Cervical dystonia

Authors: P. Kaňovský
Authors‘ workplace: Neurologická klinika LF UP a FN Olomouc
Published in: Cesk Slov Neurol N 2007; 70/103(4): 358-370
Category: Minimonography


The cervical dystonia is probably the most frequently seen type of idiopathic torsion dystonia. In the case of cervical dystonia, the abnormal involuntary contraction affects neck muscles in different distribution. It leads to the dystonic dyskinesia of the neck, which results in the dystonic posture of the head. The disorder is widely known as „spastic torticollis“, and has been described under this name in the classical neurological literature. The most frequently affected muscles are sternocleidomastoid muscle, splenius capitis muscle, trapezius muscle, semispinalis capitis and cervicis muscles, levator scapule muscle and group of scalene muscles. Less frequently are affected other neck muscles, particularly muscles of the cranio-cervical area: rectus capitis maior and minor muscles, obliquus capitis maior and minor muscles, multifidi muscles and rotatores capitis muscles. There exist an indirect evidence, that deep neck muscles, as for instance longus colli muscle, can participace on the dyskinesia. However, these muscles are actually not accessible for the palpation or electromyographic (EMG) examination. Suprahyoid and infrahyoid muscles can also participate on the dystonic dyskinesia, particularly geniohyoideus muscle and mylohyoideus muscle. Muscles, which are involved in the dyskinesia, form the so-called "muscle pattern" of cervical dystonia. Cervical dystonia manifests usually in four types, which have a typical clinical pattern, and which correspond to the particular muscle pattern: torticollis, retrocollis, laterocollis and anterocollis. Nevertheless, cervical dystonia is a variable syndrome, thus the combination or overlapping of these four types might be frequent. The clinical diagnosis of fully developed syndrome is (due to its typical clinical picture) relatively easy. On the other hand, the differential diagnosis is relatively difficult. It is necessary to exclude (using all accessible paraclinical examinations) all possible causes of secondary dystonia. To further map the clinical syndrome and its characteristics, it is necessary to use EMG methods. The needle EMG, polymyography, interference pattern analysis and turns/amplitude analysis are those which are the most helpful methods to describe the detailed characteristics of cervical dystonia. Treatment of choice of cervical dystonia is botulinum toxin A (BTX). The toxin is injected into the muscles involved in the dystonic dyskinesia, either on the basis of clinical examination and palpation or with EMG guidance. The treatment effect lasts usually up to three months, then the injection must be repeated. The treatment with BTX can be complicated by the change of muscular pattern of dystonia or development of neutralising antibodies against BTX. In such a case, the diagnostic process should be repeated and another serotype of BTX should be used for the further treatment.

Key words:
cervical dystonia – torticollis – polymyography – botulinum toxin A


1. Fahn S, Marsden CD, Calne DB. Classification and investigation of dystonia. In: Marsden CD, Fahn S (Eds). Movement Disorders 2. London: Butterworths 1987; 332-358.

2. Rothwell JC, Obeso JA, Day BL, Marsden CD. Pathophysiology of dystonias. In: Desmedt JE (Ed). Motor control mechanisms in health and disease. New York: Raven Press 1983; 851-863.

3. Rondot P. The shadow of movement. J Neurol 1991; 238: 411-419.

4. Kaji R, Shibasaki H, Kimura J. Writer´s cramp: a disorder of motor subroutine? Ann Neurol 1995; 38: 837-839.

5. Hallet M. Is dystonia a sensory disorder? Ann Neurol 1995; 38: 139-140.

6. Hallet M. Pathophysiology of writer´s cramp. Hum Mov Sci 2006; 25: 454-463.

7. Hallet M. Pathophysiology of dystonia. J Neural Transm 2006; 70: 485-488.

8. Ceballos-Baumann AO, Passingham RE, Warner T, Playford ED, Marsden CD, Brooks D J. Overactive prefrontal and underactive motor cortical areas in idiopathic dystonia. Ann Neurol 1995; 37: 363-372.

9. Ceballos-Baumann AO, Sheean G, Passingham RE, Marsden CD, Brooks DJ. Botulinum toxin does not reverse the cortical dysfunction associated with writer´s cramp. A PET study. Brain 1997; 120: 571-582.

10. Eidelberg D, Moeller JR, Ishikawa T, Dhawan V, Spetsieris P, Przedborski S, Fahn S. The metabolic topography of idiopathic torsion dystonia. Brain 1995; 118: 1473-1484.

11. Asanuma K, Karbon-Correll M, Eidelberg D. Neuroimaging in human dystonia. J Med Incest 2005, 52(Suppl 1): 272-279.

12. Day BL, Marsden CD, Obeso JA, Rothwell JC. Reciprocal inhibition between the muscles of human forearm. J Physiol 1984; 349: 519-534.

13. Deuschl G, Seifert C, Heinen F, Illert M, Lücking CH. Reciprocal inhibition of forearm flexor muscles in spasmodic torticollis. J Neurol Sci 1992; 113: 85-90.

14. Priori A, Berardelli A, Mercuri B, Manfredi M. Physiological effects produced by botulinum toxin treatment of upper limb dystonia. Changes in reciprocal inhibition between forearm muscles. Brain 1995; 118: 801-807.

15. Deuschl G, Heinen F, Kleedorfer B, Wagner M, Lücking CM, Poewe W. Clinical and polymyographic investigation of spasmodic torticollis. J Neurol 1992; 239: 9-15.

16. Reilly JA, Hallet M, Cohen LG, Tarkka IM, Dang N. The N30 component of somatosensory evoked potentials in patients with dystonia. Electroencephalogr Clin Neurophysiol 1992; 84: 243-247.

17. Mazzini L, Schiepatti M. Activation of the neck muscles from the ipsi- or contralateral hemisphere during voluntary head movements in humans. A reaction - time study. Electroencephalogr Clin Neurophysiol 1992; 85: 183-189.

18. Kaňovský P, Streitová H, Dufek J, Rektor I. Lateralization of the P22/N30 component of the somatosensory evoked potentials of the median nerve in the patients with cervical dystonia. Mov Disord 1997; 12: 553-560.

19. Kaňovský P, Rektor I, Streitová H. Dystonie: porucha programu nebo výkonu pohybu? Čes Slov Neurol Neurochir 1997; 60: 115-120.

20. Kaňovský P, Streitová H, Dufek J, Rektor I. Change in lateralization of the P22/N30 cortical component of median nerve somatosensory evoked potentials in patients with cervical dystonia after successful treatment with botulinum toxin A. Mov Disord 1998; 13: 101-112.

21. Deuschl G, Toro C, Matsumoto J, Hallet M. Movement - related cortical potentials in writer´s cramp. Ann Neurol 1995; 38: 862-868.

22. Kaji R, Ikeda A, Ikeda T, Kubori T, Mezaki T, Kohara N, Kanda M, Nagamine T, Honda M, Rothwell JC, Shibasaki H, Kimura J. Physiological study of cervical dystonia. Task specific abnormality in cintingent negative variation. Brain 1995; 118: 511-522.

23. Mavroudakis N, Caroyer JM, Brunko E, Zegers de Beyl D. Abnormal motor evoked responses to transcranial magnetic stimulation if focal dystonia. Neurology 1995; 45: 1671-1677.

24. Ridding MC, Sheean G, Rothwell JC, Inzelberg R, Kujirai T. Changes in the balance between motor cortical excitation and inhibition in focal, task-specific dystonia. J Neurol Neurosurg Psychiatr 1995; 59: 493-498.

25. Ikoma K, Samii A, Mercuri B, Wassermann EM, Hallet M. Abnormal cortical motor excitability in dystonia. Neurology 1996; 46: 1371-1376.

26. Marsden CD, Obeso JA, Zarranz JJ, Lang AE. The anatomical basis of symptomatic hemidystonia. Brain 1985; 108: 463-483.

27. Tsui JKC, Eisen A, Stoessl AJ, Calne S, Calne DB. Double - blind study of botulinum toxin in spasmodic torticollis. Lancet 1986; 8501: 245-247.

28. Traube L. Spastische form der nervosen Heiserkeit. In: Traube L (Ed). Gesammelte Beitrage zur Pathologie und Physiologie. Vol. 2. Berlin: Hirschwald 1871; 674-678.

29. Gowers WR. Manual of disease of the nervous system. Philadelphia: Blakiston 1888: 1357.

30. Destarac L. Torticollis spasmodique et spasmes fonctionelles. Revue Neurologique 1901; 9: 591-597.

31. Schwalbe W. Eine eigentumliche tonische krampform mit hysterischen symptomen. Dissertation. Berlin: G. Schade 1908.

32. Oppenheim H. Ueber eine eigenartige Krampfkrankheit des kindlichen and jugendlichen alters (dysbasia lordotica progressiva, dystonia musculorum deformans). Neurologische Zentralblatt 1911, 30: 1090-1107.

33. Flatau E, Sterling W. Progressiver Torsionspasms bei kindern. Zeitschrift des Gesamte Neurologie und Psychiatrie 1911; 7: 586-612.

34. Beck D. Dystonia musculorum deformans with another case in the same family. Proc R Soc Med 1947; 40: 551.

35. Schneider S, Feifel E, Ott D, Schumacher M, Lucking CH, Deuschl G. Prolonged MRI T2 times of the lentiform nucleus in idiopathic spasmodic torticollis. Neurology 1994; 44: 846-50.

36. Kaňovský P, Halačková H, Dufek J. Výsledky komplexní terapie cervikálních dystonií za použití botulotoxinu. Čes Slov Neurol N 1994; 57: 169-172.

37. Kaňovský P, Streitová H, Dufek J, Rektor I. Change in the pattern of cervical dystonia might be the cause of benefit loss in patients treated by botulinum toxin. Eur J Neurol 1997; 4: 79-84.

38. Fuglsang-Frederiksen A, Oestergaard L, Sjö O, Werdelin N, Winkel H, Prytz S. Turns/amplitude analysis in botulinum toxin treatment of focal dystonia. Can J Neurol Sci 1993, 20: 350-351.

39. Kaňovský P, Bareš M, Streitová H, Klajblová H, Daniel P, Rektor I. Abnormalities of cortical excitability and cortical inhibition in cervical dystonia: evidence from somatosensory evoked potentials and paired transcranial magnetic stimulation recordings. J Neurol 2003; 250: 42-51.

40. Friedmann AH, Nashold B, Sharp R. Treatment of spasmodic torticollis with intradural selective rhizotomies. J Neurosurg 1993; 78: 46-53.

41. Kaňovský P, Streitová H, Daniel P, Hekerlová R, Bareš M, Dufek J. Dlouhodobá remise cervikální dystonie navozená léčbou botulotoxinem A - signál možného ovlivnění centrálního dystonického mechanismu ? Čes Slov Neurol N 1998; 61: 124-136.

42. Bareš M, Rektorová I, Baláž M, Streitová H, Minks E, Kaňovský P, Rektor I. Long-term treatment of cervical dystonia with botulinum toxin A – retrospective assessment of the clinical and quality of life impact. Neurology 2007; 68 (Suppl 1): A381.

43. Kaňovský P. Dystonia: disorder of motor preparation or motor performance? Mov Disord 2002; 17: 1143-1148.

44. Albanese A, Barnes MP, Bhatia KP et al. A systematic review on the diagnosis and treatment of primary idiopathic dystonie and dystonia plus syndromes: report of an EFNS/MDS-ES Task Force. Eur J Neurol 2006, 13: 433-444.

45. Kaňovský P. Dystonie. In: Růžíčka E, Roth J, Kaňovský P. Dyskinetické syndromy a onemocnění. Praha: Galén 2001: 97-150.

Paediatric neurology Neurosurgery Neurology

Article was published in

Czech and Slovak Neurology and Neurosurgery

Issue 4

2007 Issue 4

Most read in this issue
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.


Don‘t have an account?  Create new account