One in a Million: A Case Report of Stiff Person Syndrome

Author:

Yadav Ruchi1ORCID,Abrol Neeraj1,Terebelo Sima2ORCID

Affiliation:

1. Department of Medicine, One Brooklyn Health, Brookdale University Hospital, Brooklyn, NY, USA

2. Division of Rheumatology, One Brooklyn Health, Brookdale University Hospital, Brooklyn, NY, USA

Abstract

Stiff person syndrome (SPS) is a rare autoimmune disease caused by lack of inhibition to excitatory neurotransmitters in the central nervous system (CNS) leading to inappropriate motor unit firing. The pathophysiology is incompletely understood; however, high titers of antiglutamic acid decarboxylase antibody (anti-GAD Ab) are strongly associated with this disease. We present a 50-year-old woman with a history of ongoing gait and balance issues for 5 years with multiple negative workups. She recently had an acute exacerbation which left her bedbound, unable to move her legs or turn from side to side. After a negative workup at an outside hospital, the patient was discharged to a subacute rehabilitation facility. She then presented to our institution due to worsening of her condition and was ultimately diagnosed with SPS which was successfully treated. We review the case presentation and treatment options in the context of a severe disabling disease presentation.

Publisher

Hindawi Limited

Subject

General Agricultural and Biological Sciences

Reference10 articles.

1. SPS: Understanding the complexity

2. Pragmatic Treatment of Stiff Person Spectrum Disorders

3. GAD antibody-spectrum disorders: progress in clinical phenotypes, immunopathogenesis and therapeutic interventions

4. Progressive fluctuating muscular rigidity and spasm (“stiff-man” syndrome); report of a case and some observations in 13 other cases;F. P. Moersch;Proceedings of the Staff Meetings of the Mayo Clinic,1956

5. Quantitative Assessment of Response to Long‐Term Treatment with Intravenous Immunoglobulin in Patients with Stiff Person Syndrome

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