J Korean Foot Ankle Soc.  2019 Sep;23(3):131-134. 10.14193/jkfas.2019.23.3.131.

Stiff-Person Syndrome: Diagnostic Difficulty

Affiliations
  • 1Department of Orthopedic Surgery, Chungnam National University School of Medicine, Daejeon, Korea. gs1899@hanmail.net

Abstract

Stiff-person syndrome is a rare disorder, and the natural history of the syndrome has not been completely ascertained. The symptoms range from mild to severe and can progress over time: the final result can be significant disability. However, this syndrome is often misdiagnosed due to a lack of understanding of the clinical manifestations. We report the case of a patient who presented with slowly progressing gait disturbance and lower extremity pain and was later diagnosed as suffering from stiff-person syndrome. The patient experienced symptomatic improvement after the administration of benzodiazepines. No recurrence of symptoms has been reported. If the characteristic clinical features and electromyography findings of the syndrome are accurately interpreted, diagnosis of patients with abnormal muscle tension can be effectively done.

Keyword

Stiff-person syndrome; Leg; Spasm; Autoimmune disease; Autoantibodies

MeSH Terms

Autoantibodies
Autoimmune Diseases
Benzodiazepines
Diagnosis
Electromyography
Gait
Humans
Leg
Lower Extremity
Muscle Tonus
Natural History
Recurrence
Spasm
Stiff-Person Syndrome*
Autoantibodies
Benzodiazepines

Figure

  • Figure 1 Clinical image (A), standing anteroposterior and lateral foot X-ray images (B, C) showing hyperextension of the metatarsophalangeal joint and flexion of the interphalangeal joint, respectively.

  • Figure 2 Spine magnetic resonance image. No specific findings other than mild bulging of the disc were observed. (A, B) Lumbar spine T1-weighted, T2-weighted and (C) cervical-thoracic-lumbar sagittal image.


Reference

1. Meinck HM, Thompson PD. Stiff man syndrome and related conditions. Mov Disord. 2002; 17:853–866. DOI: 10.1002/mds.10279.
Article
2. Moersch FP, Woltman HW. Progressive fluctuating muscular rigidity and spasm (“stiff-man” syndrome); report of a case and some observations in 13 other cases. Proc Staff Meet Mayo Clin. 1956; 31:421–427.
3. Henningsen P, Meinck HM. Specific phobia is a frequent non-motor feature in stiff man syndrome. J Neurol Neurosurg Psychiatry. 2003; 74:462–465. DOI: 10.1136/jnnp.74.4.462.
Article
4. Thompson PD. The stiff-man syndrome and related disorders. Parkinsonism Relat Disord. 2001; 8:147–153. DOI: 10.1016/S1353-8020(01)00029-3.
Article
5. Murinson BB. Stiff-man syndrome: GABA, GAD, and mechanisms of disease. Neuroscientist. 2000; 6:147–150. DOI: 10.1177/107385840000600304.
Article
6. Helfgott SM. Stiff-man syndrome: from the bedside to the bench. Arthritis Rheum. 1999; 42:1312–1320. DOI: 10.1002/1529-0131(199907)42:7〈1312::AID-ANR2〉3.0.CO;2-W.
Article
7. Barker RA, Revesz T, Thom M, Marsden CD, Brown P. Review of 23 patients affected by the stiff man syndrome: clinical subdivision into stiff trunk (man) syndrome, stiff limb syndrome, and progressive encephalomyelitis with rigidity. J Neurol Neurosurg Psychiatry. 1998; 65:633–640. DOI: 10.1136/jnnp.65.5.633.
Article
8. Choi HY, Sung DH. Stiff-man syndrome: a case report. J Korean Acad Rehabil Med. 2007; 31:232–237.
9. Vicari AM, Folli F, Pozza G, Comi GC, Comola M, Canal N, et al. Plasmapheresis in the treatment of stiff-man syndrome. N Engl J Med. 1989; 320:1499. DOI: 10.1056/NEJM198906013202220.
Article
10. Brown P, Marsden CD. The stiff man and stiff man plus syndromes. J Neurol. 1999; 246:648–652. DOI: 10.1007/s004150050425.
Article
Full Text Links
  • JKFAS
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr