Introduction: Chronic Infantile Neurological Cutaneous and Articular syndrome (CINCA) syndrome is a rare autoinflammatory disease resulting from a mutation in the NLRP3 gene. It is typically characterized by the triad of skin rash, arthropathy and central nervous system manifestations. We report a rare case of a child diagnosed with CINCA syndrome revealed by chronic headache. Observation: We reported the case of 11 year old Tunisian boy who was born to first-degree consanguineous parents. He developed in the first hour of live a brief urticarial rash. He had a personal history of sensorineural hearing loss diagnosed in the first year of life. At the age of five, he presented a progressive onset of tightening headache, with nocturnal aggravation, without nausea or morning vomiting, and no associated visual signs. Examination showed a mild intellectual disability, macrocephaly, frontal bossing and saddle back nose. Ophthalmological assessment showed persistent papilledema. The brain MRI showed cortico-subcortical atrophy. On biology, he had aseptic meningitis, persistent elevation of acute phase reactants and leukocytosis. The diagnosis of CINCA syndrome was suspected. We completed by the serum dosage of interleukin 1 Beta which was elevated at 18.6 pg/ml (normal value< 4 pg/ml). Conclusion: The CINCA syndrome must be considered in patients presenting with the association of chronic headaches, aseptic meningitis, papilledema, hearing loss, and skin rash. Early diagnosis is crucial because early prescription of anti-interleukin-1 drugs improves long-term prognosis.

Cyrine Karray
National institute of Neurology Mongi Ben Hmida
Tunisia

Thouraya Ben Younes
National institute of Neurology Mongi Ben Hmida
Tunisia

Hanen Ben Rhouma
National institute of Neurology Mongi Ben Hmida
Tunisia

zouhour miladi
National institute of Neurology Mongi Ben Hmida
Tunisia

abir zyoudi
National institute of Neurology Mongi Ben Hmida
Tunisia

hedia klaa
National institute of Neurology Mongi Ben Hmida
Tunisia

ilhem ben youssef turki
National institute of Neurology Mongi Ben Hmida
Tunisia

ichraf kraoua
National institute of Neurology Mongi Ben Hmida
Tunisia