P15-S Clinical and electrophysiological characteristics of childhood CIDP

Abstract

Background Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare type of neuropathy in childhood as the majority of polyneuropathies occurring in children, about 85%, is hereditary. Material and methods Retrospective analysis of clinical and nerve conduction study (NCS) data included 39 children aged 3.5–18 y.o. with the diagnosis of CIDP (20 girls, 19 boys). Results Except for one girl with sensory variant of CIDP all children met the clinical criteria of CIDP. There were 6/39 (15.4%) patients with pure motor variant of CIDP and 3/39 (7.7%) with atypical presentation, including cranial nerve dysfunction. Relapsing-remitting CIDP was diagnosed in 19/39 (48.7%) cases whereas progressive CIDP with slow improvement was found in 20/39 (51%) patients. The Nevo electrophysiological criteria were fulfilled in 28/39 (71.8%) patients and 7/39 (17.9%) patients met the criteria of possible CIDP. Distal CMAP duration was longer than 9 ms in at least one of the four motor nerves in 31/39 (79.5%) children. Heterogenous pattern of sensory involvement (AMNS/ASNM) was seen in 8/39 (20.5%) children. All children responded fully or partially to immunomodulatory therapy. 14/39 (35.9%) patients received IVIg, 10/39 (25.6%) steroids, 13/39 (33.3%) the combination of both and 8/39 (20.5%) additional immunosuppressive drug: 7/39 (17.9%) azathioprine and 1/39 (2.5%) methotrexat. Complete remission was found in 5/39 (12.8%) children with classical variant of CIDP. Conclusion The childhood CIDP seems to occur as pure motor or sensory more often than in adults. Incomplete improvement after therapy was observed mainly in patients with secondary axonal changes.