N. Iagăru 1, Eliza Cinteză 1
1 2nd Pediatric Clinic, Institute for Mother and Child Care “Alfred Rusescu”, University of Medicine and Pharmacy “Carol Davila”, Bucharest, Romania
Abstract
Acquired neurodegenerative diseases represent a large group of pathologic conditions. There are reports suggesting that there is a linkage between these neurodegenerative diseases and the plasma products administration. Th e authors describe a case of primary immunodefi ciency with severe neurodegenerative disease. Th e patient is a 17-year-old male who was diagnosed initially with Bruton-type agammaglobulinemia when 4 years old. Th e diagnosis was changed to probable Wiskott – Aldrich syndrome (thrombocytopenia – many episodes of purpura described, eczema, recurrent bacterial infections), although the genetic study did not confi rm WASP mutation. For 13 years he received intravenous immunoglobulin (IVIG) replacement initially monthly and then at 4 months intervals. At 16 years of age, he presented with symptoms of mental deterioration, pyramidal and extrapyramidal signs, and gait disturbance. The MRI confi rmed nervous structures abnormalities. He had no history of infection of the central nervous system and the tests for Enteroviruses were negative. Such events can appear in the clinical course of the patients receiving IVIG treatment and should be monitored for neurodegenerative disease.