Child's Nervous System

, Volume 22, Issue 4, pp 334–337

Hydrocephalus in CINCA syndrome treated with anakinra

  • D. Rigante
  • V. Ansuini
  • M. Caldarelli
  • B. Bertoni
  • I. La Torraca
  • A. Stabile
Brief Communication



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.

Case report

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.


CINCA syndrome Hydrocephalus Anakinra Interleukin 1 


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Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • D. Rigante
    • 1
  • V. Ansuini
    • 1
  • M. Caldarelli
    • 2
  • B. Bertoni
    • 1
  • I. La Torraca
    • 1
  • A. Stabile
    • 1
  1. 1.Centre of Periodic Fevers, Dipartimento di Scienze Pediatriche Medico-Chirurgiche e Neuroscienze dello SviluppoCatholic University Medical SchoolRomeItaly
  2. 2.Institute of Pediatric Neurosurgery, Dipartimento di Scienze Pediatriche Medico-Chirurgiche e Neuroscienze dello SviluppoCatholic University Medical SchoolRomeItaly

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