Stiff person syndrome with atypical features and a favourable outcome with steroids

Abstract

Stiff Person Syndrome (SPS) is a rare and disabling disorder characterised by continuous motor unit activity causing severe rigidity and episodic spasms in axial and limb muscles. It deteriorates the quality of life and causes a serious burden in the patient's life. Here we describe a male patient with stiff person syndrome having atypical features like pyramidal signs and generalised convulsions. Benzodiazepines, baclofen and IVIG did not alter the clinical status whereas oral methylprednisone had a dramatic effect and resulted in a favourable outcome.