months follow-up, CT scan showed fusion in the right C1-2 joint [Figure 1]g, [Figure 1]h. The patient is ambulant independent for his activity of daily living and returned to his work [Video 1].{Figure 1}[MULTIMEDIA:1]

Cervical myelopathy has been reported in patients with movement disorders like cervical dystonia, dystonic cerebral palsy, and Tourette's syndrome with violent motor tics.[2] Dystonia related cervical spine disease usually affects the higher levels (C3-C4), in contrast to lower level (C5-C7) involvement seen in degenerative cervical spine disease in the elderly because of lateral bending and the complex rotatory movements of the cervical spine occurring mostly at C3-C5 levels in CD.[1] Cervical myelopathy is more commonly seen in dystonic cerebral palsy than other forms of CD and strongly associated with age of the patients, and the severity of neck dystonia.[3]

Postoperative outcomes in patients with CD treated for CM varies among different studies.[4],[5],[6] Dystonic spasms can also impede postoperative healing and impaired fixation. Hence, pre and postoperative stabilization is a requisite for patients with cervical dystonia and myelopathy in order to achieve good surgical fusion. The first report of use of botulinum toxin in CD to aid in surgical correction of CM was published by Traynelis et al.[7] Botulinum toxin is shown to be effective in the perioperative reduction of severe cervical dystonia permitting cervical decompression, reconstruction and halo immobilization to aid in fusion.[6] Botulinum toxin works by inhibiting the release of the neurotransmitter acetylcholine by the nerve cells, the maximal effect of which is seen in 2–3 weeks. Botulinum toxin is thus useful in reducing the dystonic spasms, which would interfere with the postsurgical stabilization and cervical fusion.[2],[6],[7],[8],[9]

In conclusion, adequate management of the cervical dystonia per se would increase the chances of good recovery in patients operated for cervical myelopathy. Preoperative botulinum toxin injection will help in reducing the dystonic movements aiding in proper perioperative stabilization and augment fusion post operatively. Multidisciplinary coordination involving the neurologist and neurosurgeon may result in better outcomes.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


We acknowledge the patient for his support and co-operation.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


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