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
- Heat exhaustion
- Electrolyte abnormalities
- Neurologic disorders -Stiff person syndrome
- 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, emedicine.medscape.com/article/1172135-overview.