Stiffperson Syndrome

Introduction to Stiff-Person Syndrome (SPS)

Stiffperson syndrome (SPS) is an uncommon disorder characterised by progressive muscle stiffness, rigidity, and spasm involving the lumbar, trunk, and proximal limb muscles, resulting in severely impaired ambulation. It is related to a decline in the CNS level of gamma amino butyric acid (GABA). SPS is often associated with type 1 diabetes mellitus. Also, it may rarely occur as a paraneoplastic syndrome related to thyroid, renal cell, or colon malignancy. (1, 2)

Goal: Control Symptoms and Improve Mobility

Treatment of SPS is directed at the control of symptoms to improve mobility and function. Symptomatic therapy with benzodiazepines are generally considered. Agents, such as, diazepam (5-20 mg daily in three or four divided doses), clonazepam (1-2 mg orally twice daily). (3, 4)

Symptomatic Therapy

In patients with an unsatisfactory response or intolerance of escalating doses of benzodiazepines, baclofen (starting at 10 mg two to three times daily and gradually titrated up to a maximum of 80 mg daily in divided doses) could be considered. (5)

Alternative Treatment Options

In patients with inadequate symptomatic relief from both benzodiazepines and baclofen, other agents could be tried; including IVIG therapy (at a dose of 2 g/kg, given in a treatment course over two to three infusions, each usually separated by three to five days), rituximab (at a dose of 375 mg/m2 given weekly for four consecutive doses) or plasma exchange. (6, 7)

References