Case Report
Stiff Person Syndrome Masquerading as Acute Coronary Syndrome

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Abstract

Stiff person syndrome (SPS) is a rare neuroimmunological disorder characterized by severe progressive muscle stiffness in axial and lower extremity musculature with superimposed painful muscle spasms. Although chest pain is a common reason for SPS patients presenting to the emergency room, this disorder is overlooked and not part of the differential diagnosis of chest pain. Herein, we report on a middle age male presenting with classic symptoms of SPS; however, due to the rarity of this disease, he was initially thought to have acute coronary syndrome. Clinicians should consider the diagnosis of SPS in patients with fluctuating muscle spasms in the torso and/or extremities in the setting of repeated hospitalizations without subsequent symptom relief.

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Background

Stiff person syndrome (SPS) is a rare neuroimmunological disorder characterized by severe progressive muscle stiffness in axial and lower extremity musculature with superimposed painful muscle spasms. SPS prevalence is estimated to be one per million and occurs more commonly in women between ages 30 and 50.1, 2 Although chest pain is a common reason for SPS patients presenting to the emergency room (ER), this disorder is overlooked and not part of the differential diagnosis of chest pain.

Case report

A 41-year-old African American man with vitamin B12 deficiency and chronic eczema presented to the ER with worsening painful muscle spasms in his chest and back. He was in his usual state of health until 3 years ago when he started having these symptoms. The muscle spasms frequently involved his chest, lower back/legs, were sudden in onset, cramping in nature, and aggravated by voluntary movement, emotional stress and unexpected loud auditory stimuli. The patient had undergone multiple

Discussion

The uniqueness of our case resides in its presentation: an extremely rare disease (SPS) masquerading as a very common disease – ACS. The estimated incidence of SPS is 1 case per million.1 It affects women 2–3 times more often than men, and most patients present between the ages of 20 and 50.1, 2 The disease is commonly classified as classic SPS which comprises the majority of cases or variant SPS (Table 1). In patients with variant SPS, stiffness and spasms are usually anatomically limited to

Acknowledgment

Sameer Shaharyar M.D., Fulvia Banu M.D., Hamid R. Feiz M.D.

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Cited by (1)

Conflicts of interest: None.

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