Critical Cases: Stiff person syndrome!


  • 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


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


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

Work Up/Management:

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


  • 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



Rodgers-Neame, Nancy. Stiff Person Syndrome, Medscape, 5 Dec. 2019,