Abstract
Neonatal onset multisystem inflammatory disease is a rare disorder first described by Lorber in 1973. An additional 29 cases have been recorded. Two patients are described here, one with a 17 year follow-up. The typical features are a rash, fever, adenopathy, hepatosplenomegaly, and a severe, deforming arthropathy predominantly affecting large joints. The most striking feature is the onset in the neonatal period. Other associated features include inflammation, chronic meningitis, anemia, and persistent leukocytosis. Most, if not all, patients develop bizarre epiphyseal radiographic finding that are virtually pathognomonic. This disease is distinct from Still disease.
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References
Ansell BM (1981) Uncommon radiological features of chronic arthritis in childhood: A review. J R Soc Med 74:904
Ansell BM, Bywaters EGL, Elderkin FM (1975) Familial arthropathy with rash, uveitis and mental retardation. J R Soc Med 68:584
Fajardo JE, Geller TJ, Koenig HM, Kleine ML (1982) Chronic meningitis, polyarthritis, lymphadenitis, and pulmonary hemosiderosis. J Pediatr 101:738
Gadoth N, Hershkovitch Y (1979) Rheumatoid arthritis during the first year of life. Eur J Pediatr 132:115
Goldsmith DP (1985) The right stuff for a new syndrome. J Pediatr 106:441
Hassink SG, Goldsmith DP (1983) Neonatal onset multisystem inflammatory disease. Arthritis Rheum 26:668
Jacobs JC (1982) Pediatric rheumatology for the practitioner. Springer, New York, p 150
Kaufman R, Lovell D (1986) Infantile onset multisystem inflammatory disease: Radiologic findings. Radiology 160:741
Lampert F, Belohradsky BH, Foerster C, Eife R, Kollmann D, Stochdorph O, Gokel JM, Meister P, Lampert PW (1975) Infantile chronic relapsing inflammation of the brain, skin, and joints. Lancet I:1250
Lampert F (1986) Infantile multisystem inflammatory disease: Another case of a new syndrome. Eur J Pediatr 144:593
Lorber J (1973) Syndrome for diagnosis: Dwarfing, persistently open fontanelle; recurrent meningitis; recurrent subdural effusions with temporary alternate-sided hemiplegia; high-tone deafness; visual defect with pseudopapilloedema; slowing intellectual development; recurrent acute polyarthritis; erythema marginatum, splenomegaly and iron-resistant hypochromic anemia. J R Soc Med 66:1070
O'Connor D, Bernstein B, Hanson V, Kornreich H, King K (1980) Disease of central nervous system in juvenile rheumatoid arthritis. Arthritis Rheum 23:727
Prieur A-M, Griscelli C (1981) Arthropathy with rash, chronic meningitis, eye lesions, and mental retardation. J Pediatr 99:79
Prieur A-M, Griscelli C, Lampert F, Truckenbrodt H, Guggenheim MA, Lovell DJ, Pelkommen P, Chevrant-Breton J, Ansell BM (in press) A chronic, infantile, neurological, cutaneous and articular (CINCA) syndrome, a specific entity analysed in 30 patients. Scand J Rheumatol [Suppl] 66:57–68
Yarom A, Rennebohm RM, Levinson JE (1985) Infantile multisystem inflammatory disease: A specific syndrome. J Pediatr 106:390
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Torbiak, R.P., Dent, P.B. & Cockshott, W.P. NOMID — a neonatal syndrome of multisystem inflammation. Skeletal Radiol 18, 359–364 (1989). https://doi.org/10.1007/BF00361425
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DOI: https://doi.org/10.1007/BF00361425