| Chronic inflammatory demyelinating polyneuropathy (CIDP) isan immune-mediated neuropathy for which treatment with plasmaexchange, intravenous immunoglobulin, or corticosteroids usuallyis beneficial. However, one third of CIDP patients do not respondto these conventional therapies, and many who respond initially relapse and develop substantial disability after several years.Compelling data suggest that both B and T cells mediate theimmune attack on peripheral nerve, thus providing a biologic rationale for using more aggressive immunosuppressive regimens. These researchers report their experience with high-dose cyclophosphamide(200 mg/kg over 4 days) in 4 patients with severe CIDP thatwas refractory to other therapies. All patients were disabled(Rankin scores of 4 or 5) and had failed numerous trials ofstandard therapies. Interestingly, 3 patients also had failedto improve after previous treatment with intravenous cyclophosphamide(1 g/m2). All patients' Medical Research Council strength scores and Rankindisability scores improved; 2 patients improved by 3 Rankingrades. Febrile neutropenia and transient renal insufficiencyoccurred in 2 patients, and another developed line sepsis, butthere was no long-term morbidity or mortality. Comment:Current therapies for CIDP are inadequate for many patients. In this study, a 70-kg patient would have received14 g of cyclophosphamide, only slightly more than the "immunomodulating"dose (1 g/m2) of 1.5 to 2.0 g administered as a pulse monthlyfor 6 months (a regimen that many centers already have adoptedempirically). Therefore, the intensity of immunosuppressionmay be a more relevant factor than the total cumulative doseor the duration of therapy. The benefits of this approach mustbe weighed against high risks for systemic infection and for other serious complications.The risk-benefit balance may bein favor of high-dose immunosuppression if patients experience sustained remission and discontinue prednisone or other chronicimmune (and potentially toxic) treatments. — Kenneth C. Gorson, MD Dr. Gorson is Associate Professor of Neurology, Tufts UniversitySchool of Medicine, Boston. Published in Journal Watch Neurology August 22, 2002 For those with multi-focal neuropathy with conduction block the rules for treatment are different. Those patients get worse with steroids and do not do very well with plasma exchange, but do respond well to IVIg. Some patients with CIDP have a para-protein in the blood, which is an abnormal protein produced by the bone marrow. These patients may respond to CIDP treatments, but may also require treatment for the excess para-protein. To conclude, CIDP is a very variable condition. It is auto-immune and is treatable. It is exceptional for CIDP not to respond to at least one of the available drugs. The first choice of treatment is either prednisolone or IVIg. The diagnosis is difficult, much less so than GBS, depending critically on the nerve conduction studies and neuro-physiologies. Trials for immuno-suppression have shown promise. ..........cidpusa
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