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Original Contribution
November 2008

Treatment of Chronic Inflammatory Demyelinating Polyneuropathy With Pulsed Oral Steroids

Author Affiliations

Author Affiliations: Department of Neurology, University of Minnesota (Drs Muley and Parry), and Noran Neurological Clinic (Dr Kelkar), Minneapolis.

Arch Neurol. 2008;65(11):1460-1464. doi:10.1001/archneur.65.11.1460

Background  Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy that responds to various immunosuppressive treatments. Oral daily prednisone therapy is effective and inexpensive, but the long-term treatment that is usually necessary leads to serious adverse effects. Consequently, intravenous immunoglobulin and plasma exchange have been widely used to treat CIDP, making treatment expensive and inconvenient. A steroid regimen that reduces adverse effects but preserves efficacy would simplify treatment. Pulsed steroids have nongenomic actions not seen with low-dose steroids, including rapid inhibition of arachidonic acid release and of calcium and sodium cycling across plasma membranes of immune cells.

Objective  To study the efficacy, safety, and tolerability of pulsed oral methylprednisolone therapy in patients with CIDP.

Design  Open-label prospective study.

Setting  University of Minnesota Neuropathy Center, Minneapolis.

Patients  Ten patients (3 women and 7 men) with CIDP followed up for at least 22 months.

Main Outcome Measures  Neuromuscular score and Inflammatory Neuropathy Cause and Treatment (INCAT) disability score were used as outcome measures for efficacy; weight, blood pressure, changes in bone density, and steroid-related adverse effect questionnaire were used as outcome measures for safety.

Results  This steroid regimen leads to significant improvement in weakness and disability in all patients treated and to off-treatment remission in 60% of patients. Treatment was fairly well tolerated, and only 1 patient discontinued treatment because of adverse effects. Steroid-induced osteoporosis remained a problem, especially in older patients.

Conclusions  Pulsed oral methylprednisolone may be efficacious in the long-term treatment of CIDP and is relatively well tolerated. Remission can be induced in most patients, especially those with a shorter duration of disease.