Chronic inflammatory demyelinating polyradiculoneuropathy in children and adolescents-longterm follow-up in 7 patients

Introduction: Chronic inflammatory demyeninating polyneuroradicolopathy (CIDP) is defined as autoimmune-neuropathy with deterioration over more than 4 weeks. The course of the disease may be relapsing or chronic progressive. During the last 20 years, 7 patients (6 boys/1girl) with this diagnosis have been treated at the Department of Pediatrics, Graz.

Case reports: Onset of symptoms was at 4, 7 and 10 years of age in three children. The youngest boy started with a typical Guillain-Barre history and responded well to intravenous immunoglobulins (IVIG). Thereafter, he had slow, selflimited deterioration over 4 months without further therapeutic intervention and remained with moderate residual distal paresis. The 7 year old boy with primary chronic progressive course, responded well to IVIG in the first months. After a remission over 1 year a relaps was treated with highdose prednisolon and good recovery of muscle strength. Because of extensive weight gain and spontaneous fractures, therapy was changed to cyclosporine A (CyA). This lead to sustained remission and CyA could be withdrawn after another 2 years. Our third child was 10 at the beginning of symptoms. IVIG was ineffective and he was subsequently treated with prednisolone, PE, CyA, mycofenolate mofetil and rituximab. After 7 years, there is still no possibility to reduce the immunosuppressive therapy.

In 3 of the 4 adolescent patients the course was relapsing, 2 of them responded very well to IVIG. One was treated with PE and IVIG due to severe manifestation and immobility. He had a stable remission after having been switched to MMF. In 1 patient the course was primary chronic progressive with partial benefit of IVIG. Due to rather mild symptoms application of other immunosuppressants was postponed.

Conclusion: In the first two decades of life CIDP is rather uncommon. As illustrated by our cohort, the course of disease can be variable and therapeutic measures have to be selected carefully for the individual patient. All but one of our patients had initial response to IVIG. PE was applied in two patients with severe phenotype. Due to the short-term effect of IVIG, the risks of repeated PE and the severe side effects of longterm corticoids, a therapy with one of the new immuno-suppressants should be considered when a prolonged medication is required.