Chronic Inflammatory Demyelinating Polyradiculoneuropathy in Children and Their Response to Treatment

Abstract

Purpose of the study: To collect data on both the natural history of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in children and their response to treatment.

Study design: Retrospective multicentre study, using standardised criteria for the evaluation of hospital records and semiquantitative scoring of disability.

Results: A total of 21 patients (age range 2-14 years) were observed clinically over a median period of four years. At the peak of the disease, 12 children were unable to walk. None of the patients required artificial ventilation. Most of the children were treated with corticosteroids or high-dosage immunoglobulins (2 g/kg body weight), or both. Nine experienced spontaneous as well as treatment-related remissions and relapses; in twelve the degree of disability changed exclusively parallel to modifications of the treatment. Corticosteroids were used as the first-line drug for 11 patients and were effective for eight; prolonged treatment (up to 2 years) was usually necessary. Administration of high-dosage intravenous immunoglobulins was used for 14 children, resulting in significant and rapid clinical improvement in 12. In eight patients the treatment with immunoglobulins had to be repeated at regular intervals for up to four years. At the last follow-up visit, 12/21 patients were off treatment for 3 months to 11 years, showing none or only slight symptoms and signs; of those still receiving treatment three were in a stable condition, five exhibited significant fluctuation of symptoms, and one was unable to walk unaided.

Conclusion: In the majority of children with CIDP, the protracted and debilitating course of the disease can be alleviated by treatment with either corticosteroids or immunoglobulins. For patients resistant to this treatment an escalating regimen with plasmapheresis, immunosuppressive drugs or interferon-( should be considered.

Key words

Children - Chronic inflammatory demyelinating polyradiculoneuropathy - Hereditary corticoid - responsive neuropathy - Oligoclonal IgG bands - Corticosteroids - Immunoglobulins - Plasma exchange