Pseudodystonic Emergencies

  • Beom S. Jeon
  • Jong-Min Kim
Part of the Current Clinical Neurology book series (CCNEU)


Patient 1: A 6-year-old boy presented to the outpatient department for head tilt. Since the age of 2, head tilt to the right side had been noted. When he was 4 years old, cervical spine imaging was performed. Congenital laminar fusion on the right at the C2-3 vertebrae was found. At age 6, posterior in situ fusion of the C2-3 vertebrae with iliac bone graft was performed. However, head tilt was not relieved. The patient was referred to a neurologist. Examination revealed head tilt to the right with painful contraction of the neck muscles. Manual rotation of the neck to either side and straightening of the neck caused severe pain. The images obtained before and after operation were reviewed. There was an enhancing lesion anterior to atlas, axis, and C3 vertebra. It appeared to be an inflammatory lesion, whose exact etiology was not clear. Anti-inflammatory drugs and muscle relaxant were started. Painful contractions of neck muscles were somewhat reduced, resulting in mild improvement in head tilt.


Down Syndrome Juvenile Rheumatoid Arthritis Acute Rheumatic Fever Head Tilt Congenital Muscular Torticollis 
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  1. 1.
    Fahn S. Concept and classification of dystonia. Adv Neurol 1988;50:1–8.PubMedGoogle Scholar
  2. 2.
    Fahn S, Bressman SB, Marsden CD. Classification of dystonia. Adv Neurol 1998;78:1–10.PubMedGoogle Scholar
  3. 3.
    Meinck HM, Thompson PD. Stiff man syndrome and related conditions. Mov Disord 2002;17:853–866.PubMedCrossRefGoogle Scholar
  4. 4.
    Arimura K, Sonoda Y, Watanabe O, et al. Isaacs’ syndrome as a potassium channelopathy of the nerve. Muscle Nerve 2002;S11:55–58.CrossRefGoogle Scholar
  5. 5.
    Mandel H, Tirosh E, Berant M. Sandifer syndrome reconsidered. Acta Paediatr Scand 1989;78:797–799.PubMedCrossRefGoogle Scholar
  6. 6.
    Uziel Y, Rathaus V, Pomeranz A, Solan H, Wolach B. Torticollis as the sole initial presenting sign of systemic onset juvenile rheumatoid arthritis. J Rheumatol 1998;25:166–168.PubMedGoogle Scholar
  7. 7.
    Fiorillo L, Robinson JL. Localized tetanus in a child. Ann Emerg Med 1999;33:460–463.PubMedCrossRefGoogle Scholar
  8. 8.
    Berry DS, Moriarty RA. Atlantoaxial subluxation related to pharyngitis: Grisel’s syndrome. Clin Pediatr 199;38:673–675.Google Scholar
  9. 9.
    Bredenkamp JK, Maceri DR. Inflammatory torticollis in children. Arch Otolaryngol Head Neck Surg 1990;116:310–313.PubMedGoogle Scholar
  10. 10.
    Visudhiphan P, Chiemchanya S, Somburanasin R, Dheandhanoo D. Torticollis as the presenting sign in cervical spine infection and tumor. Clin Pediatr 1982;21:71–76.CrossRefGoogle Scholar
  11. 11.
    Shafir Y, Kaufman BA. Quadriplegia after chiropractic manipulation in an infant with congenital torticollis caused by a spinal cord astrocytome. J Pediatr 1992;120:266–269.CrossRefGoogle Scholar
  12. 12.
    Merello M, Garcia H, Nogues M, Leiguarda R. Masticatory muscle spasm in a non-Japanese patient with Satoyoshi syndrome successfully treated with botulinum toxin. Mov Disord 1994;9:104–105.PubMedCrossRefGoogle Scholar
  13. 13.
    Curtis BH, Blank S, Fisher RL. Atlantoaxial dislocation in Down’s syndrome; report of two patients requiring surgical correction. JAMA 1968;205:464–465.PubMedCrossRefGoogle Scholar
  14. 14.
    Brougham DI, Cole WG, Dickens DR, Menelaus MB. Torticollis due to a combination of sterno-mastoid contracture and congenital vertebral anomalies. J Bone Joint Surg Br 1987;71:404–407.Google Scholar
  15. 15.
    Kiwak KJ, Deray MJ, Shields WD. Torticollis in three children with syringomyelia and spinal cord tumor. Neurology 1983;33:946–948.PubMedGoogle Scholar
  16. 16.
    Grogaard B, Dullerud R, Magnaes B. Acute torticollis in children due to atlanto-axial rotary fixation. Arch Orthop Trauma Surg 1993;112(4):185–188.PubMedCrossRefGoogle Scholar
  17. 17.
    Subach BR, McLaughlin MR, Albright AL, Pollack IF. Current management of pediatric atlantoaxial rotatory subluxation. Spine 1998;23(20):2174–2179.PubMedCrossRefGoogle Scholar
  18. 18.
    Schwarz N. The fate of missed atlanto-axial rotatory subluxation. Arch Orthop Trauma Surg 1998;117:288–289.PubMedCrossRefGoogle Scholar
  19. 19.
    Jagoda A, Riggio S, Burguieres T. Cephalic tetanus: a case report and review of the literature. Am J Emerg Med 1988;6:128–130.PubMedCrossRefGoogle Scholar

Copyright information

© Humana Press Inc. 2005

Authors and Affiliations

  • Beom S. Jeon
    • 1
  • Jong-Min Kim
    • 1
  1. 1.Department of NeurologySeoul National University HospitalSeoulSouth Korea

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