Childhood acute and chronic immune-mediated polyradiculoneuropathies

Immune-mediated polyradiculoneuropathies are divided into Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). In children subacute inflammatory demyelinating polyradiculoneuropathy is included in CIDP. Immune polyradiculoneuropathies are not exclusively demyelinating, and axonal forms also responding favourably to immunotherapy occur. Evidence-based data on efficacy of therapy in children is lacking, relying on retrospective data, open label studies on small numbers of children, and mainly adult derived data. Immunotherapy (intravenous human immunoglobulin [IVIg] and plasmapheresis) shortens GBS recovery time with most children recovering completely. Childhood CIDP usually responds to corticosteroids and slow tapering is required to prevent relapses. IVIg and plasmapheresis are also effective. CIDP children resistant to steroids, IVIg, and steroid-dependent patients present a therapeutic challenge. Immunosuppressive agents including methotrexate, azathioprine and cyclosporine are helpful in some cases. Anecdotal reports of treatment with interferons alpha or beta and monoclonal antibodies against specific B-cell antigens (Rituximab, Alemtuzumab) have been described in limited case reports. Childhood CIDP prognosis is mostly favourable. However, a proportion of cases have residual neurological deficit.