Abstract
Background A 28-year-old woman presented to hospital after an episode of severe lower back spasms that occurred during a stressful family gathering. She had a history of progressive difficulty bending forward and increasing lumbar lordosis.
Investigations Physical examination, spine MRI scan, abdominal and pelvic ultrasound, electromyogram, nerve conduction studies, cerebrospinal fluid analysis, breast examination, Pap smear, transabdominal and endovaginal ultrasound.
Diagnosis Stiff-person syndrome with high titer of antibodies against glutamic acid decarboxylase.
Treatment Benzodiazepines and intravenous immunoglobulins.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 12 print issues and online access
$209.00 per year
only $17.42 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
References
Moersch FP and Woltman HW (1956) Progressive fluctuating muscular rigidity and spasm (“stiff-man” syndrome); report of a case and some observations in 13 other cases. Mayo Clin Proc 31: 421–427
Murinson BB (2004) Stiff-person syndrome. Neurologist 10: 131–137
Murinson BB et al. (2004) Markedly elevated GAD antibodies in SPS: effects of age and illness duration. Neurology 63: 2146–2148
Ameli R et al. (2005) A neuropsychological assessment of phobias in patients with stiff person syndrome. Neurology 64: 1961–1963
Burns TM et al. (2003) Clinically disparate stiff-person syndrome with GAD65 autoantibody in a father and daughter. Neurology 61: 1291–1293
Dalakas MC et al. (2000) The clinical spectrum of anti-GAD antibody-positive patients with stiff-person syndrome. Neurology 55: 1531–1535
Meinck HM and Thompson PD (2002) Stiff man syndrome and related conditions. Mov Disord 17: 853–866
Thomas S et al. (2005) Stiff person syndrome with eye movement abnormality, myasthenia gravis, and thymoma. J Neurol Neurosurg Psychiatry 76: 141–142
Solimena M et al. (1990) Autoantibodies to GABA-ergic neurons and pancreatic beta cells in stiff-man syndrome. N Engl J Med 322: 1555–1560
Raju R et al. (2005) Analysis of GAD65 autoantibodies in Stiff-Person syndrome patients. J Immunol 175: 7755–7762
Schiff D et al. (2003) Anti-GAD antibody positive stiff-limb syndrome in multiple myeloma. J Neurooncol 65: 173–175
Rosin L et al. (1998) Stiff-man syndrome in a woman with breast cancer: an uncommon central nervous system paraneoplastic syndrome. Neurology 50: 94–98
Saiz A et al. (1999) Anti-amphiphysin I antibodies in patients with paraneoplastic neurological disorders associated with small cell lung carcinoma. J Neurol Neurosurg Psychiatry 66: 214–217
Sommer C et al. (2005) Paraneoplastic stiff-person syndrome: passive transfer to rats by means of IgG antibodies to amphiphysin. Lancet 365: 1406–1411
Rhee P et al. (2005) Tetanus and trauma: a review and recommendations. J Trauma 58: 1082–1088
Khasani S et al. (2004) Hyperekplexia and stiff-man syndrome: abnormal brainstem reflexes suggest a physiological relationship. J Neurol Neurosurg Psychiatry 75: 1265–1269
Arimura K et al. (2002) Isaacs' syndrome as a potassium channelopathy of the nerve. Muscle Nerve (Suppl 11): S55–S58
Vasconcelos OM and Dalakas MC (2003) Stiff-person syndrome. Curr Treat Options Neurol 5: 79–90
Dalakas MC (2005) The role of IVIg in the treatment of patients with stiff person syndrome and other neurological diseases associated with anti-GAD antibodies. J Neurol 252 (Suppl 1): I19–I25
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Competing interests
The authors declare no competing financial interests.
Rights and permissions
About this article
Cite this article
Wilson, R., Murinson, B. Sudden spasms following gradual lordosis—the stiff-person syndrome. Nat Rev Neurol 2, 455–459 (2006). https://doi.org/10.1038/ncpneuro0259
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1038/ncpneuro0259
