NMP010 Chronic demyelinating polyneuropathy in children

Abstract

Childhood chronic inflammatory demyelinating polyneuropathy (CIDP) is characterized with large clinical, physiological, and immunological variability. Progressive course is rare in children. Long-term follow up of 4 children with early onset CIDP is presented. A two years old boy manifested progressive weakness and areflexia associated with CNS involvement developed after viral infection (patient 1), with relapses during intercurrent infections and development of severe axonal damage on EMG. Three-year-old boy (patient2) manifested initially progressive muscle weakness during 6 months with spontaneous regression, followed by 2 severe relapses at the age of 5 and 6 years. Decreased ASA activity was present in the patient 1 (17.6), and his family members (24.1−40nmol/mg/h). Arterial hypertension up to 20/12kPa was present in two patients in initial phase associated with muscle stiffness, occasional meningism, and left ventricular (LV) hypertrophy in one of them (patient 4). Subsequently, they both developed 2 mild relapses, at the age of 3.5−6 years. Clinical outcome was excellent in 2 cases and good in 2 cases although clinical course, therapy response and electrophysiological outcome was quite different in the only patient with low arylsulphatase A activity. Demyelination might develop in central and peripheral nervous system associated with arterial hypertension or inflammatory myopathy in patients with progressive course of CIDP.