1 – Minor symptoms or signs of neuropathy but capable of manual work 2 – Able to walk without the support of a stick but incapable of manual work 3 – Able to walk with a stick, appliance or support 4 – Confined to bed or chair bound 5 – Requiring assisted ventilation 6 – Dead Approximately 10% of patients relapse, which may require a second treatment with Ig. A second dose of IVIg must only be on the advice of and after assessment Patient with GBS variant demonstrates bulbar or autonomic features and significant disability AND [Group 2] The progressive nature of the weakness indicates a trajectory to significant disability. Relapse in GBS treatment-related fluctuation with initial improvement and subsequent deterioration post IVIg treatment (neurologist review required). The Ig request is made by a neurologist or neurologist has been consulted (including by teleconference) and has approved a second dose AND Patient has demonstrated an Initial response followed by recurrent weakness with no alternative explanation as demonstrated by deterioration in a recent Medical Research Council (MRC) Sum (12) Score compared to the MRC Sum (12) Score post Ig treatment (Kleyweg et al 1991). Kleyweg, RP, van der Meché, FGA, Schmitz, PIM 1991, ‘Interobserver agreement in the assessment of muscle strength and functional abilities in Guillain-Barré syndrome’, Muscle Nerve, vol. 14, pp. 1103–1109. running 2 – Able to walk 10 metres or more without assistance but unable to run 3 – Able to walk 10 metres across an open space with help 4 – Bedridden or chairbound 5 – Requiring assisted ventilation for at least part of the day 6 – Dead Qualification score is defined as > 1. Revised values for the GBS disability score are shown opposite and endorsed by the SWG (Lancet 1978;2:750-753). Progressive weakness is being used as a criterion to require that disease progression has a trajectory to significant disability. Text comments will be used to capture this information. Patients with GBS variants may be able to walk yet still have significant disability and risk of progression, not measured by GBS Disability Score. In this instance, description of symptoms will be used. It was noted that disease progression is difficult to measure or set parameters for a rate, therefore a textual description would be best way to assess.
• Time until recovery of walking with aid • Time until off ventilation ( for those ventilated) • Treatment related fluctuation • Death or disability Approximately 10% of patients relapse, which may require a second treatment with Ig. A second dose of IVIg must only be on the advice of and after assessment by a neurologist. Optional secondary outcome measures • Time until walking unaided • Time until recovery of walking with aid • Time until off ventilation ( for those ventilated) • Treatment-related fluctuation • Death or disability Dose Induction - 2 g/kg in 2 to 5 divided doses. Aim for minimum dose to maintain optimal functional status. Refer to the current product information sheet for further information. The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient. Initial therapy for GBS with significant disability and progression. Initial - 2 g/kg in 2 to 5 divided doses. Refer to the current product information sheet for further information. Relapse in GBS with significant disability and progression post IVIg treatment (neurologist review required) Initial - 2 g/kg in 2 to 5 divided doses. Refer to the current product information sheet for further information. The dosing approach is the same for both indications. Induction dose is unchanged. This script regarding using minimal dose was deleted as inappropriate for this condition - the dose is standard for all patients ( unless lean body mass is being used). As above - standard dosing applies.