Progressive neurodegeneration in patients with primary immunodeficiency disease on IVIG treatment

Bibliographische Detailangaben
Veröffentlicht in:	Clinical immunology (Orlando, Fla.). - 1999. - 102(2002), 1 vom: 10. Jan., Seite 19-24
1. Verfasser:	Ziegner, Ulrike H M (VerfasserIn)
Weitere Verfasser:	Kobayashi, Roger H, Cunningham-Rundles, Charlotte, Español, Teresa, Fasth, Anders, Huttenlocher, Anna, Krogstad, Paul, Marthinsen, Lars, Notarangelo, Luigi D, Pasic, Srdjan, Rieger, Christian H L, Rudge, Peter, Sankar, Raman, Shigeoka, Ann O, Stiehm, E Richard, Sullivan, Kathleen E, Webster, A David, Ochs, Hans D
Format:	Aufsatz
Sprache:	English
Veröffentlicht:	2002
Zugriff auf das übergeordnete Werk:	Clinical immunology (Orlando, Fla.)
Schlagworte:	Journal Article Multicenter Study Research Support, Non-U.S. Gov't

Beschreibung
Zusammenfassung:	(c)2001 Elsevier Science. We have identified 14 patients with diverse primary immunodeficiencies who have developed progressive neurodegeneration of unknown etiology. All patients had received immunoglobulin replacement therapy for a mean duration of 6.5 years (range of 0.5-13.5 years) at the time of first neurological symptoms. Diagnostic tests of blood and cerebrospinal fluid analyses included chemistry, cultures, PCR for viral genomes, and cytology. In addition, neuroimaging and electrophysiologic studies were performed. Brain tissue histology (n = 5) revealed nonspecific encephalitis with microglial infiltration and neuronal loss. Twelve patients died 6 months to 15 years (median 4.3 years) after onset of neurologic findings. No evidence of any infectious disease that could have explained our patients' progressive encephalopathy was found either during their lifetimes or postmortem. These patients may have had an unusual manifestation of primary immunodeficiency diseases, an autoimmune reaction against neuronal tissue, a yet undefined infectious agent, or a complication of IVIG therapy. To help determine the etiology of this rare complication, an international surveillance system for primary immunodeficiency patients who develop progressive neurodegeneration of unknown cause is recommended
Beschreibung:	Date Completed 11.02.2002 Date Revised 07.12.2022 published: Print CommentIn: Clin Immunol. 2002 Jan;102(1):1. - PMID 11781060 Citation Status MEDLINE
ISSN:	1521-7035