HPI

• A 28 yo F presents with severe abdominal wall spasms causing shortness of breath
• Spasms  sporadic over  days and worsen with stress/anxiety
• Occasional a/w limb spasms
• A full cardiac and pulmonary work previously been negative
• A family history is notable for autoimmune disorders
• Symptoms improve with IV benzodiazepines and muscle relaxants
• Pt known to be positive for glutamic acid decarboxylase antibodies

PE:

BP 112/72; Pulse 80; Temp 98.6 °F; Resp 18; SpO2 98%; Wt 99.8 kg (220 lb); BMI 40

General: Resting comfortably in no acute distress; speaking in full sentences

Cardiac: Normal rate, regular rhythm, no murmurs

Pulmonary: Lungs are clear to auscultation bilaterally; no respiratory distress.

Abdominal: Soft, nontender, no rigidity Neuro: GCS 15; no increase in tone; sensation and strength intact

DDx

• Dehydration
• Heat exhaustion
• Electrolyte abnormalities
• Neurologic disorders -Stiff person syndrome

Work Up/Management:

• ECG showed normal sinus rhythm
• Prior cardiac and pulmonary noted unremarkable

Conclusion

• This patient had an exacerbation of Stiff Person Syndrome, an extremely rare autoimmune disorder of the nervous system that causes severe muscle spasms that can lead to orthopedic injuries.
• Anxiety triggers the spasms
• The diagnostic feature is positive GAD antibodies
• Acute treatment includes IV benzodiazepines, but this is a temporary fix
• The disease is progressive and ultimately leads to the patient being bed-bound
• Patients with severe symptoms often need IVIG therapy

Reference

Rodgers-Neame, Nancy. Stiff Person Syndrome, Medscape, 5 Dec. 2019, emedicine.medscape.com/article/1172135-overview.