Stiff-person syndrome: a case report

Authors

Keywords:

STIFF-PERSON SYNDROME, MUSCLE RIGIDITY, SPASM, PARANEOPLASTIC SYNDROMES.

Abstract

Introduction: stiff-person syndrome (SPS) is a rare immune-mediated central nervous system disorder characterized by painful contraction, stiffness and spasms affecting the axial and peripheral musculature in a progressive manner.

Case report: a 47-year-old male patient, affected by stiffness and weakness in the right lower extremity, progressing in weeks to the other lower extremity and in months to the axial and proximal musculature of the four extremities; making ambulation, trunk flexion and arm movement difficult. He presented lumbar hyperlordosis and muscle spasms. Electromyography showed continuous muscle activity in agonist and antagonist muscles. Stiff person syndrome (SPS) was diagnosed. The treatment applied was immunomodulator (steroids and intravenous immunoglobulins) and symptomatic with benzodiazepines, baclofen and physical therapy, the evolution was satisfactory.

Conclusions: SPS is not frequent in neurological-clinical practice, its definitive diagnosis depends on specific complementary studies, and its clinical manifestations make it detectable allowing timely management and prevention of disabilities associated with these patients.

Downloads

Download data is not yet available.

Author Biographies

Reinier Cardentey-Sánchez, Universidad de Ciencias Médicas de Pinar del Río. Hospital Abel Santamaría Cuadrado.

Especialista de primer grado en Neurología. Hospital Abel Santamaría Cuadrado.

Mayelin Pimienta-Medina, Universidad de Ciencias Médicas de Pinar del Río. Hospital Abel Santamaría Cuadrado.

Especialista de primer grado en Neurología. Hospital Abel Santamaría Cuadrado.

Marité García-Llano, Universidad de Ciencias Médicas de Pinar del Río. Hospital Abel Santamaría Cuadrado.

Especialista de primer grado en Medicina General Integral y Neurología. Jefe de Departamento de Neurología en Hospital Abel Santamaría Cuadrado.

Ernesto Cruz-Menor, Universidad de Ciencias Médicas de Pinar del Río. Hospital Abel Santamaría Cuadrado.

Especialista de primer grado en Neurología. Especialista de segundo grado en Neurofisiología. Profesor Asistente. Hospital Abel Santamaría cuadrado.

Joel Luis Mesa-Tejido, Universidad de Ciencias Médicas de Pinar del Río. Hospital Abel Santamaría Cuadrado.

Especialista de primer grado en Neurología. Hospital Abel Santamaría Cuadrado.

References

1. El-Abassi R, Soliman MY, Villemarette-Pittman N, England JD. SPS: Understanding the complexity. Journal of the Neurological Sciences. [Internet]. 2019 [citado 01/07/2021]; 404 (2019): 137–149. Disponible en: https://www.researchgate.net/profile/Nicole-Villemarette-Pittman/publication/334527723_SPS_Understanding_the_complexity/links/5d935e90458515202b781a7a/SPS-Understanding-the-complexity.pdf

2. Moerch F, Woltman HW. Progressive and fluctuating muscular rigidity and spasm (stiffman syndrome): report of a case and some observations in 13 other cases. Proc Staff Meet Mayo Clin [Internet]. 1956 Jul [citado 01/07/2021]; 31(15): 421-7. Disponible en: https://pubmed.ncbi.nlm.nih.gov/13350379/

3. Muacevic A, Adler J, Ortiz J, Ghani M, Tambo W, Bashir F, et al. Stiff-Person Syndrome: A Treatment Update and New Directions. Cureus [Internet]. 2020 [citado 01/07/2021]; 12(12): 11995. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7793517/

4. Bernardo F, Rebordão L, Rêgo A, Machado S, Passos J, Costa C, et al. Stiff person spectrum disorders: An illustrative case series of their phenotypic and antibody diversity. Journal of Neuroimmunology [Internet]. 2020 [citado 01/07/2021]; 341: 577192. Disponible en: https://www.jni-journal.com/article/S0165-5728(19)30636-8/fulltext

5. Guajardo G, Cea G. Síndrome de persona rígida confundido por trastorno conversivo: un error común. Caso Clínico. Rev. méd. Chile [Internet]. 2019 [citado 01/07/2021]; 147(6): 799-802. Disponible en: http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S003498872019000600799&lng=es

6. Caffrey D, Finn CT, Song SM, Burton III F, Arsan C. Stiff Person Syndrome and Psychiatric Comorbidities: A Systematic Review. Journal of the Academy of Consultation-Liaison Psychiatry [Internet]. 2021 [citado 01/07/2021]; 62(1): 3-13. Disponible en: https://www.sciencedirect.com/science/article/pii/S0033318220302395

7. Dade M, Berzero G, Izquierdo C, Giry M, Benazra M, Delattre J-Y, et al. Neurological syndromes associated with Anti-GAD antibodies. International Journal of Molecular Sciences. [Internet]. 2020 [citado 01/07/2021]; 21(10):3701. disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7279468/

8. Baizabal-Carvallo JF, Jankovic J. Autoimmune and paraneoplastic movement disorders: An update. Journal of the Neurological Sciences [Internet]. 2018 [citado 01/07/2021]; 385: 175-184. Disponible en: https://pubmed.ncbi.nlm.nih.gov/29406902/

9. Gómez-Fernández L, Maragoto-Rizo C, Jiménez-Gil FJ. Síndromes de actividad continua de las fibras musculares originados en el sistema nervioso. Rev Mex Neuroci [Internet]. 2013 [citado 01/07/2021]; 14(6): 349-355. Disponible en: http://previous.revmexneurociencia.com/wp-content/uploads/2013/11/Nm136-08.pdf

10. Balint B, Meinck HM. Pragmatic treatment of stiff person spectrum disorders. Movement Disorders Clinical Practice [Internet]. 2018 [citado 01/07/2021]; 5(4): 394-401. Disponible en: https://movementdisorders.onlinelibrary.wiley.com/doi/full/10.1002/mdc3.12629

11. Vaiyapuri A, Kashyap P, Kashyap N, Muthusamy H, Unnikrishnan R, Alqahtani M. Is stiff person syndrome benefited by physical therapy intervention? Summary of case reports. BioMed Research International [Internet]. 2019 [citado 01/07/2021]; 2019: 1-7. Disponible en: https://www.hindawi.com/journals/bmri/2019/5613680/

12. Sarva H, Deik A, Ullah A, Severt WL. Clinical spectrum of stiff person syndrome: a review of recent reports. Tremor and Other Hyperkinetic Movements [Internet]. 2016 [citado 01/07/2021]; 6: 340. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4790195/

13. Xie Y-y, Meng H-m, Zhang F-x, Maimaiti B, Jiang T, Yang Y. Involuntary movement in stiff-person syndrome with amphiphysin antibodies: A case report. Medicine [Internet]. 2021 [citado 01/07/2021]; 100(3): 24312. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7837982/

14. Hicken J, Ramirez D, Rigby M, Minasian A. Stiff-Person Syndrome: Seeing Past Comorbidities to Reach the Correct Diagnosis. Case Rep Neurol Med [Internet]. 2021 [citado 01/07/2021]; 2021: 6698046. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7868164/

Published

2021-11-05

How to Cite

1.
Cardentey-Sánchez R, Pimienta-Medina M, García-Llano M, Cruz-Menor E, Mesa-Tejido JL. Stiff-person syndrome: a case report. Rev Ciencias Médicas [Internet]. 2021 Nov. 5 [cited 2025 Aug. 9];25(6):e5218. Available from: https://revcmpinar.sld.cu/index.php/publicaciones/article/view/5218

Issue

Section

CASE REPORTS