Table 2.

Escalation Treatment Approach in Stiff Person Syndrome Spectrum Disorders

First-line Therapies	Second-line Therapies	Third-line Therapies	Fourth-line Therapies
Intravenous immunoglobulin	Plasma exchange*	Combination of therapies#	Stem Cell Therapies
Subcutaneous immunoglobulin	Rituximab	Cyclophosphamide
Plasma exchange*	Mycophenolate mofetil
Corticosteroids**	Azathioprine
	Combination of therapies#

^*Therapeutic plasma exchange can be used in setting of acute exacerbations, as bridge therapy to other immune treatments, and/or as part of maintenance immune treatment regimen.

^**Try to avoid long-term use of steroids given increased risk of patients with stiff person syndrome spectrum disorders developing diabetes

^#Some patients with stiff person syndrome spectrum disorders require multiple immune therapies. For example, intravenous immunoglobulin and rituximab or mycophenolate mofetil.