Stiff Person Syndrome Presenting with Sudden Onset of Shortness of Breath and Difficulty Moving the Right Arm: A Case Report

Bradley Goodson; Kate Martin; Thomas Hunt

Disclosures

J Med Case Reports 

In This Article

Abstract and Introduction

Abstract

Introduction: First described in 1956, stiff person syndrome is characterized by episodes of slowly progressive stiffness and rigidity in both the paraspinal and limb muscles. Although considered a rare disorder, stiff person syndrome is likely to be under-diagnosed due to a general lack of awareness of the disease in the medical community.
Case presentation: A 27-year-old Hispanic woman presented to our emergency department with a sudden onset of shortness of breath and difficulty moving her right arm. Her physical examination was remarkable in that her abdomen was firm to palpation and her right upper extremity was rigid on passive and active ranges of motion. Her right fingers were clenched in a fist. Her electromyography findings were consistent with stiff person syndrome in the right clinical setting. Stiff person syndrome is confirmed by testing for the anti-glutamic acid decarboxylase antibody. Her test for this was positive.
Conclusion: Stiff person syndrome may not be a common condition. However, if disregarded in the differential diagnosis, it can lead to several unnecessary tests being carried out causing a delay in treatment. This case report reveals some of the characteristic features of stiff person syndrome with an atypical presentation.

Introduction

In 1956, Moersch and Woltman of the Mayo Clinic described an unusual condition of muscle stiffening and difficulty walking. They coined it "stiff man syndrome".[1] A more appropriate name "stiff person syndrome (SPS)" was later suggested, as the condition affects both sexes, possibly more women than men. Although considered a rare disorder, SPS is under-diagnosed due to a general lack of awareness in the medical community.

Patients with SPS usually experience a prodrome of stiffness and rigidity in the axial muscles of their cervical or lumbar spine. There is a gradual worsening and progression of the condition over time which involves the proximal limb muscles. Pain may be an associated symptom, but significant stiffness and rigidity are the classical features of the disorder. Some symptoms are reported to cause spinal deformities, such as exaggerated lumbar lordosis.[2] Ambulation can be dangerous because the normal postural reflexes of patients become replaced by stiffness, thus placing them at greater risk of fractures. Sometimes, the severity of proximal limb muscle stiffness can overwhelm that of the axial muscles, leading to presenting symptoms of arm or leg rigidity. Such a case is described in this report.

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