SciELO - Scientific Electronic Library Online

 
vol.41 issue1Dyke-Davidoff-Masson Syndrome as a cause of hemiatrophy cerebral: About a clinical caseTherapeutic failure in relapsing-remitting multiple sclerosis: Definition and algorithm for management, an expert consensus author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand

Journal

Article

Indicators

Related links

  • On index processCited by Google
  • Have no similar articlesSimilars in SciELO
  • On index processSimilars in Google

Share


Acta Neurológica Colombiana

Print version ISSN 0120-8748On-line version ISSN 2422-4022

Abstract

ESPINOZA, Stefany Lizet; CANALES, Diego Leomar; BURGOS, Maria Alexandra  and  SAQUISELA, Víctor Vladimir. Spectrum of stiff-person syndrome: Case series from a hospital of national importance. Acta Neurol Colomb. [online]. 2025, vol.41, n.1, e1924.  Epub Mar 12, 2025. ISSN 0120-8748.  https://doi.org/10.22379/anc.v41i1.1924.

Introduction:

Stiff person syndrome (SPR), a rare disease of the central nervous system with immune-mediated pathophysiology. Associated with anti-GAD antibodies (80%) and other autoimmune pathologies such as DM2 and hyperthyroidism. Clinically characterized by predominantly axial and peripheral muscle rigidity, exacerbated muscle spasms to tactile or auditory stimuli, and psychiatric disorders. Diagnosis is challenging; however, it is clinical. Favorable response to benzodiazepine and immunoglobulin therapeutics supports the stated diagnosis.

Cases presentation:

We present 3 cases of women attended at a referral hospital, Lima-Peru; mean age 40 years. Clinical presentation characterized by stiffness predominantly in the lower limbs, hyperreflexia, pain with functional limitation in walking, muscle spasms. They have a history of hyperthyroidism and vitiligo, in addition to psychiatric disorders, especially anxiety. All met clinical criteria and two cases met laboratory criteria as ANTI-GAD65 positive; broad differential diagnoses were performed. All received immunoglobulin and benzodiazepines with favorable response.

Discussion:

SPR, a rare and immune-mediated clinical entity of subacute onset characterized by muscle stiffness and spasms, which are sudden and precipitated. The 3 cases in mention presented this clinical picture, and response to immunomodulatory treatment. Reports of this entity are scarce.

Conclusions:

We present a series of cases of the spectrum of PRRS from a National Reference Hospital, of which 2 patients presented ANTI GAD65 and characteristic clinical picture, with adequate differential diagnosis and medical treatment that was evidenced with favorable clinical response. Knowledge of this pathology is of great importance to improve the prognosis and quality of life of these patients.

Keywords : Stiff Person Syndrome; Muscular rigidity; Antibodies; Benzodiazepines; Immunoglobulin; Peru.

        · abstract in Spanish     · text in Spanish     · Spanish ( pdf )