Abstract

Stiff person syndrome (SPS) is a chronic, progressive neurological disorder characterized by painful muscle spasms and rigidity with high titres of circulating anti-GAD antibodies.

Intravenous immunoglobulin (IVIg) has been proven to be very effective in reducing the symptoms associated with SPS, most notably stiffness. Patients report being able to walk for the first time in months or even years and partake in activities of daily living including showering and household chores. A reduction in falls is also noted in patients receiving IVIg. Most patients report a drastically improved quality of life after receiving IVIg with significant improvement in pain, general mental health, social functioning, vitality and energy.

At present, there is no standardised method of monitoring the effectiveness of IVIg treatment in patients with stiff person syndrome. We devised a standardised questionnaire with a combination of modified Rankin scale and Flanagan's QOLS and prospectively studied 12 SPS patients receiving IVIg therapy. We named this the Birmingham Response to IVIg Treatment (BRIT) scale.

The BRIT scale can be used to monitor IVIg efficacy in a variety of chronic neurological conditions (especially ones without other monitoring tools, eg: INCAT score for CIDP) and would be a very useful addition to the neurology practice.