Severe Chronic Inflammatory Demyelinating Polyneuropathy Ameliorated following High-dose (3 g/kg) Intravenous Immunoglobulin Therapy

Abstract

We report the case of a 53-year-old woman with severe chronic inflammatory demyelinating polyneuropathy (CIDP) who developed progressive tetraplegia with respiratory failure despite receiving a standard dose of intravenous immunoglobulin therapy (IVIg), steroid pulse therapy, plasma exchange, and cyclosporine. We administered high-dose IVIg (3 g/kg; 0.6 g/kg/day for 5 consecutive days at monthly intervals). The patient's muscle weakness gradually improved after IVIg. She recovered completely 2 years after the onset of symptoms. The effects of IVIg treatment in individuals with CIDP may vary in each patient. In patients with refractory CIDP receiving standard-dose IVIg, repeated high-dose IVIg treatment can be considered.

Keywords: chronic inflammatory demyelinating polyneuropathy (CIDP); high-dose intravenous immunoglobulin therapy (IVIg); pentaplegia; respiratory failure.

Figure 1.

The MRI findings. T1-weighted MRI with contrast agent in the most advanced stage at 7 months from admission. Enhancement was clearly observed (a) in the lumbar nerve root and nerve (a), the facial nerve (b) and the trigeminal nerves (c) (arrows).

Figure 2.

The clinical course and therapy. The initial administration of standard-dose IVIg, plasma exchange (PE), steroid pulse therapy (mPSL) with oral prednisolone (PSL) did not improve the patient’s symptoms. The additional administration of cyclosporine A (CyA) was not effective. The patient developed pentaplegia requiring respirator support during hospital months 6 to 8. After the introduction of high-dose IVIg (0.6 g/kg/day for 5 consecutive days) combined with mPSL and PSL, the patient showed a marked improvement from pentaplegia and was weaned off the artificial ventilator after the repeated administration of high-dose IVIg. After 12 months of hospitalization, the dose of IVIg was changed to the standard dose and the oral PSL dose was gradually decreased. She was able to walk at 1 year after the initiation of therapy. Although she complained of the recurrence of slight muscle weakness, the IVIg administration schedule was changed from once per month to once per year. The patient has not had any recurrences in the past 8 years. The total dose of IVIg was 2,875 g, and no marked adverse reactions were observed.