Summary
Definition
History and exam
Key diagnostic factors
- involuntary twisting or deviation of the neck
- neck pain
- presence of alleviating maneuver
- abnormal head posture
- otherwise normal neurologic exam
Other diagnostic factors
- head tremor
- insidious onset
- asymmetrical hypertrophy of neck muscles
- normal range of motion (ROM) of neck (early in course)
Risk factors
- female sex
- middle age
- family history of cervical dystonia
- exposure to dopamine-blocking drugs
- history of physical trauma
Diagnostic tests
1st tests to order
- clinical diagnosis
Tests to consider
- cervical x-rays
- CT or MRI of the brain
- CT or MRI of the neck
- genetic testing
- serum ceruloplasmin, urinary copper excretion
- electromyography
Treatment algorithm
without pain or functional impairment
with functional impairment or pain or diminished quality of life
refractory to botulinum toxin therapy
Contributors
Authors
David B. Sommer, MD, MPH
Director, Movement Disorders
Community Neuroscience Services
Westborough, MA
Disclosures
DBS declares that he has no competing interests.
Acknowledgements
Dr David B. Sommer would like to gratefully acknowledge Dr Mark A. Stacy, a previous contributor to this topic.
Disclosures
MAS has received speaking honoraria and consulting fees from Allergan, the manufacturer of Botox; he has received research grant support from Ipsen, manufacturer of Dysport, and Merz, manufacturer of Xeomin; he is an author of a reference cited in this topic. Duke University has received funding from Allergan for a continuing medical education program.
Peer reviewers
Sherita Lynn Holmes, MD
Pediatric emergency medicine
Emory University/Children’s healthcare of Atlanta
Atlanta, GA
Disclosures
SLH declares that she has no competing interests.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
References
Key articles
Albanese A, Bhatia KP, Cardoso F, et al. Isolated cervical dystonia: diagnosis and classification. Mov Disord. 2023 Aug;38(8):1367-78.Full text Abstract
Albanese A, Asmus F, Bhatia KP, et al. EFNS guidelines on diagnosis and treatment of primary dystonias. Eur J Neurol. 2011 Jan;18(1):5-18.Full text Abstract
Rodrigues FB, Duarte GS, Marques RE, et al. Botulinum toxin type A therapy for cervical dystonia. Cochrane Database Syst Rev. 2020 Nov 12;11:CD003633.Full text Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Arthritis of cervical spine
- Neck mass
- Cerebral mass, lesion or infarct
More DifferentialsGuidelines
- Isolated cervical dystonia: diagnosis and classification
- EFNS guidelines on diagnosis and treatment of primary dystonias
More Guidelines
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