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[Stiff-person syndrome: a case report].

I Cabo-López¹, M Negueruela-López, P García-Bermejo

¹Servicio de Neurología, Fundación Jiménez Díaz, Madrid, España. icabol@yahoo.es

Revista De Neurologia

|September 10, 2008

Summary

Stiff-person syndrome, an autoimmune disorder, involves muscle rigidity and spasms. Early diagnosis and treatment with corticosteroids and antispastic agents can lead to symptom improvement.

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Area of Science:

Neurology
Immunology
Autoimmune Diseases

Background:

Stiff-person syndrome is a rare autoimmune neurological disorder characterized by axial muscular rigidity and spasms.
It is often associated with glutamic acid decarboxylase (anti-GAD) antibodies, which affect GABAergic transmission.

Observation:

A case of a 29-year-old female presenting with psychogenic mutism who subsequently developed generalized stiffness, hyperreflexia, and abdominal muscle contractions.
Laboratory findings revealed anti-GAD antibodies, along with antibodies against parietal cells, microsomal/TPO, and thyroglobulin, and oligoclonal IgG bands in CSF.

Findings:

The patient's clinical presentation and serological markers were consistent with stiff-person syndrome, complicated by psychiatric comorbidity.
Treatment with benzodiazepines, antispastic agents, and corticosteroids resulted in partial remission of symptoms within two months.

Implications:

This case highlights the importance of considering stiff-person syndrome in patients with unexplained stiffness and spasms, as it is a treatable condition.
The presence of anti-GAD antibodies is not exclusive to stiff-person syndrome and can be found in other autoimmune disorders, necessitating comprehensive diagnostic evaluation.