Hydrocephalus in CINCA syndrome treated with anakinra

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...

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Bibliographic Details
Published in:Child's nervous system 2006-04, Vol.22 (4), p.334-337
Main Authors: Rigante, D, Ansuini, V, Caldarelli, M, Bertoni, B, La Torraca, I, Stabile, A
Format: Article
Language:English
Subjects:
Arthritis - complications
Arthritis - immunology
Child
Child, Preschool
Chronic Disease
Fever - complications
Fever - immunology
Hearing Loss, Sensorineural - complications
Hearing Loss, Sensorineural - immunology
Humans
Hydrocephalus - complications
Hydrocephalus - drug therapy
Hydrocephalus - immunology
Immune System Diseases - complications
Immune System Diseases - drug therapy
Immune System Diseases - immunology
Immunologic Factors - therapeutic use
Infant
Infant, Newborn
Interleukin 1 Receptor Antagonist Protein - therapeutic use
Male
Meningitis - complications
Meningitis - immunology
Syndrome
Treatment Outcome
Urticaria - complications
Urticaria - immunology
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Summary: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.
ISSN:0256-7040
1433-0350
DOI:10.1007/s00381-006-1280-3