Hydrocephalus in CINCA syndrome treated with anakinra

Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery
D RiganteA Stabile

Abstract

Chronic infantile neurologic, cutaneous, articular (CINCA) syndrome is a rare congenital autoinflammatory disease characterized by neonatal-onset chronic meningitis, hydrocephalus, sensorineural hearing loss, persistent urticarial rash, deforming arthritis, and recurrent fever. This clinical entity is believed to result from dysregulation of cytokine production. No recommended treatment protocol exists so far for CINCA syndrome. We report a 7-year-old child affected with CINCA syndrome in whom no therapy had resulted effective. Anakinra, an interleukin-1-receptor antagonist, was administered in a 1-year period with complete inflammatory symptom remission and dramatically ameliorated laboratory tests. This optimal response has been supported by the demonstration of a stabilized hydrocephalus upon magnetic resonance imaging and by an overall improvement of the neurodevelopmental issues. This paper emphasizes and discusses the medical approach with anakinra in CINCA syndrome presenting with hydrocephalus in which a consistent control of the neurological picture can be obtained.

References

Jan 1, 1987·Scandinavian Journal of Rheumatology. Supplement·A M PrieurB M Ansell
Dec 10, 2002·Trends in Molecular Medicine·Michael F McDermott
May 4, 2005·The Journal of Experimental Medicine·Charles A Dinarello
Jun 28, 2005·Fetal Diagnosis and Therapy·Israel GoldsteinImad R Makhoul
Jun 30, 2005·BioDrugs : Clinical Immunotherapeutics, Biopharmaceuticals and Gene Therapy·John Waugh, Caroline M Perry

❮ Previous
Next ❯

Citations

Nov 21, 2007·Annals of the Rheumatic Diseases·D E FurstM E Weinblatt
May 3, 2007·Nihon Rinshō Men'eki Gakkai kaishi = Japanese journal of clinical immunology·Hiroshi Sugiura, Tadashi Matsubayashi
Aug 26, 2014·Autoimmunity Reviews·Donato RiganteLuca Cantarini
Feb 6, 2016·Fluids and Barriers of the CNS·Marc R Del Bigio, Domenico L Di Curzio
Jul 10, 2012·Acta otorrinolaringológica española·David LoboRafael Ramírez-Camacho
Dec 4, 2015·Clinical Rheumatology·Donato RiganteLuca Cantarini
Mar 21, 2007·Joint, Bone, Spine : Revue Du Rhumatisme·Thierry LequerréXavier le Loët
Jul 1, 2009·Developmental Medicine and Child Neurology·Gina A Montealegre Sanchez, Philip J Hashkes
Jan 6, 2009·British Journal of Haematology·Evelien J BodarAnna Simon
Jan 31, 2009·Arthritis and Rheumatism·Tammy M MartinJames T Rosenbaum
May 19, 2007·Pediatric Neurology·Tracy V TingPhilip J Hashkes
Nov 21, 2015·International Journal of Rheumatic Diseases·Donato RiganteRaffaele Manna
Apr 14, 2016·Clinical Reviews in Allergy & Immunology·Donato RiganteLuca Cantarini
Apr 3, 2015·Clinical Rheumatology·Vittoria BascheriniLuca Cantarini
Aug 24, 2013·Mediators of Inflammation·Antonio VitaleLuca Cantarini
Nov 28, 2013·International Journal of Rheumatology·Francesco CasoLuca Cantarini
Aug 19, 2014·Mediators of Inflammation·Donato RiganteLuca Cantarini
Oct 14, 2014·Autoimmunity Reviews·Luca CantariniDonato Rigante
Jan 10, 2017·Expert Review of Clinical Immunology·Donato Rigante
Nov 21, 2013·Modern Rheumatology·Francesco CasoDonato Rigante
Nov 22, 2018·Frontiers in Pharmacology·Giulio Cavalli, Charles A Dinarello
Jul 29, 2020·Journal of Perinatology : Official Journal of the California Perinatal Association·Domenico Umberto De RoseDonato Rigante
Feb 23, 2020·Mediators of Inflammation·Carla GaggianoLuca Cantarini
Dec 15, 2020·Advances in Therapy·Ludovico Luca SicignanoRaffaele Manna
Jan 22, 2021·Internal and Emergency Medicine·Ludovico Luca SicignanoRaffaele Manna
May 8, 2020·Trends in Cardiovascular Medicine·Giuseppe LopalcoFlorenzo Iannone
May 8, 2021·Rheumatology·Jurgen SotaUNKNOWN Autoinflammatory Diseases Alliance (AIDA) and the Autoinflammatory Diseases Working Group of the Italian Society of Rheumato

❮ Previous
Next ❯

Related Concepts

Related Feeds

Auditory Perception

Auditory perception is the ability to receive and interpret information attained by the ears. Here is the latest research on factors and underlying mechanisms that influence auditory perception.