Cervical dystonia, also called spasmodic torticollis, is a focal dystonia in which the neck muscles contract involuntarily, turning, tilting or pulling the head into an abnormal position. It is the most common focal dystonia. It usually starts in adulthood, is often painful, and frequently comes with a jerky head tremor. Many patients find that touching the chin or the back of the head briefly relaxes the pull.
Botulinum toxin injections are the first-line treatment.[1] When injections stop working, deep brain stimulation is the most effective option. A pooled meta-analysis of 208 patients found that DBS improved the standard cervical dystonia scale (TWSTRS) by 58.8% on average, with 61.3% improvement in disability and 46.6% in pain; 89% of patients were responders.[2]
Symptoms of cervical dystonia
- The head turns to one side, tilts toward a shoulder, or pulls forward or backward
- One shoulder rises
- Neck and shoulder pain, often the most disabling symptom
- An irregular, jerky head tremor (dystonic head tremor, see headtremor.com)
- Symptoms worse when walking, standing, under stress or when tired, and better lying down
- Sensory tricks: a light touch to the face or head eases the pull
Types of cervical dystonia: the head and neck patterns
Specialists describe the abnormal posture precisely, because it decides which muscles to inject and helps plan surgery. The modern system separates movements of the head on the neck (“-caput”) from movements of the neck itself (“-collis”).
| Pattern | Direction of pull |
|---|---|
| Torticollis / torticaput | Rotation: the chin turns toward one shoulder (the most common pattern) |
| Laterocollis / laterocaput | Tilt: the ear moves toward the shoulder |
| Anterocollis / anterocaput | Forward flexion: the head and neck bend forward |
| Retrocollis / retrocaput | Extension: the head pulls backward |
| Lateral shift / sagittal shift | The head moves sideways or forward on the shoulders |
Most patients have a combination of these. Anterocollis and complex patterns are harder to treat with injections because the muscles involved are deep, and are one reason patients are referred for DBS.
What causes cervical dystonia?
In most adults no cause is found (idiopathic). Some cases are genetic, for example in THAP1 (DYT6), GNAL or ANO3 dystonia; some follow medication exposure (tardive dystonia) or, rarely, neck trauma. Cervical dystonia is not caused by a problem in the neck bones or the muscles themselves. The fault lies in the brain circuits that control head posture.
How is cervical dystonia treated?
- Botulinum toxin injections every few months into the overactive muscles. Level A evidence supports them as first-line therapy.[1]
- Physiotherapy and posture retraining, combined with injections.
- Oral medications, with modest effect in adults.
- Deep brain stimulation of the globus pallidus internus (GPi) or the subthalamic nucleus (STN) for patients who no longer get adequate relief. See treatment for cervical dystonia.
How well does DBS work for cervical dystonia?
A randomized, sham-controlled trial in 62 patients with medication-refractory cervical dystonia, published in Lancet Neurology, found significantly greater improvement with pallidal stimulation than with sham stimulation.[3] Two later meta-analyses pooled the published patients:
- 208 patients, mean follow-up 23 months: TWSTRS total improved 58.8%, severity 53.9%, disability 61.3%, pain 46.6%; 89% improved by at least 25%.[2]
- 86 patients, mean follow-up 22 months: average improvement 56.6%; GPi and STN gave similar results; patients operated at a younger age improved more.[4]
These are literature figures. Individual results vary.
Our results. In our own case series, patients treated with DBS for cervical dystonia, Meige syndrome and myoclonus-dystonia have shown more than 90% improvement in their symptoms, based on clinical assessment and documented on video. Individual results vary.
Patient videos: cervical dystonia after DBS
These patients were treated at Neuromodulation in Turkey and gave written consent for their videos to be published.
Frequently asked questions
Is cervical dystonia the same as torticollis?
Spasmodic torticollis is the older name for cervical dystonia. Strictly, torticollis describes only the rotation pattern, while cervical dystonia includes all patterns: rotation, tilt, forward and backward pull.
Can cervical dystonia go away?
A minority of patients have a remission, usually in the first years, but most need long-term treatment.
What happens when botulinum toxin stops working for cervical dystonia?
Some patients develop a weaker response over time, or the effect lasts too briefly. The options then include adjusting the injection plan, and for disabling symptoms, deep brain stimulation.
Does DBS help the neck pain of cervical dystonia?
Yes. In the pooled analysis of 208 patients, pain scores improved by 46.6% on average after DBS.[2]
Does DBS help dystonic head tremor?
Yes, DBS can control dystonic head tremor. Prof. YILMAZ’s published series on double-target DBS for rare tremor syndromes included patients with dystonic tremor.[5] More on headtremor.com.
Sources
- Simpson DM, Hallett M, Ashman EJ, et al. Practice guideline update summary: Botulinum neurotoxin for the treatment of blepharospasm, cervical dystonia, adult spasticity, and headache. Neurology. 2016;86(19):1818–1826. Source
- Tsuboi T, Wong JK, Almeida L, et al. A pooled meta-analysis of GPi and STN deep brain stimulation outcomes for cervical dystonia. J Neurol. 2020;267(5):1278–1290. doi:10.1007/s00415-020-09703-9 Source
- Volkmann J, Mueller J, Deuschl G, et al. Pallidal neurostimulation in patients with medication-refractory cervical dystonia: a randomised, sham-controlled trial. Lancet Neurol. 2014;13(9):875–884. Source
- Hua X, Zhang B, Zheng Z, et al. Predictive factors of outcome in cervical dystonia following deep brain stimulation: an individual patient data meta-analysis. J Neurol. 2020;267(6):1780–1792. doi:10.1007/s00415-020-09765-9 Source
- Yılmaz A, et al. Double-target (Vim + PSA) deep brain stimulation for rare tremor syndromes, including dystonic tremor. Stereotact Funct Neurosurg. 2024. doi:10.1159/000539162 (PMID 38934181) Source