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Chronic inflammatory demyelinating polyneuropathy (CIDP) typically responds to corticosteroids, plasmapheresis, and IV immunoglobulin (IVIg).1 Plasmapheresis may potentiate subsequent IVIg control of CIDP,2 and in Guillain–Barré syndrome patients the benefits of plasmapheresis may be enhanced by subsequent IVIg infusion.1 We describe two patients with CIDP who became unresponsive to conventional immunosuppressants but improved with a repeated combination of plasmapheresis and IVIg, suggesting additional synergistic effects of these treatments.
Case reports.
Patient 1.
A previously healthy 7-year-old girl with no other systemic diseases (see table E-1 on the Neurology Web site) developed areflexic quadriparesis over 4 days after a viral infection. Nerve velocities were slow (see table E-2 on the Neurology Web site), CSF protein was elevated, and a sural nerve biopsy (month 19, after prolonged immunosuppression) showed changes of chronic demyelination and remyelination but no inflammation.
The CIDP initially improved with monthly IVIg, but during a 16-month period it became progressively unresponsive to even weekly infusions. After the patient became nonambulatory late in month 18, 5 alternate-day plasma exchanges allowed her to walk with assistance but did not improve IVIg efficacy (0.8 g/kg/week) (figure). In the middle of month 20, despite having undergone another 3 weeks of alternate-day plasmapheresis, co-administered with IV methylprednisolone, prednisone, and cyclosporine, she had quadriplegia with facial diplegia, diplopia, and dyspnea.
Figure. Response to IV immunoglobulin …
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